Archives françaises de pédiatrie
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From 1975 to 1986, 75 neonates were treated at the Institute Gustave-Roussy for a malignant solid tumor, comprising 1.7% of the overall oncopediatric population treated during the same period of time. Of these 75 patients, 47 (62%) presented with neuroblastomas, 15 (20%) with mesenchymal tumors, 9 (12%) with germ cell tumors and 4 with various other types of tumors. Treatment consisted of surgery in 63 patients, chemotherapy in 43 and radiotherapy in 18. ⋯ Neuroblastomas did the best with a crude survival rate of 89%. The young age of the patients resulted in two types of problems: immediate tolerance and long term sequelae. The therapeutic regimen should take into account the good prognosis of the majority of these tumors in order to decrease delayed effects.
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A critical analysis of the evolution during the first 24 hours was undertaken in 41 children and adolescents (age: 10.1 +/- 4.6 years) treated for diabetic ketoacidosis. Three of 4 children presented with ketoacidosis revealing diabetes. One of 4 was less than 6 years of age. ⋯ Insulin doses were 2 UI/kg/24 hours and were inversely correlated with the admission pH (r = -0.6; p = 0.0001). This study shows the efficacy of a treatment taking into account the pathophysiology of diabetic ketoacidosis and the knowledge of the complication risk factors, by foreseeing the adjustments to be done with respect to individual and/or at risk situations. These precise descriptive data, collected on a large group of patients, establish a reference basis to follow evolution in the course of the treatment of diabetic ketoacidosis in children.
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Hyperoxaluria type I (HPI) is a metabolic disorder secondary to liver alanine glyoxylate aminotransferase deficiency. Renal failure occurs due to the excessive production and precipitation of oxalate in the kidney. Combined liver-renal transplantation is the correct treatment for this condition when end-stage renal failure occurs as with renal transplantation alone the risk of recurrence of the same pathology in the transplanted kidney would be high. We report the case of a 4 year-old child with HPI suffering from terminal renal failure in whom a hepato-renal transplantation was performed: six months later, creatinine clearance was 62 ml/min/1.73 m2 and liver function tests were normal.