Part 18-1. The Digestive System

PART XVIII The Digestive System

Section 1. Clinical Manifestations of Gastrointestinal Disease

CHAPTER 251 Normal Digestive Tract Phenomena

GI Function

; swallowing in fetus at 12 weeks

; nutritive sucking in neonate at about 34 Weeks

; coordinated oral and pharyngeal movements necessary for swallowing solid

  - within 1st mo or 2 of life in term infant

; preferences for sweet and salty foods at 1 mo

; *interest in solids at 4 mo

Normal Anatomic Variations

; short lingula frenulum (tongue tie)

; surface furrowing (geographic of scrotal tongue)

; bifida uvula

Regurgitation

; result of gastroesophageal reflux

; *occurs in the 1st 12-18mo

; definition

    - effortless emesis, dribble out of infant's mouth, forceful, volume 15-30ml

; remain happy after an episode of regurgitation

; ★Complication

 ① failure to thrive

 ② pulmonary disease( apnea or aspiration pneumonitis )

 ③ esophagitis

※85  Pathologic Regurgitation?

; Cx이 있는 경우

Stools

; consistency

    1) Meconium - earliest stools after birth, dark, viscous, gumlike material

  2) Transition stools after 4-5 days, green brown often containing curds

; *frequency 0-7/day

    - breast fed infants

           / frequent, small, loose st early, infrequent, soft after 2-3weeks

; color little significant except

    ① presence of blood

  ② absence of bilirubin products

; ★Toddler's Diarrhea

  - intermittent loose stools, between 1 and 3 yrs

  - drink frequently(esp. juice) and snack throught the day

  - typically, during the days and not overnight, often excessive fluid intake

Proturberant Abdomen

; 원인

    ① weak abdominal musculature

  ② relatively large abdominal organs

  ③ lodortic stance

; palpation of abdomen

  ① *liver 1st yr of life

        - common to palpate the liver up to 2cm below the Rt. costal margin

    - soft in consistency

    - Riedel lobe : thin projection of Rt. lobe

    ② spleen tip soft

  ③ vertebral column

  ④ pulsation of aorta

  ⑤ normal stool LLQ in descendingor sigmoid colon

GI Blood Loss

   ① swallowed maternal blood

   ② nasal or oropharyngeal bleeding

   ③ red dyes in foods or drinks

Jaundice

# Indirect hyperbilirubinemia

    ; common in breast fed infants

  ; benign in full terms, golden yellow color

# Direct hyperbilirubinemia

    ; *pathologic, more than 15-20% of total bilirubin

  ; intra- or extrahepatic, greenish-yellow color

Chapter 252. Major Symptoms and Signs of Digestive Tract Disorders

Table 252-1 Some Nondigestive tract Causes of GI Sxs in children

Disordered Ingestion

Transfer Dysphagia

; 원인

    ① part of generalized neurologic or muscular problem - Botulism, diphtheria, cerebral palsy

  ②painful oral lesions - acute viral stomatitis or trauma

  ③ nasal air way obstruction

Dysphagia

; primary motility disorder

    - rare

; secondary motility disorder

    - repair of TEF, collagen vascular disorder

  - achalasia : rare in children

    - esophageal web, tracheobronchial remnant : in infancy

    - esophageal stricture secondary to chronic GER, esophagitis

    - Schatzki ring, esophageal foreign body

Regurgitation

; effortless movement of stomach content into esophagus & mouth

; result of gastroesophageal reflux through incompetent or immature LES

Anorexia

; hunger and saiety center - hypothalamus

Vomiting

; center - medullary vomiting center

                   / directly by afferent innervation

                   / indirectly by CRTZ(chemorecepter trigger zone) & higher CNS center

; obstruction below the second part of duodenum - bile stained vomitus

★Table 252-2 Differential Diagnosis of Emesis during childhood

Table 252-3 Causes of Gastrointestinal Obstruction 

Diarrhea

; excessive loss of fluid and electrolyte in the stool

; stool output

    - amount 5g/kg/day in young infants ( cf. adult 200g/day )

; stool osmolality - greater than 50 mOsm

; small bowel disease

    - *voluminous diarrhea

; large bowel disease

    - less voluminous

    - *dysentery

           / frequent, bloody stool with tenesmus, urgency

# Pathophysiology of secretory diarrhea

    ; secretagogue such as cholera toxin

           --> binding to receptor on surf. epithelium

           --> stimulating intracellular accumulation of cAMP or cGMP

    ; intraluminal fatty acids & bile salts

           --> same mechanism

    ; congenital microvillous inclusion disease

㉿Table 252-4 Mechanism of diarrhea 

Constipation

; hard stool passed with difficulty every 3rd days

; ★True Constipation In Neonatal Periods

    - hirschsprung disease, intestinal pseudoobstruction, hypothyrodism

; defects either in filling or emptying the rectum

    - hypothyroidism, opiate use, hirschsprung disease

# ♥Diarrhea로 오인되는 경우

    ; involuntary encorporesis

    ; constipation이 심할 때 Prox, colon으로 부터 watery content가 hard retained stool주위로 침투하여 rectum으로 pass

★Table 252-6 Important Causes of Constipation

Abd. pain

# 2 fibers

    ; A fiber in skin & muscle - sharp & localized pain

  ; C fiber from viscera, peritoneum, muscle - poorly localized dull pain

           --> cell bodies in dorsal root ganglia

           --> cross midline

           --> ascend medulla, midbrain, thalamus

           --> pain perception in cortex of postcentral gyrus

# visceral pain : dermatome 관련

    ; epigastrium : liver, pancreas, bil. tree, stomach, upper GI

    ; umbilicus : distal small intestine, cecum, append., prox. colon

    ; suprapubic : *distal large intestine, urnaty tract, pelvic organ

# parietal pain

    ; C fiber of dermatome T6-L1

    ; more localize & intense

# ★Stimulus provoking pain

  ; tension or stretching

    ; tissue metabolites

★Table 252-7 Recurrent Abdominal Pain in Children

★Table 252-8 Distinguishing Features of Acute GI Tract Pain in Children

GI hemorrhage

※79 site  : lower eso., stomach, colon

※81 DDx

    ; hematemesis

           - esophagus, stomach, duodenum에서 bleeding origin시 vomiting 시  blood 출현

           - coffe grounds

    ; hematochezia

           - stool내 red or maroon blood

           - distal bleeding site나 distal ileum상방의 massive bleeding

    ; melena

           - distal ileum상방의 mild to mod. bleeding

    - duo.상방의 major bleeding

           - blackened stools of tarry consistency

# *신생아기 이후의 infant, child의 TMC 직장 출혈 : anal fissure

# *학동전기에 2nd common 직장 출혈 : Juvenile polyp

Table 252-9  Differential Diagnosis of GI Bleeding in childhood    

Abdominal Distention And Abdominal Mass

# Cause Of Abdominal Distention

    ; diminished tone of the wall musculature

  ; increased contents (fluid, gas, solid)

# Cause Of Abdominal Mass

    ; constipation, fecal mass, anomalies, cysts, inflammation, neoplasm, liver cirrhosis

Section 2  Oral cavity

; 모든 child는 birth시부터, 1세 전에는 oral health counselling 받아야함.

; visual oral inspection by dentist : 18-24 Mo

CHAPTER 253. Development of The Teeth

; 12wk fetal life시 epith. band. ( dental lamina )로 시작

; permanent teeth는 5th gest. mo부터 crypt형성.( central incisors )

; 1st, 2nd, 3rd permanent molar는 각 4 mo, gestation, 1yr, 4-5yr에 나타남.

; 2nd bicuspids : 10 Mo

253.1 Anomalies Ass. with Tooth Development

Anodontia

# *total anodontia : ectodermal dysplasia

# partial anodontia

    ; dist. of initiation site (palatal cleft)

  ; genetic failure

           - ★albright osteodystrophy, chondroectodermal dysplasia, cleidocranial dysostosis, Hallermann-Streiff, oto-palato-digital type I, william

    ; 부위

           - 3rd molar

    - maxillary lat. incisor

    - mandibular 2nd molar

Supernumerary Teeth

    ; maxillary central incisor 많다.

    ; teeth position & eruption 방해하므로 제거

Macrodontia, Microdontia

Twinning

: two teeth joined together

Amelogenesis Imperfecta

    ; faulty production of organic matrix

Dentinogenesis Imperfecta

    ; poorly calcified dentition

Localized Dist. Of Calcification

    ; hypocalcification, hypoplasia

Mottled Enamel

    ; *fluoride content of drinking water > 2.0 ppm

           --> *ameloblastic dysfunction

           --> mottled enamel

Mineral Deficiency

    ; Vit. D def. rickets, Vit D resistant rickets

※$ Discolored teeth

1) Neonatal hyperbilirubinemia

    ; *blue to black of primary teeth

2) Tetracyclines

    ; *brownish yellow discoloration, hypoplasia of enamel

    ; *Pregnant women과 8세 이전에서는 CIx.

3) *Porphyria ; red brown

Teething

; irritable

※81,82객 Delayed Eruption

    1. all teeth

           ; systemic or nutritional disturbance

           i) hypopituitarism

           ii) hypothyroidism

           iii) cleidocranial dysostosis

           iv) 21-trisomy

           v) Progeria

           vi) Albright osteodystrophy

           vii) Incontinenta pigmenti

           viii) rickets

           ix) Multiple syndrome ; Hunter, Dubowitz, Goltz, de Lange, Gardner, Maroteaux-Lamy

    2. single or small groups teeth

           i) malpositioning of teeth

           ii) supernumerary teeth

           iii) cysts

           iv) retained primary teeth

*advanced dentition ; hyperthyroidism, precocious puberty

★Natal Teeth

    ; eruption at birth

    ; *indidence 1/2,000

  ; 대개 two in mandibular central incisor

  ; gingival margin에만 attach ( No root or no bony support )

  ; *prematurely erupted primary teeth이므로 X-ray확인 전에는 supernumerary로 생각하면 안됨

  ; *asso. disease

         - cleft palate

         - Pierre Robin synd.

         - Ellis van Creveld synd.

         - Hallermann-Streiff synd.

    ; *영향

         - looseness & movement에 의한 2차적인 통증이나 수유 거부

         - nursing시 marternal discomfort : nipple biting

         - detached시 asp. 위험

         - tongue : laceration & tip amputation *(Riga-Fedes ds.)

# Neonatal Teeth

    ; *eruption within 1mo

Exfoliative Failure

    ; permanent teeth eruption 때 primary teeth 탈락 안된 경우

Chapter 254. Disorder Of Teeth Ass. With Other Conditions

1) Osteogenesis imperfecta

    ; hereditary opalescent dentin(=dentinogenesis imperfecta)

2) ※$ Cleidocranial Dysostosis

    ; delayed teeth eruption

    ; primary teeth abnormally retained

    ; permanant teeth unerupted

    ; common supernumerary teeth (esp. premolar area)

    ; erupted teeth

           - *no hypoplasia but variation in size, shape

3) Ectodermal dysplasia

    ; teeth & alv. process가 totally or partially absent

4) congenital syphilis ; permanent teeth에 영향

  i) screw driver-shaped incisors with central notch

  ii) Hutchinson's incisor

  iii) muberry molar : lobular occlusal surf. & narrow pinched crown

Table 254-1 Dental Problems Associated with Selected Medical Conditions    

CHAPTER 255. Malocclusion

# ★Occlusal deformity

    ; leading cause of loss of teeth

class I : normal

class II : *retrognathia - ▲common

class III : prognathia

Cross Bite

Open & Closed Bite

Dental Crowding

Thumb Sucking

    ; *beyond 6 yr --> poor Px

Chapter 256. Cleft lip & Palate

Cleft lip : mesenchymal layer의 hypoplasia로 medial nasal & maxillary prc.의 결합부전

Cleft palate : palatal shelves가 fusion부전

★Incidence & Epi.

1. cleft lip /c or /s cleft palate

    ; *1/600

    ; *male dominant

    ; possible etiology

           - maternal drug exposure

           - syndrome-malformation complex

           - isolated-unknown

           - genetic factor

2. cleft palate alone  

    ; *1/1000

3. Increased incidence of associated congenital malf. & impairment in development

Clinical Manifestations

; *unilat. (Lt. > Rt.) or bilateral

Treatment

1. *▲Immediate Problem : Feeding

    ; "Plastic obturator"의 잇점

  ① fluid control에 도움

  ② reference plan for suction제공

    ③ stability for lat. arch seg. 제공

2. * Surgical Closure

1) cleft lip

    ; *2 mo with satisfactory weight gain & without any oral, respiratory or systemic infection

    ; Z-plasty

    ; revision of initial repair - 4~5yr

    ; nasal corrective surgery - adolescence

2) cleft palate

    ; criteria of the timing

           a) cleft의 width

    b) existing palatal seg.의 adequacy

    c) surrounding area의 morphology

    d) soft P. & pharyngeal wall의 neuromusc. function

    ; goal

       a) cleft seg.의 union

    b) intellignet & pleasant speech

    c) nasal regurgitation ↓

    d) growing maxilla 에 injury방지

    ; *prior to 1 yr to enhance normal speech development

    ; beyond 3 yr

           - contoured speech bulb을 사용해야 한다.

Preoperative and Postoperative Management

    ; feeding *within 6 hr of operation

    ; fluid or semifluid diet for 3wks

Complication

 1. recurrent O.M.

 2. hearing loss

 3. excessive dental decay

 4. displacement of max. arch & malposition of teeth

 5. speech defects -- emission of air from nose, hypernasal quality

Palatopharyngeal Incompetence

    ; inability to form an effective seal between oropharynx and nasopharynx during swallowing or phonation

    ; abnormalities in structure or pharynx or muscles

Clinical Manifestation

    ; hypernasal speech ~ p, b, d, t, h, v, f, s

    ; constricting movement of nares during speech

    ; inability to whistle, gargle, blow out candle, balloon inflation

    ; loss of liquid through the nose when drinking with head down

    ; otitis media

    ; hearing loss

    ; Oral inspection

           - cleft palate or short palate with large oropharynx

    - absent, grossly asymmetric, or minimal muscular activity of the soft palateand pharynx during phonation or gargling

           - submucous cleft t

            / suspected by bifid uvula, palpable notching in post. border of hard palate

Diagnosis

    ; true lat. view

       - one film:at rest, another film:phonation 'u' as 'boom'

           - normal function / soft palate contacts the post. pharyngeal wall

           - *this patient / absent contacts

Treatment

; pharyngoplasty

 

Chapter 257. Syndromes with Oral Manifestation

※$ Pierre Robin Sequence

1. micrognathia

2. glossoptosis ( & pseudomacroglossia )

3. high arched or cleft palate

    ; normal size tongue, foreshortened floor of the mouth, reduced buccal cavity

           --> air passage obstruction on inspiration

    ; Treatment

       - *in a prone or partially prone position

           - *no necessary of temporary suturing of ventral surface to lower lip

           - no necessary of trachostomy

                   (due to sufficient mandibular growth within a few month)

Mandibulofacial Dysostosis (=Treacher Collins synd. or Franceschetti synd.)

; facial Characteristics

    - downward sloping palpebral fissure toward outer canthi, colobomas of lower eyelids, shunken cheekbone, blind fistula opening between angle of mouth and ears, deformed pinna, atypical hair growth toward cheeks, receding chin, large mouth

; facial clefts, abnormalites of ears, deafness

; hypoplastic mandible

; autosomal dominant

Unilat. Hypoplasia Of Mandible

; partial paralysis of facial n. macrostomia, blind fistula, deformed ear lobes

; severe facial asymmetry, malocclusion

Facial asymmetry

Ankylosis of TM Joint

CHAPTER 258. Dental Caries

Etiology

1. *Streptococcus Mutans

    ; enamel surf. cavitates

           --> oral bacteria invasion underlying dentin (esp. lactobacilli)

           --> mixed bacterial infection

           --> destruction of tooth    

2. frequency of carbohydrate consumption

    ; sucrose in chewing gum

Clinical Manifestation

1. pits & fissure on occlusal surf. of molar teeth

2. contact surface between teeth

3. necks of teeth : rare, but ↑ in nursing bottle caries

4. ♥BBTD (Baby Bottle Tooth Decay)

(=nursing bottle caries)

    ; occurs from sleeping with the nursing bottle

    ; occurs *before 18 mo

    ; only severe dental disease common in children less than 3 yr

    ; develop additional cavities on smooth surfaces of teeth

    ; Treatment

           - breast-feeding or water bottles

Complication

; *pulpitis-> toothache-> dental abscess, periapical abscess-> sepsis, facial cellulitis

Treatment

; dentoalv. unit에 localize시는 extraction or pulpectomy,

; *dentoalveolar unit넘어서면 antibiotics

1) infection in vital area

    ; *submandibular space -> Ludwig angina

   facial triangle -> cavernous sinus thrombosis

   periorbital space -> orbital involv.

    ; parenteral antibiotics

2) Immunocompr. host, impaired wound healing, endocarditis 위험시

    ; parenteral antibiotics (PC or EM, clindamycin, vancomycin)

Prevention

1. fluoridation communal water (*1.00 ppm )으로 유지

    ; *▲effective

Table 258-1  Supplemental Floride Dosage Schedule     

2. oral hygiene : brushing of teeth

3. diet : 1세까지 weaning 하여 nursing bottel caries예방

4. dental sealants & plastics

5. high risk pt. identification

   i) Sjogren synd.

   ii) Mikulicz ds.

   iii) chr. GVH ds.

   iv) Xerostomia유발 drug 장기 사용자

CHAPTER 259. Periodontal Disease

; gingiva, alv. bone, perodontal lig.

Gingivitis

# cause

    ; poor oral hygiene, ANLL, DM, neutropenia, thrombocytopenia, scurvy, hormonal change ass. with puberty & preg.

Teething

; from low grade fever to significant discomfort

Eruption gingivitis

Acute necrotizing ulcerative gingivitis (ANUG : Vincent Infection : Trench Mouth)

Etiology

; ass. with bacterial flora (spirochetes, fusobacterium)

; etiology, not fully understood

; in young adults & adolescent (esp. protein malnutrition)

    -> facial structure necrosis (*cancrum oris, noma)

♥Clinical Manifestation

1. *necrosis & ulc. of erythematous gingiva

2. *adherent grayish pseudomemb. over affected gingiva

3. fetor oris

4. cervical lymphadenopathy

5. malaise

6. fever

Treatment

; two phase

    1) acute : 48시간 이내 호전

      i) antibiotics (PC or EM)

     ii) local debridement     

     iii) oxygenating agents 

     iv) analgesics 

  2) 2nd phase : acute phase가 irreversible periodontal ds. 야기시

Herpetic Gingivostomatitis

※94 $ Phenytoin Induced Gingival Overgrowth ( PIGO, Dilantin Hyperplasia )

Pathogenesis

1. direct stimulatory action on gingival fibroblasts

2. gingivitis

*Clinical Manifestation

; *10-30% of DPH-treated pt.

