Archives francaises de pediatrie最新文献

Background: Infants suffering from respiratory syncytial virus (RSV) infection can have severe responses that require intensive care. This study compares the epidemiologic patterns and the severity of respiratory diseases produced by RSV strain subtypes A and B.

Population and methods: The prevalence of RSV subgroups was studied over 8 consecutive outbreaks from 1982 to 1990. The files of 73 infants aged from 1 to 24 months admitted because of RSV infection between October 1987 and March 1990 were studied. The criteria of severity were tachypnea and/or the use of the accessory muscles for respiration, apnea and/or cyanosis, hospitalization for > 8 days, hypercapnia and/or acidosis, oxygen therapy, use of corticosteroids and/or bronchodilators, nutritional difficulties.

Results: A total of 374 RSV strains were isolated: 142 were group A and were 232 group B. Subtype A predominated during the winter 1987-1988 and subtype B during 1983-1984, 1984-1985 and 1989-1990. Some indicators of severity, such as degree of respiratory distress, duration of hospitalization and levels of oxygen saturation, were correlated with A subgroup infections, but the difference in the severity index for the two subtypes was not statistically significant.

Conclusions: The results are not consistent with other previous reports, perhaps because different indicators of severity were used in this study and infants less than one month old or having other underlying diseases were excluded.

Background: The procedures used to locate pancreatic endocrine tumors have only limited success in infants and children in whom the nodules may be small. Portal catheterization may therefore be useful.

Case report: A child aged 6 yrs 4 months was admitted because of several recent episodes of pallor and sweating associated with hypoglycemia. Further investigation showed moderate hyperinsulinemia and low blood levels of ketone bodies and branched amino-acids after a 15 hr fast. Celiac angiography was normal. The hypoglycemic episodes recurred despite treatment with diazoxide for 6 months. A transparietal portal catheterization was therefore performed. Selective pancreatic venous sampling showed high concentrations of insulin in two small veins draining one part of the head of the pancreas (117 and 89 microU/ml). The head of pancreas was removed 16 months later. Extemporaneous examination revealed an adenoma 0.8 cm in diameter. This patient has completely recovered, 8 months after surgery.

Conclusion: Transparietal portal catheterization can detect pancreatic areas with high insulin secretion. It may also help the interpretation of celiac angiographs in children, as diagnosis may be obscured by the normal rich vascularity of the pancreas in these patients.

Background: Periventricular leukomalacia in preterm neonates is usually due to abnormal cerebral blood flow before and/or during delivery. It is diagnosed during the first month of life. This study describes a case of late-developing periventricular leukomalacia.

Case report: A girl was delivered by cesarean section at the 23rd week of gestation that was complicated by placenta previa. She weighted 880 g and immediately required resuscitation. She did not develop respiratory distress syndrome. She was intubated and ventilated for the first two weeks of life and developed bronchopulmonary dysplasia requiring corticosteroid treatment. Extubation was possible on day 43. The neonate had been given antibiotics for the first 10 days of life and indomethacin on day 8 because of a patent ductus arteriosus. Repeated craniosonography showed moderate bilateral intraventricular hemorrhage and occipital areas of hyperechogenicity that disappeared at the end of the first month of life. Repeated electroencephalograms revealed no positive rolandic sharp waves until the infant was 42 days old. Craniosonography on day 48 showed areas of heterogeneous hyperechogenicity and NMR imaging showed bilateral frontoparietal leukomalacia with loss of white matter. The baby died on day 71.

Conclusions: Late periventricular leukomalacia is exceptional and usually seen after postnatal abnormalities in cerebral blood flow and/or prolonged hypoxemia. Both these risk factors were absent in this patient.

Background: Chronic relapsing microangiopathic hemolytic anemia is rare in children. This report describes a case associated with thrombocytopenia following idiopathic thrombocytopenic purpura.

Case report: A 4 year-old girl was admitted for acute idiopathic thrombocytopenic purpura (platelet count: 12,000/mm3) without anemia or fragmented red cells. The patient was given intravenous gammaglobulins without success, followed by prednisone (2 mg/kg/day). The platelet count was normalized, but decreased when the treatment was discontinued. The patient developed acute intracranial hypertension at the age of 5 yr 8 mo, following two cerebral hematomas. The platelet count was 9,000/mm3. A second course of intravenous gammaglobulins and prednisone was unsuccessful, so a splenectomy was performed. One year later, the patient was admitted because of diffuse purpura, anemia and jaundice. Hematologic findings were: Hb 8.4 g/dl, reticulocytes 448,200/mm3, fragmented red cells 16%, platelets 15,000/mm3, WBC 22,400/mm3. Seroimmunologic investigation showed a high titer of antinuclear antibodies. Examination for viral etiology was negative. Intravenous gammaglobulins had a transient effect on platelets, reticulocytes and fragmented red cells. The patient was then given vincristine plus prednisone; they were only effective when high doses were used. A second intracerebral hemorrhage occurred when the patient was given low doses of drugs. After 3 other hematologic relapses, the vincristine was stopped without further complication.

