Unusual Protein-Losing Enteropathies In Children

Gastrointestinal protein loss in children has been described in a rapidly growing list of disease states. The site and extent of the protein loss can be studied by utilizing immunologic and isotopically labeled protein methods. The isotopes used are iodinated po1yvinylpyyrrolidone (131PVPI), iodinated serum proteins, and chromium-labeled proteins. These technics have shown that the loss of serum proteins into the intestina11umen plays an important part in their normal degradation. When serum proteins are lost into the gastrointestinal tract, whether in normal or abnormally large amounts, they are rapidly catabolized into their constituent amino acids. These amino acids are then reabsorbed and made available for protein resynthesis by the liver. The maximal protein synthesis ability of the liver is approximately double its normal rate. In many of the severe protein-losing enteropathies the rate of intestinal protein loss greatly exceeds the ability of the liver to respond, and hypoproteinemia, edema, and associated conditions such as anemia and growth failure result. We have recently seen 4 unusual cases of protein-losing enteropathy in children. Two of these exhibit lesions not previously described as the cause of gastrointestinal protein loss. Case Reports Case I: Congenital Ileal Stenosis: Since birth, K. F., a 4-year-old male, had intermittent, foul-smelling diarrhea which did not improve with changes in diet. Weight and height were below the third percentiles on the standard growth charts. The patient appeared quite malnourished, and there was minimal pitting edema in the lower extremities and a protuberant abdomen. Laboratory findings included hemoglobin of 8.7 per 100 ml; hematocrit 28 per cent; normal white blood count and differential; normal urinalysis; total serum protein 4.2 mg/100 ml; albumin 1.9 mg/100 ml; globulin 2.3 mg/100 ml; negative sweat test. Labeled tracers (131I and 131PVPI) indicated the gastrointestinal tract was the site of abnormally high protein loss. The barium enema findings were normal with a normal-appearing terminal ileum. A small bowel series (Figs. 1 and 2) disclosed dilated proximal loops and a single stenotic area in the mid-ileum. At surgery a single stenosis of the mid-portion of the ileum was found with proximal dilatation. Thirty-six inches of ileum were removed because of the marked dilatation. A jejunal biopsy was interpreted as normal. The removed ileum contained superficial ulcerations. Histologic study (Fig. 3) revealed a short area of muscular hypertrophy and absence of inflammatory cells, findings typical of those seen m congenital ileal stenosis. Following surgery gram-negative bacteremia and renal shutdown developed, and the patient died. Comment: Ileal stenosis as the cause of abnormal gastrointestinal protein loss has not been reported. The patient described above had an isolated, incomplete, presumably congenital ileal stenosis which was responsible for his symptoms from birth.