Archives francaises de pediatrie最新文献

Background: The immediate medical causes of childhood death in developing countries are low birthweight, inadequate nutrition and serious infectious diseases. This report describes the main diseases causing infant mortality in Algeria.

Population and methods: 1% of Algerian household was investigated in December 1989 in a cross-sectional single-interview survey. Sampling was simple random stratified based on the population dispersion, with a second degree cluster for district and household. Questionnaires included 6 data on the deaths: birthdate, day of death, sex, associated factors, diagnosis of the mother, and diagnosis of the survey team.

Results: 32,945 births were registered between 1985 and 1989. There were 1,608 deaths. The main factors linked to these deaths were acute diarrhea (18.4%), acute respiratory diseases (13.8%), premature birth (11.6%). Premature birth was the main factor (31.9%) linked to mortality before the age of 1 month. Factors linked to mortality were not influenced by sex but were influenced by population dispersion. The mortality rate and the factors linked to it remained unchanged between 1985 and 1989.

Conclusions: These results will help target the national program to reduce infant mortality.

Background: A hemolytic-uremic syndrome associated with methylmalonic aciduria and homocystinuria is seen during the first weeks of life. A molecular defect in the CbIC mutation has been found. This report describes a new case with this association.

Case report: A girl, the second in this family, was born at term: her birth weight was 2,100 g, height was 47 cm and head circumference 31.5 cm. She was admitted at 32 days of age with hemolytic anemia and fragmencytosis, renal failure and thrombocytopenia. The renal failure required peritoneal dialysis followed by hemofiltration. The signs of pancytopenia of central origin and liver failure seen at that time raised the possibility of an intracellular defect of B12 metabolism. Chromatography of the amino acids and organic acids in the urine and plasma revealed homocystinemia, hypomethioninemia, homocystinuria and methylmalonic aciduria. The deficient B12 metabolism was confirmed in fibroblasts which showed deficits in both methyl and adenosyl-cobalamin synthesis. The metabolic disturbances were completely resolved after intravenous administration of hydroxy-cobalamin (2,000 micrograms per day) and folinic acid (25 mg per day) for 5 days. But the neurological abnormalities persisted, with retinitis pigmentosa and major leukodystrophic changes seen by MRI, and the infant died one month later.

Conclusion: This new case emphasizes the importance of systematically screening all cases of neonatal hemolytic-uremic syndrome for this autosomal recessive disorder.

Background: Granulocytic sarcoma is more frequent in adults; it can be a tumoral localization of acute non-lymphoblastic leukemia. This report describes an infantile case revealing an acute myeloid leukemia.

Case report: A 14 month-old girl was admitted because of enlarged bilateral cervical lymph nodes. They had increased in volume over the past month and were accompanied by fever and anorexia. The liver and spleen were moderately enlarged. Hematologic data were: Hb = 7.5 g%; leucocytes = 14,900/mm3; platelets = 614,000/mm3. There were no abnormal cells. X-rays and MRI showed a mediastinal mass. Bone scintigraphy was normal but the myelogram showed a few atypical cells that were also seen in a biopsy of the cervical lymph node. Electron microscopic examination of these cells showed that they were myeloid in origin. This was confirmed by immunohistochemistry. The cytogenetic examination showed many chromosomal abnormalities in the lymph node and myelogram. The child is now recovered after nine months of chemotherapy.

Conclusion: Diagnosis of acute myeloblastic leukemia in children is difficult when it is revealed by granulocytic sarcoma without major infiltration of bone marrow. The clinical presentation as a mediastinal mass is rare. The cytogenetic study was determinant for diagnosis.