Abstract

A–1–AT fecal clearance and random fecal A–1–AT were measured in children with symptoms of protein loosing enteropathy (PLE) and in normal subjects.Material and methods: 44 children with PLE at the age (1/12 – 18 yr) were examined. In 8 children – clearance A–1–AT (Bernier J. and co., Lancet II, 763, 1978) was done, in 36 children - random A-1-AT (Crossley and Elliot, Br Med J 1977, 428). Control group 18 healthy children at the same age.Results: In 6 healthy controls A–1–AT clearance: x ± SD = 5.58 ± 1.76 ml/24 h. A-1-AT clearance was elevated in children with PLE: intestinal lymphangiectasia (n = 4,65.2 – 236 ml/24h) Crohn's disease (n =1, 114.6 ml/24h). Barret's disease (n = 1, 14.3 ml/24h), protracted diarrhea (n= 1, 19.1 ml/24h), pericarditis constrictiva (n = 1, 800ml/24h).Mean random A–1–AT concentration in 12 healthy controls was: x ± SD = 1.37 ± 0.32.In children with PLE random A–1–AT were elevated: intestinal lymphangiectasia (n = 4, 8.69 ± 1.39 mg/1g), Crohn's disease (n = 7, 11.18 ± 3.67 mg/1g) ulcerative colitis (n = 4, 6.13 ± 4.06 mg/lg), hypogammaglobulinemia (n = 3, 6.21 ± 2.97 mg/1g), Viskott–Aldrich (n = 1, 6.75 mg/1g), protracted diarrhea (n = 9, 3.69 ± 0.79mg/1g), hepatitis neonatale (n = 1, 4.33 mg/1g), cystic fibrosis (n = 2, 2.34; 6.36 mg/1g), coeliac disease (n = 4, 2.79 ± 0.81 mg/1g), uncompensated heart failure (n = 1, 29.51 mg/1g).A significant correlation between random A–1–AT concentration and A–1–AT clearance was found (n = 13, r= 0.997, p< 0.001).Conclusions: 1) A–1–AT is a reliable endogenous marker for intestinal protein loss. 2) In gastrointestinal disorders A–1–AT excretion is an indicator of the disease activity and severity of the intestinal damage. 3) Random A–1–AT concentration is a valuable and simple screening test for PLE.

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