[Hydrops of the gallbladder in children].
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Biomedical subjects
Publications and source records attributed to B Labrune.
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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.
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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.
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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.
In a 10-year-old girl admitted to hospital for polyuria and polydipsia, the central nervous system origin of the symptoms was demonstrated by low antidiuretic hormone levels (inferior to 1 pg/ml) and reduction of diuresis after administration of 1-deamino-8D-arginine-vasopressin (DDAVP). A study of the girl's family history showed that the disease was hereditary and autosomal dominant. Intranasal instillations of DDAVP twice daily constitute the best known treatment of the condition.
Two children (4 2/12 and 7 years old, respectively) presented with features of recurrent multifocal periostosis. Radiologic findings consist of periosteal appositions, preferentially located on the long bones and are associated with general and local inflammatory signs. No causal agent can be identified. Complete clinical recovery occurs spontaneously sometimes after several relapses. The relationship between these patients' history and Caffey's syndrome is discussed.
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A girl, who died at 25 days of age, was found to have a partial monosomy due to a 11q23 leads to 11qter deletion. The main clinical findings were trigonocephay, facial dysmorphia, and congenital heart disease. A review of developmental and dysmorphic features of the seventeen recognized cases is presented.
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In a six-year-old girl, suffering from an auto-immune haemolytic anaemia, routine radiological examination revealed the presence of a mediastinal tumour which was removed surgically and proved to be a multiple tissue polycystic dysembryoma. Haemolysis and signs of anti-erythrocyte auto-immunisation disappeared after the operation and total and stable cure obtained with a follow-up of 15 months. The target antigen of the anti-erythrocyte autoantibody could not be found within the tumour. However, the latter contained lymphoid tissue and a considerable quantity of antibody. Although indirect, these findings offer arguments in favour of the secretion of autoantibodies by the dysembryoma.