Oligomeganephronia in an Adult Presenting With Features of Chronic Kidney Disease

Abstract

We report the case of a 29-year-old man with oligomeganephronia (OMN) who presented with symptoms of chronic kidney disease (CKD). Radiological and ultrasound images showed bilateral mildly small and uneven kidneys. Right renal biopsy was performed. Under light microscopy, the number of glomeruli in histological samples was decreased and the remaining glomeruli were markedly enlarged without mesangial proliferation. The obsolescent glomeruli had findings compatible with segmental sclerosis. Tubules were dilated and enlarged, and there was a presumptive primitive duct enclosed by a smooth muscle collar. Dysplastic changes in the biopsy specimen suggested that the cause of the patient's small kidneys was a congenital anomaly rather than acquired kidney disease. OMN is one of the probable causes of CKD in adults. Angiotensin type II receptor blockers may be an effective treatment for focal segmental glomerulosclerosis due to OMN.

Diagnostic Image of Oligomeganephronia - A temporary Caseillustrated Review

“…Survival beyond puberty without renal transplantation in OMN patients is considered unusual [8]. In the last decade, however, a series of cases with late onset of OMN have been described [2,6,10,11,18], contrary to the previous statement.…”

“…All patients eventually develop progressive renal insufficiency, which leads to complete insufficiency between within a few months from birth to the beginning-middle of adolescence [4][5][6][7][8][9][10][11][12][13][14]. In recent years there are increasing reports of cases with late onset of symptoms -between 20 and 36 years of age [2,[10][11][12][13][14][15][16][17][18][19][20]. Final diagnosis is established on the basis of histology -missing corticomedullary borders, reduced number of cortical pyramids and calyces (up to 6), fewer nephrons greatly increased in size (two to three times bigger), frequent hialinization of said nephrons [4,9,19,20].…”