Protein-losing enteropathy

normal enteric protein loss : 10-20% albumin turnover

1. 원인 : Tab 285-8

2. 병태생리

* 몇가지 기전

① inflammed or ulcerated mucosa를 통한 protein loss : UC, CD

② disordered mucosal cell structure : celiac sprue

③ increased lymphatic pressure : granulomatous or neoplastic involvement of lymphatics

④ dilated lymph vessel : idiopathic intestinal lymphangiectasia

3. 진단 1) 125I-labeled serum album scan등..

2) a1-AT(serum & stool)

normal stool a1-AT : 2.6mg/g↓

* random fecal AT assay : simple screening method for enteric protein loss

Intestinal lymphangectasia

1) 정의 : 비정상적으로 확장된 small intestinal lymphatic channels을 통한 enteric protein

loss로 인하여 hypoalbuminemia, edema, lymphocytopenia, malabsorption,

chylous effusion등이 생기는 질환.

2) 임상적 특징

asymmetric edema, chylous effusion, diarreha, steatorreha(mild)

hypoalbuminemia, IgGAM, transferrin, ceruloplasmin↓, hypocalcemia

vit B12 흡수장애

lymphocytopenia(400-1000/mL)

lymphangiogram : hypoplastic peripheral & visceral lymphatics

3) 진단

① small intestinal bipsy

② radiolabeled macromolecules을 이용한 increased enteric protein loss확인

4) 치료

low-fat diet, MCTG