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Clin Exp Nephrol (2012) 16:325328
DOI 10.1007/s10157-011-0560-8
CASE REPORT
Oligomeganephronia in an adult without end stage renal failure
Yoshinobu Fuke Seiichiro Hemmi
Mamiko Kajiwara Minako Yabuki
Takayuki Fujita Masayoshi Soma
Received: 13 August 2011 / Accepted: 27 October 2011 / Published online: 25 November 2011 Japanese Society of Nephrology 2011
Abstract A 23 year-old male was investigated for hypertension, moderate renal insufciency, persistent proteinuria and bilateral small kidneys. The renal pathological features were diagnostic with greatly enlarged glomeruli (the mean diameter was 325 lm, 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 ndings of OMN in patients, such as renal insufciency, persistent proteinuria, and bilateral small kidneys.
Keywords Oligomeganephronia Adult
Multiple anomalies End-stage-renal-failure (ESRF)
Introduction
Oligomeganephronia (OMN) is a congenital anomaly of bilateral renal hypoplasia, histopathologically featuring a striking reduction in the number of nephrons and markedly enlarged glomeruli [14]. The main manifestations of early-stage OMN are polydipsia, polyuria and proteinuria [3]. This is followed in affected patients such as those in
early-mid adolescence by progressive renal insufciency, and ultimately to end-stage renal failure (ESRF) [2, 3]. Here, we describe a 23 year-old male, who presented with hypertension, persistent proteinuria and moderate renal insufciency.
Case report
A 23 year-old male was referred for hypertension, moderate renal insufciency and persistent proteinuria. Proteinuria had been detected on routine examinations at the age of 16. He was a full term baby of normal weight (3,200 g). His growth and development were normal. There was no family history of renal diseases apart from one kidney stone in the elder brother.
Physical examination showed the following: height 163.0 cm, weight 66.3 kg, blood pressure 160/94 mmHg, pulse rate 63 beats/min, temperature 36.5C. He had a complete hearing loss (congenital) in the left ear. Eyesight tests showed 1.2 in the right eye but 0.2 in the left eye, although there was no abnormal nding on bilateral fundus examinations. There was no abnormality found in other...