Clinical and pathological features of patients with Oligomeganephronia

Objective:To investigate the clinical manifestation and pathological characteristics of Oligomeganephronia(OMN). Methodology:From June 2003 to July 2008,eight patients with OMN were reviewed retrospectively.The clinical and pathological feature of those patients has been analyzed.Eight age-and sex-matched patients with microhematuria served as control group.The number,diameter,area and volume of glomerulus were measured calculated in both two groups. Results:(1) Patients with OMN including 7 male and 1 female were characterized by early-onset with age from 5 to 27.All of them were negative family history of kidney disease.(2)Proteinuria was obviousely noted as the nonnephrotic range in all patients,with negative hematuria.There were 2 patients with hypertension and 6 with impaired renal function.The positive ratios of urine NAG and urine osmolarity were 100% and 75.0%.(3)Histological observation: the glomerular numbers in patients with OMN were decreased significantly compared with that in control group(P0.01).The average volume of glomerulus in OMN was 2.93 times lager than that in control group.The global or segmental scleroisis was noted in all patients.The tubluer hypertrophy and interstitial fibrosis were found in 87.5% and 75% cases. Conclusion:OMN is renal hypoplasia with early-onset-age and negative family history.Proteinuria,chronic renal insufficiency and tubular dysfunction were main clinical manifestations.The histological characteristics was prominent decrease in number and augmentation in volume of glomerulus,combined with tubluer hypertrophy and interstitial fibrosis.