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 |
|---|---|
| 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 |
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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 journal › Article
}
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
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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 -