scholarly journals The Unpredictability of Idiopathic Membranous Nephropathy: An Illustrative Case Report

2018 ◽  
Vol 8 (2) ◽  
pp. 103-106
Author(s):  
Murray L. Levin ◽  
Shubhada Ahya

The case of a female patient with primary membranous nephropathy is presented. She was treated with corticosteroids and chlorambucil after conservative therapy had failed and went into remission for 5 years. Her nephrotic syndrome recurred but did not respond to the same regimen. She had another complete remission after treatment with corticosteroids and cyclosporine, but the nephrosis recurred after 7 years. Again, she failed to respond with retreatment of steroids plus cyclosporine. She was treated with alternate-day steroid plus mycophenolate and, once again, had a complete remission. She was maintained on low-dose mycophenolate for 7 more years. The mycophenolate had to be discontinued because of cytomegalovirus colitis. Treatment with ganciclovir abolished the colitis. She is still in remission 10 years later. The case is discussed with regard to current knowledge of the immune pathogenesis of membranous nephropathy as well as the unknowns of the immunogenesis of the disease.

2011 ◽  
Vol 11 ◽  
pp. 1041-1047 ◽  
Author(s):  
Ramón Peces ◽  
Jorge Martínez-Ara ◽  
Carlos Peces ◽  
Mariluz Picazo ◽  
Emilio Cuesta-López ◽  
...  

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.


2012 ◽  
Vol 31 (2) ◽  
pp. 112-117 ◽  
Author(s):  
Yong Chul Kim ◽  
Tae Woo Lee ◽  
Hajeong Lee ◽  
Ho Suk Koo ◽  
Kook-Hwan Oh ◽  
...  

2013 ◽  
Vol 88 (3) ◽  
pp. 473-475 ◽  
Author(s):  
Laura Maria Andrade Silveira ◽  
Andreia Nogueira Ramos ◽  
Isadora Rosado do Amaral ◽  
Vitoria Regina Pedreira de Almeida Rego

Congenital Hypertrichosis Lanugionsa is a rare autosomal dominant genetic disorder, with fewer than 50 cases reported in the literature. It is characterized by excessive lanugo hair, sparing only the mucous membranes, palms and soles. It may be associated with other organic abnormalities and should form part of the dermatologist's current knowledge. We discuss some aspects of the syndrome in question arising from the case report of a 2-year-old female patient, black, with classic clinical presentation, with no other associated congenital abnormalities.


2018 ◽  
Vol 19 (1) ◽  
Author(s):  
Eriko Yamaguchi ◽  
Kazuhiro Yoshikawa ◽  
Izaya Nakaya ◽  
Karen Kato ◽  
Yoshikazu Miyasato ◽  
...  

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