[Should we defend french? A plea for bilingualism].
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Biomedical subjects
Publications and source records attributed to P Mozziconacci.
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In 40 children suffering from a form of chronic juvenile arthritis (CJA), the authors found encouraging results after immuno-suppressive treatment. Indeed, major corticotherapy, often necessary in these forms, could be stopped in nearly half the cases. The immediate improvement in the clinical signs of the disease was very clear and the signs of corticoid intoxication regressed, and in particular growth was normally resumed in many of the children. While the immediate infectious and hematologic consequences are generally benign, the occurrence of malignant hemopathies at a distance, seen in 3 cases, mean that use of this type of therapy should be totally reconsidered during CJA. The authors feel that because of this grave oncogenic risk, the immunosuppressive treatments should not be reserved merely for forms that involves the vital prognosis in the more or less short run.
This study deals with 82 cases of systemic-type chronic juvenile arthritis, 27 of which were followed up over a period of more than 7 years, the development of inflammation of the disease, the extnet of destructive bone lesions and the final functional prognosis. Their conclusion is that, contrary to the traditional notion, this condition often continues to develop beyond adolescence and it often brings about deep-set deterioration of the bones (especially at the wrist and the hip), which has a heavy bearing on the functional prognosis.
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We report in a five month old baby a case of the cutaneomucosal and lymph node syndrome described by Kawasaki, remarkable for the onset of multiple aneurysms involving the coronary and the limb arteries. This syndrome has rarely been observed except in Japan where it seems frequent. Possibly is benign forms, without heart complications, producing a picture of febrile erythema of viral type, have not received sufficient attention. The relationship between Kawasaki's disease and periarteritis nodosa in infants is then discussed. The clinical and pathological existence of the fatal forms seems undoubted, but the generally benign course of Kawasaki's disease contrasts with the severe course of polyarteritis nodosa in infants. It is possible that the usually benign forms of Kawasaki's disease represent only the initial stage of periarteritis nodosa in infants, from which they may recover, and we have only identified until now the severe forms which are rapidly fatal. The etiology of Kawasaki's syndrome, like periateritis nodosa remains unknown.
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A case of systemic hyalinosis is reported and the main features of this unusual affection are quoted. The systemic hyalinosis is a syndrome exhibiting the following symptoms : a distressing arthogryposis syndrome, a diffusely stiff skin, some subcutaneous and submucosal fibrotic nodular lymps, repeated suppurative original infections and a facial dysmorphy. Biopsies of nodules show an active proliferation of the connective tissue associated with some peculiar hyalin changes of the collagen fibres. Most cases including the present one die in the first years. The pathogenesis is presently unknown. The systemic hyalinosis seems to be an autosomic recessive heritable disorder and is reasonably settled in the large group of the hereditary diseases of the connective tissue.
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