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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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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In 1988 and 1989, 79 children have been treated for induction of acute leukemia. 68 presented an acute lymphoblastic leukemia (ALL) and 11 an acute non-lymphoblastic leukemia (ANLL). The complete remission rate was 92% (96% in ALL, 73% in ANLL). Fever occurred in 50% of the children, with positive blood cultures in 11 of them. ⋯ Four patients were transferred into the intensive care unit. After 8 days, the treatment of ALL was continued in the outpatient clinic in more than 50% of the cases. The treatment of ANLL is frequently complicated by hemorrhages and sepsis and needs adapted supportive care in a specialized unit.
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Medulloblastoma is a frequent brain tumor in childhood. Recent advances in histogenesis, management of early and late side-effects, and prognosis are discussed in this review.
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The increasing aggressivity of the modern technology connected with neonatal resuscitation had led to the recognition by paediatricians of the existence of pain in the newborn, and of the necessity to treat this condition. The neurophysiological development of the neonate is such that it allows pain perceptions even in premature infants; a number of clinical parameters have therefore been described for pain recognition in neonates. An antalgic therapeutic scheme to be used for treatment of pain in the intensive care unit is proposed.