[Macroscopic hematuria caused by glomerulonephritis with mesangial IgA deposits in adults].
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Biomedical subjects
Publications and source records attributed to D Droz.
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We report the case of a renal transplantation performed with the kidney of an asymptomatic female carrier of Fabry's disease. The recipient, her daughter, had normal alpha-galactosidase levels. Eight years after transplantation, the characteristic lesions of the glomerular epithelial cells, noted as early as 11 days after transplantation, are unchanged on the successive biopsies. This observation suggests that 1) some heterozygotes (perhaps all of them) have glomerular changes, 2) the glomerular changes are not modified if the kidney is placed in a normal enzymatic "environment".
Renal biopsies were performed in 211 patients which 10 days of excretory urography or renal arteriogrpahy in which diatrizoate, iothalamate or ioxithalamate had been used. In 47 renal specimens, osmotic nephrosis of the proximal tubular cells was found. Previous renal function had been normal in 10 patients, moderately impaired in 19, and severely impaired in 18. Tubular atrophy and/or necrosis was associated with histological features in 29 of 47 patients. Diffuse osmotic nephrosis was more often found in patients biopsied soon after roentgenography and also with severe renal insufficiency, but was not necessarily associated with declining renal function. The mechanism(s) by which contrast media may induce osmotic nephrosis remains unclear.
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Adult Still's disease (ASD) is a rare systemic disorder characterized by fever, arthralgia, cutaneous rash, and lymphadenopathy, with high polymorphonuclear leucocytosis and low glycosylated ferritinaemia. Kidney involvement has been reported rarely. We present a patient with ASD who developed haemolytic uraemic syndrome (HUS). The 42-year-old patient was admitted for unexplained fever related to ASD according to Yamaguchi's classification criteria. As Still's disease was resistant to prednisone, high-dose intravenous immunoglobulins (IV Ig) were administered. During the follow-up the patient developed acute renal failure and non-immune haemolytic anaemia with high levels of antiphospholipid antibodies (IgG anticardiolipin antibodies and anti-beta2 glycoprotein 1 antibodies). Renal biopsy disclosed thrombotic microangiopathy (TMA) with arteriolar and glomerular involvement. Treatment with steroids and intravenous IV Ig was reinitiated but renal function worsened towards end-stage renal failure. In this case, we suggest that antiphospholipid antibodies could have promoted arteriolar and glomerular TMA. HUS may be the cause of acute renal failure in Still's disease.
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Extended development cycles put companies at risk through loss of sales, margins, market share, and credibility as innovators. The author suggests constructing early-stage models to provide a dress rehearsal for new products and help companies develop products in less time.
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