Nephrotic syndrome in autosomal dominant polycystic kidney disease.
Urinary protein excretion is generally less than 1 g/24 h in autosomal dominant polycystic kidney disease (ADPKD), and the association of the nephrotic syndrome with this condition is considered rare. A patient with ADPKD associated with nephrotic-range proteinuria is described. She exhibited a relatively rapid impairment of her renal function. An open renal biopsy revealed focal segmental glomerulosclerosis (FGS) with features consistent with secondary FGS. Twenty-one patients with ADPKD and nephrotic syndrome were retrieved from the literature. Fourteen of them (including this case) had a histopathologic evaluation, and FGS was the dominant diagnoses (five patients). Next in frequency were minimal-change disease and membranous nephropathy, with two patients each. Five other patients had a variety of diagnoses. Thus, it is difficult to ascertain if these associations are coincidental or represent a specific pathogenetic relationship. The evaluation of the data also suggests that the presence of proteinuria and nephrotic syndrome accelerates the course of ADPKD toward ESRD.
Contreras
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G
Department of Medicine, University of Miami School of Medicine, FL, USA.
Mercado
A
A
Pardo
V
V
Vaamonde
C A
CA
D000368:Aged; D018450:Disease Progression; D005260:Female; D006801:Humans; D009404:Nephrotic Syndrome / Q000150:complications* / Q000473:pathology; D016891:Polycystic Kidney, Autosomal Dominant / Q000150:complications* / Q000473:pathology