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idiopathic nephrotic syndrome

Takao Saito, Masayuki Iwano, Koichi Matsumoto, Tetsuya Mitarai, Hitoshi Yokoyama, Noriaki Yorioka, Shinichi Nishi, Ashio Yoshimura, Hiroshi Sato, Satoru Ogahara, Yoshie Sasatomi, Yasufumi Kataoka, Shiro Ueda, Akio Koyama, Shoichi Maruyama, Masaomi Nangaku, Enyu Imai, Seiichi Matsuo, Yasuhiko Tomino
BACKGROUND: We designed a prospective and randomized trial of mizoribine (MZR) therapy combined with prednisolone (PSL) for idiopathic membranous nephropathy (IMN) with steroid-resistant nephrotic syndrome (SRNS). METHODS: Patients with IMN were divided into 2 groups, and MZR combined with PSL was administered for 2 years. PSL was initially prescribed at 40 mg/day and tapered. MZR was given once-a-day at 150 mg and 3-times-a-day at 50 mg each to groups 1 and 2...
October 25, 2016: Clinical and Experimental Nephrology
Claire Dossier, Nathanael Lapidus, Florian Bayer, Anne-Laure Sellier-Leclerc, Olivia Boyer, Loic de Pontual, Adrien May, Sylvie Nathanson, Christine Orzechowski, Tabassome Simon, Fabrice Carrat, Georges Deschênes
BACKGROUND: The etiology of idiopathic nephrotic syndrome (INS) remains partially unknown. Viral infections have been reported to be associated with INS onset and relapse. The aim of this study was to describe the epidemiology of a population-based cohort of children with INS and propose a spatiotemporal analysis. METHODS: All children aged 6 months to 15 years with INS onset between December 2007 and May 2010 and living in the Paris area were included in a prospective multicenter study...
October 24, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Claudio Bazzi, Tomoko Usui, Virginia Rizza, Daniela Casellato, Maurizio Gallieni, Masaomi Nangaku
BACKGROUND: The clinical course of idiopathic membranous nephropathy (IMN) varies from spontaneous remission of nephrotic syndrome (NS) to end-stage renal disease (ESRD). Selecting patients with high risk of progression for immunosuppressive therapy is mandatory. METHODS: 86 IMN subjects were followed for median of 69 months (range 6-253). Receiver operating characteristic curve and Cox proportional hazards model were used to evaluate prognostic factors for progression, defined as ESRD or eGFR reduction ≥50% of baseline...
October 20, 2016: Nephrology
Abdulmoein E Al-Agha, Abrar M Alnawab, Tala M Hejazi
To determine the various etiologies of primary and secondary hyperlipidemia among children visiting the pediatric endocrine clinic. Methods: This is a retrospective, cross-sectional, cohort study conducted at King Abdulaziz University Hospital (KAUH), Jeddah, Kingdom of Saudi Arabia from January 2010 to 2015 that included 253 children aged from birth to 12 years old. Data were obtained by reviewing medical reports of patients who presented with hyperlipidemia to the clinic, and their laboratory investigation results using KAUH electronic "Phoenix" system...
November 2016: Saudi Medical Journal
Agnieszka Antonowicz, Piotr Skrzypczyk, Beata Kępa, Małgorzata Pańczyk-Tomaszewska
: Toxocariasis is a common zoonosis caused by infection with Toxocara canis or cati larvae. Ocular toxocariasis is one of the forms of infestation found in 1/1,000 - 1/10,000 children. Children with idiopathic nephrotic syndrome (INS) are at high risk of infections, also parasitic infestations, which can, in turn, cause relapses of the disease. A CASE REPORT: We present a case of a 6-year-old boy with steroiddependent nephrotic syndrome. The disease started at age of 2, the boy had 9 relapses of INS, and was treated with oral prednisone, levamisole, and cyclophosphamide...
October 19, 2016: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
Imen Gorsane, I Helal, I Yacoub, F Ben Hamida, E Abderrahim, T Ben Abdallah
Focal and segmental glomerulosclerosis (FSGS) is a heterogeneous entity. Previous few studies have evaluated the efficacy of calcineurin inhibitors in primary FSGS and have suggested positive benefit. In this single-center, retrospective study (1975-2014), we report our experience in Tunisian adults with primary FSGS treated with cyclosporine A (CsA). It includes patients histologically proven FSGS and managed in the Charles Nicolle Hospital at Tunis, Tunisia. The dose of CsA was adjusted to maintain a whole blood trough level of 80-150 ng/mL...