1. gross enlargement of gingiva

2. edema & erythema of gingiva

3. secondary inf. abscess formation

4. migration of teeth

5. inhibition of exfoliation of primary teeth & subsequent impaction of permanent teeth

Treatment

prevention, oral hygiene, gingivectomy

Juvenile Periodontitis

; 특징 rapid alveolar bone loss

; 관련 flora

   i) Capnocytophaga

   ii) Actinobacillus

   iii) Haemophilus

   iv) Bacteroides

; esp. permanent incisor, 6 yr molar

Acute Pericoronitis

; incompletely erupted tooth의 crown을 partially cover하는 gingiva flap의 infla.

 

CHAPTER 260. Dental Trauma

Dental Injury

; preschool (1-3yr)  : fall, child abuse

; school age (7-10yr) : bicycle, play ground

; adolescent (16-18yr) : fight, athletic injury, automobile accident

Injuries To Periodontal Structures

Concussion

Subluxation

Intrusive Luxation

Extrusive Luxation

Evulsion

    ; *replanted within 30min after injury

           --> more than 90% success rate

CHAPTER 261. Common Lesions of Oral Soft Tissue

Oropharyngeal Candidiasis (OPC, Thrush, Moniliasis)

; Candida albicans

Clinical Manifestation

; white plaques covering oropharyngeal mucosa

    - detach 시 inflammation & hemorrhage

Prognosis

    ; self-limited

Treatment

    ; *topical Nystatin (1,000,000u 4x/d)

# Myelosuppressed Pt.

    ; systemic candidiasis초래 가능

  ; *Multiagent Regimen For Prophylaxis

         1) debriding all mucous membrane surface with *povidone-iodine swabstick 4 times a day

           2) swabbing with on large cotton pledget saturated with *500,000u of nystatin 4 times a day

# ★Chronic OPC

    ; *endocrinopathy, specific cndida immunodeficiency syndrome(ass. with cutaneous & nail involvement), acquired immunodeficiency, nutrient problem, broad spectrum antibiotics

Aphthous Ulcers (Canker Sores)

    ; solitary or multiple painful ulceration

    ; labial, buccal, or lingual mucosa and sublingual, palatal, or gingival mucosa

    ; minor ulcer ~ *2-10mm in diameter     --> heal spontaneously in *7-10days

    ; major ulcer ~ greater than *10mm in diameter --> *10-30days

    ; coalesce into plaques --> *7-10days

Etiology

    ; activation and accumulation of cytotoxic T cells

           --> altered local regulation of the cell-mediated immune system

♥ Predisposing factors

1. trauma

2. emotional stress

3. low serum iron or ferritin levels

4. vitamin B12 or folate deficiency

5. malabsorbtion in ass. with celiac or crohn disease

6. mensturation

7. food hypersensitivies

8. allergic or toxic drug reactions

* 14일 이상 지속시는 biopsy

Treatment

1. *0.2% aqueous chlorhexidine gluconate mouthwash

2. topical anesthetics

    1) viscous lidocain(Xylocain)

    2) oral rinse with a solution of elixir of diphenhydramine, viscous lidocain, 0.5% dyclonine hydrochloride

3. topical corticosteroid in a mucosal adhering agents

    ; 0.1% triamcinolone in Orabase

4. topical tetracycline mouthwash

5. systemic therapy

    1) corticosteroids

    2) colchicine

    3) dapsone

Table 261-1  Differential Diagnosis of Oral Ulceration 

Bohn Nodules

; small cystic lesions along buccal & lingual aspects of mand. & maxillary ridges of neonate

; mucous gl. remnant에서 발생

Dental Lamina Cysts

; small cystic lesion located along the crest of mandibular & maxillary ridges of neonate

Mucocele

; raised bluish vesicle

; commonly in lower lip

; caused by traumatic laceration of minor sal. gl. duct

Fordyce Granule

; multiple, yellowish-white granule in clusters or plaque like area on buccal mucosa or lips

Herpes Labialis

Cheilitis

; dryness of lip, scaling & crackling

    - characteristic burning sensation

; fever

; sensitivity to contact substances + photosensitivity to sun ray

; Tx - bland ointment application

Black hairy tongue (Lingua nigra)

; elongation of filiform papillae in front of V-line

; *원인

    - prolonged antibiotics esp. oral troche

  - bismuth medication

Geographic tongue (Migratory glossitis)

; benign, asymptomatic lesion

; one or more smooth, bright-red patch

    - yellow, gray, white membranous margen

; some have anemia, DM, Reiter disease, seborrheic dermatitis, pustular psorisis

; spont. regression, recurrence

Fissured Tongue (Scrotal Tongue)

; deep grooves on dorsal tongue

    - pebbled or wrinkled appearance

; *ass. with

    - infection, trauma, malnutrition, *low vitamin A level

; food prticles & debris trapped in fissures

    - irritation, inflammation, halitosis

    - Tx

           / careful cleansing with mouth rinse & soft-bristled toothbrush

※82 Etio of macroglosia

①hemangioma, cyst, tumor

②cretinism

③acromegaly

④Beckwith syndrome

⑤gargoyism 

※87 tongue tie OP time : after 8-10 Mo

Chapter 262. Diseases Of Salivary Glands And Jaws

# ♥Bilat. Enlargement Of Submaxillary Gland

    ; cystic fibrosis

    ; malnutrition

    ; transiently during acute asthmatic attack

    ; AIDS

# Enlargement of parotid gland

    ; *chrnic vomiting & aspiration in achalasia or bulimia에 의해 발생할 수도 있다.

# Benign Salivary Gl. Hypertrophy Ass. With Endocrinopathy

    i) thyroid ds.

    ii) diabetes

    iii) disorder of pituitary-adrenal axis

※81주 Increased secretion of saliva

1. reflux to anticipated feeding or pain

2. irritative lesion in mouth

3. in conjunction with nausea

4. administration of *mercurial compound

5. encephalitis, chorea

6. irritation of teething  

7. neurologic impairment - drooling

Recurrent Parotitis

; healthy children에서, 대개 unilat.

; *pain (-), 봄에 호발, 2-3주후 spontaneous subside

Suppurative Parotitis

; Staph. aureus, 대개 unilat.

; Sx. : fever, tender painfully swollen gland

Ranula

; sublingual area에 cyst

; large, soft mucous containing swelling

Xerostomia (dry mouth)

★Etiology

  ; fever, dehydration, anticholinergic drugchr. GVH ds, Mikulicz ds., Sjogren synd., tumoricidal radiation

Caffey Ds.

Osteomyelitis

NB : Premaxillary suture

CH : mandible

Reticuloendotheliosis ( Histiocysosis X )

Neoplasms

Benign Tumors

; ossifying fibroma : pinless, unilat. soft tissue swelling

Cysts Of Jaw

; with multiple basal cell nevoid synd.

Malignant Tumors

; Burkitt lymphoma, lymphosarcoma, osteogenic sarcoma, rarely fibrosarc.

CHAPTER 263. Diagnostic Roentgenograms in Dental Assessment

1) Panoramic R. : single image of upper & lower jaw 제공

   ① include

      i) mandible condyle

      ii) inf. border of mandible

      iii) max. sinuses

   ② indication

       ; unerupted teeth, cyst of jaw, missing teeth, Fx., supernumery teeth

2) Cephalometric R.

   ① child's facial growth assess, sequently

   ② relation of uper & lower jaws

      relation of jaws to cranial base

      aligment of incisor teeth

      relation of  teeth to supporting bone

       -> in planning orthodontic care & orthognathic surgical procedure

3) Intraoral dental R.

      .. one section of mouth

     Ix.   ① dental caries

          ② extent of dental trauma

          ③ position of supporting bone relative to teeth

          ④ stage of periodontal ds.

          ⑤ dental anomaly immediately around teeth

Section 3. The Esophagus

CHAPTER 264. Development & Function of The Esophagus

# ★swollowing ; 20wk of gestation

# ★sucking & swallowing coordination : 33-34 wk of gestation

# 3 types of eso. waves

    i) primary

           ; swallowing하여 food를 stomach으로 보내는 synchronizing waves

           ; propulsive efforts

    ii)secondary

           ; local distention으로 시작

           ; residual food이 있는 esophagus를 empty시키려는 propulsive efforts

    iii) tertiary

           ; *non propulsive

           ; 다수가 나타나면 abnormal이며, *chest pain발생

# Common Sx. of eso. ds.

    ; cough or choking with swallowing

    ; regurgitation or vomiting

    ; dysphagia

    ; complete inability to swallow

    ; pain on swallowing(odynophagia)

    ; hematemesis

# Diagnostic evaluation

    ; barium swallow roentgenogram

    ; fluoroscopy

    ; esophageal manometry

    ; pH meter : *most sensitive to detect reflux of acid

    ; radionuclide scan

    ; esophagoscopy

Chapter 265. Atresia & Tracheoesophageal Fistula

Incidence : 1/3,000 - 4,500 ( 1/3 이 premature )

♥ Type (5)

  1. distal TEF with EA (87%)

  2. EA without TEF (8%)

  3. H-type (4%)

  4. Prox. TEF with EA ( <1%)

  5. both TEF ( <1%)

※81,83 Clinical Manifestation

# ♥ Suspected Case

    1) maternal polyhydramnios

    2) catheter cannot be inserted into stomach

    3) excessive oral secretions

    4) choking, cyanosis, coughing at feeding

    ; type 에 따라 다양한 증상

    ; H-type

           - recurrent aspiration pneumonia

           - delayed for days or even months

    ; ※81, 83 ass. anomalies - 50%

           - *cardiovascular anomaly : ▲

           - VATER syndrome

           1) vertebrae defect

           2) anal atresia or duodenal atresia

           3) tracheoesophageal fistula with atresia

           4) extremities anomlies : radial upper limb hypoplasia

           5) renal defects

Diagnosis

; *pulmonary aspiration이 예후에 영향을 미치므로 delivery room에서 진단되는 것이 이상적이다.

; *inability to pass a catheter into stomach

    - *abruptly stop 10-11cm from upper gum line

    --> X-ray

           / coiled cath. in upper eso. pouch

           / dilated esophagus with air

; esophagogram : water soluble dye *1cc 이내

; H-type : videoesophagogram, bronchoscopy

Treatment

    ; surgical emergency

1. prone position

2. constant suction

3. temp. control, resp. function, management of asso. anomalies

4. ♥Occasionally Stage Op.

    1) 1st step : fistula ligation + insertion of gastrostomy tube

  2) 2nd step : anastomosis of two ends of esophagus

           --> *anastomosis 8-10일 후 oral feeding

       --> *10일째 esophagography하여 anast. 의 adequacy 결정

# Structural malformtions of trachea가 흔하다.

    ; tracheomalcia, recurrent aspiration pneumonia, reactive airway disease

# *Fistula 가 없으면, normal tracheal development이지만 다른 질환 흔하다.

    ; *esophageal stenosis, severe GER

# *Sequelae

    ; failure to thrive, slow feeding, coughing, choking

Chapter 266. Other Disorders Of Esophagus

Laryngotracheoesophageal Cleft

; TEF와 비슷하나 *aphonia (+)

 Dx. : endoscopy

External Compression

1.* TMC mass : subcarinal enalrged LN

    ; Tbc, histoplasmosis, other forms of Pul. suppuration, lymphoma

2. vascualr anomaly in the mediastinum

Esophageal Duplication Cysts

; 2/3가 Rt.

; epith.은 any portion of intestine가능

; ulceration발생하면 esophagus 와 communication

    - 주로 gastic mucosa origin일 경우

; Dx. - barium esophagogram

; neurenteric cyst : glial element가진 dupl. cyst로 vertebral anomaly 증가

Congenital Stenosis & Webs

; solid food첨가시 dysphagia

※81,87주 Dysphagia Due To Neuromuscular Disease

Table 266-1. Neuromuscular Disorders that may cause dysphagia

Cricopharyngeal Dysfunction

    ; *spasm of cricopharyngeal m or achalasia of superior esophageal sphincter

    ; intermittent dysphagia, increased pressure in the pharynx

           --> *post. pharyngeal diverticulum

Dx. : videoesophagogram of manometry

Tx. : myotomy of C.P. muscle

Cricopharyngeal Incoordination Of Infancy

; 출생후 sucking잘하나 삼킬 때 choke & asp.

; 6 Mo까지 careful feeding by spoon or gavage시 호전

Bulbar palsy

; supranuclear : jaw jerk 증가,  spastic CP sign

; lower motor neuron : flaccid bulbar P & facial diplegia -> Moebius synd.

Paralysis Of Sup. Laryngeal N.

; Eti. : unusual intrauterine position

; C/M : dysphagia, eso. motiltity 감소, head turning a side

; Px : 1년내 spontaneous recovery

Transient Pharyngeal Musxle Dysfunction

; palatal dysf. 동반

; delayed develop. or C-P시

; 수일-수주 지속

Diffuse Esophaeal Spasm

; cause of chest pain & dysphagia in adolescents

Chapter 267. Achalasia (Megaesophagus)

; primarily *adolescents and adult

; fewer than *5% under 4yr

# Pathogenesis

    ; *decreased ganglion cell surrounded by infl. cell

           --> heightened response to methacholine

           --> degeneration hypersensitivity

※95$ Fig.267-1

Clinical Manifestation

; swallowing diff.

; regurgitation

; cough

; poor Wt. gain

; reported in sibilings

; *asso. with adrenal insufficiency, alacrima

Diagnosis

    ; *esophageal manometry - confirm

        --> *incomplete or absent relaxtion of the LES with swallowing

           --> lack of primary or secondary propulsive peristaltic waves

           --> increased LES pressure

Treatment

1. nifedipine ; indicated when brief delay in definite therapy

2. intrasphincter injection of botulism toxin ; as long as 6mo

3. simple bougienage ; temporary

4. *surgical division of muscle fibers (Heller myotomy) ; permanant

Chapter 268. Hiatal Hernia (Partial Thoracic Stmach)

2 Types

1. Sliding hernia

    ; *▲common

    ; GE junction slides into the thorax

    ; congenital

    ; ★ Tx - not hernia but GER

2. Paraesophageal hernia

    ; stomach insinuated bedside GE junction

  ; following fundoplication for GER

    ; fullness after eating & upper abd. pain

    ; ★infarction of the herniated stomach - rare

㉿Chapter 269. Gastroesophageal Reflux (Chalasia)

Etiology 

1. Contributing factor to competency of LES

    1) abd. position of the sphincter

    2) angle of insertion of esophgus

    3) *sphincter pressure ; major mechanism of reflux

2. Norml person

    ; reflux가 있지만, swallowing of saliva로 protect

3. decresed gastric emptying and reduced acid clearance

Clinical Manifestation

1. excessive vomiting

    ; 85% during 1st wk of life

    ; 10% by 6wk

           --> ※87 by 2yr as upright posture

                           ; 60% Sx disappear

                           ; remainder until 4yr

2. ♥ Increased Incidence

    ; *cerebral palsy, down synd., developmental delay

3. delayed gastric emptying & vomiting

4. aspiration pn. (1/3)

5. chronic cough, wheezing, recurrent pn.

    ; Sx persist until later childhood

6. rumination

7. growth & wt. gain 장애 (2/3)

8. Esophagitis

    ; hemorrhage

           --> hematemesis but melena rare

    ; iron deficiency anemia

    ; substernal pain rare but dysphagia

           --> irritability, anorexia

    ; stricture formation (5%)

    ; death due to inanition and pn. (5%)

# ♥Sandifer Syndrome

    - opisthotonus, abnormal head posturing ass. with reflux

    - GER ass. with severe esophagitis, IDA, vomiting, head tilt trait

9. laryngospasm, apnea, bradycardia ; rare

Diagnosis

1. Clinical Assessment With Response To Therapy : Mild

2. ※89 barium esophagography under fluoroscopy ; severe or complex cases

    1) hiatal hernia ; gastric fold above diaphragm

    2) GER ; no demenstrated

                   --> enough barium, special maneuver

    3) severe esophagitis ; ragged mucosal outline

3. *Esophagoscopy With Biopsy ; ▲specific

    ; early changes

           - increased thickness of the germinative layer

           - increased length of dermal papillae

    ; severe changes

           - intraepithelia neutrophils, eosinophils

           - ulcer formation

           - presence of columnar epithelium (Barrett esophagitis)

4. gstric scintiscans

5. continuous monitoring of distal esophageal pH

    ; not needed for routine diagnosis

    ; atypical symptoms or unusual events가 있는 경우

※87주 Treatment

1. *prone position(young infant) & raising head of the bed and keeping the child(older infant)

2. thickening the feeding with cereals

3. Drug therapy

    1) Metoclopramide ; 0.15mg/kg/dose qid (S.E. - drowsiness, restlessness, EPS)

    2) Domperidone ; acts only peripherally

    3) Cisapride ; 0.2 mg/kg/dose qid

※96 if esophagitis

    1) antacid

    2) H2 receptor blocker or omeprazol 를 추가

           ; omeprazol - most effective depression of acid secretion

4. Operation

    1) ♥Indication

           ; prolongd trial of intensive medical Tx.에 no response

           ; recurrent aspiration, apnea가 no response

           ; stricture formation due to reflux esophagitis

    2) *Nissen fundoplication or variation

5. Percutaneous gastrojejunostomy

Prognosis

    ; 90% no reflux

※88 Rumination

; repetitive gagging, regurgitation, mouthing, reswallowing of materal

; uncommon but serious form of chr. regurgitation

; 6-12 Mo에 common, growth failure동반 가능

Etiology

    ; unknown

    ; altered interaction with the environment

           - prolonged lack of stimulation

           - altered relationships with caregivers

※89 Treatment

1. behavioral therapy ; regular eye contact - mother와 intensive relationship중요

2. medical treatment of reflux

3. antireflux surgical procedure

Chapter 270.  Esophagitis

Peptic Esophagitis

; *▲common form of esophagitis

; pain, blood loss, stricture

Retroesophageal Abscess

; Etiology

    - extension of retropharyngeal abscess, eso. perforation, spinal O.M., pleuritis, pericarditis, diphtheria of pharynx, suppuration of mediastinal LN

