[AA-amyloidosis in Muckle-Wells syndrome].
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Biomedical subjects
Publications and source records attributed to T N Ianushkevich.
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A SLE course has been analyzed retrospectively in pregnancy and after delivery for 34 patients (44 pregnancies). Aggravation of the disease was registered in 6 cases (13.5%). Only two of them received prophylactic corticosteroid maintenance. Two SLE patients with unaffected kidneys had no pregnancy-related SLE exacerbations though they developed nephropathy of the pregnant. After delivery the disease aggravated in 12 patients (27.3%). Five patients had moderate exacerbations within half a year after the delivery, seven patients developed severe exacerbations within 3-11 postnatal months. Five out of seven women presented nephrotic syndrome. One of the patients died of rapidly progressive glomerulonephritis. Three women had renal damage after the delivery as initial condition. SLE exacerbations were reported more frequently in young patients (under 30, in 15 of 31 pregnancies) than in older ones (over 30, in 3 of 13 pregnancies). Postnatal SLE aggravations can be prevented by large doses of prednisolone (20-30 mg/day) administered for 2-3 weeks.
Renal function was assessed in 8 males at reproductive age suffering from lupus nephritis (LN). Normal renal function was registered in 5 patients, hypofunction in 3 males. Endocrinological examinations (evaluation of secondary sexual characters, of hypophyseal and sex hormone profile, sexological questionnaires, spermograms) failed to distinguish significant differences between SLE males and renal patients without SLE suffering from hypogonadism (13 males with uremic hypogonadism, 10 males with azoospermia induced by cytostatics). Endocrinological changes revealed may result from uremia and immunodepressive therapy. In 5 patients these abnormalities were corrected by parlodel and zinc sulfate. The authors came to the conclusion that feminization is not universal in LN males.
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The authors analyze the 10-year experience gained with the use of steroid pulse-therapy for the gravest forms of lupus nephritis--rapid-progressing lupus nephritis and active lupus nephritis associated with the nephrotic syndrome. Ultrahigh doses of prednisolone and methylprednisolone (1000 mg i. v. for 3 days) was monotherapy or as a constituent part of multimodality treatment were given to 30 patients including 27 women and 3 men aged 18 to 48 years. Of these, 12 patients had rapid-progressing lupus nephritis and 18 active lupus nephritis. The short-term treatment results were estimated after 1 to 3 months, whereas the long-term ones after 12 months to 9 years. Analysis of the treatment results allows the following conclusions to be drawn: the use of steroid pulse-therapy was monotherapy is only justified in patients suffering from active lupus nephritis with the nephrotic syndrome without renal failure and only at the disease debut. In rapid-progressing lupus nephritis and long active lupus nephritis with the phenomena of renal failure, the positive effect can only be attained after combination of steroid pulse therapy and high doses of prednisolone per os or long intake of cytostatics per os or in the form of cytostatic pulses.
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