Pediatrie最新文献

The acronym CHARGE names a syndrome described by Pagon et Graham in 1981, which associates mainly ocular anomalies, i.e. coloboma, and uni or bilateral choanal atresia. We report three observations of children with choanal atresia confirmed by CT scan. One child exhibited all the signs of the CHARGE syndrome including cerebral involvement at autopsy.

Thanks to the technical progresses during the last decade and to the active participation of parents, parenteral nutrition may be undertaken at home in patients with chronic digestive or extra-digestive diseases with serious undernutrition. This technique being highly sophisticated and demanding, we have undertaken a study of the quality of life of 44 children submitted to home parenteral nutrition and followed by the four registered French centres of pediatric nutrition. This study shows that home parenteral nutrition improves the quality of life of both children and parents as compared with the preceding period in the hospital. However, because of its constraints, it requires a careful evaluation of the parents' capacity to cope with, before to be undertaken.

Eight young children with renal failure, undergoing continuous peritoneal dialysis (CDP) and presenting an anemia (hemoglobin level [Hb] 57 to 89 g/l) were treated by subcutaneous recombinant human erythropoietin (rHu EPO) twice weekly. The initial dose of 75 U/kg was adjusted to induce progressive increase of Hb with a target level of 100-120 g/l. Treatment duration was 24 weeks in five of these children and 10 to 13 weeks in the three others. In seven cases out of eight, anemia was corrected. The target Hb level was reached in 3 to 21 weeks with rHu EPO doses of 150 to 300 U/kg/w (mean: 200 U/kg/w) for four children without recent transfusion; then the median maintenance dose was 135 U/kg/w (range: 50-300 U/kg/w). In only one patient, Hb never reached a level higher than 77 g/l despite weekly dose of 350 U/kg, a reticulocytosis of 5.6%, rHu EPO treatment lasting up to 24 weeks and the absence of iron deficiency. In any case, no transfusion was necessary after the first day of rHu EPO treatment. In three patients, the increase of a preexisting hypertension required the adaptation of antihypertensive treatments. One patient presented a marked thrombocytosis. In conclusion, twice-a-week subcutaneous injections of 75 to 150 U/kg of rHu EPO appear to be well tolerated and effective in the treatment of anemia of CPD children.

The aim of the study was to determine in young pupil the physiological effect of a 1 hour earlier awakening (summer time) by measuring: the salivary cortisol level. Results showed that the cortisol circadian rhythm synchronized with summer clock time after remaining synchronized with winter time (previous rhythm) for 15 days at least.

Modifications of the total ear reconstruction technique allowing a two-stage procedure is described. However this is only possible if the soft tissue has not yet been damaged by previous plastic surgery or tympanoplasties. The framework sculpture is improved in order to obtain a posterior conchal wall at the first stage without using a skin graft. A tissue expander is used during the operative time in order to improve the framework coverage. A bone anchored hearing aid is preferred to tympanoplasty when necessary, for bilateral microtia. This method is effective and does not compromise the skin qualities. The osteointergrated implant is put in place during the second external ear reconstruction stage. The total hospitalisation period is no longer than 8 days.

The Peutz-Jeghers syndrome includes periorificial melanin spots and digestive adenoma, transmitted according to an autosomal dominant mode. We report on a case discovered in a 13 year-old girl presenting with anaemia.

The authors report on a case of a preterm infant who suffered from pneumonia due to genital mycoplasma infection. Hepatic dysfunction was associated. Tetracyclines were successful.