; Clinical Manifestation

    - dyspnea, brazzy cough, dysphagia, swelling of neck

; Diagnosis

    - increased retrotracheal space by lat. neck X-ray

; Treatment

    - prompt surgical drainage

Esohphageal candidiasis (moniliasis)

    ; *▲common esophgeal infection

    ; not limited to immunocompromised patients

    ; Torulopsis glabrata

    ; frequently no oral candidiasis

    ; Diagnosis

           - barium esophagography

                   / mucosal irregularities

           - esophagoscopy and biopsy

                   / most sensitive and accurate diagnostic studies

    ; Treatment

           - ketoconazole

                   / 3-6 mg/kg/24hr as a single daily oral dose

           - amphotericin and/or flucytosne

                   / immunocompromised and systemic infection

Corrosive esophgitis

1. Agents

    ; *ingestion of household cleaning products - ▲common

    ; alkalis(70%), acids(20%), bleaches, detergents, microwave overheated baby bottles, button mercuric oxide batteries

    1) Alkalis

           ; severe, deep, liquefaction necrosis on all layers of esophagus

           ; severe gastritis

           ; household liquid alkali agents - 8-10% base

           ; industrial strength alkali agents - 80% base

           ; no taste --> significant amount ingestion

    2) Acids

           ; toilet bowl cleaners, drain decloggers, rust & stain removers

           ; coagulative necrosis and thick eschar --> injury on mucosa, superficial muscularis layers of esophagus

           ; severe gastrits

           ; bitter --> limiting ingestion

2. Peak age ; less than 5yr

3. Tissue injury sequence

    ; early saponification, necrosis, thrombosis ( days 1-5 )

    ; further necrosis ( days 4-7 )

    ; collagen formation ( days 8-12 )

    ; healing or stricture formation ( days 8-42 )

# *esoph. perforation시기 : 7-26일

Clinical manifestations

; salivation, refusal to drink, nausea, vomiting, epigastric pain, oral burns or ulcerations, fever, leukocytosis

♥Treatment

1. Emergency Management

    ; *oral adm. of large quantities of fluid (water or milk)

           - *neutralization, induced emesis, gastric lavage - contraindication

    ; endotracheal intubation or tracheostomy ; edema of pharynx, larynx, airway

2. hospitaliztion

3. NPO

4. ※96 esophagogastroscopy with flexible fibroptic endoscope

    ; all patients *within 48hr

           --> indentify those /s burns who not need F/U

           --> determine the severity of esoph. burn & gastric antral ulceration

5. drug therapy

    ; ampicillin - suspected infection

           cf) perforation with mediastinitis ; broad spectrum antibiotics and mediastinal drains

    ; *prednisolone - at now, no indication

6. intraluminal stents

    ; risk of stricture

           1) circumferential ulcerations

           2) white plaque

           3) sloughing of the mucosa

    ; prevention is more important - early detection

7. colonic interposition

Chapter 271. Esophageal Perforation

Etiology

    - 대개 instrumentation 시

  - eso. pr. ↑시 : violent retching, automobile accidents, compression in birth canal

; Site  

    - children : *Lt. of distal eso. (95%)

  - neonate : Rt.

Clinical Manifestation

    - vomiting후 severe substernal pain, cyanosis attack

# Mallory-Weiss synd.

    - violent retching시 eso. mucosa & submucosatearing hematemesis

CHAPTER 272. Esophageal varices

; portal hypertension의 Cx.

※$ Chapter 273. Foreign Body In Esohagus

Coins ; *▲common in esp. less than 5yr

※81 Physiologic narrowing

    i) below cricopharyngeal m.

    ii) at the level of aortic arch

    iii) just above diaphragm

Clinical Manifestation

1. provoke coughing, drooling, choking

2. pain, dyspnea, dysphagia

Diagnosis

1. radiopaque foreign body ; coin & flat object on *edge in lat. film

2. symptomatic foreign body ; endoscopy

3. radiolucent foreign body ; barium swallow esophagography

Treatment

1. removal of object under direct vision with esophagoscopy

    ; ★Emergency - open safety pins, disc batteries(corrosive injury within 4hr)

2. removal by Foley catheter

3. asymptomatic coin ; observation during 24hr

Section 4. Stomach and Intestines

CHAPTER 274. Normal Development, Structure, Function

Development

8 wk ; peristalsis

10wk ; re-enters of bowel to abdomen

12-13wk ; full development of blood vessels & nerve supply to gut

# acid secretion

    ; low in the first 5hr

    ; increase by 24hr

    ; pepsin secretion peak during the first 10day

    ; decrease from 10-30days

# disaccharide activity

    ; 12 wks (+)

  ; sucrose  24wk

    ; maltose  32wk

    ; lactose  36wk

           -> decline after the first few years of life

# fat absorption

    ; term 시 less efficient than infant & child

    ∵ lower bile salt synthesis & transport rate

Normal structure

; submucosa : matrix for lymphoid & vascular plexus in duodenum ,Brunner gl. 포함.

※87 4 Types Of Epith. Cells In Small Intestine

    1. columnar absorptive cells ; dominate

    2. goblet cells ; secrete mucus

    3. endocrine cells ; secrete intestinal H.

    4. paneth cells : unknown

    5. ‘m’ cells

           ; over areas of lymphod aggregation

           ; absorb intact, potentially antigenic proteins

; jejunal epith. 의 complete renewal time : *5-6 일

Normal function

; intraluminal digestion

    - 주로 exocrine pancreas 에 의해

    - *secretin, cholecystokinin

           / *synth. & secretion of HCO₃^- & digestive enzyme 

; CHO, protein, fat : 주로 SI의 upper portion에서 흡수

; Na, K, Cl, water : 대부분 SI에서

; bile salt, Vit B12 : terminal ileum

; iron : *duodenum, prox. jejunum

CHAPTER 275. Pyloric Stenosis and Other Congenital Anomalies of The Stomach

# hallmarker of gastric obstruction

    : nonbilious vomiting, other Sx. abdominal pain, nausea

# sign of gastric outlet obstruction : abdominal distention and bleeding

# *▲common cause of nonbilious vomiting : infantile hypertrophic pyloric stenosis

# DDx of CHPS

    ; pyloric atresia, antral webs, gastric duplication, gastric volvulus

    ; GER, peptic ulcer disease, salt-wasting adrenogenital syndrome, bezoars, metabolic & motility abnormalities

※91~96 275.1  Hypertrophic Pyloric Stenosis

; incidence : 3/1,000 in US

; more common in *whites, less common in blacks, rare in Asian

; *M : F=4 : 1 (esp. 1st born )

# mother ; CHPS

    --> 20% of male, 10%of female CHPS incidence

; increased incidence of type B or O blood group

; asso. with congenital defects : TEF

Etiology

Unknown

    ; abnormal muscle innervation

    ; *breast feeding

    ; maternal stress in third trimester

    ; *elevated PG

    ; reduced level of pyloric nitric oxide synthetase

    ; *infant hypergastrinemia

    ; *asso. with eosinophilic gastroenteritis, trisomy 18, Turner syndrome, Smith-Lemli-Opitz syndrome, Cornelia de Lange syndrome

Clinical Manifestation

1. vomiting

    ; initial Sx.

    ; *nonbilious

  ; may or may not projectile initially --> progressive immediately after a feeding

    ; *usually starts 3 wk (1st wk ~ 5th mo)

2. hunger and wants to feed again after vomiting

3. *hypochloremic metabolic alkalosis

4. *maintained serum K⁺ but total body K⁺ deficit

5. ♣Jaundice ; 5%

    ; decreased level of glucuronyl transterase

    ; increased enterohepatic circulation of bilirubin

    ; *수술후 72시간내 소실

3. dehydration, lethargy, weight loss, oldman app.

4. hypochromic alkalosis : 감소 (s-Na, K, Cl)증가 (pH, CO2)

Diagnosis

1. *palpation of mass(60-80%) : confirm Dx.

    ; firm, movable, nontender, 2cm in lenth, olive shapped

    ; site

           - from left side, above and to the right of umbilicus in midepigastrium beneth liver edge

  ; easier to palpate after vomiting

2. visible peristalsis after feeding, just before vomiting

3. ♣Abdominal ultra

    ; *pyloric muscles thickening > 4mm

    ; *pyloric length > 14mm

    ; 90%

4. Barium studies

  ; 'string sign' fine elongated pyloric channel

  ; 'shoulder sign' bulge of the pyloric muscle into the antrum

    ; 'double track sign' parallel streak of barium in narrow channel

※82 Differential Diagnosis

; fed by inexperienced or anxious caretakers

; inadequate maternal-infant bonding relationship

; emaciated infant 시 visible peristalsis

; chalasia, hiatal hernia

; adrenal insufficiency

    - metabolic acidosis, elevated serum potassium, urinary sodium concentration

; inborn error of metabolism

    - *emsis with alkalosis (urea cycle)

    - acidosis(organic acidemia), lethergy, coma, seizure

; gastroenteritis

; pyloric memb. or pyloric dupulication

; Duod. stenosis

; achalasia

Treatment

1. Correcting the fluid, acid-base, and electrolyte losses

    ; 0.45%-0.9% saline

    ; 5-10% dextrose with KCl 30-50mEq

    --> ★till   1) rehydrated

                           2) *serum bicarbonate < 30 mEq

                           3) alkalosis corrected

2. Surgical procedure

    ; *Ramstedt pyloromyotomy

3. Postop. Care

    ; feeding *within 12-24hr and advanced to maintenance oral feedings within 36-48hr

    ; ♥if persistent vomiting(after postoperation)

           ① incomplete pyloromyotomy

     ② gastritis

     ③ hiatal hernia

           ④ chalasia

           ⑤ other cause of the obstruction

4. Conservative medical Tx

    ; *slow improvement, higher mortality

    ① *small frequent feeding thickened with cereal

  ② semiupright position for 1hr or after feeding

  ③ sedation

  ④ adm. of cholinergic blocking agents

  ⑤ parenteral adm. of fluids

5. Endoscopic balloon dilation : trial

Prognosis

; op. mortality  0-0.5%

; medical Tx. ; higher mortality

275.2 Congenital Gastric Outlet Obstruction

; resulting from pyloric atresia and antral web

; uncommon

; 1% of all atresia and diaphragms ass. with epidermolysis bullosa

; M=F

Clinical Manifestation

; nonbilious vomiting, feeding difficulties

; abdominal distention of 1st day of life

; polyhydramnios, LBW

; rupture of stomach (12시간 이내 일어나 수 있다.)

; antral web : less dramatic

Diagnosis

; plain abdomen : large dilated stomach

; UGI : diagnostic 'pyloric dimple'

; endoscopy

Treatment

; correction of dehydration and hypochloremic alkalosis

; nasogastric decompression

; OP repair

275.3 Gastric Duplication

; uncommon *cystic or tubular structures within wall of the stomach

; smaller than 12cm

; not communicated with stomach lumen

; along with lesser curve of stomach

Clinical Manifestation

; gastric outlet obstruction과 연관

; 33%에서 palpable

; Tx

    - Op repair

275.4 Gastric volvulus

# Triad

① sudden of severe epigastric pain

② intractable retching with emesis

③inability to pass a tube into stomach

# Normal stomach

    ; tethered longitudinally by the gastrohepatic, gastrosplenic, gastrocolic lig, gastrophrenic lig. and retroperitoneal attachment of duo.

    --> volvulus ; 위의 attachment중 하나가 없거나 stretch 되어 rotate되어 발생

# *Asso. defects

    ; *intestinal malrotation, diaphragmatic defects, asplenia

# Clinical Manifestation

    ; infancy

           - nonbilious vomiting

           - rapidly strangulation & perforation

    ; older children

           - Hx of emesis, abdominal pain, early stiety

# Diagnosis

    ; plain abdomen

           - dilated stomch

    - mesenteroaxial volvulus

                   / double fluid level

                   / `beak' near the lower esophageal junction

           - organoaxial volvulus

                   / single air fluid level

                   / no ‘beak’

# Treatment

    ; emergency op.

    ; *chronic volvulus : endoscopic correction

CHAPTER 276. Intestinal Atresia, Stenosis, and Malrotation

General Consideration

1. Incidence of intestinal obstruction : 1/1,500 live birth

2. Classification

    1) partial or incomplete : stenosis, constricting bands, duplication, incomplete volvulus

        complete : atresia

  2)  intrinsic : atresia, stenosis, meconium ileus, aganglionic megacolon

    extrinsic : malrotation, constricting bands, intraabd. hernia, duplication

  3)         simple : failure of progression of aboral flow of luminal contents

     strangulation : ass. with impaired blood flow --> bowel infarction and perforation

3. Pathology of intestinal obstruction

  ; accumulation of ingested food, gas, secretion prox. to obstruction

           - distension of bowel

           - decreased intestinal absorption & increased secretion of fluid and electrolytes

           - isotonic intravascular depletion

           - hypokalemia

           - increase in contractile activity proximal to obstruction

           - marked decrease with hypoactive bowel sound

           - nausea and vomiting due to fluid accumulation & hypomotility

           - decreased blood flow

           - loss of mucosal integrity, bacterial proliferation

           - endotoxemia, bacteremia, sepsis

4. ※81,82 Polyhydramnios 있을시

    ; high intestinal obstruction (esp. esophageal atresia)

        --> gastric aspiration immediately after birth

           --> 15-20ml or more gastric fluid or bile stained

           --> suggestive of a high intestinal obstruction             

5. Atresia

    ; *common ileum(50%), duodenum(25%), jejunum

    ; rare in colon, stomach

    ; 33% of neonatal intestinal obstruction

  ; M=F

Clinical Manifestation

; cause, level of obstruction, time between obstruction and evaluation에 따라 다양하게 나타난다.

; classic Sx

    - nausea and vomiting, abdominal distension, obstipation

; Sx. onset

  - Duodenal obstruction : 수시간내,

  - lower intestine : 24시간 이후

; high intestinal obstruction

    - large volume frequent, bilious emesis

  - intermittent pain relieved by vomiting

  - localized in epigastrium and periumbilical area

  - little abdominal distension

; low intestinal obstruction : feculent emesis

  - mod. or marked abdominal distension

    - diffuse pain

Diagnosis

1. Hx. & P/Ex.

2. Laboratory study

    ; not diagnostic

  ; hypochloremic metabolic alkalosis

  ; marked leukocytosis /c or /s thrombocytopenia, metabolic acidosis, hematochezia

           --> bowel infarction

  ; s-amylase & lipase

           - R/O pancreatitis

3. Imaging

  ; plain simple abdomen (S+E) & lat.

         - calcification in peritoneal cavity : meconium peritonitis

         - ground glass appearance in RLQ with trapped air : meconium ileus

           - normal child

                   / 출생 즉시 air in stomach

                   / air within 1hr reach proximal portion of small bowel

                   / *air in distal part of colon as early as 3rd hr or as late as 18hr

  ; ultrasonography

  ; contrast studies

Management

; initial treatment

    - fluid resuscitation and stabilizing

; nasogastric decompression

; broad spectrum antibiotics

; surgical repair

    - strangulating infarction이 의심되면, 즉시 수술

    - medical Tx 12-24시간에 improve없을 때

276.1 Duodenal Obstruction

Duodenal Atresia

; incidence

    - 1/1000

    - 25-40% of all intestinal atresia

  - 1/2 prematurity

; forms

    1) *membrane - ▲common

  2) short fibrous cord connecting blind pouches

  3) gap between non-connecting ends

; site - ampulla of vater 직하부

; 동반질환

    - *Down Syndrome (20-30%)

    - malrotation (20%)

    - esophageal atresia (10-20%)

  - congenital heart disease (10-15%)

    - anorectal and renal anomalies (5%)

Cause Of Duodenal Obstruction

   1. atresia

   2. incomplete rotation of midgut

   3. annular pancreas

   4. duodenal web(=windsock web)

           - malrotation많이 동반

   5. preduodenal portal vein compression

Clinical Manifestation

; *bilious vomiting /s abdominal distension

    - noted in 1st day of life

    - *hallmark

; visualed peristaltic waves

; Hx. of hydramniosis

; jaundice 1/3

; duodenal ileus

Diagnosis

; U/S in Utero

; plain abdomen

    - ※92-92$ ‘double bubble sign’ in erect view

    - no gas below obstruction

; contrast studies

    - *usually not need 

    - *occasionally exclude malrotation & volvulus

Treatment

; Initial Treatment

    - naso- or orogastric decompression & IV fluid replace

; asso. anomalies evaluation

    - echocardiography, radiology

; surgical repair

    - duodenoduodenostomy

  - gastrostomy or jejunal tube to drain

    - division & reposition in Ladd band with malrotation

           / balloon catheter로 coexisting duodenal obstruction을 evaluation

    - duodenoduodenostomy in annular pancrease

    - duodenoplasty in duodenal diaphragmatic obstruction

276.2 Jejunal and Ileal Atresia and obstruction

# Etiology of jejunal and ileal obstruction

  1) atresia & stenosis

  2) meconium ileus

  3) Hirschsprung disease

  4) intussusception

  5) Meckel's diverticulum

  6) intestinal duplication

  7) strangulated hernia

# Cause of jejunoileal atresia

    ; *intrauterine vascular accidents

# Classification

    type I (20%) ; intraluminal diaphragm(continuity is maintained)

      II (35%) ; small diameter solid cord

      IIIa(35%) ; blind loop with small mesenteric defects

      IIIb ; extensive mesenteric defects and loss of normal blood, simply to the distal bowel "apple peel" appearance

      IV ; multiple segment of bowel atresia

# Meconium Ileus

      cystic fibrosis 의 10% 이하에서 발생 ( family Hx. )

      last 20-30cm of ileum

       :collapsed and filled with pellets of pale colored stools, impacted in the ileum

      perforation시 meconium peritonitis

      DDx with meconium plug SD(Lt. colon무증상)

# Hirschsprung disease와 동반 5%

     : aganglionic segment가 entire colon과 terminal ileum

Clinical Manifestation

   abdominal distension and bile stained emesis : 1st day of life                

   fail to pass meconium : 60-75%

   jaundice : 1/5-1/3

   polyhydramnios(25%)

Diagnosis

   U/S in Utero

   Plain abdomen

     1) meconium ileus

        typical hazy or ground glass app. in RLQ with small bubble gas

        mod. dilated bowel loop with air fluid level

     2) meconium peritonitis

        peritoneal calcification in flank

        multiple air fluid levels

     3) Pneumomediastinum의  의심 소견

        ① 24시간내에 rapid abdominal distension

        ② liver percussion시 less dullness

        ③ free fluid within abdomen 'foot-ball sign'

   contrast studies

Treatment

   fluid & E' balance & antibiotics

   surgical repair laparotomy ileostomy colostomy

   Gastograffin enema q 8-12 hr in meconium ileus(50%성공)

276.3 Malrotation

incomplete rotation of the intestine

가장 흔한 Malrotation은 Cecum이 RLQ로 move안되어

  1) volvulus

  2) duo. obstruction by Ladd bands

    #80 동반 질환

 Non-rotation

  1) Midgut volvulus

  2) gastroschisis

  3) omphalocele

  4) Bochdalek hernia

Mal-rotation

  1) annular pancreas

  2) duo. atresia or stenosis

C/M

대부분 1년 이내, 때때로 1주 이내에

volvulus : bilious emesis with acute bowel obstruction

recurrent abd. pain or vomiting in older

malabsorption or protein losing enteropathy

20-50% aSx.

