[Kawasaki's syndrome. Major therapeutic advances provided by gammaglobulins].
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to B Labrune.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
In France, the combination of diabetes insipidus, diabetes mellitus, optic atrophy, and deafness (DIDMOAD) is designated as Wolfram syndrome. An analysis of 14 personal cases and previous reports showed that the syndrome develops gradually and specified the most common order of occurrence of the various components as well as the other abnormalities (e.g., of the urinary tract) which may be found. Wolfram syndrome is an inherited condition (recessive autosomal transmission). The lack of association with HLA antigens seems to have been established (in the few cases where HLA typing was performed). The prognosis of Wolfram syndrome is grim, with the occurrence of each additional component adding to the severity of the disease.
This report concerns a transient and isolated hyperphosphatasemia in a 33 month-old infant. Such a case is asymptomatic and benign. There is an important increase in enzymatic activity which includes both fractions (bone and liver). Pathophysiology is still unclear. This biological data is worth being known in order to avoid useless and always normal investigations.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
The authors report a case of acquired polydipsia in an infant. The case was unusual in its presentation, its late onset (without anorexia, nor vomiting), and the normal salt-water balance contrarily to what is observed in water intoxication. The course was favourable after progressive conditioning.
Three main concepts came out of this prospective study of 103 cases (51 girls and 52 boys) of renal diabetes followed up from 1955 to 1975 and reviewed in 1984: contrary to what is still sometimes written, renal diabetes does not evolve to diabetes mellitus; renal diabetes does not seem to progress over the years; the mode of genetic transmission, when present, which is rare, is obscure but it seems to occur in a recessive rather than a dominant fashion.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
The authors report a new case of lactobezoar in a 45 day-old infant. Vomiting was the presenting sign. The diagnosis was made using radiologic examination of the gastro-intestinal tract. Medical treatment (digestive rest and parenteral rehydration) resulted in a favourable outcome. The factors favouring such a situation and its management are analysed.
Explore the source record for details and available documents.
Congenital cervical thymic cysts are a very rare cause of tumor of the neck in children. They can exceptionally induce a laryngeal compression. Actual identification of the tumor may sometimes be made only after surgery and histologic examination. Evolution is benign. Their existence seems to be related to the persistence of thymic remnants in the neck.
Explore the source record for details and available documents.
The authors report a case of diffuse angiomatosis of bone associated with focal epilepsy. This case confirms the usual absence of cerebromeningeal angiomatosis in cases with diffuse angiomatosis of bone and establishes a relationship between angioma of the dome of the skull and focal epilepsy.
Explore the source record for details and available documents.