September 2016: Saudi Journal of Kidney Diseases and Transplantation
Rutger J Maas, Jeroen K Deegens, Bart Smeets, Marcus J Moeller, Jack F Wetzels
Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are the key histological findings in patients with idiopathic nephrotic syndrome (INS). Although MCD and idiopathic FSGS are often considered to represent separate entities based on differences in their presenting characteristics, histology and outcomes, little evidence exists for this separation. We propose that MCD and idiopathic FSGS are different manifestations of the same progressive disease. The gradual development of FSGS in patients with non-remitting or relapsing INS has been well documented...
October 17, 2016: Nature Reviews. Nephrology
Mercedes Acevedo Ribó, Francisco Javier Ahijado Hormigos, Francisco Díaz, Marta Romero Molina, Maria Angeles Fernandez Rojo, Maria Antonia Garcia Rubiales, Eugenio García Diaz
This case report describes a patient with a previous history of autoimmune pancreatitis secondary to IgG4-related disease, who developed an overt nephrotic syndrome due to membranous nephropathy, surprisingly idiopathic. In all the previously described cases with both concurrent diseases, membranous nephropathy was considered to be secondary to the IgG4-related disease based on the absence of anti-PLA2R1 autoantibodies, and nephrotic syndrome usually remitted after treatment with steroids alone. However, in our patient positivity of serum anti-PLA2R1 autoantibodies together with a normal serum IgG4 level, and the absence of the other most commonly associated diseases were compatible with an idiopathic membranous nephropathy...
October 14, 2016: Clinical Nephrology
Elisabeth M Hodson, Sophia C Wong, Narelle S Willis, Jonathan C Craig
BACKGROUND: The majority of children who present with their first episode of nephrotic syndrome achieve remission with corticosteroid therapy. Children who fail to respond may be treated with immunosuppressive agents including calcineurin inhibitors (cyclosporin or tacrolimus) and with non-immunosuppressive agents such as angiotensin-converting enzyme inhibitors (ACEi). Optimal combinations of these agents with the least toxicity remain to be determined. This is an update of a review first published in 2004 and updated in 2006 and 2010...
October 11, 2016: Cochrane Database of Systematic Reviews
Manal A Safan, Nesreen G Elhelbawy, Dina A Midan, Heba F Khader
BACKGROUND: The most common cause of nephrotic syndrome (NS) is idiopathic nephrotic syndrome (INS), also called nephrosis. Although most patients respond to steroid therapy, there is unequal response to treatment suggesting the involvement of genetic factors. The current study was conducted to evaluate the influence of two single nucleotide polymorphisms (SNPs) in ABCB1 (C3435T and C1236T) on the steroid treatment response in INS children. MATERIALS AND METHODS: Genotyping of ABCB1 C3435T and C1236T polymorphisms by real time PCR were conducted on 120 INS children, 80 steroid sensitive (SS) and 40 steroid resistant (SR)...
October 8, 2016: British Journal of Biomedical Science
K Dahan
Membranous nephropathy (MN) accounts for about 20% of cases of nephrotic syndrome in the adult. Thickening of glomerular capillary walls results from subepithelial formation of immune deposits containing IgG and the membrane attack complex of complement, which is the major mediator of proteinuria, and antigens. Idiopathic forms of MN (IMN) represent 70 to 80% of all cases. A major breakthrough was the identification of the podocyte antigen PLA2R as the target of circulating antibodies in about 70% of IMN, which confirmed that the disease was auto-immune in nature...
May 25, 2016: La Revue de Médecine Interne
Yasser Gamal, Ahlam Badawy, Salwa Swelam, Mostafa S K Tawfeek, Eman Fathalla Gad
Glucocorticoids are primary therapy of idiopathic nephrotic syndrome (INS). However, not all children respond to steroid therapy. We assessed glomerular glucocorticoid receptor expression in fifty-one children with INS and its relation to response to steroid therapy and to histopathological type. Clinical, laboratory and glomerular expression of glucocorticoid receptors were compared between groups with different steroid response. Glomerular glucocorticoid expression was slightly higher in controls than in minimal change early responders, which in turn was significantly higher than in minimal change late responders...
October 3, 2016: Fetal and Pediatric Pathology
Claire Dossier, Agnès Jamin, Georges Deschênes
Steroid sensitive nephrotic syndrome is marked by a massive proteinuria and loss of podocytes foot processes. The mechanism of the disease remains debated but recent publications suggest a primary role of EBV. EBV replication in the peripheral blood is found in 50% of patients during the first flare of the disease. The genetic locus of steroid sensitive nephrotic syndrome was also identified as influencing antibodies directed against EBNA1. EBV is able to establish, latent benign infection in memory B cells that display phenotypes similar to antigen-selected memory B cells...