Dx.

    X-ray duo. obstruction : double bubble sign

    Barium enema malposition of cecum(10%에서 정상)

    UGI : malposition of lig. Treitz

 

Tx.

    surgical

    fluid Tx.후 laparotomy

    division of transduodenal bands

    straightened LI in Lt. & SI in Rt. side abdomen

    repair 후 persistent Sx.시

       ` Pseudo-obstruction-like motility disorder'

Chapter 277. Intestinal Duplications, Meckel's Diverticulum And Other Remnant  Of The Omphalomesenteric Duct

277.1 Intestinal Duplication

; well-formed tubular or spherical structures

; mesenteric border에 존재

# Classification

    1) localized duplication

  2) duplications ass. with spinal cord defects & vertebral malformation

           ; hemivertebra, ant. spina bifida, connection between lesion and cervical thoracic spine

  3) duplications of the colon

    4) multiple duplications (10-15%)

# Site of localized duplications

 1) *Ileum ; ▲common

 2) Jejunum

 3) Ileocecal region

 4) Esophagus

# Neuroenteric cysts

    ; intrathoracic duplications of esophagus & stomach

    ; dysphagia & resp. Sx.야기

Pathology

1) Saccular type

    ; gastric mucosa lining(-), not communicated to bowel lumen, peptic ulcer(-)

2) tubular structure within the wall

    ; *gastric mucosa lining(+), communicated to bowel lumen

  ; *peptic ulcer, bleeding, perforation가능

Clinical Manifestation

    ; infants와 early childhood에 주로 발생

    ; depend on size, location, gastric mucosa lining

    ; bowel obstruction, lead point of intussusception, site of volvulus

    ; ulcer, perforation, bleeding, abd. pain, vomiting, palpable mass

  ; respiratory distress( neurogenic cyst )

    ; prolapse of rectum : diarrhea, constipation

Diagnosis

    ; Hx. & P/E

    ; barium studies

    ; U/S

    ; CT : intrathoracic lesion

    ; MRI : spinal cord lesion

    ; Radioisotope 99mTc scanning : ectopic gastric tissue

Treatment

    ; surgical repair

277.2 Meckel's Diverticulum and other Remnants of the Omphalomesenteric Duct

; remnant of the embryonic yolk sac(=omphalomesenteric duct or vitelline duct)

; *2-3% of all infants

; *▲common residual structure

; *▲frequent congenital GI anomaly

Pathology

; 3-6cm outpouching along of ileum 50-75cm prox. to ileocecal junction on antimesenteric side

; 최소 35%에서 ectopic gastric or panc. tissue

Clinical Manifestation 

; *usually arise within 1st 2yr, common during 1st decade

; ※93$ painless rectal bleeding by ulceration of adjacent normal ileal mucosa

    - not neutrlized by pancreatic bicarbonate

; stool

    - brick colored or currant jelly colored

; anemia

    - self-limited because contraction of splanchnic vessels

; partial or complete obstruction

    - by lead point of an intussusception

           / *▲common mechanism

    - by intraperitoneal bands

           --> internal herniation or volvulus

; diverticulitis

    - similar to appendicitis

    - perforation & peritonitis

Diagnosis

# ※82 99mTc Meckel radionuclide scan

    ; *▲sensitive study

    ; *enhanced scan by cimetidine, glucagon, gastrin

           - *sensitivity 85%, specificity 95%

Treatment

; Complication

    - hemorrhage

  - diverticulitis -> perforation, peritonitis

  - intussusception

  - volvulus

  - Littre hernia : indirect inguinal H. 에 M. div. 호발

; surgical excision

    - choice

Chapter 278. Motility Disorders And Hirschsprung Disease

278.1 Chronic Intestinal Pseudo-obstruction

; anatomic lesion없이 intestinal obstruction의 증상과 증후를 보이는 질병군

; primary or secondary

; transiently or permanently

; Site

   SI > colon > stomach > esophagus

Pathology

; wide spectrum from abnormal myoelectric activity to abnormalities of nerves(intestinal neuropathy) or musculature(intestinal myopathy)

; ★congenital form

    - *spordically onset

    - *disorganized or hypo- or hyperganglionosis

    - *outer longitudinal muscle layer is replaced by fibrous material

Clinical Manifestation

; symptom *within first few months of life

; *수일내에 증상 보이는 환아

    - *2/3는 premature이며, 약 40%는 malrotation

; 75%는 첫 1년에 경험

; ★abd. distension & vomiting

    - *▲common (75%)

; constipation, failure to thrive, abd. pain (60%)

; diarrhea (30-40%)

; UTI (15%)

; urinary tract dilatation (30%)

; wax and wane symptom

Diagnosis

; anatomic obstruction이 없으면서 compatible symptome이 있어야 한다.

; plain abdominal X-ray

  - air fluid level

  - microcolon ( in neonate )

; contrast studies

    - slow passage of barium

; gastrointestinal manometry

    - involved intestine에 따라 abnormal motility pattern을 보인다.

    - *anorectal motility는 정상을 보여 Hirschsprung diseae와 감별할 수 있다.

; radionuclide scan

    - delayed gastric emptying

; exploratory laparotomy

    - *진단을 위해서는 필요가 없다. 만약 시행된다면, furture adhesive obstruction with exacerbation of symptoms의 가능성이 있다.

Differential Diagnosis

    1. Hirschsprung Ds.

    2. Other Causes Of Mech. Obst.

    3. Psychogenic Constipation

    4. Neurogenic Bladder

    5. SMA Synd.

    6. Hypothyroidism

    7. Narcotics

    8. Scleroderma

    9. Chagas Ds.

    10. Hypokalemia

    11. Diabetic Neuropathy

    12. Amyloidosis

    13. Porphyria

Treatment

; *Nutritional Support

    - ★mainstay of treatment

    - *30-50%는 partial or complete pareteral nutrition

; prokinetic drugs

    - cisapride(5-HT receptor antagonist)

    - *erythromycin(motilin receptor angonist)

; oral AB for bacterial overgrowth

; Tx of constipation

    - enema, suppositories, bowel softner

; Tx of peptic symptom

    - acid suppressors

; surgery

    - *gastrostomy or placement of jejunostomy을 제외하고는 도움이 되지 않는다.

    - *colectomy

           / *abnormalities가 colon에만 국한되어있는 경우에 고려

; *bowel transplantation or electromechanical pacing

278.2 Superior Mesenteric Artery Syndrome (Wilkies SD. Cast SD. Arteriomesenteric Duodenal Compression Syndrome)

# ♥Definition

; extrinsic compression of the duodenum (2nd and 3rd portion) after weight loss (loss of supporting fat) and in supine position between SMA ant. and Aorta post. against spine

; classic description

    - *adolescent가 정형외과 수술을 위한 body cast후 vomiting 발생

; asso. factors

    - anorexia, prolonged bed rest, Wt. loss, abd. surgery, lumbar lordosis

Diagnosis

; plain abd. X-ray

    - cutoff of the duodenum just to Rt. of the midline

    - proximal duodenal and gastric dilatation

Treatment

; *positioning in lat. & prone position

; Prokinetic agents

    - Cisapride

; nasojejunal tube

; TPN

; surgical intervention

278.3 Congenital Aganglionic Megacolon (Hirschsprung Ds.)

; caused by abnormal innervation of bowel, beginning in internal anal sphincter & extending proximally

; *▲common cause of neonatal obstruction of colon

  - all neonatal obst. 의 33%

; *male : female = 4 : 1

; incidence 1/5,000

; associated congenital defects

  1. 21-Trisomy

  2. Laurence-Moon-Biedl-Barat Synd.

  3. Waardenburg Synd. & Megasystis - Megaureter

  4. Cardiovascular abnormalities

; premature infant : rare, familial incidence ( 4-8% ) : 가끔

Pathology

; arrest of neuroblastoma migration from proximal to distal

    --> absence of ganglion cells

    --> increase nerve ending in aganglionic bowel

    --> high concentration of acetylcholinesterase

; *absence of Meissner and Auerbach plexus and hypertrophied nerve bundle with high concentration of acetylcholinesterase between muscular layer and submucosa

; *knockout of endothelin B receptors

; involved lesion

    - *rectosigmoid (75%)

  - anus to hepatic flexure 까지의 colon (15%)

  - entire colon (10%)

Clinical Manifestation

; usually begin at birth with delayed passage

    - noraml meconium passage

           / 99% of fullterm within 48hr

    - some infant는 정상적으로 meconium passage후 chronic constipation을 보이기도 한다.

; vomiting, abd. distention

    --> decreased blood flow

    --> bacterial overgrowth

    - enterocolitis by Cl. difficile, Sta. aureus, anaerobes, coliforms

    - *enteroclitis 발생 전에 발견해야 morbidity & mortality감소

; failure to thrive with hypopreteinemia from protein-losing enteropathy

; *palpable large fecal mass in LLQ but empty in rectum

; BMF는WMF보다 not severe

★Table 278-1 DDx of Hirschsprung diseae and functionl constipation

# ♥DDx In Infancy

    ; small left colon synd. in infant of DM mother   

    ; meconium plug synd.                       

    ; ileal atresia with microcolon                  

    ; malabsorption synd                         

Diagnosis

# Rectal manometry and rectal suction biopsy

    ; *▲reliable indicators

# Rectal manometry

    ; measures pressure of internal anal sphincter muscle while balloon is distended in rectum

    ; normal

           - reflex drop in internal sphincter muscle pressure

    ; patients

           - *fail to drop or paradoxical rise in internal sphincter muscle pressure

    ; *normal 이면 exclude Hirschsprung disease, but equivocal or paradoxical response이면 punch or suction biopsy

           - ※96신생아에서 false positive

           - dentate line 2cm이내에서는 시행하지 않는다.

    ; *more than 90% accuracy

# X-ray

    ; presence of transitional zone

           - not usually present before 1 to 2wks

    ; ※96 without prepration to prevent transient dilatation

    ; *barium enema는 진단 목적 보다는 evaluating other diseae & determining extent가 더 의의가 있다.

    ; 24hr delayed film more helpful

           - significant barium

# ※주 Barium Enema소견

    ; abrupt caliber change between gang. & agang. section of bowel

  ; irregular sawtooth contractions of agang. seg.

  ; parallel transverse folds in dilated prox. colon

  ; thickened, nodular, edematous prox. colon ass. with protein-losing enteropathy

  ; failure to evaduate barium

# ※82,83 Classic Feature 잘 안보이는 경우

    ; newborn

           - prox. colon과 distal agang. colon사이의 size disparity 나타낼 시간적 여유 부족

  ; entire colon

  ; ultrashort segment

Treatment

; operative intervention

; limited laparotomy with multiple biopsy with colostomy in most distal portion of normal gangl. colon

; temporary colostomy and then definite repair at 6-12 mo

    - three surgical options

  ① Swenson

           / anastomose normal proximal bowel to retum 1-2cm above the dentate line

  ② Duhamel

           / create neorectum

           / anterior agnaglionic half with normal sensation & posterior ganglionic half with normal propulsion

  ③ Boley

           / endorectal pull-through procedure

# ultrashort segment

    ; limited to internal sphicters

    ; normal rectal suction biopsy but abnormal rectal motility

    ; internal sphicter muscle을 포함한 biopsy

# total colon aganglionosis

    ; ileal-anal anastomosis with preserving agang. colon

    ; Duhemel op.

Prognosis

; generally satisfactory for fecal continence

; early colostomy 시 mortality 4%

; enterocolitis 후 mortality 33%

Chapter 279. Ileus, Adhesion, Intussusception, And Closed Loop Obstruction

279.1 Ileus

; failure of intestinal peristalsis without evidence of mechanical obstruction

★Etiology

    1. abd. surgery - *▲common

  2. infection, (Pn., AGE, peritonitis)

  3. metabolic abn. (uremia, hypikalemia, acidosis)

  4. drugs(*vincristine, loperamide)

Clinical Manifestation

; increasing abd. distension

; abd. pain initially minimal

Diagnosis

; plain abd.

    - multiple air fluid level thoughout the abdomen

; contrast radiography

    - slow movement of barium through the patent lumen

Treatment

; correction of underlying causes

; nasogastric decompression

; prokinetics

    - cisapride, erythromycin

279.2 Adhesion

    fibrous bands

    post. op. SI obstruction after abd. surgery : 2-3%

    single after 2 weeks

Clinical Manifestation

   pain & distension nausea vomiting

   fever & leukocytosis : necrotic bowel and peritonitis

Diagnosis

   P/E bowel sound hyperactive and abdomen flat initially

     then, bowel sound disappear and abdomen distension

   Plain abd.

   contrast radiography

Treatment

   nasogastric decompression

  IV fluid resucitation

   Broad spectrum antibiotics

   surgical intervention

279.3 Intussusception

; Incedence 1-4/1,000 

; M:F=4:1

; *▲common intestinal obstion between 3mo and 6yr

    - *rare in younger than 3mo, decrease in frequency after 36mo

Etiology & Epidermiology

; unknown

; seasonal incidence

    - *peaks in spring & autumn

; correlation with adenovirus infection

# Lead Points

    - *more common in very young and older patients

    - 5-10%

    - *inverted appendiceal stump, Meckel's diverticulum, intestinal polyp, duplication, lymphosarcoma

    - uncommonly

           / H-S purpura with intramural hematoma

  - rare

           / *post. op.(always ileoileal), dehydrated patients with cystic fibrosis, metastatic tumor, hemangioma, foreign body, parasitic infection, fecolith, after cancer chemotherapy

Pathology

# Site

  1. ileocolic & ileoilecolic ; *▲often

  2. cecocolic

  3. ileal

# intussusceptum

    ; upper portion of bowel, invaginated into the lower

    ; *dragging its mesentery along with it into the enveloping loop

# intussuscipiens

    ; lower portion of bowel

# *after 24hr

    ; *intestinal gangrene and shock

Clinical Manifestation

; sudden onset of severe paroxysmal coliky pain in previously well child

; initially maybe comfortable and play normally between paroxysm of pain

  --> progressively weaker and lethargic

    --> moaning sound

  --> shock like state

           / fever, weak and thready pulse, respiration shallow and grunting

; vomiting

    - more frequent early

; stool

    - normal during 1st few hr of symptom

    - *blood in first 12hr (= current jelly stool (60%))

           / infrequently not at all

; *sl. tender saussage mass(70%)

    - increase size & firmness during paroxysm of pain

    - *▲often in RUQ & long axis cephalocaudal

  - if felt in epigastrium

           / long axis or transverse

    - *30% not palpable

; abd. distension & tenderness

  - more acute

; ileoileal intussusception

    - less typical Sx

    - SI obstruction Sx. Sn.

; *recurrent intussusception

    - *5-8%

    - *more common following hydrosttic than surgical reduction

; chronic intussusception

    - symptom exist in milder form at recurrent interval

    - following acute enteritis

    - older children

Diagnosis

1. clinical Hx. & P/E

2. plain abd.

    ; mass density

3. barium enema

  ; *filling defect or cupping in the head of barium

    ; *coiled-spring sign

    ; *ileoieal intussusception은 진단이 되지 않고, Plain abdomen상 lesion위쪽으로 gaseous distension으로 의심하는수 밖에 없다.

4. air enema

    ; Dx. & Tx.

    ; *safer with less risk of perforation

    ; as accurate as barium enema

    ; less irradiation

5. real time sonography

    ; useful Dx.

  ; target or donut configuration of bowel

         - hypoechoic rim & dense central echogenic core

         - *rim thickness 0.6 cm↑

           - *rim thickness 1.6 cm 이상시 수술 필요

Differential Diagnosis

1. gastroenteritis

2. enterocolitis

3. Meckel's diverticulum

4. anaphylactoid purpura

Treatment

; reduction이 emergency procedure

    - after diagnosis & rapid preparation for operation with fluid & blood for shock and water & electrolyte to replace losses

; hydrostatic or pneumatic pressure under fluoroscopic or ultrasonographic guidence with consultation and close proximity of surgeon

; more than 74% of cases

    - short duration

  - no sign of prostration, shock, peritoneal irritation, pneumatosis intestinalis

※92주 Reduction Findings Of Intussusception

    ; free filling of small intestine

    ; disappearance of mass

  ; passage of flatus or feces

  ; improvement of infant's condition

# ♥Surgical Indication

    ; prolonged intestinal obstruction with peritonitis

    ; ileoileal type

Prognosis

; 24시간 이내는 recover

; 2일 이후는 mortality증가

; spontaneous reduction during preparation for op.

; unTx.시 거의 fatal

; ★Recurrence Rate

    - after barium enema : 10%

    - surgery (manual) ; 2-5%

    - resection : 0%

; *lymphosarcoma, polyp, meckel's diverticulum는 barium enema에 의한 reduction이 거의 안된다.

279.4 Closed loop Obstruction

    defects in the mesentery ( internal hernia )를 통해 SI loop가 pass & trap

    vascular engorgement of trapped bowel ,intestinal ischemia & gangrene

 

C/M

   transient or recurrent or spontaneous relieve

   bilious vomiting

   abd. distension, abd. pain

   peritoneal sign : bowel ischemia

 

Dx. plain rad. sign of small bowel obstruction

              free air : perforation

 

Tx. :   prompt surgical relief

CHAPTER 280. Foreign Bodies and Bezoars

280.1 Foreign Bodies in the stomach and intestine

 peak incidence 6Mo-3yrs

종류

   1. young children  : coin

   2. older children : fish or chicken bone

 

risk factor

   1. after alcohol consumption or cold liquid

   2. psychiatrically impaired pt.

 

outcome

   80-90% spontaneous pass (보통 4-6일, 또는 3-4주)

   10-20% endoscopic removal or conservative Tx.

   1% or less surgical intervention

   1% or less perforation

         site : physiologic sphincters

                acute angulation

                congenital gut malformation

                previous bowel surgery

 

Dx.   plain rad.(chest X-ray 포함) & contrast rad.

