[Adoption and chronic hepatitis B carrier state]. / Adoption et portage chronique du virus B.

AIM: Because there are few adoptable children in France, parents, for the last 20 years, have turned to international adoption. Alerted by the generally poor health of these children, we paid particular attention to their health problems and especially to infection by hepatitis B virus (HBV). POPULATION AND METHODS: The 60 internationally-adopted children seen from June 1993 to June 1997 were included in this study. All had hemogram and serum iron dosage, and search for intestinal parasites and tuberculosis was performed in each child, as were HBs antigen and HBs antibody screening. When HBs antigen was positive, HBe antigen and antibodies, HBV DNA and hepatitis C and delta serology were also studied. RESULTS: Six out of the 60 children were HBV chronic carriers. The six presented HBs antigen and five out of the six presented viral DNA. One child was co-infected with delta virus. Serum aminotransferase was normal in three children and increased in the three others. DISCUSSION: Some internationally adopted children are exposed to chronic infection by HBV. This concerns children coming from countries known for the high frequency of the disease, but also children with long stay in Eastern European nurseries. Chronic HBV carriage puts the child, as well as the family and other children in institutions and/or schools at risk, thus necessitating preventive measures.

[Idiopathic thrombocytopenic purpura: long-term outcome]. / Purpura thrombopénique idiopathique chez 87 enfants: évolution à long terme.

BACKGROUND: Idiopathic thrombocytopenic purpura (ITP) has usually an excellent prognosis even when no specific therapy is given. We studied, in 1996, the outcome of 87 patients with ITP observed between 1973 and 1995. PATIENTS AND METHODS: These 87 cases represented all the thrombocytopenic purpura observed during 23 years in the same pediatric unit not specialized in hematology. The outcome of these children was investigated in 1996 by phone or writing to the patients themselves or to their physician. Adequate information was obtained for 63 patients (72.4%). RESULTS: Two cerebral hemorrhages were observed, one was lethal while the other, observed after several years of course, healed completely. All these patients became free from clinical signs but 11 were not cured in 1996. None of these 63 patients had developed autoimmune disease. One patient died in a traffic accident, his thrombocytopenic purpura had been cured. CONCLUSION: In spite of two cerebral hemorrhages, ITP remains in the great majority of cases a benign disease not requiring grave therapeutic decisions.

[Giant cell hepatitis and autoimmune hemolytic anemia: efficacy of splenectomy on hemolysis]. / Hépatite à cellules géantes et anémie hémolytique auto-immune: efficacité de la splénectomie sur l'hémolyse.

BACKGROUND: The previously reported cases of giant cell hepatitis with autoimmune hemolytic anemia were improved by prednisone plus azathioprine. CASE REPORT: A 14-month-old boy suffered from giant cell hepatitis with auto-immune hemolytic anemia and positive direct Coombs test. Prednisone and azathioprine administration improved the liver disease but failed to control hemolysis so that repeated blood transfusions were necessary. Persistance of severe degree of hemolysis required splenectomy that was promptly and definitively effective. Azathioprine and prednisone were pursued for a total duration of five years. Twelve years after the onset of the disease, the child is well without any treatment. CONCLUSION: This is the first reported case of such an association in which poorly controlled auto-immune hemolytic anemia benefited from splenectomy.

[Acquired giant lobar emphysema in an artificially ventilated premature infant. Cured by corticosteroid therapy]. / Emphysème lobaire géant acquis chez un prématuré sous ventilation artificielle. Guérison par la corticothérapie.

A case of acquired giant lobar emphysema in a premature triplet born at 31 weeks gestational age and treated by artificial ventilation is reported. Corticosteroid therapy was remarkably successful.

[Bilateral newborn testicular torsions]. / Les torsions testiculaires néonatales bilatérales.

A case of bilateral spermatic cord tension in a neonate is reported. Clinical findings are compared with those in 22 previously published cases. Obstetrical history is usually unremarkable. Torsion occurs prior to birth or immediately after birth. The two cords are involved simultaneously or successively. Edema of the scrotum is found in recent spermatic cord tension, whereas subsequently the tests are extremely hard and occasionally severely atrophied. Prognosis is poor since castration or poor testicular viability was the outcome in most cases.

[Severe neurologic course of galactosemia. Default of myelisation caused by deficient synthesis of UDP-galactose?]. / Evolution neurologique sévère d'une galactosémie. Défaut de myélinisation par synthèse insuffisante en UDP galactose?

We describe the unfavourable neurological outcome of a galactosemic patient who however received an appropriate diet from the neonatal period. The magnetic resonance imaging showed significantly abnormal myelinization of the brain. The biochemical hypotheses at the origin of this abnormal cerebral myelinization are discussed in our patient. The 1-14C incorporation in the TCA precipitable glycoproteins reached 21% of the average value observed in 6 controls; it ranged from 37% to 59% for galactosemic patients without neurological disturbances. These results, which have to be confirmed, suggest that some galactosemic patients have a very low UDP-galactose synthesis.

[Acute parvovirus myocarditis with fatal outcome]. / Myocardite aiguë à parvovirus d'évolution létale.

We report a new complication related to parvovirus B 19: acute lethal myocarditis in the context of a familial epidemic. Serological studies confirmed the existence of a recent infection and the B 19 structural proteins were detected by immunocytochemical techniques on myocardial sections.

[Hematologic anomalies and triploidy]. / Anomalies hématologiques et triploïdie.

Major macrocytosis seems to be suggestive of triploidy in dysmature infants with multiple malformations. This marker may prove ethically useful since its presence may justify withholding special therapies until results of the karyotype are obtained.