Pédiatrie
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Regarding a case of beta-ketothiolase deficiency revealed by ketoacidosis with hyperglycinemia, the authors show the way to diagnose and to treat this disease. Ketoacidosis without hyperglycemia or lactacidemia suggested this diagnosis. ⋯ The treatment of acute episodes consisted of acidosis control and exclusive glucides intake before diagnosis. Afterwards, a controlled proditic diet and L-carnitine must be given and fasting must be avoided.
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One of the aspects of prematurity in neonates is the respiratory distress syndrome. Although treatment with mechanical ventilation reduced the mortality rate, bronchopulmonary dysplasia still develops in many neonates. We have attempted to reduce intubation and mechanical ventilation by using, in the delivery room, humidified and warmed gas with fractional inspired oxygen as low as possible to obtain SaO2 between 85 and 95%. ⋯ Seven out of 26 infants (27%) born between 30 and 32 weeks required mechanical ventilation. In contrast, ventilation was necessary for eight out of 16 premature neonates born before the 29th week of gestation. Mortality rate was 6% (4/66) in the latter group (< 29 weeks), and only one neonate developed bronchopulmonary dysplasia.
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A survey carried out by questionnaire was performed among parents accompanying their children to the emergency department of the Lenval's children hospital in order to determine their opinion on the quality of the reception and care. The questionnaire was distributed to 2,382 parents. ⋯ A large majority of the parents (99%) expressed their satisfaction with the care and reception in the emergency department. The necessary improvements following this survey concern the duration of waiting prior to the consultation and the quality of the reception in the radiological and admittance departments; it is hoped that the radiological and admittance areas will be included within the emergency department, in the future.
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The authors report on a case of cardiocirculatory arrest in a 4-month old infant, related to severe vitamin D deficiency rickets with hypocalcemia. Despite medical follow-up, this infant, who was breast-fed during the first 3 weeks of life, did not receive any vitamin D prophylaxis. This observation shows the potential severity of vitamin D deficiency rickets. Thus, systematic prophylaxis should be applied as soon as the infant is born, even in breast-fed children.
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[Search for early indications for reintubation after mechanical ventilation weaning of the newborn].
Early predictive factors for successful extubation were investigated, in order to determine the best moment for respiratory weaning of the newborn, and the risk of subsequent reintubation. PaO2/FiO2 ratio, PCO2 and respiratory rate were measured 2 h after extubation in 100 newborn infants. There was no statistically significant difference for the PaO2/FiO2 ratio and PCO2 between infants who were successfully extubated (group 1) and those who required subsequent reintubation (group 2). By contrast, the respiratory rate 2 h after extubation was significantly higher in group 2, and a respiratory rate greater than 70/min appears to be the earliest ventilatory modification predictive of the need for further mechanical ventilation before the occurrence of hypoxemia and respiratory acidosis.