 

Tx. regular diet

    endoscopic removal

     1. older children : > 5cm in diameter, 2cm in chickeness, 10cm in length

     2. infants : > 20mm in diameter, 3cm in length

     3. needle, hair, pin, hobby pin, open safety pin

   surgery : perforation sign

   laxatives : CIx. (intestinal motility증가로 perforation증가)

280.2 Bezoars

    accumulation of exogenous matter in the stomach or intestine

    most in female, 2nd decade, with underlying personality problem

           or  neurologically impaired

    trichobezoars( hairs ), phytobezoars( plants & animal )

    lactobezoars( casein or calcium in premature )

 

C/M

    Sx. of gastric outlet or partial intestinal obstruction sign,

    vomiting, anorexia, Wt. loss, abd. pain or distension halitosis

    동반질환 : chr. gastritis, IDA, hypoproteinemia, steatorrhea

 

Dx.

    P/E : patchy baldness and a firm mass in LUQ

    plain or contrast rad. or ultra

    endoscopy

 

Tx.

    endoscopic disruption or removal

    surgical intervention

    lactobezoars : 24-48시간 feeding하면 resolve

Chapter 281 Anorectal Malformations

Incidence 1/4,000

Embryology & Pathogenesis

    ; origin cloaca

Pathology And Classification

Male

Perineal Fistula

    ; ▲simplest defect in both sexes

    ; bucket-handle type malformation or blck ribbon type stucture in perineum

    ; well-formed sacrum

    ; affecting other organ - 10%

    ; *repaired without protective colostomy

Rectourethral Fistula

    ; *▲frequent anorectal defect in male patients

    ; flat-looking perineum

    ; hypodevelopmental sacrum

    ; protective colostomy during newborn periods

Rectovesical Fistula

    ; poorly developed sphincteric mechnism

    ; flat-looking perineum

    ; poor prognosis of bowel function

    ; mandatory colostomy

Imperforate Anus Without Fistula

    ; blind retal loop 2cm above perineal skin

    ; *well-developed sacrum, sphincteric mechanism

    ; good functional prognosis

    ; *ass. with down syndrome

Rectal Atresia

    ; normal anus & anal cannal

    ; obstruction 2cm above perineal skin

    ; good functional prognosis

    ; protective colostomy

Female

Vestibular Fistula

    ; *▲frequent defect in females

    ; normal sacrum

    ; excellent prognosis

Persistent Cloaca

    ; fuse into single common channel

    ; short common channel(<3cm)

           - well-developed sacrums & good sphincters

    ; longer than *3cm channel

           - more complex defect

           - poor sphincteric mechanism & poor sacrum

           - hydrocolpos

           - *diverting colostomy

           - *90% urologic defect

                   / *colostomy전 반드시 urinary evaluation

 

	No colostomy	Colostomy
Males	Perineal Fistula	Rectourethral Fistula Rectovesical Fistula Imperforated Anus without Fistula Rectal Atresia
Females	Perineal Fistula	Vestibular Fistula Persistent Cloaca Imperforated Anus without Fistula Rectal Atresia

 

Diagnosis & Early Management

# ♥▲Important Decision Regarding Newborn With Anorectal Malformation

    ; whether needs diverting-decompression colostomy and emergency urinary diversion for associated obstructed uropathy

Males

    ; perineal or rectourinary fistula는 16-24hr이내에 meconium이 pass되기때문에 진단하기 어렵다.

    ; gauze test

    ; clinical sign(+)이면 24hr observation

           - and then radiologic evaluation

                   / *cross-table lateral film in prone position for determining position of rectal pouch

                           --> *skin으로부터 1cm이상이면 colostomy

    ; *all patients need abdominal ultrasound evaluation

Females

    ; meticulous perineal inspection : 90%

  ; single perineal orifice or palpable pelvis mass

           - persistent cloaca

Associated Defects

    ; *50% of children with anorectal anomalies

    ; ★Urologic Problem

       - *90% 이상 : male rectovesical fistula and persistent cloaca

       - 10% 이하 : rectoperineal fistula

           - *evaluation before performing a colostomy

    ; *good correlation between degree of sacral development and final functional prognosis

    ; spinal or sacral abn., esophageal or duodenal atresia, cardiovascular defects

Treatment

; perineal fistula : anoplasty without protective colostomy during infant

; other fistula

    - protective colostomy during infant

  - corrective surgical repair, later in life (1-12mo)

; posterior sagittal approach

    - uses an electric muscle stimulator to idendify the sphincteric mechanism

    - direct exposure to internal anatomy

    - avoid potential damage to important sturcture and nerves

Prognosis

; perineal fistula & rectal atresia : good

; males rectourethral bulbar F. : 80% bowel control, 3-4yrs

; both sexes imperforated anus without fistula : same above

; male rectourethral prostatic F. : 60% bowel control, 3yrs

; *male rectovesical fistula : 20% bowel control, 3yrs

; female rectovestibular F. : 90% bowel control, 3yrs

; persistent cloaca with common channel (<3cm) : 80% bowel control, 3yrs

; *         "        "              "    (>3cm) : most fecal incontinence

Complication

; fecal or urinary incontinence

; constipation

    - *more severe in lower and simpler defects

Chapter 282. Ulcer Disease

; uncommon in children

    - ※93 2세이전 DU=GU

    - 2세이후 DU>GU

Pathology And Physiology

# ★Predisposing Factors

    1. gastric acidity DU(high) GU (nl or low)

    2. blood type O

    3. high serum pepsinogen I

    4. genetic factor

           ; family Hx in duodenal ulcer (25-50%)

    ; concordance for duodenal ulcer in monozygotic twin (50%)

    5. cigarette smoking

    6. climatic condition

    7. diet(alcohol) 

    8. emotional strain

    9. tissue resistance

        ; cell turnover, mucus production, bicarbonate secretion, local PG synthesis, tissue assult

           ; factors lowing resistance

                   - anoxia, poor perfusion, bile and drugs (esp, NSAID)

    10. Helicobacter pylori

    ; present in gastric mucosa

           ; cause chronic lymphonodular gastritis

           ; eradication of H. pylori

                   - asso. with healing & lone-term cure of recurrent peptic ulcer

# Pathology

    ; superficial abrasion, deep erosion, penetraion, hemorrhage, perforation

    ; abrasion

           - 1cm ro less in diameter

           - fibrinus coat of leukocyte & red cell cover fibrinoid necrosis surround by inflammatory cells

    ; acute or chronic gastric obstruction

    ; ※93 Sites

     DU : post. part of bulb

     GU : lesser curve or antral area

282.1 Primary (Peptic) Ulcers

; ★usually chronic, duodenal, related to H. pylori

Clinical Manifestation

; variable & non-specific

; vomiting, G-I blood loss, pain, familial incidence

# 1st 1 mo

  ; ※93 2 most common Sx.

        - GI hemorrhage

        - perforation

# neonate - 2yr

    ; *recurrent vomiting, slow growth, GI hemorrhage

# preschool age

  ; *periumbilical postprandial pain

           - often elicited

    ; vomiting, hemorrhage

# 6 yr↑

    ; adult와 유사

    ; epigastric abdominal pain, acute or chr. GI blood loss(hematemesis, hematochezia, melena)

    ; IDA

  ; *male ↑

  ; strong family Hx.

    ; characteristics of pain

           - dull or aching than sharp or buring as adult

           - last from minutes to hours

           - frequent exacerbation and remission lasting from weeks to months

           - common nocturnal pain

    ; *history of typical ulcer pain with relief after taking antacids

           - *33%

Diagnosis

# UGI

    ; acute Sx.이 아닌 경우, *most useful available test

    ; *DU 75%, GU 0% detect

    ; true deformity of bulb -> past uler ds.의 good sg.

    ; spasm of bulb는 normal

  ; DDx

           - duodenitis, irritability of bulb, pylorospasm

# ※93 Gastroduodenoscopy

    ; Indication

           - X-ray상 questionable or absent in Sx. pt.

    - X-ray상 healing 되었어도 Sx. 지속

    - ulcer crater의 prolonged presence

    - acute UGI bleeding시 blood clot제거할 수 있다면 choice

# Selective Abdominal Angiogram

    ; active, severe UGI bleeding시 -> vasoconst. agent of embolization

# Gastric Acid Analysis

  ; recurrent  severe ulcer or multiple ulcer시

  ; gastrin level check for Z-E synd.

Differential Diagnosis

    ; esophagitis, giardiasis, GE reflux, Meckel's diverticulum, pancreatitis, inflammatory bowel ds. cholelithiasis, appendicitis, recurrent abdominal pain of childhood

Treatment

# Goal Of Therapy

  i) healing of ulcer 촉진

  ii) relieve pain

  iii) prevent Cx.

# Recurrence

    ; 6세이하 25%, 6세이상 70% 

1. normal diet

  ; aspirin, alcohol, smoking금지

  ; bland diet, cola, coffee, spiced food의 금지가 healing촉진시키지는 않음.

2. antacid .... mainstay

    ; *liquid form > tablet

  ; full-dose

           - 100mEq of stomach acidity/m² 중화하는 15ml/ dose antacid

           - *소아 용량 : 1ml/kg/dose

    ; *1-3hr after a meal & at bedtime

           - *▲effective

    ; bed time snack 금지

  ; therapy for : 4-6 wk

    ; side effect

       - Mg

                   / diarrhea

       - aluminum

                   / constipation

                   / *phosphate deletion & aluminum toxicity

                           : dietary phosphate와 bind하여 P의 흡수 장애로 인해 발생

                           : *anorexia, osteomalacia, osteoporosis

       - calcium antacid

                   / buffering effect 끝난후 acid분비 증가

       - NaHCO3

                   / chr. use 시 systemic alkali, Na load

3. H₂ antagonist

    ; 12세 ↑

  ; single dose at night -> recur 예방

  ; cimetidine : (20-40 mg/Kg/day #4) meal time, bed time

  ; ranitidine : (4-6 mg/Kg/day #2)

4. sucralfate : local coating action

5. omperazole

    ; *potent H+-K+ ATPase pump inhibition

    ; single dose in delayed released capsule

5. anticholinergic drug

    ; gastric acid secretion inhibit

    ; *dry mouth, blurred vision 나올 때까지 high dose 필요하므로 first therapy로는 부적절

6. ※96주 Tx. for H. pylori

    ; *not responding to therapy and recurrent disease일 경우 H. pylori에 대한 검사 요

    ; *Amoxicilin, metronidazole, bismuth for 2wks

7. Surgery

    ; ★Indication

           - perforation

     - intractable pain or chronic bleeding

    - embolization이나 cautery로 조절되지 않는 blood loss more than 30% within 48hr from hemorrhage

           - nasogastric drainage로 72시간내 호전되지않는 gastric outlet obstruction

    ; vagotomy + pyloroplasty

282.2 Secondary Ulcers

; *primary ulcer보다 2배 이상

Stress Ulcers

; often multiple, ass. with hemorrhage gastritis

; Causes

    1. infants : *sepsis, resp. or cardiac insufficiency or dehydration

  2. older children : trauma, life threating condition

    3. Curling ulcer : burn (13%)

  4. Cushing ulcer : head trauma, surgery

; perforation or acute massive painless bleeding

    - often initial symptom

Drug-related Ulcer

;Aspirin or NSAID(25%), alcohol and smoking, steroid

Treatment

; similar to primary U.

; maintaining gastric pH > *3.5

; *Misoprostol

    - PG analog

    - *prevent delvelopment of gastric ulcers in chronically taking NSAID

282.3. Zollinger-Ellison Syndrome

; multiple recurrent duodenal & jejunal ulcers

; gastric secretion ↑ & hypertrophy of gastric fold on X-ray, diarrhea

; islet cell tumor or hypertrophy -> ↑↑ gastrin (-> acid↑)

; Treatment

    - chronic cimetidine therapy or omperazole

  - gastrectomy

CHAPTER 283. Inflammatory Bowel Disease

; unpredictable exacerbations and redission

; dimodal distribution

    - 15~25yr, 50~80yr

    - *▲common during adolescence and young adult

; genetic & environmental components

    - *asso. genetic disorders

           / Turner syndrome, Hermansky-Pudlak syndrome, glycogen storage disease type Ib, immunodeficiency disorders

; DDx of CD & UC

    - clinical, radiologic, endoscopic, histopathologic findings을 종합하여 진단

    - 10%는 정확히 진단하지 못한다.

    - 치료에 있어 공통적인 부분도 있지만 refinements에 있어 차이가 있어 구분이 중요하다.

; extraintestinal manifestation

    - *more common in CD

    - *peripheral arthritis, erythma nodosum, anemia는 bowel disease의 activity와 연관성

    - pyoderma gangrenosum도 bowel disease의 activity와 약간의 연관성

    - arthritis            

           / three pattern

                   : migratory peripheral arthritis involving primarily large joints

                   : ankylosing spndylitis

                   : sacroiliitis

           / *peripheral arthritis는 nondesturctive

- skin manifestation

           / *erythma nodosum이 ▲common lesion

           / erythma nodosum, pyoderma gangrenosum은 high likehood of having arthritis

- glomerulonephritis, hypercoagulable state, cerebral thromboembolism, uveitis

           / common in childhood

283.1 Chronic Ulcerative Colitis

; colonic mucosa에 국한되고 UGI는 sparing되는 idiopathic chr. inflammatory disorder

; always begins in the rectum

; ulcerative proctitis - localized in rectum

; 20-50% of UC

    - less systemic mani. & less response to Tx.

; pancolitis

    - involving entire colon

Etiology

; unknown

; immunologically mediated Rx.으로 생각

Incidence

; 1/10,000( Japan, South Africa) 15/10,000( northern Europe, USA )

; *male slightly more frequent

Clinical Manifestation

; insidious onset with gradual progression or fulminant course

; mild dysentery (bloody diarrhea with mucus)

    - *▲typical presentation

; tenesmus, urgency, crampy abd. pain(esp. with bowel movement), nocturnal bowel movement

    - more severe colitis를 의미

; *fever, anemia, hypoalbuminemia, leukocytosis, stool frequency (>6/day)

    - *fulminant colitis를 의미

; chronicity( > 3-4 wks)가 진단에 중요한 역할

; anorexia, wt. loss, growth failure (more typical in Crohn diasease)

; ★more common extraintestinal symptom than CD

    - pyoderma gangrenosum (necrotic skin lesion), sclerosing cholangitis, CAH, ankylosing spondylitis

; IDA

    - chronic blood loss로 발생

; folate deficiency

    - *sulfasalazine으로 치료하는 환아에서 흔함.

    - folate 흡수를 방해하기 때문.

; clinical course

    - exacerbtions often without apparent explanation

; colon cancer risk

    - *increase 8-10yr of disease and then increase by 0.5-1% per year

    - childhood onset not increase risk further

    - Prevention

           / ★10yr duration후에는 biopsy every 1-2yr

Differential Diagnosis

; infectious colitis, crohn disease

㉿Table 283-1 Comparison of crohn disease and ulcerative colitis

★Table 283-2 Infectious agents mimicking inflammatory bowel disease

; colitis of HUS, Behcet syndrome, cathartic colitis, radiation procitis, human immunodeficiency virus-associated colitis, ischemic colitis, protein intolerance

Diagnosis

; not confirm test

; typical presentation in bsence of identifiable specific causes

; chronicity - 3-4 wks

    - firm diagnosis

; laboratory finding

    - anemia, hypoalbuminemia

    - elevated ESR

           / maybe normal even with fulminant colitis

    - leukocytosis

           / only seen with more severe colitis

    - *antineutrophil cytoplasmic antibodies

           / *65% of UC, 20% of CD

# Endoscopic examination

    ; more sensitive for mild disease

    ; typical features

           - concentric, diffuse colitis starting in rectum & extending proximally for variable distance

           - no skip area

    ; colonoscopic exam

           - fulminant case인 경우에는 금기이다.

           - microulcers giving appearance of diffuse abnormality

                   / ulcer가 없는 경우에는 CD일 경우가 높다.

           - *earlist : granularity and loss of nl. vascularity

        - severe change : spontaneous friability, erythema, edema of mucosa, blunting of mucosal fold

           - *very severe change : pseudopolyp

# perianal disease or anal fissures with diarrhea는 CD의 가능성이 더 높다.

# Biopsy

    ; acute and chronic mucosal inflammation

    ; typical findings

           - cryptitis, crypt abscess, separation of crypt by inflammation cell, foci of acute inflammatory cell, edema, mucous depletion, brancing of crypts

# Plain Abdomen

    ; loss of haustral markings in air-filled colon, marked dilatation with toxic megacolon(>6cm in diameter)

# Barium enema

    ; diffuse, concentric disease with finely spiculated border

           --> microulceration

    ; earlies change - fine granularity followed by more coarse granularity

           --> progressing severe disease

                   - *‘collar button ulcers’

    ; thickened mucosal folds, completely obliterated to give smooth-appearing surface

           - *“lead pipe colon”

           - reversible with treatment

    ; normal terminal ileum

           - except) pancolitis

                   / *dilated terminal ileum with patulous ileocecal valve : “backwash ileitis”

Treatment

# aim

    1) control Sx.

  2) reduce risk of recurrence

# spontaneous improvement in Sx. : 20-30%

# ※96 Drug Medication

    ; mild colitis때 사용.

    1) aminosalicylate

           - sulfasalazine 50-75mg/Kg/day #2-4, no more than 2 or 3g/day

                   / sulfur moiety가 active ingredient 5-aminosalicylate와 연관되어 있어 소장에서는 흡수되지 않고, 대장에 이르러 bacteria에 의해 2개의 components로 분리 된다.

                   / *점차로 용량을 증량하여 치료 1주경에 full-dose

                   / remission이 생기더라도 계속 사용

                   / *only drug shown to prevent relapses

                   / Side Effects

                           : *hypersensitivity, skin rash(typically morbiliform), hepatopathy, fever, exfoliative dermatitis(stevens-Johns syndrome)

                           : others - reversible leukopenia, hemolysis, pancreatitis, headache, nausea & vomiting, bloody diarrhea

                           : bloody diarrhea 는 disease progression과 DDx 가 어렵다.

           - olsalazine

         / sulfasalazine에 side effect을 가진 환아의 10-20%는 다른 5-aminosalicylate에도 역시 side effect을 나타낸다.

                   / diarrhea가 common side effect

    2) Hydrocortisone enemas

           - 100mg once a day for 2-3wks

    3) Corticosteroids

           - *severe pancolitis이거나 5-aminosalicylate에 반응하지 않는 경우

           - prednisone 1-2mg/Kg/day (below 40mg) for 3-4mo

                   / *not severe symptom인 경우에는 single morning dose로 사용하고, severe colitis이면, daily divided dose 로 사용한다.

