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.

Vascular compromise prior to intestinal manifestations of Crohn's disease in a 14-year-old girl.

Vascular manifestations as extraintestinal symptoms of Crohn's disease are rare and only occasionally reported in children. A 14-year-old girl with vascular compromise prior to intestinal manifestations of Crohn's disease is described. The vascular symptoms were due to segmental narrowing of several major arteries as shown by angiography. This kind of vascular involvement in our patient is different from the pattern described in Crohn's disease and resembles Takayasu's disease. Recently, it has been suggested that Crohn's disease could be mediated by multifocal gastrointestinal infarction due to chronic focal mesenteric arteritis at the level of the muscularis propria of the gut. In Takayasu's disease, a granulomatous inflammation of the vasa vasorum of affected vessels is frequently found. An intramural arteritis, granulomatous in nature, could be the common pathway in both Crohn's and Takayasu's diseases. Until the etiologies of both diseases are uncovered, the interrelation between them will remain subject to speculation.

Acute liver injury after protracted seizures in children.

Three children were observed to have extensive liver injury following protracted seizures. Two recovered with supportive care and one died from central nervous system complications. When first measured, the levels of aminotransferases were minimally elevated, but they increased to 250 to 8,000 times normal within 12 to 24 h after the seizure episode. They fell to near normal over the next 8 to 11 days in the survivors, and to one sixth of the peak level by 4 days in the patients who died. A percutaneous liver biopsy from one child demonstrated centrolobular necrosis consistent with severe ischemic injury. Common causes for liver dysfunction, including viral hepatitis, drug hepatitis, and Reye syndrome, were excluded on clinical, serologic, and histologic grounds. We reason that hepatic injury resulted from ischemia. We speculate that prior treatment with anticonvulsants, which are capable of inducing mixed-function oxidases in the liver, aggravated the ischemia-reperfusion injury by increasing the production of reactive oxygen intermediates and reducing cytoprotective mechanisms. Prevention of such injury should be directed toward control of seizures and early respiratory support when seizures occur, not restructuring medication regimens.