Conclusion: The criteria for systemic lupus erythematous were not satisfied, despite the presence of antinuclear antibodies. A congenital deficiency of an unidentified plasma factor that reverses microangiopathic hemolysis and thrombocytopenia was not demonstrated in this patient, who could not be given fresh frozen plasma.

Background: The childhood emergence and development of motor behavioural skills is imperfectly understood despite intensive study. This report is of the development of motor skills and social behaviour in young children.

Population and methods: 17 infants (9 boys and 8 girls) in good health, aged 8 to 11 months at the beginning of the research, were studied. They were observed in groups of 6 for 30 minutes, in a special area fitted out with climbing structures, once every two weeks for one year. Their movements and behavior were filmed.

Results: 1) Motor skills which allow the "conquest" of climbing structures and complex social behaviors (multimodal interactions, imitations, cooperations, anticipations of behaviors) had developed by the time the children were 12 months old and were first examined. 2) From 12 to 14-15 months old, they consolidated these motor skills and the frequency of imitations and multimodal interactions increased markedly. 3) From 14 to 18 months old, they assumed the standing position more and more during ascents and descents and the frequency and diversity of imitations and cooperations increased sharply. 4) There was relatively little aggressive behavior at all ages.

Conclusions: Motor skills and social behavior appear earlier and are more complex in children moving freely among their peers in a special setting than previously reported for developmental scales.

Background: The cardiac involvement in cystic fibrosis includes the rare cardiomyopathy seen in infants and changes in left ventricular performance in older children.

Population and methods: 67 patients, 6 to 34 months-old (mean: 16.7), 37 male and 30 female, with cystic fibrosis, were studied. Their Shwachman score was < 70. None showed any clinical manifestations of left ventricular insufficiency, but 6 patients had right ventricular insufficiency. Echocardiography was performed on 58 patients; it showed dilation of the right ventricle in 32 of them. Left ventricular perfusion was studied with thallium 201 tomoscintigraphy and left ventricular ejection fraction with 99mTc ventriculography.

Results: The left ventricular ejection fraction was < 45% in 17 patients and scintigraphy showed hypofixation in 6 of them. In contrast, only 4 of the 50 patients with left ventricular ejection fraction > 45% had thallium hypofixation.

Conclusions: Resting perfusion abnormalities are more frequent in patients with a low left ventricular ejection fraction. These perfusion abnormalities suggest that myocardial fibrosis complicates the advanced stages of cystic fibrosis.

Background: Malabsorption of oraliron is rare, and more frequently suspected than proved. It could be due to prolonged iron deficiency.

Case reports: Case no. 1: A boy was admitted at the age of 5 months for recurrent bronchitis. His hemoglobin was 8.2 g/dl, mean corpuscular volume (MCV) 60 micron3, mean corpuscular hemoglobin (MCH) 15 ng and mean corpuscular hemoglobin concentration (MCHC) 25 gHb/dl. The serum iron was 1 microgram/dl, iron binding capacity (IBC) was 284 micrograms/dl and ferritin was 14.9 ng/ml. Dietary iron was inadequate. The patient was given ferrous sulfate but iron deficiency persisted at the ages of 11 months and 3 years, probably due to poor compliance. Similar hematologic data (Hb: 6.4 g/dl, MCV 55 micrograms/m3, MCH 13.9 ng, MCHC 24 gHb/dl) were found at the age of 9 years. The patient was then given ferrous sulfate orally as test but the serum iron levels were unchanged during the 4 hours following ingestion. A parenteral iron preparation (iron-dextran, 500 mg) improved the hematologic data. 6 months later, a new oral test with ferrous sulfate improved the serum iron level. Case no. 2: A boy with complex congenital cardiopathy was operated on in the neonatal period and given oral iron at the age of 9 months because of anemia with microcytosis and hypochromia. This anemia was still present at 17 months and was associated with normal or high serum ferritin. Electrophoresis of hemoglobin was normal. At the age of 4 yr 5 mo, Hb was 9.7 g/dl, MCV 62.8 micrograms/m3, MCH 18.4 ng, iron 16 micrograms/dl and ferritin 94.1 ng/ml. An oral test with ferrous sulfate failed to increase the serum iron. The patient was then given parenteral iron-dextran without benefit, and a second oral test remained ineffective. After a second course of parenteral iron-dextran, Hb was 11.5 g/dl, MCV 74.1 micrograms/m3, MCH 23.7 ng while the serum iron remained low (23 micrograms/dl) and ferritin increased to 587 ng/ml. A third oral test with ferrous sulfate was still ineffective, as was a test using 4 mg/kg iron.

Conclusion: The first patient suffered from iron malabsorption, presumably due to iron deficiency. The second patient could have abnormal metabolism and/or abnormal ferritin.