Abstract
Segmental “hypoplasia” of the kidney is a form of severe renal atrophy commonly associated with vesicoureteric reflux (VUR). The scarred segments contain atrophic tubules and hyperplastic blood vessels; glomeruli are often absent or sparse. These lesions have been identified in 21 patients (12F, 9M), 6-27 yr: 13 (62%) hypertensive (HT) and 8 normotensive (NT); 13 with prior urinary tract infection; 18/19 (95%) with radiographic VUR; 8 (38%) with impaired renal function (GFR <40 ml/min/1.73 m2); none with severe proteinuria. Renal atrophy commonly progressed after surgical repair of VUR, even in absence of infection. Lesions were bilateral in 5 HT patients and 2 others had unilateral agenesis; bilateral in only 1 NT patient. HT responded to nephrectomy in all patients. Histologic examination of the scars showed no differences between HT and NT patients in numbers of sclerotic glomeruli or in severity of interstitial fibrosis, arteriolar sclerosis, and inflammatory cell infiltration. Examination of cortical tissue adjacent to scars showed HT patients to have more severe tubular atrophy and vascular change, possibly as the result of HT. Thioflavin-T staining showed the sclerotic glomeruli and hypertrophic arterioles to contain more JGA granules in the scars of HT than of NT patients. Segmental “hypoplasia” appears to be an acquired abnormality, although its association with renal dysplasia in two specimens indicates an occasional origin during intrauterine life.
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Shindo, S., Bernstein, J. & Arant, B. RENAL SEGMENTAL “HYPOPLASIA” (ASK-UPMARK): PATHOLOGIC ASPECTS. Pediatr Res 14, 1002 (1980). https://doi.org/10.1203/00006450-198008000-00174
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DOI: https://doi.org/10.1203/00006450-198008000-00174