September 28, 2016: Pediatric Research
Alastair J Rankin, Emily P McQuarrie, Jonathan G Fox, Colin C Geddes, Bruce MacKinnon
BACKGROUND: The reported incidence of venous thromboembolism (VTE) in patients with nephrotic syndrome (NS) varies widely, as does the approach to prophylactic anticoagulation. We aimed to assess the incidence of VTE in patients with primary NS in order to inform a sample size calculation to determine if a future clinical trial will ever be feasible. METHODS: All adults undergoing native renal biopsy for NS between 2008 and 2013 yielding a diagnosis of primary glomerulonephritis were identified...
September 24, 2016: Nephron
Kaori Kikunaga, Kenji Ishikura, Chikako Terano, Mai Sato, Fumiyo Komaki, Yuko Hamasaki, Satoshi Sasaki, Kazumoto Iijima, Norishige Yoshikawa, Koichi Nakanishi, Hitoshi Nakazato, Takeshi Matsuyama, Takashi Ando, Shuichi Ito, Masataka Honda
BACKGROUND: Little is known regarding the epidemiology of idiopathic nephrotic syndrome (INS) in East Asia. Previous studies have suggested higher incidence of INS in Asian children, though decreasing trend of its incidence has also been shown. METHODS: We conducted a nationwide study of Japanese children aged 6 months to 15 years with INS. Children who were newly diagnosed with INS between 1 January 2010 and 31 December 2012 were eligible. Children with congenital nephrotic syndrome or nephrotic syndrome secondary to nephritis were excluded...
September 2, 2016: Clinical and Experimental Nephrology
Amal A Al-Eisa, Mohammad Z Haider
BACKGROUND: Vitamin D activity is controlled by vitamin D receptors (VDRs), which are affected by different genetic polymorphisms, including TaqI and Apal restriction fragment length polymorphisms (RFLPs), which have been reported to be associated with several diseases. The aim of this study was to determine the frequency and the association of VDR gene polymorphisms with idiopathic nephrotic syndrome (INS) and steroid responsiveness in Kuwaiti children. SUBJECTS AND METHODS: Genotypes of the VDR TaqI gene polymorphism and the Apal gene polymorphism were analyzed using polymerase chain reaction-RFLP in 78 INS patients and 56 matched controls...
2016: International Journal of Nephrology and Renovascular Disease
Talal Alfaadhel, Daniel Cattran
BACKGROUND: Idiopathic membranous nephropathy (IMN) is a common cause of nephrotic syndrome (NS) in adults in Western countries. In 2012, the KDIGO (Kidney Disease: Improving Global Outcomes) working group published guidelines for the management of glomerulonephritis, thus providing a template for the treatment of this condition. While being aware of the impact of the clinicians' acumen and that patients may choose a different therapeutic option due to the risks of specific drugs and also of the evolving guidelines, this review details our approach to the management of patients with IMN in a Western center (Toronto)...
September 2015: Kidney Diseases
Jing Xu, Xiaofan Hu, Jingyuan Xie, Nan Chen
BACKGROUND: Membranous nephropathy (MN) is the most common cause of nephrotic syndrome (NS) in adults, accounting for about 20.0% of all NS cases. With an increasing prevalence, especially in the elderly, it has received great attention in Asia. SUMMARY: Recently, the prevalence of idiopathic MN (IMN) has significantly increased among the elderly people in Asia and other places in the world. Although the exact mechanism of IMN remains unveiled, the identification of new antigens such as PLA2R and THSD7A has greatly enhanced our understanding of its pathogenesis...
September 2015: Kidney Diseases
P Skrzypczyk, M Panczyk-Tomaszewska, J Przychodzien, M Mizerska-Wasiak, E Kuzma-Mroczkowska, E Gorska, A Stelmaszczyk-Emmel
OBJECTIVE: Renalase is a protein produced mainly by kidneys. Renalase inactivates circulating catecholamines catalyzing process of their oxidation. Studies in adults with kidney disease suggest that renalase may play a role in pathogenesis of hypertension and target organ damage. The aim of the study was to assess level of renalase in children with glomerular kidney diseases and to find the relation between renalase level and clinical parameters in this group of patients. DESIGN AND METHOD: In 78 children (47 M, 31F) aged from 3...
September 2016: Journal of Hypertension
H Xie, M Fang, H Lin, P Li, N Sun, J Chen, Y Sun
No abstract text is available yet for this article.
August 10, 2016: West Indian Medical Journal
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