Biomedical subjects
Publications and source records attributed to J Panouse-Perrin.
In a search for potential immunomodulating agents novel pyrrolo[1,2-c]pyrimidines were synthesized and their structures elucidated by spectroscopic means. Unfortunately, most of them were cytotoxic and devoid of effects on T lymphocyte lymphoblastic transformation. Furthermore, they were inactive in the locomotor activity test in mice.
A case of hereditary angioedema secondary to C1 esterase inhibitor deficiency associated with lupus-like nephritis is reported. The patient was initially treated with both corticosteroids and danazol and subsequently had Guillain-Barré syndrome together with appearance of circulating immune complexes and an increase in total complement and C1q, C3, C4, B, and C1 esterase inhibitor levels. Guillain-Barré syndrome might be secondary to danazol therapy since this drug could increase both circulating immune complex production and complement synthesis, thereby providing additional substrate for the underlying immune complex disease. Normalization of complement might therefore be hazardous in lupus with underlying complement deficiency states.
About 20 cases of acquired C1 esterase inhibitor deficiency have been reported in association with malignant lymphomas. We describe 3 such patients. The 3 patients studied were asymptomatic and had low C1q level. Danazol administration resulted in an increase of C1 esterase inhibitor in 2 patients. The complement activation in acquired C1 esterase inhibitor deficiency could be explained by interaction with pathological cells of the spleen, the blood or the bone-marrow. The mechanism of decrease of C1 INH is discussed.
Congenital deficit of the inhibitor of C1 esterase (C1 INH) usually presents by oedema of the lower limbs, abdomen and glottis (sometimes lethal), which explains its clinical denomination of angioneurotic oedema. The association of this condition with disseminated lupus erythematosis has been reported in 4 cases and with discoid lupus in 4 cases. Antinuclear factors were found in all these cases but there were only two documented cases of nephropathy (one diffuse proliferative glomerulonephritis and one local glomerulonephritis). The association of a deficit of C1 INH and membrano-proliferative glomerulonephritis has only been reported in 2 cases (one lobular glomerulonephritis and one glomerulonephritis with dense basal membrane deposits). Our case had C1 INH deficiency and proliferative lupic glomerulonephritis in the absence of other clinical and immunological signs of DLE. Nephropathy was not looked for in 9 cases of association of C1 INH deficiency and C3-shearing autoantibody (C3 NEF). A common genetic mechanism for these associations seems very improbable. The aptitude of patients with C1 INH deficiency to synthesise autoantibodies under the influence of infections factors, for example, could explain the higher incidence of lupus and glomerulopathies in these patients.
Deficiency of C1 esterase inhibitor (C1-INH) was demonstrated in 7 of 22 subjects belonging to the same French family. Immune complex glomerulonephritis without lupic symptoms was discovered in one of the C1-INH deficient subjects, and in a girl of the same family the same deficiency was associated with an insulin-dependent diabetes of sudden onset. The pathophysiological consequences of complement deficiency resulting from the lack of C1 esterase inhibitor are discussed.
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For the last 25 years, numerous attempts have been made to isolate the HBV agent responsible for hepatitis B by means of cultures 'in vitro'. We have undertaken longterm cultures of children's hepatic tissue (C.H.), conjunctive tissue (human adult H.A.F. and human embryonic fibroblasts H.E.F.) and KB cells; these were put in the presence of 7 sera HB + rich in Dane particles. These cells were trypsinized twice a week for almost 3 months and did not present any cytopathogenic effects. Electromicroscopy revealed, 15 days after infection, the presence of icosahedral particles (25 to 27 nm in diameter), free or in dense clusters, but more often empty (20 nm in diameter). These structures seemed to be made up of an assembly of capsomers approximately 5 nm in diameter, joined together in fours to form a ridge. Older cultures revealed clusters of icosahedrons some of which degenerated spontaneously; others were surrounded by proteinic structure having a fringed aspect. Certain rare particles of 35 to 45 nm in diameter are similar to full Dane particles. EID immunological results were positive in the case of sera of patients convalescent from hepatitis B, containing anti-HBc antibodies, on C.H. cells the 27th and 40th days, and negative with anti-HBs antibodies. By immunofluorescence we observed 12 to 20 days after infection of the cells, a clear fluorescence at first nuclear, then essentially cytoplasmic, by means of fluorescent anti-HBc sera of human or animal origin. With the fluorescent anti-HBs antibodies, the reaction is weak and solely cytoplasmic although in DRI, with H.E.F. and KB cells, we obtained from time to time weakly positive results in HBs. The relations between the morphological structures and the immunological results observed are discussed.
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