                   / *1-3mo내에 alternate-day dose로 바꾼다.

           - Side Effects

                   / growth retardation, adrenal suppression, cataracts, aseptic necrosis of femoral head, DM, risk of infection, cosmetic effects

    4) azthioprine, 6-MP, metronidazole, cyclosporine

# ♥Surgical Therapy

    1) Steroid 치료에 반응을 하지 않거나 dependent인 경우

    2) fulminant disease

           ; 3-4wks간의 치료에 불구하고, 반응을 하지 않는 경우

           ; > 6-8 stool/day, passage of stools during night, fever, leukocytosis, anemia, hypoalbunminemia

Prognosis

; *remission and exacerbation

; *대부분의 환아가 초기 medical management 에 잘 반응한다.

; risk of colon cancer

    - after 8-10 yrs, 0.5-1% /yrs

  - proctitis alone에서는 발생않으며, mucosal dysplasia거쳐 발생

  - 10년 이후에는 매 1-2년마다 colonoscopy와 biopsy시행

; toxic megacolon

    - most serious acute Cx.

    - impending perforation sign

283.2 Crohn Disease (=Regional Enteritis, Regional Ileitis, Granulomaous Colitis)

; Mouth에서 Anus까지의 위장관의 idiopathic chr. inflammatory disorder

    - eccentric & segmental with skip area

    - transmural gastrointestinal involvemnent

; site

    - initial ileum & colon

           / ileocolitis

           / *▲common

    - small bowel alone

           / terminal ileitis

           / 50%

    - colon alone

           / granulomatous colitis

           / 10%

Incidence

; rare in 1st 10 year of life

; bimodal age distribution

    - 1st peak beginning late teens

; *과거 10년동안 빈도가 증가하고 있지만, UC는 stable

; 3-4/100,000

; prevalence 30-100/100,000

Clinical Manifestation

# Region of Bowel Involved, Degree of Inflammation, Presence of Complication(Stricture Or Fistular)에 따라 증상이 다르다.

    ; ileocolitis

           - crampy abd. pain, diarrhea sometimes with blood

    ; ileitis

           - RLQ abd. pain alone

    ; colitis

           - bloody diarrhea, tenesmus, urgency

    ; gastric or duodenal involve

           - recurrent vomiting, epigastric pain

    ; partial SI obstruction Sx.

           - crampy abd. pain, borborygmi, abd. distension

           - sudden gurgling of intestinal contents후 symptom relief가 있으면, stricture의심

    ; ureteral obstruction Sx.

# *Systemic Manifestations

    ; more common than UC

    ; *fever, malaise, fatigability, growth failure delayed sexual development, amenorrhea

# perianal disease

    ; more common than UC

    ; tags, fistula, abscess

# fistula

    ; enteroenteric, enterocolonic, enterovesical, enterovaginal, enterocutaneous, perianal

# intraabd. abscess

    ; hepatic, splenic, psoas, anorectal, perianal

# *Extraintestinal Sx

    ; more common than UC

    ; oral aphthous ulcer, peripheral arthritis, erythema nodosum, digital clubbing, episcleritis, renal, GB stone

    ; *correlates with presence of colitis

# short bowel syndrome

    ; bile acid malabsorption with steatorrhea, Vit. B₁₂ malabsorption 

    ; chronic steatorrhea --> oxaluria with secondary renal stones

    ; bile acid depletion --> cholelithiasis

Differential Diagnosis

★Table 283-3 Chronic Inflammatory Intestinal Disorders

Diagnosis

; typical clinical features, ruling out specific entities that mimic Crohn disase, demonstrating chronicity (1-2yr)

    - commonly weight loss

    - linear growth retardation이 1-2년정도 먼저 발생해야 한다.

    - pale, decreased energy level, poor appetite

           / latter findings

# Laboratory Findings

    ; anemia often with IDA

    ; often elevated ESR, maybe normal

    ; *thrombocytosis(>600,000/mm³) more commonly

    ; normal or mild leukocytosis

    ; low albumin

    ; elevated stool α₁-antitrypsin

# Plain Abdomen

    ; normal

    ; partial SI obstruction

  ; thumb printing of the colon wall

# UGI

    ; small bowel involvment 가 있으면, 먼저 선행되어야 한다.

    ; aphthus ulcer, thickened nodular folds, narrowing of lumen

    ; linear ulcer --> cobblestone appearance

    ; terminal ileum

           - *▲common involved area

    ; eccenteric regions

           - skip areas

    ; fistula between bowel, sinus tracts, strictures

# Barium Enema

    ; smilar mucosal change of colon

    ; cecum, ascending colon

           - *▲common involved area

# Ultrasonography & CT

    ; intraabd. abscess

# MRI in pregnancy

# Colonoscopy with biopsy

  ; more helpful

    ; patchy, nonspecific inflammatory changes(erythema, friability, loss of vascular pattern), aphthus ulcer, linear ulcer, nodularity, strictures

           - *위의 소견으로 Crohn disease을 의심할 수 없지만, deeper layer에 병변이 있는 경우에는 의심해야한다.

    ; *noncaseating granuloma

           - *▲characteristic histologic finding

    ; *transmural inflammation

           - *▲characteristic histologic finding

Treatment

# *small bowel involvment만 있는 경우

    ; *prednisone 1-2mg/kg/day(max. 40mg)가 first treatment

# Steroid Cream In Perianal Ds

    ; sitz baths같이 해야 한다.

# Aminosalicylate

    ; sometimes more effective

    ; delayed release 5-Aminosalicylate(Asacol) 50mg/Kg/day (max. 3g/day)

# azathioprine 1-2mg/kg/day (or its metabolite 6-MP)

    ; poor response to steroids or steroid dependent에 때때로 효과적

# metronidazole

    ; effective in intractable Crohn disease

# Azathioprine + Metronidazole

    ; helpful in prianal fistulas

# cyclosporine, ciprocloxacin

# restricting diet 는 피해야 한다.

# Acute with severe pain, anorexia, fever, abdominal tenderness, elevated white cell count가 있는 경우

    ; microperforation을 포함하는 infectious process을 rule out하기 어렵기 때문에 intravenous steroid + AB(for gram-negative & anaerobes)을 준다.

    ; ultrasonography or abdominal CT를 시행한다.

# Nutional therapy

    ; *effective primary therapy

    ; High calorie oral supplement가 성공적이지 못한 이유

           - early satiety or exacerbation of symptom(abdominal pin, vomiting, diarrhea)

    ; TPN

           - effective in nutrional repletion & in quieting active disease

    ; ♥Continuous Adminstration Of Nocturnal Nasogastric Feeding

           - complex enteral feedings

                   / 500-1000kcal nightly

                   / 50-80kcal/kg/night monthly every 4months

           - elemental diet

                   / *superior in inducing remission

                   / continuous as 24hr or overnight

                   / ♥Advantages

                           1) free of side effects

                           2) avoids problems ass. with corticosteroid therapy

                           3) simultaneouly addresses nutritional rehabilitation

                   / disadvantages

                           1) early relapse on discontiuing treatment

                           2) no response to perianal & colon disease

                   / ♥Indication

                           : severe nutritional depletion

                           : severe growth failure

                           : unreponsive to conventional treatment

                   / *remission온 후에는 delayed-release 5-minosalicylate 혹은 alternate-day steroid로 유지요법을 한다.

# Surgery

    ; ♥신중해야된다. 그이유는

           - recurrance rate after bowel resection is high(>50% by 5yr)

           - increase risk of requiring additional surgery

           - potential complication

                   / fistula, stricture, anastomotic leak, postoperative partial small-bowel obstruction secondary to adhesions, short-bowel syndrome

    ; Indication

           - localizing disease of small bowel or colon unresponsive to medical Tx.

           - bowel perforation

    - stricture with symptomatic partial bowel obstruction

    - intractable bleeding

    ; *removing bowel up to margins that are free of disease

           - better outcome than most involved areas

    ; symptomatic small-bowel stricture

           - *stricturoplasty rather than resection

    ; severe perianal disease

           - *colectomy with conventional ileostomy

                   / continent ileostomy 혹은 endorectal pull-through는 권장되지 않는다.

Prognosis

; *high morbidity & low mortality active full life

    - 치료에도 불구하고 설명되지 않는 재발이 일어난다.

; up to 15%

    - permanent decrease in linear growth

; *시간이 갈수록 involved region of intestine 이 커지고, complication이 흔하다.

; *결국에는 거의 대부분의 환자가 수술을 요하고, 재수술의 빈도도 높다.

    - *symptom onset에서 수술의 시기까지의 기간이 소아에서 더 짧다.

283.3 Behcet Syndrome

          multisystem vasculitis, very rare in children

Clinical Manifestation

         1. aphthous stomatitis : 2-10 mm in diameter, mouth, post. pharynth

            covered by white yellow membrane, painful

         2. erythema nodosum

         3. arthritis

         4. 그외 genital ulcer, CNS involvement, myositis, iridocyclitis

            segmental lesion in colon or distal small bowel

Treatment

         1. corticosteroid

         2. immunosuppressive : chlorambucil, cholichine

         3. resection : intestinal obstruction시

Chapter 284. Dietary Protein Intolerance (Food Allergy)

          immunologically mediated response to dietary antigen

          several immunologic mechanism

           i) immediate anaphylactic hypersensitivity involving IgE Ab

           ii) Ab dependent cytotoxic hypersensitivity involving Ig M or Ig G

           iii) immune complex hypersensitivity

           iv) cell mediated hypersensitivity

Clinical Manifestation

  GI 어디라도 침범 가능

         1. mouth : recurrent shallow, mucosal ulceration & perioral dermatitis

         2. stomach : i) intragastric antigen -> hm, edemaous inflammation

                     ii) acute vomiting & watery, bloody diarrhea

                     iii) fatal anaphylactic shock (rare)

         3. small intestine

            i) acute watery diarrhea

               ; immediate response to antigen ingestion

               ; w/wo vomiting and abd. cramps

            ii) chronic diarrhea & failure to thrive

               ; after ingestion of milk, soy, egg, fish

               ; anorexia, chronic diarrhea, retarded growth

            iii) excessive enteric protein & blood loss

               ; hypoproteinemia, iron deficiency without obvious GI Sx.

                 in older infant에서 breast milk의 weaning

                 milk formula feeding이 withdrawl시

                 eosinophilia(+), cow's milk withdrawal 시 Sx. improve

         4. colon

            pancolitis causing profuse bloody diarrhea in young infant

            diarrhea stool : abundant eosinophils (+)

            eosinophil gastroenteritis와 감별되어야함

Diagnosis

         1. clinical Dx.

           #86 withdrawal of milk후 acute Sx.은 48시간이내 소실, chronic Sx.은 1주이내

             rechallenging시 with caution

     #81 2. dietary Ag에 대한 GI response보기 위한

            ① skin tests,

            ② circulating Ab titers

            ③ complement assay

            ④ copro Ab titer -> of not proven Dx. value

         3. 50% of cow's milk intolerance pt.에서 soy protein에도 intolerant

       DDx. ; enteric infection, lactose intolerance, other N-S infl. bowel ds

Treatment

         1. prolonged breast feeding하면 후에 cow's milk intolerance 감소

         2. offending food removal (48, 72시간내 증상 호전)

            i) non-milk containing formula in young infant

               (soy feeding, hydrolyzed milk protein feed)

            ii) enteric protein & blood loss

                -> fresh milk를 processed (evaporated or powdered) milk로

         3. oral sodium cromoglycate : 많은 음식에 대한 intolerance있을 때

                                        intestinal Sx. suppress

Prognosis

 variable 이나 대개 transitory

         50% 는 1년내, 대개 2년내 소실

Chapter 285. Eosinophilic Gastroenteritis

         주로 stomach, upper SI에 infl. of eosinophil

         때로 esoph. distal SI도 침범

         Sx. : abd. pain, V. D. delayed growth & Wt. gain

               때로 atopic Sx. - rhinitis, asthma, peripheral eosinophils

               excessive protein loss (alb. Ig)

               bowel thickening & granuloma : rare

         대개 chr. debilitating course,  corticosteroid Tx.

CHAPTER 286. Malabsorptive Disorders

Table 286-1  Generalized Malabsorptive States in Children  

# Characteristics of malabsorption syndrome

    ; abd. distension                        

  ; pale, foul smelling, bluky stools        

    ; wasting of muscles, esp, prox. m. group

    ; poor weight gain or weight loss

    ; growth retardation

# Disaccharidase deficiency

    ; *▲common congenital disorder affecting digestive enzyme

Table 286-2. Specific Defects of Digestive-absorptive Function in Children  

286.1 Evaluation Of Children with Suspected Intestinal Malabsorption

Clinical Manifestation

 malabsorption과 다른 Ds.를 DDx.하는 주된 방법

1) Hx.

   i) family Hx.

   ii) time of onset

   iii) relation to dietary content & intake

   iv) description of stool

   v) body Wt. & length

2) signs of malnutrition

   i) muscle wasting

   ii) edema

   iii) mouth sores

   iv) smooth tongues

   v) excessive bruising

3) rectal exam. 중요 : initial step

   i) anus & rectum assess

   ii) stool exam. for gross microscopic, chemical study

       a. panc. insufficiency 시 excessive droplet of fat & undigested meat fiber

       b. intestine ds.시 crystalline aggregates of monoglycerides & fatt acid

Laboratory Manifestations

Fat

# Microscopic Examination Of Stool For Fat

    ; *▲useful screening test

    ; Sudan red stain

    ; *more than six to eight droplets per LPF - abnormal

         1) absorptive function

  ## 86   1. fat

            i) fecal fat balance study

               20g/day 이상 섭취후 최소 4일간 collection

               15% ↑ excretion in NB        -+

               10% ↑ excretion in older CH  -+ 시 abn.

            ii) serum carotene conc.

                < 50 ug/dl : malabs.

                > 100 ug/dl : normal

            iii) direct stool microscopy for fat content

Carbohydrate

# Benedicts test

    ; *clinitest reagents(reducing substance)을 이용하여 carbohydrate in stool을 검사

    ; stool sugar 양 > 0.5%시 abn.

    ; *sucrose제외하고 dietary sugar는 reducing sugar이므로 양성 반응을 보임.

  ; sucrose 검사시는 stool을 HCl로 heating시켜 가수분해

# Fresh Stool pH

    ; *< 5.6이면 abnormal

    ; unabsorbed sugar가 bacteria에 의해 oraganic acid생성 증가

# ★Stool Electolyte Contents

    ; *2x[sodium]+[potassium]+50

    ; *< 290mOsm/L이면 osmotic diarrhea, carbohydrate malabsorption

# Breath Hydrogen Test

: indirect

      hydrogen conc. in expired air는 sugar p.o. 2g/Kg (max 50g)

      -> USI에 서 흡수 안되면 distal intestine에서 bacteria에 의해

         hydrogen gas형성 -> absorb -> expiration

      첫 2시간내 breath hydrogen이 20ppm이상시

Protein

Table 286-3 Gasrointestinal Causes of Hypoproteinemia 

   endogenous fecal protein source 때문에 balanced study는 부정확

 i) fecal nitrogen : rough, quick

 ii) enteric protein loss : choice

     51CrCl iv 4일후 stool collection하여 0.8%이상 loss시 abn.

 iii) fecal clearance of serum α1-antitrypsin : simple

      48hr collection하여 15ml/day 이상 clearance 시 abn.

Other Nutrient

 iron, folic acid in RBC, s-Ca, Mg, Vt. D, & metabolite Vit. A, Vt. B12

 localization of size of malabsorption

 i) iron, D-xylose : upper SI

    bl. level < 25mg/dl - 1hr after oral xylose 시 small intestine lesion

              14.5g/m²시 prox. intestinal mucosal lesion

 ii) Vt. B12, bile salt : distal bowel

     -> Schilling test : radioactive B12 oral

     -> 24hr 후 urine excretion 측정

        urinary excretion이 <5%시 abn.

Diagnostic Procedures

Microbiologic

: giardiasis 1⁰ inf. cause

Small Bowel Biopsy

i) celiac ds.

ii) abetalipoproteinemia, acrodermatitis enteropathica

    eosinophilic gastroenteritis, congenital villus atrophy

iii) giardiasis, lymphangiectasia, gamma globulin deficiency

     viral enteritis, tropical sprue, cow's milk or soy intolerance

Hematologic

i) iron deficit

ii) megaloblastic ... folate, Vt. B12 deficit

iii) acanthocytosis ... abetalipoproteinemia

iv) lymphocyte defect or neutropenia, ass. with Shwachman-diamond synd.

Imaging Procedures

i) plain abd. & barium enema : intestine stasis 의 siz. 원인

ii) 99mTc scan : duplication, Meckel's diverticulum

iii) US, panc. mass, biliary tree abn.

iv) ERCP

disaccharidase assay

286.2. Chronic Malnutrition

  exocrine pancreatic insuff.

  * malnutrition의 가장 흔한 원인( Kwashiorkor, marasmus )

  intestine은 protein caloric malnut.에 resistant

286.3. Liver & Biliary Disorders

           bile salt 감소시 +- steatorrhea

                            +- bone lesion due to Vt. D malabsorption

                            +--Vt. E abs. ↓ -> chronic neuropathy

286.4. Intestinal Infections

# Giardiasis

    ; *▲common infectious causes

    ; diarrhea, vomiting, bloating, gas

    ; *day care children(toddlers) - special risk

# coccidiosis due to  Isospora belli, I. hominis

# intestinal hookworm

286.5. Immunodeficiency

           : defective resistance to enteric microflora

 

      (1) Congenital deficiency

         1) congenital sex-linked panhypogammaglobulinemia ( Bruton )

            mild intermittent diarrhea ... 2세내 대개 호전

            giardia ↑

         2) Ig A deficiency

            TMC congenital immunodeficiency

            IBD, nodular lymphoid hyperplasia, celiac ds. : common

            giardia ↑

         3) combined immunodeficiency : disaccharide activity ↓

            severe diarrhea & malabsorption -> high mortality in this group

         4) neutropenia & neurophil dysfunction

            neutropenia pt.에서 NEC는 cause of fever, RLQ pain

            chronic granulomatous ds. 환자에서 granuloma는 intestine에 파급되어

            diarrhea, malabsorption유발

     

      (2) Acquired immunodeficiency

         ① oral eso. -> Candida / HSV / CMV / EBV

         ② enteric

            i) AIDS ; C. albicans / CMV / blastocytosis / C. difficle / Cryptosporidium

            ii) BM transp ; C. diff / adeno V / Rota V

286.6 Stagnant Loop Synd.

       ( Blind loop synd. Bacterial overgrowth synd.)

