Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access
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Case Report

Volume 6, Number 8, August 2015, pages 362-366

Oligomeganephronia in an Adult Presenting With Features of Chronic Kidney Disease


Figure 1.
Figure 1. An intravenous pyelogram had revealed smaller (right) and small (left) kidneys without hydronephrosis. Size of renal pelvis and calices of bilateral kidneys seemed mildly small. Abdominal computed tomography showed uneven surface of smaller (right) and small (left) kidneys without apparent thinning of renal cortex.
Figure 2.
Figure 2. (a) An enlarged glomerulus with slightly hyperplastic juxtaglomerular apparatus but without proliferative changes or thickening of the glomerular basement membrane (periodic acid-methenamine silver, original magnification × 28). (b) An obsolescent glomerulus with thickened Bowman’s capsule, adhesion of tufts, crescent-like proliferation of epithelial cells, hyaline deposits (H), and remaining open tufts (periodic acid-methenamine silver, original magnification × 68). (c) Some tubules are atrophic with a thickened tubular basement membrane, while other tubules are relatively enlarged. In the center, a peculiar area contains a presumptive primitive duct (P) and numerous small vessels (periodic acid-methenamine silver, original magnification × 20). (d) A presumptive primitive duct enclosed by a smooth muscle collar (hematoxylin-eosin, original magnification × 80). (e) Epithelial cells of the presumptive primitive duct (P) are cytokeratin positive, as are other tubular epithelial cells (immunostain with Becton Dickinson-CAM5.2, original magnification × 40). (f) The muscle collar of the primitive duct (P) and the walls of small vessels and scattered muscle-like cells are positive for smooth muscle actin (Immunostain with DAKO-anti-smooth muscle actin antibody, original magnification × 22).


Table 1. Reported Cases of Oligomeganephronia in Adults
ReportsAge; sexGestation; birth weight (weeks; g)Initial blood pressure (mm Hg)Urine protein (g/day)HematuriaInitial serum Cre (mg/dL)eGRF (mL/min/1.73 m2)Kidney (right/left) (mm)Other anomalyTherapy
Cre: creatinine; eGFR: estimated glomerular filtration rate; ACE-I: angiotensin-converting enzyme inhibitor; ARB: angiotensin type II receptor blocker; CCB: calcium channel blocker; RBC: red blood cell.
Kawanishi et al [3], case 136; male37; 2,700166/1130.18None2.6523.7Atrophic (89/93)NoneACEI, ARB, CCB, αβ-block, aspirin
Kawanishi et al [3], case 219; female41; 2,41095/600.53RBC 1 - 4/HPF1.1453.4Atrophic (87/80)NoneACE, ARB, dilazep
Kawanishi et al [3], case 321; male41; 3,405132/700.65None1.2066.3Normal (106/105)NoneARB
Fuke et al [4]23; maleFull term; 3,200160/943.86None1.6146.9Atrophic (70/85)Complete healing loss of the left earARB
Alves et al [5]33; maleNot describedNot described3.3RBC 5/HPF2.0931.8Atrophic (72/102)NoneNone
Present report29; male39; 3,100126/683.96RBC 1 - 4/HPF1.6143.8Atrophic (101/88)Ventricular septal defectARB