Teaching neurology in Germany.
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Biomedical subjects
Publications and source records attributed to R Janzen.
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A case of Whipple's disease is reported in detail. Pathological changes of duodenal mucosa are demonstrated and the diagnostic importance of duodenoscopy is stressed.
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Drugs that interfere with the immune response in vitro, such as azathioprine (AZ), have been used extensively since 1964 in clinical therapeutic trials of autoimmune diseases. However, few adequately controlled studies are available concerning the concurrent effect of AZ on the immune and clinical responses to treatment of rheumatoid arthritis (RA). Thirty patients suffering from classical seropositive RA received AZ (1.5 - 2.0 mg/kg/day) and placebo in a controlled clinical cross-over study for two 12-week periods. Other treatments were kept constant throughout the entire 6 months. In terms of the clinical responses of joint count and grip strength patients receiving AZ improved markedly, in contrast to the placebo group. After 2 months, joint scanning revealed no progress of the disease in patients undergoing AZ treatment. Corresponding with the remarkably beneficial clinical effect of AZ, a significant drop in immunoglobulins was observed. However, since AZ failed to suppress in vivo specific antibody synthesis in RA, the question remains as to whether this drug actually does interfere with the autoimmunogenesis of the disease.
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The case of a 27-year-old woman with pancytopenia, revealing acute monocytic leukaemia and haemolytic anaemia, is described in detail. The underlying cause for the red cell destruction was found to be a pyruvate kinase (PK) instability. Further investigation into three generations of her family (n = 12) disclosed a hereditary PK instability. This was proven by performing biochemical studies to elucidate mutants representing a structurally defective enzyme. Since conversions of pancytopenia with acquired red cell enzyme deficiency into leukaemia have been described, our observation emphasizes that hereditary red cell enzymopathy might also be associated with adult acute leukaemia.
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