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Nephrotic syndrome and idiopathic membranous nephropathy associated with autosomal-dominant polycystic kidney disease

Author
Peces, Ramón; Martínez-Ara, Jorge; Peces, Carlos; Picazo, Mariluz; Cuesta-López, Emilio; Vega, Cristina; Azorín, Sebastián; Selgas, Rafael
Entity
UAM. Departamento de Medicina; Instituto de Investigación Sanitaria Hospital Universitario de La Paz (IdiPAZ)
Publisher
Hindawi Publishing Corporation
Date
2011-05-05
Citation
10.1100/tsw.2011.94
The Scientific World Journal 11 (2011): 1041–1047
 
 
 
ISSN
2356-6140 (print); 1537-744X (online)
DOI
10.1100/tsw.2011.94
Funded by
This work has been supported in part by grants from the Instituto de Salud Carlos III from Ministerio de Ciencia e Innovación (EC08/00236) and Programa Intensificación Actividad Investigadora (Agencia Laín-Entralgo/CM) to R.P.
Project
Gobierno de España. EC08/00236
Editor's Version
http://dx.doi.org/10.1100/tsw.2011.94
Subjects
ADPKD; Glomerulonephritis; Membranous nephropathy; Mycophenolate mofetil; Nephrotic syndrome; Proteinuria; Medicina
URI
http://hdl.handle.net/10486/672733
Rights
© 2011 with author

Abstract

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
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Google™ Scholar:Peces, Ramón - Martínez-Ara, Jorge - Peces, Carlos - Picazo, Mariluz - Cuesta-López, Emilio - Vega, Cristina - Azorín, Sebastián - Selgas, Rafael

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  • Producción científica en acceso abierto de la UAM [14677]

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All the documents from Biblos-e Archivo are protected by copyrights. Some rights reserved.
Universidad Autónoma de Madrid. Biblioteca
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