Nephrotic syndrome in autosomal dominant polycystic kidney disease

Gabriel Contreras, A. Mercado, V. Pardo, C. A. Vaamonde

Research output: Contribution to journalArticle

20 Citations (Scopus)

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 languageEnglish
Pages (from-to)1354-1359
Number of pages6
JournalJournal of the American Society of Nephrology
Volume6
Issue number5
StatePublished - Jan 1 1995
Externally publishedYes

Fingerprint

Autosomal Dominant Polycystic Kidney
Nephrotic Syndrome
Focal Segmental Glomerulosclerosis
Lipoid Nephrosis
Proteinuria
Kidney
Membranous Glomerulonephritis
Chronic Kidney Failure
Biopsy
Proteins

Keywords

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

ASJC Scopus subject areas

  • Nephrology

Cite this

Nephrotic syndrome in autosomal dominant polycystic kidney disease. / Contreras, Gabriel; Mercado, A.; Pardo, V.; Vaamonde, C. A.

In: Journal of the American Society of Nephrology, Vol. 6, No. 5, 01.01.1995, p. 1354-1359.

Research output: Contribution to journalArticle

Contreras, Gabriel ; Mercado, A. ; Pardo, V. ; Vaamonde, C. A. / Nephrotic syndrome in autosomal dominant polycystic kidney disease. In: Journal of the American Society of Nephrology. 1995 ; Vol. 6, No. 5. pp. 1354-1359.
@article{319a916fba6c44ebbf7ec33975d651e6,
title = "Nephrotic syndrome in autosomal dominant polycystic kidney disease",
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.",
keywords = "Autosomal dominant polycystic kidney disease, Focal segmental sclerosis, Nephrotic syndrome, Progression to ESRD, Proteinuria",
author = "Gabriel Contreras and A. Mercado and V. Pardo and Vaamonde, {C. A.}",
year = "1995",
month = "1",
day = "1",
language = "English",
volume = "6",
pages = "1354--1359",
journal = "Journal of the American Society of Nephrology",
issn = "1046-6673",
publisher = "American Society of Nephrology",
number = "5",

}

TY - JOUR

T1 - Nephrotic syndrome in autosomal dominant polycystic kidney disease

AU - Contreras, Gabriel

AU - Mercado, A.

AU - Pardo, V.

AU - Vaamonde, C. A.

PY - 1995/1/1

Y1 - 1995/1/1

N2 - 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.

AB - 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.

KW - Autosomal dominant polycystic kidney disease

KW - Focal segmental sclerosis

KW - Nephrotic syndrome

KW - Progression to ESRD

KW - Proteinuria

UR - http://www.scopus.com/inward/record.url?scp=0028844484&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0028844484&partnerID=8YFLogxK

M3 - Article

VL - 6

SP - 1354

EP - 1359

JO - Journal of the American Society of Nephrology

JF - Journal of the American Society of Nephrology

SN - 1046-6673

IS - 5

ER -