Abstract
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 language | English (US) |
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Pages (from-to) | 1354-1359 |
Number of pages | 6 |
Journal | Journal of the American Society of Nephrology |
Volume | 6 |
Issue number | 5 |
State | Published - Jan 1 1995 |
Externally published | Yes |
Keywords
- Autosomal dominant polycystic kidney disease
- Focal segmental sclerosis
- Nephrotic syndrome
- Progression to ESRD
- Proteinuria
ASJC Scopus subject areas
- Nephrology