       Eti.

         1. incomplete bowel obstruction

           a. congenital i) malrotation with duodenal band

                       ii) stenosis

                       iii) diverticulum

           b. acquired  i) postop. intestinal adhesions

                       ii) longstanding Crohn's ds.

         2. neuromuscular dysf. -> motility disorder

             i) intestinal pseudo-obstruction

             ii) hollow viscus myopathy

 

      Pathogenesis

         i) enteric bacteria가 peristalsis에 의해 incompletely cleared되어 upper SI 에 colonize

            -> bile salt deconjugation하여 dietary fat의

               ineffective intraluminal processing & steatorrhea

         ii) Vt. B12와 결합 : 흡수 방해

         iii) microvillus brush border memb. damage

         iv) disaccharide activity 감소

  

      C/M

         i) chronic incomplete bowel obst. Sx. : distension, pain, vominting

         ii) steatorrhea ... pale foul bulky stool

             megaloblastic anemia (Vt. B12 def.)

             diarrhea from disaccharide deficiency

 

      Tx.

         i) oral bactrim : temporary improvement

         ii) definite Tx. : operative correction

286.7 Short Bowel Synd.

          small intestine (w/wo loss of some large intestine )의

          congenital or postnatal loss후의 malabsorption & malnutrition disorder

 

      (1) Congenital

          ass. with malrotation atresia, gastroschisis

 

      (2) Massive intestinal resection

          NB :small bowel  2-3m의 25% 이상의 resection시

               fluid-nutritional problem 초래 가능

            * resection후 infant의 소화, 흡수 기능은 2년 지나냐 자연 개선

               ileum : sole site of absorption for bile salts -> fat 흡수

          resection시 ileocecal sphincter보존 이유

            i) colonic flora의 retrograde flow를 impede

            ii) 남은 small bowel mucosa에 nutrient contact를 prolongation

 

         Cx. after massive resection

            i) gastric acidity & retrograde bacterial contamination of intestinal lumen

                  : absorption decrease 

            ii) bile salt loss : fat & fat soluble Vt. malabsorption

                colon에서 bile salt가 water & Enzyme secretion increase

            iii) hyperoxaluria after distal SI resection

                 -> but nephrolithiasis : rare in early CH

            iv) CMI : normal but Ig ↓

            v) mid & distal jejunum & ileum resection 시 fluid balance 유지할 필요없음

 

         Tx.

            1. TPN

            2. liquids or liquid formulas : isotonic initially & frequent small feeding

              excessive water 금지

              steatorrhea시 MCT

              constant intragastric infusion이 bolus feeding보다 net fluidretention 을 증가

              glucose than disaccharide (<5g/dl유지 - fluid isotonicity때문)

              Vit. supplementation : A, D, E, K

              vit. K -> PT monitor하여 supplement

              s-Ca, Mg, K, phosphorus monitor & supplem.

              large ileal resection 시 Vt. B12 100ug inj. monthly for life

              antidiarrheal agents : rarely helpful

              cholestyramine : colon도달전 bile acid 와 결합하여 fecal Na, water loss 감소

                 but massive resection 이나 severe steatorrhea시 aggrevation

              antacid : not - proven

              oral antibiotics for stagnant loop synd.

              intellectual function : preserved

286.8 Gluten-Sensitive Enteropathy (=Celiac Disease)

    permanent intestinal notolerance to gliadin, 밀에 함유된 단백질

    causes severe small intestinal mucosal lesions in susceptible individuals

Pathogenesis 

① genetic and environment factor 관여

     ass. with class HLA B8 and class II HLA antigen DR3, DQ, W2, DR4

     80-90% HLA B8 (+)

  ② Breast feeding시  risk decrease

  ③ enteric infection (human adeno v. 12)시 risk증가

     mechanism of gliadin damages to small intestine : unknown

      -> celluar & cell mediated immune response자극

Clinical Manifestation

Table 286-4  Active Childhood Celiac Disease -42 Cases 

wide range

   typical : irritability, anorexia, chr. diarrhea in later in 1st year

   constant features : decreased rates of Wt. gain & linear growth

Evaluation

    ; anemia & hypoproteinemia

    ; ★Serologic Test

           - *antigliadin antibodies

                   / not reliable enough

           - *serum IgA-endomysial antibodies

                   / confirm diagnosis

                   / exception) IgA-deficient patients

: common, iron, folate, vit B12 deficiency

   ii) hypoalbuminemia & circulating γ-globulin

   iii) steatorrhea : 4 day balanced study상 fat excretion > 10%

   iv) fasting s-carotene level ↓ (< 50ug/dl)

       low serum 25-OH-vit D, calcium, vit. A level

   v) PT ↑

   vi) diffuse small bowel mucosal damage

       -> flat oral GTT, blood xylose conc. < 25mg/dl

   vii) barium contrast ; diffusely dilated SI, coarse mucosal folds

   viii) bone X-ray : osteoporosis, rickets (rare)

        selective Ig A deficiency & IDDM ↑

        adult와 달리 child atophy는 증가 안함

   ix) Ab to gliadin+- Ig G -> sensitive

                    +- Ig A -> specific

#81   USI mucosa에 diffuse lesion -> peroral suction biopsy

      LM 상  

i) short, flat villi

              ii) deepend crypts

              iii) irregular vaculated surface epi. with lymphocytes in epith, layer

      Rota virus, giardia, tropical sprue같은 inf. 시

       villous flattening & elongate crypt초래 가능하나  enterocyte의 marked abn.(-)

  i) mucosal suction biopsy : duodenal & jejunal mucosal lesion

   ii) clinical & lab. response to a gluten free diet

   iii) reappearance of lesion after gluten challenge

        -> 최소 치료후 2년후 시행, ∵ mucosal healing후

Pathology

       i) tropical sprue

       ii) protein intolerance

       iii) immune deficiency

       iv) eosinophilic gastroenteritis

Treatment

   i) gluten free diet ( wheat, rye, barley )

   ii) 급성기에는 fat-soluble vit, iron, folate보충

   iii) lactase deficiency 시 disaccharide restriction

Prognosis

i) gluten free diet로 mood & appetite증가후 diarrhea 감소 대개 치료 1주후부터 호전

ii) preadolescence와 adolescence동안에는 celiac ds. children이 상당량의dietary gluten의 증상 없이 tolerable

iii) adult pt.에서 intestinal malignancy빈도 더 높다.

286.9 Immunoproliferative Small Intestinal Ds.

      Pathology : thickened mucosal folds, duo. or jejunal nodularity

                     lymphoplasmacytic infiltrates

                         

      C/M  initial : intermittent diarrhea. abd. pain

            later : persistent chronic diarrhea, malabsorption

                                  Wt. loss, digital clubbing, growth failure

 

      Dx. : endoscopic biopsy of multiple duodenal & jejunal mucosal sizes

            serum marker of Ig A

 

      Tx.

         1. early lesion : 6 Mo 이상의 TC therapy

         2. prelymphomatous stage : cyclophosphamide w/wo prednisone

         3. lymphoma : combination of cyclophosphamide & TC

                       doxorubicin, teniposide, prednisone

 

      Px. : early Tx. of AB - excellent

              later Tx.       - poor

286.10 Other Malabsorptive Syndrome

      1. Intestinal lymphangiectasia

         dilatation of lymphatic vessels

         leakage of lymph into intestinal lumen

         steatorrhea, protein losing enteropathy, fat malabsorption, chylous ascites

         hypoalbuminemia, hypogammaglobulinemia, edema, lymphocytopenia

         causes :

           surgical damage to lymphatic vessels, chr. RHF, constrictive pericarditis

           retroperitoneal tumor, malrotation with lymphatic obstruction

           Turner SD, Noonan SD

         Dx. elevated fecal ?1-antitrypsin

           Rad. uniform symetric thickening of SI

 

      2. Microvillus Inclusion Disease ( Congnital Microvilus Atrophy)

         intractable watery diarrhea and severe malabsorption at birth

         inherited in AR pattern

         Dx. villus atrophy, crypt hypoplasia, microvillus inclution in enterocyte

         Tx. somatistatin analog octreotide

             TPN bowel transplantation

      

      3. Autoimmune Enteropathy

         chr. diarrhea and malabsorption after 6 Mo

          그외 arthritis, membraneous GN, thrombocytopenia, hemolytic anemia

         Dx. villus atrophy, crypt hyperplasia, chr. inflammatory cellin

              lamina propria antienterocyte Ab (IF)    

         Tx. Prs, Azathioprine, cyclophosphamide, cyclosporine

 

      4. Tufting Enteropathy

         persistent watery diarrhea in 1st weeks of life

         Dx. SI biopsy focal epithelial tufts(80-90%) : shortening of microvilli

 

      5. Tropical Sprue

         fever, malaise후 watery diarreha

         수일후 chr. malabsorption, internittent diarrhea, anorexia,

         malnutrition sign : night blindness, glossitis, stomatitis, cheilosis

           hyperpigmentation, edema, megaloblastic anemia,

 

         Tx.

           antidiarrheal agent

           nutrient : folate

           broad spectrum antibiotics : sulfonamide, TC (for 3-4wks)

 

      6. Wolman Disease

          lethal lipoidosis : lipid accumulation

          vomiting, hepatosplenopathy, steatorrhea

286.11 Enzyme Deficiency

Enterokinase Deficiency

           complete absence of pancreatic preteolytic activity

            ( enterokinase가 pancreatic trypsinogen의 essential activator )

           severe diarrhea, failure to thrive, hypoproteinemia

           duodenal juice상  +- trypsin activity (-)

                             +- lipase, amylase : normal

Disaccharidase Deficiencies

           SI의 brush border memb. surf.에 존재

           대개 secondary

           disaccharide intolerance시 response유사

            incomplete disacch. hydrolysis at brush border

            -> sugar ↑in distal lumen & organic acid, H+

            ->  i) sugar & organic acid가 water를 끌어 들임

                   ; watery diarrhea & frothy stool, low pH, excessive sugar in stool

                ii) excoriate the buttock

           bloating, borborygmi (+), steatorrhea rare

            infancy이후 abd. pain

 

           Dx. i) clinitest (+) : if reducing sugar (lactose)

                ii) mucosal biopsy for disaccharidase activity

                iii) hydrogen breath test after oral sugar load

                iv)stool PH < 6

Lactase Deficiency

; congenital absence : rare

    - *usual mechanism : developmental pattern of lactase activity

           / 정상적으로 lactase activity는 late in fetal life에 증가하여 3yr이후에 감소한다

; AR

; *lilely depletion secondary to diffuse mucosal disease

; Symptoms 

    - onset in response to ingestion of lactose, sugar in milk

    - *explosive watery diarrhea

           / ass. with abdominal distension, borborygmi, flatulence, excoriated diaper area

    - recurrent, vague, crampy abd. pain

    - episodic midabdominal pain in school- and proschool-aged child

; Treatment

    - milk elimination & lactase preperation

Sucrase-Isomaltase Deficiency

; AR

; Symptoms

    - bloating, watery diarrhea, excoriation of buttock

    - *no recurrent abdominal pain

; *clinitest상 (-) - suctose는 reducing sugar 아님

; breath test상 ↑H2 after sucrose ingestion

; small intestinal mucosa - normal

286.12 Defects Of Absorption Of Transport

Glucose-Galactose Malabsorption

Eti.     i) congenital : AR,  renal tubular epithel.도 mild effect

        ii) acute viral enteritis : severe chronic diffuse mucosal damage

C/M : watery stool after glucose, breast milk, conventional formula

Tx :  fructose는 tolerate : restriction of glucose & galactose

Abetalipoproteinemia

    ; AR

  ; Clinical Manifestation

           - *severe fat malabsorption from birth

           - failure to thrive during 1st yr

           - pale, foul smelling, bulky stool

           - distended abdomen

           - absent DTR due to peripheral neuropathy

    ; Diagnosis

       - *acanthocytes in PBS & very low levels of S-cholesterol(<50mg)

           - TG accumulation in villus of duodenal mucosa

    ; Treatment

           - large amount fat sol. vit. A, D, E, K

    - massive vit E. (100mg/Kg/24hr시) neurologic degeneration

    - MCT

Homozygous Hypobetalipoproteinemia

Chylomicron Retention Ds.

Amino Acid Transport Defects

    1) cystinuria ; no GI Sx

    2) ★Harnup ds

           ; malabsorption of tryptophan

            --> *ataxia, intellectual deterioration, pellagra like rash, diarrhea

    3) methionine malabsorption

           ; diarrhea, sweet urine with ↑α-hydroxybutyric acid           

    4) blue diaper synd. ; defective absorp. of tryptophan

Vitamin B₁₂ malabsorption

1. juvenile pernicious anemia : intrinsic factor 형성 (-)

2. transcobalamine II defect : vit B12의 intestinal transport protein장애

3. Imerslund : defective ileal absorp. of vit B12

Congenital Malabsorption Of Folic Acid

 megaloblastic anemia, cerebral degeneration

Chloric Losing Diarrhea

 cong. defective ileal chloride transport ass. maternal polyhydramnios

 severe watery diarrhea since birth

 -> hypo K, hypochloremia, alkalosis

Congenital Sodium Diarrhea

 diarrhea since birth

 maternal polyhydramnios

 acidosis

Vitamin D Dependent Rickets

  AR, malabs. of Ca

Primary Hypomagnesemia

Acrodermatitis Enteropathica

    ; Etiology

           - *zinc deficiency secondary to zinc malabsorption

    ; ★Clinical Manifestation

           - rash around mucocutaneous junction & extremity

    - alopecia

           - chronic diarrhea 때로 steatorrhea

    - unTx.시 failure to thrive

  ; intestinal mucosal biopsy

           - *Paneth cell

    ; Treatment

           - *oral zinc sulfate 1-2mg element zinc/kg/24hr

           - after treatment rapid healing skin lesion, diarrhea

Menkes (Kinky Hair) syndrome

; AR

; Characteristics

    - growth retardation, abn. hair, cerebellar degeneration, early death

; Pathogenesis

    - unknown

    - *cellular copper transport defect

; s-Cu & ceruloplasmin ↓, cellular Cu ↑

Bile Acid Malabsorption

Drug Induced Absorptive Defects

1. MTx : arrest of enterocyte mitosis -> mucosal lesion

2. neomycin (large amount) : affect mucosal structure

3. sulfasalazine : folic acid abs. ↓

4. cholystyramine : hypoCa, steatorrhea초래 <- bind to Ca & bile acid

5. phenytoin : Ca abs. ↓ -> rickets

Chapter 287. Chronic Diarrhea

# definition of diarrhea

    ; excessive loss of stool water & electrolyte

  ; stool volume 15mg/kg/day in infant, 200g/day at 3 years

# Chronic diarrhea

    ; consistent increase in stool frequency

  ; decrease in stool consistency or increase in stool volume

Characteristics Of Diarrhea

Osmotic diarrhea : feeding이나 medication을 중단시 Diarrhea 멈추면 common

Secretory diarrhea : fasting시에도 diarrhea 지속, rare and primarily in infancy

     stool frequency > 5/day, watery & large volume (more than 75%)

     through day and night,persistence a diarrhea beyond 24-48hr

     require hospitalization, complete bed rest, IV hydration

# Diarrheal stool

    ; osmolality 280-330mOsm/L 

    ; osmotic gap = 290-2(Na++K+)

  ; ★osmotic diarrhea

           - *low Na⁺ (<50mEq/L) osmotic gap (>160mEq/L)

    ; secretary diarrhea

           - high Na+ (>90mEq/L) osmotic gap(<20mEq/L)

287.1 Osmotic Diarrhea

Table 287-1 Differential Diagnosis of Chronic Osmotic Diarrhea on the Basis of  Pathophysiology  

Table 287-2 Differential Diagnosis of Chronic Osmotic Diarrhea on the Basis of Age of onset 

Table 287-3 Patterns of Presentation of Chronic Osmotic Diarrhea  

287.2 Secratory Diarrhea

Table 287-4 Differential Diagnosis of Chronic Secretory Diarrhea on the Basis of  Pathophysiology  

Diagnosis

   1. congenital transprot defects

          : begin at birth or in the 1st few days with anorexia, vomiting

            formula change시 transient improvement

   2. protracted diarrhea (intractable diarrhea of infancy )

     1) infection : Ab to HIV

     2) anatomic or functional intestinal obstruction : UGI

     3) secretory tumor : S-VIP level, U-VMA, US, CT, MRI

     4) immune def.

     5) disorder of epi. turnover or differentiation : lab.

   3. biopsy : early in evaluatin  duodenal fluid culture & EM

Factitious Diarrhea

   1. phenolphthalein(Ex-Lax)

      turn pink on alkalinization-turn colorless with acidification

   2. laxatives

      measuring stool electrolyte, Mg, S,

      chromatographic analysis of anthracene derivatives

   3. low stool osmolality (less than 280mOsm/L) : add water to stool

287.3 General Therapeutic Consideration

 Tx. for specific cause

 adequate nutrition balance with bowel rest

 protein-calorie balance

 fluid-electrolyte balance

 diet restriction : postenteritis lactose intolerance in infancy

   : 4-6주 동안 lactose free milk formula or soy based formula준다.

 control of chronic non-specific diarrhea

   regular diet ingestion & nonprotein fluid 90ml/kg/day

   oral loperimide 0.1-0.2mg/kg/day #2-3

 Broad spectrum antibiotics : metronidazole, TC, CM, AP, EM with NM for 2wk

Treatment Of Secretory Diarrhrea

    ; IV nutrition and complete bowel rest

    ; Refeeding

           - *BW가 50%ile 이 된 후에 refeeding

           - benefit on mucosal growth of enteral nutrients

    ; ★Drug Therapy

           - opiates or opiate derivatives

                   / decrease fluid transient throuth guts

           - loperamide

                   / high antidiarrheal specificity

           - cholestyramine

                   / anion exchange resin for bile acids

                   / protect against direct bile-induced injury, bile acid-induced secretion in colon

        - *somatostatin

                   /*reduce secretory diarrhea caused by neuroendocrine tumors and villous atrophy

           - *PG synthesis inhibitor

                   / indomethacin, bismuth subsalycylate, 5-aminosalycylate

                   / decrease diarrhea in inflammatory diarrhea

           - immunosuppressive agents

                   / autoimmune enteropathy

Chapter 288. Recurrent Abdominal Pain Of Childhood

; Incidence : 10% of preschool and school-aged children

2세이하는 organic cause가 많으며, older children에서는 10%에서 organic cause이다.

