Biomedical subjects
Publications and source records attributed to R Jean.
The distribution of 29 HLA antigens has been compared in 60 unrelated children presenting a dust allergy and in 300 healthy controls. We observed an increased frequency for HLA-Aw19 and HLA-B5 in patients. Yet, the differences are not very significant and there is probably no association between one HLA gene and the dust allergy.
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Two children presented with an acute mycoplasma infection associated with a significant increase of antistreptolysin level. A severe nephropathy occurred, rapidly resulting in renal failure with histologic lesions of membrano-proliferative glomerulonephritis. Both patients had persisting low complement levels with low C3 and normal C4. The relationship between mycoplasma and streptococcal infections and the abnormality of complement and the renal disease is discussed.
Report of a case of lymphocytic parotitis associated with important inflammatory syndrome, anti-salivary antibodies and rheumatoid factor. The study of the cases of literature suggests that it might be the mode of onset of a Sjögren's syndrome.
Case report of a 7 year old girl, anaemic since birth. The anaemia, of variable intensity, is associated with a moderate reticulocytosis. Bone marrow films, show a marked erythroblastosis with conspicuous morphological abnormalities and internuclear chromatin bridges. The erythrocinetic pattern is that of ineffective erythropoiesis. The morphologic features are those of Heimpel and Wendt's type I congenital dyserythropoiesis. The erythrocytes are lysed by some acidified sera, thus showing a membrane anomaly which is unusual in type I. However the characteristics of the lysis are different from those of type II (HEMPAS) erythrocytes.
Report of the cases of two infants presenting with lymphoid hyperplasia of the colon, occuring after intestinal obstruction associated with enterocolitis. In one case, it was histologically proved. These cases emphasize the problem of the real meaning of lymphoid hyperplasia of the colon, as well as the meaning of its relationships with enterocolitis. Evolution was benign and it seems that there is no peculiar symptomatology.
A complex syndrome was observed in two sisters. It associated important and dysharmonious dwarfism, craniofacial dysmorphy (blepharophimosis and microstomy), osteo-chondro dystrophy (vertebral column, pelvis, coxofemoral joints), oligophrenia and a muscular syndrome of myotonic type. The myotonic muscular syndrome is characterized by the prevalence at the face. Clinical and electric myotonia is clear. Voluntary muscular contractions induce prolonged myotonic responses. However there is no basal activity as observed in other cases. Curarization does not induce any disappearance of myotonic discharges. No specific histological change was shown: Schwartz-Jampel's muscular syndrome is not univocal; however it seems to depend on an abnormality of neuro-muscular activity, at the level of the end plate.
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