Gastrointestinal disease in the hemolytic-uremic syndrome

Gastroenteritis is recognized as a precursor of the hemolytic-uremic syndrome (HUS), but colitis as a major feature of this syndrome is less well established. The records of 12 consecutive patients, aged 1-40 yr, in which a secure diagnosis of the HUS could be made on the basis of acute azotemic renal failure with hemolytic anemia and thrombocytopenia, were reviewed with respect to GI disease. GI complaints initiated the disease in all patients; 12 had bloody diarrhea, 8 vomited, and 7 had cramping abdominal pain. These symptoms persisted 2 days to 6 wk before the diagnosis of the HUS was made. Prominent abdominal tenderness was elicited in 8 subjects: 2 had classical findings of peritonitis, and 5 had hepatomegaly. Proctoscopy, performed in 4 patients, revealed friable mucosa, 4; lax anal sphincter tone, 2; a rectal ulceration; and a dirty gray pseudomembrane. Transaminases were elevated in all patients studied; SGOT and SGPT ranged from 58 to 468 and 89 to 149 IU/liter, respectively. Plain abdominal X-ray demonstrated ascites, 3; massive colonic dilatation, 1; and thumb printing of the sigmoid colon, 1, while barium enema, performed in 2 patients, showed evidence of diffuse colitis, 2, and a 2X2 cm filling defect in the cecum. The illness was mistakenly diagnosed as appendicitis, ulcerative colitis, cecal polyp, pseudomembraneous colitis, or intussusception. Eleven patients recovered normal GI function with support for renal failure, but 1 died as a consequence of his intestinal disease. We consider intestinal and liver disease to be regular features of the HUS.