Human leukocyte antigens in Turkish pediatric celiac patients.

With the aim to determine the frequency of human leukocyte antigen phenotypes of celiac disease in Turkey, thirty celiac patients fulfilling the European Society of Pediatric Gastroenterology and Nutrition criteria were included in the study. The mean age of the study population was 5.8 +/- 4.3 years and of the control subjects was 32.6 +/- 6.7 years. The human leukocyte antigens -A, -B, -DR and -DQ were studied serologically by micro lymphocytotoxic reaction. It was found that human leukocyte antigens A-25(10), -B8, -DR18(3) and -DQ2 were more significantly frequent in the celiac population than in the control group. Children with antigen -B8 showed a five times higher risk for celiac disease and those with antigen -DQ2 showed a nine times higher risk. It was determined that human leukocyte antigen -B4 had a protective role in celiac disease. The study suggests that the human leukocyte antigen -A25(10) is a phenotype particularly encountered in Turkish pediatric celiac patients.

A case of vertical transmission of hepatitis A virus infection.

We present a case of hepatitis A infection in a 2.5-month-old male who became icteric after 18 d of birth. The diagnosis of hepatitis A was made by compatible clinical symptoms, laboratory results and liver biopsy showing evidence of hepatitis, and confirmed by detection of anti-HAV IgM antibodies. Because the mother had an acute icteric hepatitis A 1 week before delivery, and the viraemic phase of hepatitis A infection is very short, approximately 7 d, we suggest that the infant was infected by his mother, before birth.

[Hennekam syndrome]. / Syndrome de Hennekam.

UNLABELLED: Hennekam syndrome is a disorder comprising intestinal lymphangiectasia, facial anomalies and moderate mental retardation. Eight cases have been previously reported. CASE REPORT: A 17-month-old girl was admitted to hospital for peripheral edema. On physical examination, she presented with a normal mental development. Facial anomalies were noted including a flat face, depressed and broad nasal bridge, puffy eye lids, mild down-slanting palpebral fissures, hypertelorism, epicanthal folds, bulbous nasal tip, small mouth, and low set ears. A simian line and haemangiomas on the arms, trunk and left limb were also noted. There was no organomegaly. Laboratory investigations showed iron deficiency anemia, hypoproteinemia, hypogammaglobulinemia and an elevated level of alpha-1 antitrypsin excreted in the feces. Endoscopic investigation and the small bowel biopsy showed findings consistent with lymphangiectasia. The patient did well on 24 hour enteral nutrition including medium-chain triglyceride rich diet and infusion of human albumin. CONCLUSION: We have aimed to remind that Hennekam syndrome should be included in differential diagnosis when intestinal lymphangiectasia are associated with facial anomalies.

[Treatment of chronic viral hepatitis B in children with moderate doses of alpha interferon]. / Traitement de l'hépatite chronique à virus B de l'enfant par des doses modérées d'interféron alpha.

AIM: The alpha interferon treatment criteria have not been established in children with chronic hepatitis B. We report the results of a prospective study. METHODS: Between 1988-1992 14 children (2 girls and 12 boys) with chronic hepatitis B received 3 million U/m2 of interferon alpha three times a week for 6 months. All patients underwent a liver biopsy that showed a pattern of chronic active hepatitis. One patient had cirrhosis. Hepatitis B surface antigen, hepatitis Be antigen and hepatitis B virus DNA had been positive in the serum in all for at least 6 months and anti-delta antibodies were negative in all. Pretreatment aminotransferase levels were at least 1.5 times the upper limit of normal. RESULTS: After treatment patients were followed up for at least one year (mean: 21.5 +/- 8.3 months). At the end of treatment HBV DNA was negative in 13 out of 14 patients and reappeared in one; HBeAg seroconversion was observed in 11 patients with the appearance of anti-HBe antibodies. Six patients lost the HBs antigen within 1 to 14 months after treatment. Anti-HBs antibodies did not appear in any patients and aminotransferase level normalized in 13 patients. Thirteen patients underwent liver biopsy after treatment which showed improvement in 12. CONCLUSIONS: Treatment with alpha interferon at doses of 3 MU/m2 is effective in children with active hepatitis B. Long-term follow up is needed to evaluate the effectiveness of this therapy.