[Emergency treatment of an acute form of leucinosis by peritoneal dialysis].
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Biomedical subjects
Publications and source records attributed to B Labrune.
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Report of a case of a neonatal hypoglycemia detected at the 11th hour of life, secondary to a Langherans polyadenomatosis in a girl with an hemihypertrophy. Pre- and post-operative tests are reported. In case of neonatal hypoglycemia, the criteria leading to the diagnosis of hyperinsulinism, i.e. the only neonatal hypoglycemia with surgical treatment are reviewed. Except in the case of adenoma, the pancreatectomy should be performed subtotally. The best time for surgery is as soon as the 3rd week, in order to preserve the cerebral development.
Two new cases of Proteus syndrome are reported. This congenital syndrome, first described in 1983, comprises gigantism of extremities, body hemihypertrophy, pigmented nevi and multiple tumors (subcutaneous, lipomas, hamartomas). This syndrome belongs to the same group as Recklinghausen disease, Maffucci or Klippel-Trenaunay syndromes. The prognosis is not well known but mostly depends on functional and psychologic consequences of important deformations.
The authors report the association of neonatal hyperinsulinism (nesidioblastosis) and congenital hemihypertrophy in an infant. At the age of 13 6/12 years fibro-adenomas of the breast occurred. The diagnosis of Beckwith-Wiedemann syndrome is discussed.
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