C7 deficiency, abnormal platelet aggregation, and rheumatoid arthritis.
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Biomedical subjects
Publications and source records attributed to D Bontoux.
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Albuminemia, calcemia, phosphoremia and alcaline phosphatasemia were measured in three groups of 52 subjects each : rheumatoid arthrits, inflammatory rheumatisms other than rheumatoid arthritis and lumbarthrosics serving as a reference group. Calcemia and albuminemia were significantly lower in patients suffering from rheumatoid arthritis, whose calcemia corrected in relation to albuminemia is, on the other hand, normal : the increase in corrected calcemia pointed out by Kennedy, was not noted. Corrected calcemia was also normal in ankylosing spondylitis, but it was significantly higher in polymyalgia rheumatica. Phosphoremia was shown to be normal but alkaline phosphatases were higher than normal in the three groups.
Mac Duffie's syndrome includes a hypocomplementaemic allergic vasculitis with essentially cutaneous and articular manifestations. Its pathogenesis, still incompletely elucidated, involves the precipitation of immune complexes in the walls of the all vessels. The problem remains as to whether the alteration in the complement system is merely a reflection of this formation of immune complexes, or if it is primary, favourising the chronic infections which produce such complexes. The significance of the presence of low molecular weight precipitins reacting with C1g in the serum of a number of patients remains uncertain. The case reported, with its new clinical features, does not provide an answer to these questions but emphasises the possible role of chronic infections in the origin of this syndrome.
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The authors report their experience of 61 injections of osmic acid into the knee during the course of rheumatoid polyarthritis. The results stated to be very good and good, are classified by the disappearance of pain, swelling and stiffness, or a improvement in any one of these criteria. As observed in 67% of cases at the third month the result following changed with time. While these favourable results persisted into the fifth month, there was a remarkable maintenance of them to the 30th month in those patients who it was able to follow so long. The most distinct and lasting results were seen in those joints which showed the least radiological change. Tolerance of the injection of osmic acid was satisfactory apart from several painful reactions which improved in the 24 hours following injection. Two cases of thrombo-phlebitis were seen and one case of rupture of the synovium. If the results obtained with osmic acid appear less impressive than those obtained with isotopes, the simplicity of the method attracts attention as it needs no complicated equipment and is applicable to young invalids.
From September 1975 to September 1976, eleven patients suffering from rheumatoid arthritis in whom gold salts were either inactive or contraindicated were treated with levamisole. Treatment was interrupted in one case because of an allergic rash and in one as a precaution. There was an improvement in six, taking the form of a decrease in clinical symptoms and signs and a slowing of sedimentation rate. Certain immunitary changes were seen, in the sense of a reinforcement of humoural and cellular immunity. Fear of sudden agranulocytosis should lead to caution in further clinical trials.
The authors analysed thirty cases of hyperparathyroidism seen between 1969 and 1975 at Centre Hospitalier Universitaire at Poitiers. They noted the similarity of their observations with those which have generally been made in this condition. The only peculiarities of this study were a low incidence of calculi and a particularly high incidence of chance discovery by routine blood calcium measurement. The diagnostic value of blood calcium levels and blood citrate levels is confirmed in the same way as the advantage of a quantitative bone biopsy even although a few false positives limit the specificty of the test.
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The authors report a series of 9 cases of meningoradiculitis after tick bites and compare them with 56 other cases in the literature. Clinically, the bite is followed by a free interval, then more or less extensive local erythema, pain then paralysis is undoubtedly the most typical presentation. Uni- or bilateral paralysis of the 7th cranial nerve was found in more than 50% of cases. Erythema may exceptionally be absent. There may be forms with pain alone. Finally, there may sometimes be pyramidal signs or signs of brain irritation. As concerns the course, one should note the absence of respiratory complications, and although there are usually no or minimal sequelae, one should note the slowness of the recovery in certain paralytic cases. In the laboratory, pleocytosis is constantly found in the C.S.F. As concerns physiopathology, there are 3 theories, virus, allergic and toxic.
