Orthotopic liver transplantation for autoimmune hepatitis and cryptogenic chronic hepatitis in children.

BACKGROUND: Autoimmune hepatitis (AIH) and cryptogenic chronic hepatitis (CCH) are important causes of liver failure in children, frequently necessitating orthotopic liver transplantation (OLT). The aim of this study is to review disease progression and potential differences between subgroups of children with AIH and CCH. METHODS: The medical records of 65 children diagnosed with AIH or CCH between 1980 and 1998 were evaluated. RESULTS: The median age at presentation was 9 years, 8 months (range 4 months-19 years), and the median follow-up period was 8 years (range 3 months-18 years, 10 months). Forty-one patients (63%) were female. Twenty-eight patients were Hispanic, 28 were Caucasian, 8 were African-American, and 1 was Asian. Forty-three patients (66%) were diagnosed with type 1 AIH, 8 (12%) with type 2 AIH, and 14 (22%) with CCH. Forty patients (62%) underwent OLT (51% of those with type 1 AIH, 75% of those with type 2 AIH, and 86% of those with CCH). Thirteen (33%) of the transplanted patients experienced disease recurrence. African-American patients experienced a significantly higher rate of disease recurrence post-OLT than did Hispanic patients. Seven patients (11%) died, two without OLT, and five posttransplantation. CONCLUSIONS: AIH and CCH frequently necessitate OLT in children. CCH is a more aggressive disease than Type 1 AIH among children with these disorders. Ethnicity influences the rate of disease recurrence after liver transplantation.

Intestinal strictures presenting with gastrointestinal blood loss.

The typical manifestations of intestinal strictures include abdominal distention, bilious vomiting, hematochezia, diarrhea, disaccharide intolerance, and occasional growth failure. However, chronic gastrointestinal (GI) blood loss from ulcers at the site of the stricture has not been noted as a major feature. We report three patients in whom an intestinal stricture presented with minimal evidence of obstruction, but with GI bleeding and anemia. Our experience indicates that intestinal strictures with ulcers must be considered in the differential diagnosis of blood loss, and that surgical intervention may be required if GI blood loss is chronic and laboratory results are negative.

Budd-Chiari syndrome associated with antiphospholipid antibodies in a child: report of a case and review of the literature.

Budd-Chiari syndrome, hypertension, and thrombocytopenia developed in a 6-year-old girl as manifestations of primary antiphospholipid antibody syndrome (APS). She improved with systemic corticosteroid and anticoagulation therapy. Anticardiolipin antibodies were found in the patient, her mother and 3 siblings, suggesting the importance of genetic factors. The clinical features of an APS in children is reviewed.