Nephrotic syndrome in autosomal dominant polycystic kidney disease

G. Contreras, A. Mercado, Victoriano Pardo, C. A. Vaamonde

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21 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)1354-1359
Number of pages6
JournalJournal of the American Society of Nephrology
Issue number5
StatePublished - Jan 1 1995
Externally publishedYes


  • Autosomal dominant polycystic kidney disease
  • Focal segmental sclerosis
  • Nephrotic syndrome
  • Progression to ESRD
  • Proteinuria

ASJC Scopus subject areas

  • Nephrology


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