ANCA (Antineutrophil cytoplasmic antibody) positive Glomerulonephritis in Children 1800

Autor: Jacques Lemire, William R. Griswold, Henry F. Krous, Reznik Vm, D Franzon
Rok vydání: 1998
Předmět:
Zdroj: Pediatric Research. 43:307-307
ISSN: 1530-0447
0031-3998
DOI: 10.1203/00006450-199804001-01823
Popis: ANCA (Antineutrophil cytoplasmic antibody) positive glomerulonephritis (GN) is uncommon in children. We report the clinical and pathologic findings of 7 children with ANCA positive GN. ANCA titers were performed on serum using either indirect immunofluorescence (IF) or ELISA. Mean age at presentation was 10.4 years (range 5-13 years). Symptoms at presentation included fever, malaise, abdominal pain, scleritis, arthritis and conjunctivitis. Only one patient had gross hematuria and none had edema. Initial laboratory tests showed microhematuria and proteinuria in all patients. Anemia was prominent with a mean hematocrit of 24%; all patients had a high ESR (mean value 90 mm/hr). Elevated serum creatinine and reduced GFR were seen in 4 of the 7 patients. Proliferative GN was seen on biopsy in each case; four of the biopsies showed prominent crescent formation. Four of the 7 patients had pauci-immune immunofluorescence findings; granular deposits of Ig and complement were seen in 2 cases. Extrarenal manifestations were common including sinusitis, pulmonary hemorrhage and pancreatitis. Two patients had positive ANCA-C; these patients had clinical findings consistent Wegener's Granulomatosis. Five patients had positive ANCA-P. Four of the 7 patients have developed ESRD; each of these patients has received a renal transplant. Mean post transplant follow up is now 27 months. Average serum creatinine is 1.1 mg/dl (range 1.1 - 1.24) and estimated GFR (Schwartz equation) is 69 ml/minute/1.7M2. Mean urine protein-creatinine ratio is 0.24 (range 0.18-0.27). Pretransplant geometric mean ANCA titer was 1/40; post transplant titer was 1/10 (p
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