[Methylmalonic acidemia. A new cause of severe ketoacidosis. Study of a case].
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Biomedical subjects
Publications and source records attributed to R Perelman.
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Two cases, in children of distinct families, of a particular form of "dry syndrome", are described. This syndrom, which associates xerophthalmy, xerostomy and cutaneous dryness, is congenital and familial. He looks different from previously described diseases or syndroms which include one or several of these three components.
A case of chronic neurogenic hypernatremia with arhinencephaly has been studied. The clinical and biological picture of neurogenic hypernatremia is reviewed. Hormonal studies have been performed, to try to clear up the physiopathology of this syndrom.
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No immunodeficiency could be found in a case of generalized BCG infection. The therapeutic trials were unsuccessful, and the child died at the age of 19 months.
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In a three and a half old girl, we have noticed the apparition: first, of on idiopathic pulmonary hemosiderosis; then, five months later, of a rheumatoid arthritis with LE cells and antinuclear antibodies of very difficult detection. The meaning of this articular involvement is discussed: rheumatoid arthritis with LE cells, or rheumatoid arthritis marking the beginning of a systemic lupud erythematosus. The literature concerning the association of an hemosiderosis and a connectivite is reviewed.
OBJECTIVE: The objective of this study was to compare the relative effects of both antepartum and intrapartum maternal plasma glucose concentrations on neonatal plasma glucose levels. STUDY DESIGN: This was a prospective, nonrandomized study. Two hundred thirty-three pregnant insulin-requiring patients with diabetes received intensive diabetic treatment aimed at maintaining the 2-hour postprandial plasma glucose level < 150 mg/dl and the intrapartum plasma glucose level < 100 mg/dl. The neonatal plasma glucose level was monitored during the first 48 hours of life and the incidence of neonatal hypoglycemia was compared with the average antepartum and intrapartum maternal plasma glucose concentrations. RESULTS: The lowest incidence of neonatal hypoglycemia occurred, as expected, among infants of patients who had the lowest mean antepartum and intrapartum plasma glucose levels. However, the intrapartum plasma glucose concentration had a stronger association with decreased neonatal hypoglycemia than the antepartum plasma glucose levels. CONCLUSIONS: Even in the presence of poor antepartum diabetic control tight regulation of the intrapartum plasma glucose levels will significantly reduce the incidence of neonatal hypoglycemia.