 `functional abdominal pain' : recurrent abdominal pain without organic cause

Etiology

   ①not ass. with specific structural or biochemical causes

   ②chr. peripheral pain

   ③psychosocial stress

   ④personality pattern

   ⑤reinforcement of illness behavior

Clinical Manifestation

   non-specific

   variants

    1. irritable bowel syndrome

    2. nonulcer dyspepsia

   nocturnal pain은 organic cause이지만 functional pain일수도

   periumbilical pain

   distracted from pain

   good periods with remission of Sx.

   recurrence without apparant cause or stressful period

Diagnosis

   normal P/E & Hx. of good growth with rectal exam.

   pale appearance

   initial nondirect interview technique

   family medical Hx.

   Lab. : CBC, ESR, U/A, stool exam. for parasite

   plain X-ray, US, UGI, esophagogastroduodenoscopy

Differential Diagnosis

      ① organic cause

      ②chronic constipation

      ③ parasite infection

      ④lactase deficiency

      ⑤ GU disorder : hydronephrosis

      ⑥ Crohn disease

      ⑦recurrent pancreatitis

      ⑧ peptic ulcer, H. pylori infection

      ⑨ abdominal migraine or epilepsy

Treatment

   reassurance of the children and family member

   medication usually unheplful

   biofeedback & relaxation technique

   lactose free diet

Chapter 289. Acute Appendicitis

; *▲common cause of abd. surgery in child

 2세 이상의 어린이에서 emergency op. Ix.

   i) acute appendicitis

   ii) trauma to viscera

   iii) intussusception

   iv) adhesive bowel obstruction

   v) lesion of ovary

  risk of perforation  i) 1-4yr : 74%

                     ii) 5-8yr : 66%

                     iii) adolescents: 30-40%

Epidermiology

    2세이하 unusual, 1세이하 rare

    peak age : teenage, young adult years  M>F

    season : 가을, 봄

Etiology

1. luminal obstruction :TMC

   i) hard concretions (appendicial fecalith)

      crushable fecal impaction in appendix

      appendicial calculi (hard, calcified fecaliths) : radioopaque 15-20%

   ii) narrow appendicial mesentary : distal torsion- acute ischemic necrosis

   iii) hyperplasia of submucosal lymphoid tissue by inf.

   iv) viral or bacterial (Yersinia, salmonella, shigella)

   v) cystic fibrosis or carcinoid tm.

2. nonobstructive lesion : rare

Pathology

   1. young CH : rapid course

      3 phases

        I) acute obstruction of appendix

           -> mucus secretion -> mucosal vessels compression

           -> ischemia, necrosis, ulceration

        ii) bacterial invasion & inf. of appendicial wall

            infl. cell infiltration in all layer

        iii) app. wall necrosis perforation at tip of appendix.

           distal to obstruction fecaliths

   2. older CH

      대개 omentum과 adj. ileum이 inflammatory appendix에 adhere prior to perfo.

      -> wide spread fecal spillage를 예방

      -> localization abscess in Rt. iliac fossa

  ### infant, young children시 appendicitis가 quickly perf.

         & generalized peritonitis 호발 이유

         ; omentum이 small하고 ineffective in localizing the infection

   3. bacterial invasion of the mesenteric v. - portal v. sepsis

      - liver abscess formation

Clinical Manifestation

# classic triad

    ; pain, vomiting, fever

    ; pain

         - initial stage : periumbilical

    ; emesis

           - follows onset of pain

    ; *symptoms sequence가 중요하다

           - *pain preceding emesis and fever followed by crampy pain of hyperperistalsis

# anorexia, low grade fever, constipation, diarrhea(infrequent), urinary frequency, urgency

# perforation

    ; *usually over 36-48hr from onset of symptom

    ; if delayed over 36-48hr, *65% perforation rate

    ; risk factors

   ii) fecaliths

   iii) young age

   iv) 36O 이상의 temp.

   v) WBC ↑ : 14,000 ↑

   vi) (+) family Hx. of appendicitis

Hx.   pallor, flushing, physical activity, abd. movement

       pulse rate & rectal temp.관찰

Diagnosis

Physical Examination

    ; inspection : swelling, movement of abdomen, pain on coughing

    ; maximal tender point

        - older CH : McBurney point

        - young CH : right iliac fossa

  ; rebound tenderness : needlessly painful sign

  ; Psoas sign : post. situated appendix시 Lt. side로 눕히고 hip extension시 pain

  ; *▲important physical sign

           - *persistent direct tenderness to palpation and rigidity of overlying rectus muscles

constant localized significant degree of tenderness 

  먼저 abd. exam.후 general exam. -> 마지막 recta exam.

                                      특히 very young female adolescent

  equivocal 한 경우 4-6시간후 reexam.요함.

 . 15%에서 normal appendectomy

Auscultation : n'l or hyperactive -> hypoactive bowel sound

Laboratory Findings

   1. CBC : leukocytosis : neutrophilia with left shifting, eosinophil ↓

   2. U/A : pyuria ; ureter & bladder 가까운 app.시

                    WBC 30/HPF RBC 20/HPF까지

   3. X-ray i) small bowel distension or obstruction

            ii) calcified appendicolith

            iii) soft tissue mass effect

            iv) scoliosis concave to the Rt. side

            v) paralytic ileus      

   4. Graded compression ultrasound

      Ix. i) accurate tool to diagnose nonperforated app. (edematous & enlarged app.)

         ii) perforation & abscess formation

         iii) ileocecal enteritis -> RLQ pain시 appe. R/O 가능

            (bacterial ileocecitis시 terminal ileum과 cecum의

                mural thickening과 enlarged mesenteric LN)

   5. Barium enema or abdominal CT

Differential Diagnosis

   1. enteric inf. : Y. enterocolitica, campylobacter jejuni

   2. acute flare up of crohn's ds

   3. intussusception in older CH

   4. Meckel diverticulum

   5. mesenteric adenitis

   6. generalized viral infection : midabd. pain, eating시 악화, neutropenia

   7. RLL pneumonia

   8. UTI

   9. DKA

  10. bleeding from Rt. ovary, graafian follicle, torsion of undescended of testis

  11. primary peritonitis

  12. hematologic ds.: leukemia (esp. in relapse), hemophilia, sickle cell ds. H-S purpura

  13. constipation, meconium ileus

  14.constipation

Treatment

   1. emergency appendectomy  for early acute appe.

   2. localized app. abscess시 ext. drainage우선 -> 나중 수술

   3. fluid replacement : generalized peritotitis시 fluid & E. balance교정

      i) no sign of dehydration

         Ringer lactated sol. : 5% of body Wt. (1/2 - 술전, 1/2 - 술후)

      ii) dehydration sign (+) : 7% of body Wt.

      iii) severe dehydration : 10-15% of body Wt.

         수술전 adequate urine output유지

   4. antibiotics : rupture시 수술전부터

      triple (aminoglycoside + ampicillin + clindamycin/metronidazole/new cepha)

      for 7-10 days

 

 Px : good ; mortality 0.5-1%

★Complication

    ; 25-30%

1) wound inf.

    ; *▲common postop. Cx.

    ; perforation시 10-15%, no perforation시 0-2%

2) pelvic, subphrenic, intraabdominal abscess

    ; 대개 pelvic abscess는 adj. bowel로 rupture되어 resolve

  ; subphrenic abscess는 surgical drainage

3) liver abscess

4) prolonged paralytic ileus

5) intestinal obst. (postop.)

    ; 30일내 발생 .... nonop. management

  ; 30일후 발생 ... ischemia 증거(-)시 48hr nasogastric tube로 decomp. 효과없으면 laparotomy

6) volvulus

7) infertility

Appendix & chr. abd. pain

    fibrous band, worm, fecalith 에 의해 obst. of appe.

    ... important cause of recurre or chr. abd. pain

    DDx.

         1. urinary tract pathology

         2. GI malfunction

         3. psycholphysiologic pain

         4. hereditary angioedema

         5. porphyria

         6. familial Mediterranean fever

Chapter 290. Surgical Condition Of The Anus, Rectum, And Colon Close Observation Of Anal Area

290.1 Anal Fissure

  small laceration of mucocutaneous junction

C/M

  : Hx. of constipation

    pain on defecation

    bright red blood on stool

    skin tag

 

Tx. : Spontaneous healing 수일-수주, stool softener

290.2 Perianal Abscess and Fistula

# Two Different Groups

    1) infants with no predisposing conditions

    2) older children with predisposing conditions

Infants With No Predisposing Conditions

    ; *relatively common

    ; *infants usually boys younger than 2yr

    ; drain through orifice in the perianal area

  ; *disappear fistula before 2yr

Older Children With Predisposing Conditions

    ; older than 2yr with predisposing illness

           - drug-induced or autoimmune neutropenia, Leukemia, AIDS, DM, Crohn disease, prior rectal surgery (Hirschsprung ds. imperforated anus), sequelae from immunosuppresants

 

Causative agents : mixed

  aerobic : E. coli, Klebsiella pneumonia, S. aureus

  anaerobic : Bacteroides, Clostridium vellionella

Clinical Manifestation

: low grade fever, mild rectal pain, perianal cellulitis, pustule, drain 

Treatment

# infants

    ; *not require any treatment

    ; uncomfortable infants

           - I & D

    ; chronic fistula

           - fistulotomy under general anesthesia

# older children

    ; wide excision & drainage & antibiotics

290.3 Hemorrhoids

      : uncommon, benign, suspect portal hypertension

290.4 Rectal prolapse

; Exteriorization of the rectal mucosa through the anus

; procidentia

    - extrusion of all the layer of wall

; onset age : 1-5yrs(mean, 3yr)

; ★Predisposing Factor

    - intestinal parasites, malnutrition, acute diarrhea, UC, pertussis, Ehlers-Danlos syndrome, meningocele, cystic fibrosis, chr. constipation, postop. for imperforate anus

; Clinical Manifestation

    - painless, mild discomfort

  - protruding mass from bright red to dark red, so long as 10-12cm

; Treatment

    - manual & surgical reduction, stool softener

290.5. Pilonidal Sinus and Abscess

     dimple located in midline intergluteal cleft, at level of coccyx

Chapter 291. Tumors Of Digestive Tract

Juvenile Colonic Polyp (Retention Polyp, Inflammatory Polyp)

  TMC tumor of bowel in CH, malignant potential

  rare, 1세 이후 나타나며, 15세 이후 less 21세 이전의 3-4%

  site : prox. to descending colon (40%)

   1/2이상 2 or more erythematous friable, pedunculated

  size : 0.5mm-3cm, mucus-filled cystic gland simple layer of epithelium

 

C/M  painless rectal bleeding, IDA

   crampy abd. pain if intussusception or prolapse

  대개 spontaneous infarction & shedding

 

DDx.

   i) other form of polyposis (esp. familial)

   ii) Meckel's diverticulum

   iii) fissure

   iv) infection, colitis

   v) coagulation disorders

Dx.

   I) rectal exam : 1/3

   ii) sigmoidoscopy : smooth pedunculated lesion

   iii) air contrast barium enema

   iv) fibroptic colonoscopy

   v) saline enema with ultra

Tx. - Removal of polyp

Px. - recurrence 때때로

Familial Polyposis Syndrome

Familial Adenomatous Polyposis Coli

    ; *premalignant condition

    ; adenomatous lesion in distal large bowel

    ; late in the 1st decade or teens

           --> adenoma in 2nd or 3rd decades of life

    ; *APC(adenomatous polyposis coli) gene

           - *on long arm of chromosome 5

    ; Clinical Manifestation

           - initially asymptomatic

           - hematochezia, crampy pain, diarrhea (rare)

    ; Diagnosis

           - family Hx.

    - double contrast barium enema

    - colonoscopy : biopsy

    ; child with family Hx of APC

           - *colonoscopy annually after 10yr of age

    ; Treatment

           - genetic counselling, pancolectomy

Peuts-Jegher Syndrome

    ; *dominant inheritance, not premalignant

    ; mucosal pigmentation of lip & gum, harmatoma of stomach & small bowel

    ; 50% - no family history

    ; up to 50%

           - cancer in middle-aged adults

           - *not GI tract, not harmatoma

Gardner Syndrome

    ; dominant inherited, *premalignant

    ; *multiple intestinal polyps & tumors of soft tissue and bone, particularly mandible

    ; additional features

           - dental abnormalities, bilat. pigmented lesions in the ocular fundus, extracolonic cancers (hepatoblastoma, CNS)

Hemangioma Of Intestine

           painless bleeding : subtle, sudden, massive

           50% cutaneous hemangioma동반

Leiomyoma

           stomach, jejunum에 시작

Carcinoma

          * adenoca. predisposing lesions in gut

             ① familial polyposis syndrome

             ② Gardner synd.

             ③ idiopathic ulcerative colitis

             ④ Crohn's ds                       -+

             ⑤ chromosomal breaks관련 disorder  -+- lesser

            tumor는 대개 adult시

            site : colon

            C/M : ill health, abd. pain & mass, hemorrhage

                    종종 undifferentiated & highly malignant

Lymphosarcoma Of Intestine

           children의 digestive tract의 malignancy는 대개 lymphosar. TMC

           site : lower SI

           C/M : ill health, abd. pain, anemia

           longstanding celiac ds.시 ↑

Carcinoid Tumors

    ; enterochromaffin cell의 tumor

  ; appendix에 common, low grade malignancy, resection후 recur는 rare

  ; Clinical Manifestation

           - carcinoid syndrome

           - appendix외의 tumor는 metastasis잘 일으켜 pharmacologically active secretion

         - *episodic intestinal hypermotility & diarrhea

       - vasomotor disturbances

    - bronchoconstriction

  ; *▲important active agent : serotonin

  ; Diagnosis

           - urinary 5-hydroxyindoleacetic acid

291.1 Diarrhea From Hormon-Secreting

      C/M :  massive diarrhea, fluid & electrolyte imbalance, Wt. loss,

      Dx. presence of secretory watery diarrhea, extraintestinal manifestation

          measurement of suspect hormone or metabolic serum or urine

          various imaging technique

      Tx.  resection or hormone antagonist

★Table 291-1 Diarrhea Caused by Hormone-Secreating Tumors

Nodular Lymphoid Hyperplasia

         lymphoid follicles in the lamina propria of the gut in Peyer's patches

         submucosal nodules

         causes of diffuse SI nodular lymphoid hyperplasia :

           immunoglobulin deficiency w/wo Giardia lamblia infection

         Sx. rectal bleeding, diarrhea, abdominal cramps beginning by 3 yrs

         resolves spontaneously

         require no specific Tx.

Chapter 292. Inguinal Hernia

; *▲common condition requiring op. in the pediatric age

; incidence

    - 10-20/1,000

    - M:F=4:1

    - 50% < 1yrs(most 6 Mo)

; *indirect inguinal hernia > direct inguinal hernia(1%), femoral hernia

; *Rt. side(60%) Lt.(30%) both(10%)

; *prematurity시 30%

Embryology & Pathogenesis

    ; *persistent patency of processes vaginalis

        cf) hydrocele : obliteration prox. & patency distally

★Fig. 291-1

Clinical Manifestation

         bulge in the inguinal region and extended toward or into the scrotum only

         during crying or straining

         swelling of the scrotum

         discomfort

         intestinal obs. abd. distension, vomiting

Diagnosis

 careful Hx.

 index finger manifulation : unnecessary

 infant lie supine with extended legs and arm over heads-bulge & swelling

 older children standing

 rectal exam.

 transillumination

 silk sign palaption of spermatic cord over pubic tubercle

# ★Risk Factor

    ; positive family Hx. of hernia, cystic fibrosis, CDH, undescended testis, ambiguous genitalia, hypospadia, epispadia, ascites, congenital wall defects

    ; connective tissue disorder as Ehlers-Danlos SD, Mucopolysaccharides

    ; *testicular femminization

           - *50%이상이 inguinal hernia을 가지므로 female with inguinal hernia는 반드시 고려

Treatment

    ; operative repair

           - Tx of choice

           - not resolving spontaneouly이므로 1세이전에 elective operation

    ; contralat. op. 남아는 1세이하, 여아는 2세 이하, 2세 이하의 Lt. side H.

          not op. Ix. isolated hydrocele (hydrocele of tunica vaginalis)

Complication

         incaceration 9-20% in 1st year higher in female, premature

           need to emergency op. : reduction 24-48시간내 elective

           method to reduce incacerated inguinal hernias

            - sedation by barbiturate, chloral hydrate

              placing in Trendelenburg position

              not use ice pack

           strangulation

         Richter hernia : intestine의 antimesenteric portion이 incaceration되지만

                          intestine의 obstruction은 없는 경우

         premature infant higher incidence & incaceration

Prognosis

         postop. Cx. 2% : wound infection 1%

         recurrence rate < 1%

section 5. exocrine pancreas

Chapter 293. Embryology, Anatomy, And Physiology

293.1 Anatomic Abnormalities

Pancreas divisum

    ; *▲common anomaly

    ; failure of dorsal and ventral pancreatic anlagen to fuse

           --> tail, body, head of pancrease drain through small accessory duct of Santorini

    ; recurrent pancreatitis

    ; Treatment

           - endoscopic insertion of an endoprosthesis

Chapter 294. Pancreatic Function Tests

# ★Bentriomide (N-benzoyl-L-tyrosyl-p-aminobenzoic acid, Chymex)

    ; synthetic tripeptide for noninvasive testing of pancreatic enzyme function

    ; cleaved by chymotrypsin --> releasing *para-aminobenxoic acid (PABA)

    ; measured at 90min in serum specimen

Chapter 295. Disorders Of The Exocrine Pancreas

Disorders Associated With Pancreatic Insufficiency

Cystic Fibrosis

Shwachmann-Diamond Syndrome

Isolated Enzyme Deficiencies

Syndromes Associated With Pancreatic Insufficiency

Chapter 297. Pancreatitis

Pathogenesis

Clinical Manifestation

    ; *abdominal pain, persistent vomiting, fever

    ; pain

           - epigastric, steady

           - resulting in antalgic position : hips & knees flexed, sitting upright or lying on the side

           - increase in intensity for 24-48hr

           - time vomiting increase

Diagnosis

Treatment

Prognosis

297.1 Chronic Pancreatitis

★Etiology

    ; hereditary pancreatitis

    ; idiopathic congenital anomalies of ductal systems

    ; *hyperlipidemia(type I, IV, V)

    ; *hyperparathyroidism

    ; ascariasis

    ; cystic fibrosis

Diagnosis

# ★ERCP

    ; *any child with idiopathic nonresolving, recurrent pancreatitis