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The authors report osteomalacia in 3 cases of epilepsy and one case of coronary heart disease treated with phenobarbitone, either alone or associated with other anticonvulsants. There were clinical signs in all cases and typical radiological signs in 3 cases, a characteristic laboratory syndrome in 4 cases. In the 3 cases where it was estimated, serum levels of parathormone were high. Finally, in 3 cases where it was measured, daily urinary excretion of glucaric D acid was increased. The bony histological signs studied in 3 cases, were similar to those in deficiency osteomalacia. A study of Ca45 metabolism in one case, showed the characteristic changes found in osteomalacia. Finally, a study of the metabolism of tritiated vitamin D, or tritiated 25 OH CC, carried out in 3 cases, gave 3 different patterns; only one of them was characteristic of enzyme induction under the dependency of anticonvulsant. Started in 2 cases, treatment with 125 OH2CC, brought about a rapid fall in blood PTH levels which then rose again before falling progressively in one case, under treatment with 25 OH CC. The bony histological signs of hyperparathyroidism then regressed whilst serum PTH levels remained high. Phosphorous and calcium balance improved in only one case. Treatment with 25 OH CC in high dosage brought about clinical, radiological and laboratory cure of osteomalacia in both cases, reducing the frequency of fits in the epileptic patient.
The authors report the case of a 72 year old man admitted to hospital for thoracic pain, who was found to have an Igd myeloma of lambda type. The diagnosis, suspected, in view of anemia, raised sedimentation rate and abnormal electrophoresis of serum proteins, was confirmed by marrow examination after sternal aspiration and by the use of a specific anti-serum during immuno-electrophoresis and on Ouchteriony medium. There were radiological lesions of diffuse, decalcifying myelomatous type. The course was characterised by : 1) a constant tendency to anemia, then preterminal thrombocytopenia, whereas the leukopenia remained moderate. 2) The secondary onset of Bence-Jones proteinuria and mild albuminuria with the aggravation of pre-existing renal failure. 3) Repeated attacks of infection and the intermittent onset of mental confusion, the etiology of which was not clear. Death occurred ten months later. No autopsy was carried out. In the light of this case, the authors review the world literature and the special characteristics of IgD myeloma.
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The authors report two cases of multiple myeloma which were typical both clinically and in the laboratory but XRay examination, on the other hand, showed appearances of osteocondensation. In the first case, XRay showed both lesions of osteolysis in the cranial vault and homogeneous condensation of D11 and L1, together with the left iliac crest. In the other case, there was osteolysis of the acetabulum together with areas of osteocondensation distributed throughout the pelvis and upper ends of the femurs, with two areas of annular fibrosis circumscribing the area of osteolysis, finally, homogeneous condensation of the skull. In both cases, bone biopsy confirmed the diagnosis of multiple myeloma showing both osteofibrosis and plasma cell infiltration of the bone marrow. This also permitted the authors to note the absence of any myelofibrosis or metamorphic neo-osteogenesis. Illustrated by these two cases, condensing multiple myeloma is a rare entity, the special clinical characteristics of which reside in its fairly frequent coexistence with peripheral neuritis which is probably similar to a para-neoplastic syndrome. The radiological appearances are mainly of four types: 1) Focal areas of bony condensation. 2) Areas of annular fibrosis circumscribing osteolysis. 3) Appearances of radial spicules, or 4) Osteocondensation extending to a fairly large part of the skeleton. The laboratory signs are identical with those in other types of multiple myeloma with a few exceptions, such as, rareness of hypercalcemia, more frequent tendency to hypocalcemia, rise in alkaline phosphatase, in a few cases. Bone biopsy confirms the diagnosis. The osteofibrosis resulted here from thickening of the osteoid seams by laying down of successive layers of bony substance, irregularly calcified and, also secondarily, metamorphic neo-osteogenesis in a few rare cases which also included myelofibrosis.
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Immunoglobulin D is the fourth class of immunoglobulins secreted by the plasma cells and also, in 5% of lymphocytes, this is a surface immunoglobulin. Its serum levels are about 0.03 mg/ml, due to low secretion, intersititial diffusion and intense catabolism. IgD migrates on electrophoresis in the beta-gamma zone, on immuno-electrophoresis, in the beta2 zone. Only one disulphide bridge links the delta-chains together. The light chains, are, in most cases, lambda chain. Studies of the antigenic characteristics of the delta-chains are in progress. IgD does not take up complement, but has an antibody function. 37% of healthy subjects have anti IgD antibodies. Only 4 cases of monoclonal IgD gammopathy without malignant myeloma, have been reported. Anto-antibodies of IgD nature have been described in various connective tissue diseases or auto-immune processes.