Oligomeganephronia in an adult without end stage renal failure

Abstract

A 23 year-old male was investigated for hypertension, moderate renal insufficiency, persistent proteinuria and bilateral small kidneys. The renal pathological features were diagnostic with greatly enlarged glomeruli (the mean diameter was 325 μm, which was approximately two times larger than normal glomeruli), indicating oligomeganephronia (OMN). He also showed malrotated kidneys, expanded extrarenal pelvis, and hearing loss. Thus, these clinical and pathological features aided in diagnosing the renal disorder as OMN. This is a very rare case of OMN, which did not advance to end-stage renal failure as an adult. We believe that multiple anomalies might be suggestive findings of OMN in patients, such as renal insufficiency, persistent proteinuria, and bilateral small kidneys.

Secondary Focal Segmental Glomerulosclerosis in an Adolescent Born with a Very Low Birth Weight

“…The most frequently affected patients develop progressive renal insufficiency, leading to end-stage renal failure (ESRF), either in early childhood or early-mid adolescence. Adult OMN has only been reported in a few patients, and those who do not advance to ESRF present with secondary FSGS (17,18). Clinical and renal pathological features of such patients appear to be similar to our case.…”

Oligomeganephronia in an Adult Presenting With Features of Chronic Kidney Disease

“…We have identified only six reported adult cases [3][4][5] confirmed by renal biopsy, including the case presented here (Table 1). These six cases included five males and one female with a mean age of 28 years (range 19 -36 years).…”

“…In patients with glomerulomegaly and FSGS, angiotensin inhibitors, such as angiotensin converting enzyme inhibitors and ARB, decrease proteinuria and slow the rate of progression to end stage renal disease [17]. ARB might be effective in reducing proteinuria in FSGS due to OMN [4].…”

Diagnostic Image of Oligomeganephronia - A temporary Caseillustrated Review

“…Oligomeganephronia (OMN) is a rare condition, the awareness of which has been rising in recent years [1][2][3][4][5][6][7]. It was first described in 1962 by Royer, Habib et al [8] as a peculiar form of hypoplastic kindey.…”

“…This entity is subsequently called (congenital) oligomeganephronia [3,5]. 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].…”