scholarly journals Nephrotic syndrome in autosomal dominant polycystic kidney disease.

1995 ◽  
Vol 6 (5) ◽  
pp. 1354-1359
Author(s):  
G Contreras ◽  
A Mercado ◽  
V Pardo ◽  
C A Vaamonde
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Minimal Change Disease ◽  
Autosomal Dominant ◽  
Polycystic Kidney ◽  
Protein Excretion ◽  
Histopathologic Evaluation ◽  
Autosomal Dominant Polycystic Kidney ◽  
Nephrotic Range Proteinuria

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.

scholarly journals Factors relating to urinary protein excretion in children with autosomal dominant polycystic kidney disease.

1998 ◽  
Vol 9 (10) ◽  
pp. 1908-1914 ◽  
Author(s):  
C Sharp ◽  
A Johnson ◽  
P Gabow
Keyword(s):  
Kidney Disease ◽  
Renal Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Urinary Protein Excretion ◽  
Polycystic Kidney ◽  
Protein Excretion ◽  
Autosomal Dominant Polycystic Kidney ◽  
Overt Proteinuria ◽  
Excretion Rates

Adults with autosomal dominant polycystic kidney disease (ADPKD) who have overt proteinuria (>300 mg/d) have higher mean arterial pressures, lower creatinine clearances, larger renal volumes, and a more aggressive course of renal disease than ADPKD patients without proteinuria. This study examines the relationship between proteinuria and microalbuminuria and similar factors in ADPKD children. A total of 189 children from 81 ADPKD families was included in the analysis. The ADPKD children (n = 103) had significantly greater urine protein excretion rates than the non-ADPKD children (n = 86) (3.9+/-0.3 versus 2.8+/-0.2 mg/m2 per h, P < 0.001). Children with severe renal cystic disease (> 10 cysts; n = 54) had greater protein excretion than those with moderate disease (< or = 10 cysts; n = 49) (4.4+/-0.5 versus 3.3+/-0.2 mg/m2 per h, P < 0.05). The ADPKD children had significantly greater albumin excretion rates than the non-ADPKD children (32+/-6 versus 10+/-2 mg/m2 per 24 h, P < 0.001), and a higher percentage of ADPKD children had significant microalbuminuria (>15 mg/m2 per 24 h in boys and >23 mg/m2 per 24 h in girls) than their unaffected siblings (30% versus 10%, P < 0.05). Thirty percent of ADPKD children had albuminuria and 23% had overt proteinuria. For all ADPKD children, there was no correlation between proteinuria and hypertension. However, there was a significant correlation between urinary protein excretion and diastolic BP among children diagnosed after the first year of life (r = 0.23, P < 0.05). Therefore, proteinuria and albuminuria occur early in the course of ADPKD and may be markers of more severe renal disease.

scholarly journals Nephrotic Syndrome and Idiopathic Membranous Nephropathy Associated with Autosomal-Dominant Polycystic Kidney Disease

2011 ◽  
Vol 11 ◽  
pp. 1041-1047 ◽  
Author(s):  
Ramón Peces ◽  
Jorge Martínez-Ara ◽  
Carlos Peces ◽  
Mariluz Picazo ◽  
Emilio Cuesta-López ◽  
...  
Keyword(s):  
Renal Function ◽  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Complete Remission ◽  
Low Dose ◽  
Autosomal Dominant ◽  
Polycystic Kidney ◽  
Autosomal Dominant Polycystic Kidney ◽  
Nephrotic Range Proteinuria ◽  
Dose Prednisone

