IgA nephropathy (Berger's disease) - a clinicopathologic study in children

Clinicopathologic features and follow up of 21 children with IgA nephropathy (Berger's disease) is discussed. The disease predominantly affected males. Although no definite prediction for any age group was observed, a majority (71.5%) of patient were six years or older. Macroscopic hematuria was the presenting feature in 71.5%, while as 28.5% had microscopic hematuria. Isolated proteinuria was not observed in any. 81% of the patients continue to have recurrent episodes of macroscopic hematuria, and 76% of the patients have microscopic hematuria during the symptom free intervals. Hypertension, azotemia and nephrotic syndrome were absent in all patients at the time of onset of the disease, and have not developed in any patient during follow up (mean 5.4 yrs). Serum IgA level elevated in only one (7.7%) of the thirteen patients in whom this was tested, and did not appear to be a diagnostic test of IgA nephropathy in children. The histologic features of renal biopsy did not correlate with known duration of the disease, extent of proteinuria at the time of biopsy or the degree of IgA deposits in the renal tissues. During childhood. IgA nephropathy appears to have an excellent prognosis, but longterm outlook can be projected only by following these children into adulthood.