Archives francaises de pediatrie最新文献

Background: Neonatal hyperthyroidism is a relatively rare condition. It can be severe and difficult to treat when the maternal hyperthyroidism has recently been recognized.

Case report: A baby was born at 37 weeks of gestational age to a mother whose hyperthyroidism was only suspected at the 34th week. The mother was not given propylthiouracil until the day before delivery. At birth, the newborn presented with meconial fluid; it weighed 2,380 grams, was 46 cm long and had a head circumference of 32 cm. Clinical examination showed exophthalmos, tachycardia, hepatosplenomegaly, restlessness, moderate goiter and premature craniosynostosis. Laboratory data showed elevated serum free T4 (76 pmol/l), unmeasurable TSH, and the presence of thyroid-stimulating immunoglobulins. The infant was given propranolol (10 mg/day), and carbimazol (3.75 mg/day) from day 13. Clinical and biological improvement allowed the carbimazol to be stopped at 1 month. A relapse was treated with carbimazol plus L-thyroxine. Both drugs were discontinued at the age of 4 months. At 12 months, the baby is perfectly well and has developed normally.

Conclusion: This transient thyrotoxicosis was due to the transplacental passage of TSH receptor antibodies. Its management was difficult because the mother was treated late during her pregnancy.

Background: Kasabach-Merritt syndrome is characterized by one or more large hemangiomas associated with thrombocytopenia due to platelet trapping. The hemangiomas may be located in the abdominal viscera, in which case, treatment may be difficult.

Case report: A 2 1/2 month-old boy was admitted because of the discovery of an abdominal mass. He was anemic (hemoglobin: 6.8 g%; reticulocytosis: 288,000/mm3), thrombocytopenic (50,000/mm3), hypofibrinogenemic (0.95 g/l), with fibrin split products in the blood. Ultrasonography showed that this mass was retroperitoneal and heterogeneous. X-rays delineated the mass, which distorted the duodenal loop and pressed forward against the stomach. Surgery showed that the mass included the pancreas, the root of mesenterium and the extrahepatic bile duct. This mass was biopsied. Histological examination showed infantile type hemangioendothelioma. Abdominal angiography showed that the mass was not very hypervascularized, and there were no dilated supplying blood vessels. An aortogram showed two other small vascularized areas. The patient was given methylprednisolone, but the volume of the mass remained unchanged, while hepatosplenomegaly and jaundice developed and ultrasound showed dilated extra- and intrahepatic biliary ducts. The patient was then given interferon alpha 2b for 1 month. Embolization of the small supplying arteries was performed because of a lack of improvement after 1 week of treatment. This procedure was followed by rapid disappearance of the signs of consumption coagulopathy, a progressive improvement in cholestasis, and decrease in the mass.

Conclusion: Improvement in manifestations of this Kasabach-Merritt syndrome is difficult to assign to one of the several therapies. The rapid disappearance of consumption coagulopathy after arterial embolization suggests that this treatment was successful in our patient.

Background: Respiratory syncytial virus (RSV) infection in infancy can induce bronchial reactivity and virus-specific IgE production; these responses may favor the later development of asthma. This retrospective study examines the influences of early and severe forms of bronchiolitis on such an outcome.

Population and methods: The files of 43 infants who had spent 1 to 24 days (mean 7.8 days) in intensive care between 1986-1990 for severe bronchiolitis with apnea and/or hypercapnia and/or hypoxemia were studied. The parents of all these children agreed to answer a standard questionnaire covering perinatal events, gestational age, number and frequency of recurrent episodes of wheezing, personal and familial history of allergy and environmental conditions. 15 of the children were preterm and 12 of them were given respiratory support during the neonatal period; 5 of these patients developed broncho-pulmonary dysplasia. RSV was isolated in 20 of 39 patients.

Results: 26 of the 41 surviving patients developed at least 3 episodes of wheezing over periods ranging from 5 to 54 months (mean 27.6 months): 15 of them were admitted at least once for asthma and 17 were given long-term bronchodilators and/or corticosteroids. Of the 8 patients with histories of allergy, 6 developed asthma, as did 7 of the 9 that had been in close contact with other children, and 9 of the 15 patients who had been exposed to tobacco.

Conclusions: Not only is the development of asthma associated with risk factors (RSV infection, bronchopulmonary dysplasia, history of allergy, close contact with other children and exposure to tobacco) but the severity of the bronchiolitis per se is also correlated with this risk.

Background: The survival and outcome of infants with bronchopulmonary dysplasia (BD) depend on the patient's maturity, the severity of the BD and nutritional problems. This study evaluates the specific role of chronic pulmonary failure in the growth and development of infants recovering from BD.

Population and methods: 88 infants admitted for BD from January 1984 to December 1988, having gestational age from 25 to 41 weeks 5 days (mean: 29) and birth weight from 680 to 3,400 g (mean: 1,195) were studied. All infants were given respiratory support for 6 to 914 days (mean 84) and oxygen therapy for 28 to 1,232 days (mean: 119). 29 infants were given corticosteroids for more than 1 month. The outcome of the 80 infants with gestational ages of less than 33 weeks was compared to that of 272 infants with the same gestational age but not suffering from BD on their 28th day. The infants in both groups were examined at 2 years of age and classified as: a) handicapped (neurologic deficit, IQ < 80, hearing loss, blindness, convulsions); b) doubtful (transitory neurology dysfunction); c) normal.

Results: Of the 88 infants still living at the age of 28 days, 19 died before the age of 2 years: 16 of the 64 surviving infants who could be followed until the age of 2 years were classified as handicapped, 13 were considered doubtful and 35 were normal. The more significant risk factors for neurodevelopmental impairment were: a) the presence of porencephaly and/or ventricular dilatation on brain ultrasonography; b) head circumference < -2 SD at the end of hospital stay; c) oxygen therapy and hospitalization > 5-6 months. The group of infants with BD had a higher death rate (24% vs. 3.7 in the group without BD) and more frequent neurodevelopmental impairment at gestational ages of > 31-32 weeks.

Conclusions: BD is an extra risk for the survival and neurodevelopment of infants with gestational age > 31 weeks.

Background: Behçet's disease is very occasionally revealed by thrombophlebitis in children. Dural sinus thrombosis can be one of its complication.

Case report: A 14 year-old boy of Mediterranean origin was admitted for acute meningitis with temperature of 40 degrees C. His CSF contained 24 cells/ml and 0.33 g/l protein; it was sterile. The RBC sedimentation rate was 84 mm. Other investigations, including brain scan, were negative. The condition became worse, with a deterioration of visual acuity, attack of aphthous stomatitis and skin lesions at points of puncture. There was papilledema with retinal vascularitis. A second brain scan and MRI showed sagittal sinus thrombosis. The condition improved immediately after treatment with prednisolone and ticlopidine. The brain MRI taken 3 months later showed partial permeability of the sagittal sinus. The patient had several attacks of meningoencephalitis and stomatitis during the following 2 years. The last attack was complicated by iridocyclitis; this required cyclosporin treatment.

Conclusion: Phlebothrombosis is a classic complication of Behçet's disease and can reveal the disease. Sagittal sinus thrombosis has never before been reported as the first manifestation of the disease in children.