We report the case of a 38-year-old male with autosomal-dominant polycystic kidney disease (ADPKD) and concomitant nephrotic syndrome secondary to membranous nephropathy (MN). A 3-month course of prednisone 60 mg daily and losartan 100 mg daily resulted in resistance. Treatment with chlorambucil 0.2 mg/kg daily, low-dose prednisone, plus an angiotensin-converting enzyme inhibitor (ACEI) and an angiotensin II receptor blocker (ARB) for 6 weeks resulted in partial remission of his nephrotic syndrome for a duration of 10 months. After relapse of the nephrotic syndrome, a 13-month course of mycophenolate mofetil (MFM) 2 g daily and low-dose prednisone produced complete remission for 44 months. After a new relapse, a second 24-month course of MFM and low-dose prednisone produced partial to complete remission of proteinuria with preservation of renal function. Thirty-six months after MFM withdrawal, complete remission of nephrotic-range proteinuria was maintained and renal function was preserved. This case supports the idea that renal biopsy is needed for ADPKD patients with nephrotic-range proteinuria in order to exclude coexisting glomerular disease and for appropriate treatment/prevention of renal function deterioration. To the best of our knowledge, this is the first reported case of nephrotic syndrome due to MN in a patient with ADPKD treated with MFM, with remission of proteinuria and preservation of renal function after more than 10 years. Findings in this patient also suggest that MFM might reduce cystic cell proliferation and fibrosis, preventing progressive renal scarring with preservation of renal function.

Nephrotic Syndrome and Rapid Renal Failure in Autosomal Dominant Polycystic Kidney Disease

1990 ◽  
Vol 10 (1) ◽  
pp. 69-72 ◽  
Author(s):  
Gayle Murphy ◽  
Antonios H. Tzamaloukas ◽  
Margaret B. Listrom ◽  
Lawrence J. Gibel ◽  
Suzanne Meleg Smith ◽  
...  
Keyword(s):  
Renal Failure ◽  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Polycystic Kidney ◽  
Autosomal Dominant Polycystic Kidney

scholarly journals Nephrotic syndrome and autosomal dominant polycystic kidney disease

2012 ◽  
Vol 5 (6) ◽  
pp. 508-511 ◽  
Author(s):  
B. Visciano ◽  
R. A. Di Pietro ◽  
R. Rossano ◽  
A. Mancini ◽  
P. Zamboli ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Polycystic Kidney ◽  
Autosomal Dominant Polycystic Kidney

Overt proteinuria and microalbuminuria in autosomal dominant polycystic kidney disease.

1994 ◽  
Vol 5 (6) ◽  
pp. 1349-1354
Author(s):  
A B Chapman ◽  
A M Johnson ◽  
P A Gabow ◽  
R W Schrier
Keyword(s):  
Mean Arterial Pressure ◽  
Kidney Disease ◽  
Arterial Pressure ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Urinary Protein Excretion ◽  
Urinary Protein ◽  
Polycystic Kidney ◽  
Protein Excretion ◽  
Autosomal Dominant Polycystic Kidney

The amount of proteinuria is a prognostic indicator in a variety of glomerular disorders. To examine the importance of urinary protein excretion in autosomal dominant polycystic kidney disease, this study determined the clinical characteristics of autosomal dominant polycystic kidney disease patients with established proteinuria and the frequency of microalbuminuria in hypertensive autosomal dominant polycystic kidney disease patients without proteinuria. In 270 autosomal dominant polycystic kidney disease patients, mean 24-h urinary protein excretion was 259 +/- 22 mg/day. Forty-eight of 270 autosomal dominant poly-cystic kidney disease patients had over proteinuria (> 300 mg/day). The patients with established proteinuria had higher mean arterial pressures, larger renal volumes, and lower creatinine clearances than did their nonproteinuric counterparts (all P < 0.0001), a greater pack year smoking history (P < 0.05), and the projection of a more aggressive course of renal disease (P < 0.05). All autosomal dominant polycystic kidney disease patients with established proteinuria were hypertensive, as compared with 67% without established proteinuria (P < 0.001). Forty-nine patients with hypertension and left ventricular hypertrophy without established proteinuria were examined for microalbuminuria; 41% demonstrated microalbuminuria. Those with microalbuminuria had higher mean arterial pressure, larger renal volumes and increased filtration fraction. Therefore, established proteinuria and microalbuminuria in autosomal dominant polycystic kidney disease patients are associated with increased mean arterial pressure and more severe renal cystic involvement.

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