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idiopathic nephrotic syndrome

Rossana Malatesta-Muncher, Karen W Eldin, Laurence H Beck, Mini Michael
BACKGROUND: Idiopathic membranous nephropathy is an uncommon cause of nephrotic syndrome in children and can present treatment challenges. The current treatment options of steroids and cyclophosphamide, cyclosporine, or mycophenolate require prolonged treatment durations and the associated side effects may result in nonadherence in children, especially in adolescents. CASE-DIAGNOSIS: We report two adolescent patients with idiopathic membranous nephropathy with nephrotic range proteinuria and elevated anti-phospholipase A2 receptor levels who did not achieve remission with steroids and were later treated with rituximab...
March 15, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Shoji Tsuji, Chikushi Suruda, Masaki Hashiyada, Takahisa Kimata, Sohsaku Yamanouchi, Tetsuya Kitao, Jiro Kino, Atsushi Akane, Kazunari Kaneko
BACKGROUND: While the etiology of idiopathic nephrotic syndrome (idiopathic nephrotic syndrome [INS]; characterized by repeated relapses and comorbid allergic conditions) remains unknown, recent evidence suggests that dysfunction in regulatory T cells (Tregs) plays an important role in the development of INS as well as allergic diseases. We hypothesized that dysbiosis involving decreased butyric acid-producing gut microbiota leads to defective induction and differentiation of peripherally induced Tregs, resulting in INS relapse...
March 13, 2018: American Journal of Nephrology
Janice Crespo-Salgado, Tyrus Stewart, Diego H Aviles
No abstract text is available yet for this article.
March 13, 2018: American Journal of Nephrology
Ilan Rozenberg, Andres Kotliroff, Tania Zahavi, Sydney Benchetrit
BACKGROUND: Idiopathic membranous nephropathy (IMN) is one of the most common causes of nephrotic syndrome (NS) in Caucasian adults. Most patients have good renal prognosis, but 30-40% may progress to end stage renal disease (ESRD). OBJECTIVES: To evaluate the efficacy and safety of immunosuppressive treatment (IST) in high-risk patients. METHODS: All IMN patients diagnosed by kidney biopsy from 2004-2010 were included. Clinical and laboratory data were collected at each follow-up visit...
March 2018: Israel Medical Association Journal: IMAJ
Eunjin Bae, Sung Woo Lee, Seokwoo Park, Dong Ki Kim, Hajeong Lee, Hyuk Huh, Ho Jun Chin, Shina Lee, Dong-Ryeol Ryu, Ji In Park, Sejoong Kim, Dong Jun Park, Shin-Wook Kang, Yon Su Kim, Yun Kuy Oh, Yong Chul Kim, Chun Soo Lim, Jung Tak Park, Jung Pyo Lee
Idiopathic membranous nephropathy (MN) is the most common glomerulonephritis in elderly patients showing nephrotic syndrome. However, little is known about its treatment options and outcomes in elderly MN patients at long term follow-up. We retrospectively enrolled patients with biopsy-proven MN between April 1990 and December 2015 from eight tertiary hospitals in Korea. Among them, we excluded patients who had secondary causes of MN and subnephrotic-range proteinuria. We evaluated the presenting features and clinical outcomes and analyzed the all-cause mortality, renal outcomes, infection, and remission with respect to age...
March 3, 2018: Archives of Gerontology and Geriatrics
Y F Dong, L W Sun, B Zhang, X Y Kuang, X L Niu, Y L Kang, S Hao, P Wang, Z Li, G H Zhu, W Y Huang, Y Wu
Objective: To explore the clinical features and expression of PLA(2)R in renal tissue of children with idiopathic membranous nephropathy. Methods: Retrospective study was performed in patients with membranous nephropathy diagnosed through renal biopsy and the follow-up time was at least half a year in Shanghai Children ' s Hospital from January 2010 to February 2017. We compared their clinicopathological and pathological findings of IMN. Indirect immunofluorescence assay was used to detect glomerular PLA(2)R expression...
March 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Shojiro Watanabe, Tomomi Aizawa, Hiroyasu Tsukaguchi, Koji Tsugawa, Kazushi Tsuruga, Akemi Shono, Kandai Nozu, Kazumoto Iijima, Kensuke Joh, Hiroshi Tanaka
Recent advances in high-throughput sequencing for clinical genetic testing have revealed novel disease-causing genes, such as Crumbs homolog 2 (CRB2) for early-onset steroid-resistant nephrotic syndrome (SRNS). We report the long-term clinicopathologic observation of a Japanese female patient with SRNS caused by a newly identified compound heterozygous mutation of CRB2 (p.Arg628Cys and p.Gly839Trp located in the 10th and 11th epidermal growth factor-like domains, respectively). She was initially examined during a mass urinary mass screening for 3...
February 23, 2018: Nephrology
Shikun Yang, Aimei Li, JianWen Wang, Jun Liu, Yachun Han, Wei Zhang, Yan Chun Li, Hao Zhang
BACKGROUND: Kidney disease is a serious problem that adversely affects human health, but critical knowledge is lacking on how to effectively treat established chronic kidney disease. Mounting evidence from animal and clinical studies has suggested that vitamin D receptor (VDR) activation has beneficial effects on various renal diseases. METHOD: A structured search of published research literature regarding VDR structure and function, VDR in various renal diseases (e...
