The purpose of the present study was to evaluate the incidence of BehFet disease among Japanese children and to compare their clinical features with adult patients. A first questionnaire was sent to the department of pediatrics of 1290 hospitals in Japan. Fifty-one cases of Behget disease among children under 16 years of age were reported. From a second survey, 31 cases satisfied the criteria of the BehGet's Syndrome Research Committee of Japan. Of 31 patients (14 boys and 17 girls), three cases were the complete type, 24 cases were the incomplete type, and four cases were the possible type. As to the specific type of the disease, 10 cases were of the entero-BehGet type, two were a combination of the entero-vasculo-Behqet type, and four were the neuro-BehGet type. In the first 6 months after onset, oral ulcers were observed in 77% of cases, genital ulcers in 45%, uveitis in 10%. and skin lesions were observed in 39% of cases. During the course of the disease, the frequency of oral ulcers was 100%, genital ulcers, 58%; ocular complications, 29%; and skin lesions, 55%. Gastrointestinal signs and symptoms were more frequent in childhood Behqet disease than in adults, while ocular complications were less frequent. The diagnosis of Behqet disease in children is difficult because of the long interval before the onset of enough manifestations to satisfy the diagnostic criteria. Specific diagnostic criteria are necessary, based on a discussion of the many childhood cases of Behget disease.

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Abstract--We report a case of systemic lupus erythematosus (SLE) associated with hemolytic uremic syndrome (HUS). The patient was a 13-year-old boy who had complained of nausea and diarrhea. Abnormal urinalysis, pancytopenia and renal dysfunction were revealed. The immunological studies showed an elevation of antinuclear antibody and anti-double-stranded DNA antibody titers with a low complement level. Renal biopsy specimens showed diffuse membranous glomerulonephritis with microthrombi in the glomerular capillary lumen. He was diagnosed as having SLE with HUS. Methylprednisolone pulse therapy was performed. Renal function, proteinuria and hematuria were gradually improved. Prednisolone and an immunosuppressive agent (mizoribine) were prescribed per os. In our case, diarrheal prodrome was present, so gastro-enteritis was suggested as the trigger of HUS, but the causal agent was not detected. HUS was considered to be an accelerator in the renal lesions of SLE. There have been few reported cases in children of SLE associated with HUS.

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