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Biomedical subjects

J J Benichou

Publications and source records attributed to J J Benichou.

At least 19 recordsLinked to original sources
PubMed1991

[Diabetes mellitus, diabetes insipidus, optic atrophy and deafness].

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.

Adolescent↗
PubMed1988

[Isolated and transient hyperphosphatasemia in young children].

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.

Alkaline Phosphatase↗
PubMed1987

[Acquired polydipsia in infants].

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.

Conditioning, Psychological↗
PubMed1985

[Lactobezoar in an infant].

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.

Animals↗
PubMed1985

[Congenital cervical thymic cyst].

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.

Child↗
PubMed1984

[Disseminated bone angiomatosis and focal epilepsy].

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.

Angiomatosis↗
PubMed1984

[Shwachman's syndrome with neonatal thoracic manifestations].

The authors report a case of Shwachman's syndrome in whom neonatal respiratory distress was the initial manifestation. Metaphyseal dysplasia, which was severe at birth, progressively disappeared. By the age of 10 years, radiologic anomalies persist only in the pelvis.

Agranulocytosis↗
PubMed1982

[Perioperative management of 98 cases of type C esophageal atresia (1970-1980) ].

We report our experience about 98 cases of type C esophageal atresia admitted to our unit from 1970 to 1980. 78.6% underwent successful anastomosis; 65.3% were considered at high risk because of delayed diagnosis (34.6%), low weight (22.4%) or associated malformations (15.3%). These latest two were especially responsible for mortality. Our results are encouraging and stress the need for a high quality of surgical management, for a systematic detection of this malformation and for a delayed surgical procedure allowing a better preoperative care.

Esophageal Atresia↗
PubMed1981

[Present views on Kawasaki's syndrome (author's transl)].

Recent findings seem to confirm that the pathophysiological mechanism responsible for Kawasaki's syndrome is of immunological origin. Cardiac involvement (coronary aneurysm/thrombosis) is not uncommon, and its influence on prognosis remains a cause for concern. Improved diagnostic (ultrasonography) and therapeutic approaches, together with prolonged surveillance of the patients, appear to have reduced the death rate.

Child, Preschool↗