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  • 1
    ISSN: 1432-198X
    Keywords: Glomerulonephritis ; Factor H ; Complement ; β-1 H globulin ; Collagen type III glomerulopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A non-immune complex-mediated glomerulonephritis associated with persistent hypocomplementemia occurred in a young boy. Measurement of complement components revealed complete factor H deficiency, inherited as an autosomal recessive trait. Evaluation of the renal lesion revealed extensive deposition of type III collagen suggestive of collagen type III glomerulopathy, a recently identified cause of chronic renal insufficiency in children and adults. This report represents the first association of inherited factor H deficiency with collagen type III glomerulopathy.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-198X
    Keywords: Key words: Delayed acute renal failure ; Post-transplant period ; Unexplained etiology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. This report describes six young children (5 male) who developed delayed acute renal failure (DARF) in the early post-kidney-transplant (Tx) period in the absence of acute rejection (AR) or other diagnosable conditions. These young children, aged 16.5 ± 3.1 (12–21) months [mean ± SD, (range)] and weighing 8.5 ± 1.7 (7.1 – 11.4) kg received a primary renal Tx (5 living-related donor, 1 cadaver) between 1984 and 1992. Immunosuppression included prednisone, azathioprine, and Minnesota antilymphocyte globulin (MALG, n = 5); one patient received cyclosporine and no MALG. Initially, all patients had good urine output (UO). They became systemically ill and abruptly developed diminished UO on post-operative day (POD) 6.5 ± 1 (4 – 8). DARF was accompanied by fever (39.1 – 40.4°C, n = 6), thrombocytopenia (platelets 〈100,000/mm3, n = 6), leukocytosis, or leukopenia (white cell count 〉20,000/mm3, n = 4 or 〈1,000/mm3, n = 1). Four patients had diarrhea. Three had ascites and one was surgically explored for suspected urinary leak. None showed significant urinary obstruction by renal ultrasound. Renograms showed intact blood flow. Renal biopsy showed tubular ectasia (n = 6), vascular congestion (n = 5), focal glomerular endothelial swelling (n = 4), and capillary thrombi (n = 3). None showed AR. Five patients required dialysis for 11 ± 4 (7 – 15) days. All patients survived. One patient, treated for suspected AR with the monoclonal antibody OKT3, developed shock and lost her graft on POD 12 due to vascular thrombosis. Renal functional recovery in the remaining five patients took 14 ± 5 (6 – 20) days and their serum creatinine at discharge was 0.7 ± 0.5 (0.3 – 1.6) mg/dl. We report DARF from undetermined etiology occurring in the first 2 weeks of renal Tx in young children. Treatment is supportive care including dialysis. Recognition of this complication will help avoid risky investigations or unnecessary treatment for rejection.
    Type of Medium: Electronic Resource
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