Dense deposit disease in children: prognostic value of clinical and pathologic indicators. The Southwest Pediatric Nephrology Study Group.

Abstract

Clinical and pathological features were examined in 16 children with dense deposit disease. The children ranged in age from 5 to 15 years (mean: 9.3 years). There were nine boys and seven girls. Semiquantitative grading of renal biopsy findings was performed in these patients and compared to clinical features at the time of presentation, and at the time of latest follow-up. Initial clinical features included hypertension and decreased glomerular filtration rate in 50% of patients, nephrotic syndrome in 69%, and gross hematuria in 73%. Serum C3 concentrations were low in nine of nine patients. All but one of the patients subsequently received steroid therapy, the dosage of which varied. Of the 16 patients, six developed progressive renal insufficiency, six have normal renal function after a period of 7 to 12 years, and four have normal renal function but have been followed for less than 6 years. When these different subgroups were compared, clinical and laboratory features were not helpful outcome indicators. By contrast, poor outcome was correlated with the following pathologic features: excessive prominence of glomerular lobules, severe mesangial hypercellularity and sclerosis, severe glomerular loop obliteration, and mesangial electron dense deposit alteration. We conclude that the course of dense deposit disease is variable and that certain pathologic features may be helpful in predicting clinical outcome. Whether alternate-day prednisone therapy may have been of benefit for the patients in this study is uncertain.