Outcome of antineutrophil cytoplasmic autoantibodies-positive glomerulonephritis and vasculitis in children: A single-center experience☆,☆☆,★
Section snippets
Patient Selection
A retrospective analysis of all children presenting to the University of Michigan Medical Center between July 1989 and November 1996 with a focal and necrotizing glomerulonephritis and a positive ANCA titer was performed. Seven patients met these criteria. Data are presented as the mean ± SEM.
Glomerulonephritis was defined as the presence of hematuria (250/ μl), proteinuria (≥100 mg/dl or ≥2+), or both on urinalysis with or without renal insufficiency or hypertension. Blood pressure,
Presentation
The mean age of the seven affected children was 13.0 ± 0.9 years (range 11 to 17 years). Five (71%) patients were female. Four (57%) patients were cANCA-positive, and three (43%) were pANCA-positive. Features at presentation included constitutional symptoms (100%), sinopulmonary symptoms (71% [5 of 7]), and arthralgias (57% [4 of 7]). Microscopic hematuria was present in 100% of patients; ≥2+ proteinuria and renal insufficiency were seen in 71% (5 of 7) of patients. Two patients had microscopic
DISCUSSION
The morbidity of pediatric ANCA-associated glomerulonephritis (13 patients treated at six different centers) is high.8, 9, 10, 11, 12 Renal insufficiency at presentation is an important determinant of long-term renal outcome. The mean presenting serum creatinine in our patients (2.9 ± 0.9 mg/dl [range 0.7 to 6.9 mg/dl]) was much better than that in the studies with poor renal outcomes (10.1 and 10.5 mg/dl, respectively.8, 11 This better renal function at presentation suggests that our patients
Acknowledgements
The authors thank W. Joseph McCune, MD, for his helpful discussion regarding the treatment of these children with ANCA-associated glomerulonephritis and vasculitis.
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Glomerulonephritis in Children
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2009, Pediatric Urology: Expert ConsultClinical and biological symptoms of glomerular diseases
2005, EMC - PediatrieAntineutrophil cytoplasmic antibodies and associated diseases: A review of the clinical and laboratory features
2000, Kidney InternationalCitation Excerpt :However, in children over the age of 7 and in adolescents, the features resemble adult disease, except that subglottic stenosis and nasal deformity are more common, and fewer cyclophosphamide-related malignancies ensue72. As in adults, early recognition and treatment are important to improve the renal outcome73. Pregnancy is uncommon in the age group affected by Wegener's granulomatosis, but may trigger the onset of disease and relapse74.
Glomerulonephritis
2005, Adolescent Medicine Clinics
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From the Division of Pediatric Nephrology, Department of Pediatrics, University of Michigan, Ann Arbor, and the Department of Pediatrics, University of Rochester, Rochester, New York.
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Reprint requests: Rudolph P. Valentini, MD, Children's Hospital of Michigan, Division of Nephrology, 3901 Beaubien Blvd., Detroit, MI 48201-2196.
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0022-3476/98/$5.00 + 0 9/21/83689