February 14, 2018: Current Medicinal Chemistry
Alice Bonanni, Marta Calatroni, Matteo D'Alessandro, Sara Signa, Enrica Bertelli, Michela Cioni, Eddi Di Marco, Roberto Biassoni, Gianluca Caridi, Giulia Ingrasciotta, Roberta Bertelli, Armando Di Donato, Maurizio Bruschi, Alberto Canepa, Giorgio Piaggio, Pietro Ravani, Gian Marco Ghiggeri
AIMS: Anti-CD20 antibodies are increasingly being used to treat idiopathic nephrotic syndrome (INS) in children. While they may allow steroid and calcineurin-inhibitor withdrawal, repeated infusions of anti-CD20 antibodies are often required to maintain remission. Data on their potential toxicity in INS are needed to consider repeated infusions. METHODS: We reported data on side effects related to the use of rituximab (a chimeric anti-body; 130 pts) and ofatumumab (a humanized anti-body; 37 pts) in children with INS (steroid-dependent and steroid/calcineurin-inhibitor dependent disease) treated in a national referral center during a 9-year period (400 treatments; follow-up 1-9 years)...
February 13, 2018: British Journal of Clinical Pharmacology
Hiromichi Hara, Daishi Hirano
Long-term immunosuppressive therapy with severe adverse effects is indispensable to maintain disease remission in frequently relapsing nephrotic syndrome (NS) in children. Hence, development of new therapy with less toxicity for relapses of NS is required. We demonstrated a case of a 2-year-old boy with frequently relapsing NS, whose frequent relapses were successfully treated with azithromycin. Azithromycin treatment prevented the need for long-term immunosuppressive therapy in this case. Azithromycin could be a new treatment option for relapse of NS, with few adverse effects, in selected cases...
February 2018: Clinical Kidney Journal
Jiro Kino, Shoji Tsuji, Tetsuya Kitao, Yuko Akagawa, Sohsaku Yamanouchi, Takahisa Kimata, Kazunari Kaneko
BACKGROUND: The pathogenesis of idiopathic nephrotic syndrome (INS) remains unclear although recent studies suggest endothelin 1 (ET-1) and CD80 of podocytes are involved. We investigated the potential of antagonist to ET-1 receptor type A (ETRA) as therapeutic agent through suppression of CD80 in rat model of INS. METHODS: Puromycin aminonucleoside (PAN) was injected to Wister rats to induce proteinuria: some were treated with ETRA antagonist and others were treated with 0...
January 23, 2018: Pediatric Research
Giovanni Conti, Dominique De Vivo, Claudia Fede, Stefania Arasi, Angela Alibrandi, Roberto Chimenz, Domenico Santoro
BACKGROUND: Low birth weight (LBW) is associated with reduced nephron endowment. Clinical-pathologic features of post adaptive focal segmental glomerulosclerosis (FSGS) have been observed in subjects with prematurity and very LBW. METHODS: We aimed to investigate the correlation between LBW and outcome in a cohort of 89 children with idiopathic nephrotic syndrome (NS) (2-12 years-old at onset, followed for > 3 years), of whom 21 with LBW (birth weight < 10th percentile for gestational age, gender, ethnicity, and maternal parity or birth weight < 2500 g)...
January 19, 2018: Journal of Nephrology
Eiichiro Uchino, Daisuke Takada, Haruta Mogami, Takeshi Matsubara, Tatsuo Tsukamoto, Motoko Yanagita
Pregnancy and membranous nephropathy (MN) can occur concurrently with nephrotic syndrome. However, the pathophysiology of MN associated with pregnancy remains unclear, including the involvement of anti-M-type phospholipase A2 receptor (PLA2R) antibody, the major antigen of idiopathic MN (iMN). A treatment for the condition is also not established. We present the case of a 43-year-old pregnant female with incidental proteinuria and hypoalbuminemia. We made a diagnosis of nephrotic syndrome at 11 week gestation...
January 18, 2018: CEN Case Reports
Yen-Sung Huang, Shin-Huei Fu, Kuo-Cheng Lu, Jin-Shuen Chen, Hsin-Yi Hsieh, Huey-Kang Sytwu, Chia-Chao Wu
Idiopathic membranous nephropathy (MN) is an autoimmune-mediated glomerulonephritis and the most common cause of idiopathic nephrotic syndrome in adult humans. A tumor necrosis factor α (TNF-α)-mediated inflammatory response via TNF receptor 1 (TNFR1) and TNFR2 has been proposed as a pathogenic factor. In this study, we assessed the therapeutic response to blocking TNF signaling in experimental MN. Murine MN was induced experimentally by cationic bovine serum albumin (cBSA); phosphate-buffered saline was used in control mice...
December 19, 2017: Oncotarget
Aya Nawata, Satoshi Hisano, Shohei Shimajiri, Ke-Yong Wang, Yoshiya Tanaka, Toshiyuki Nakayama
AIMS: Nephrotic syndrome (NS) is a major manifestation of lupus nephritis (LN). The dysregulation of podocyte, glomerular basement membrane (GBM) and endothelial cell (EC) develops proteinuria in glomerular diseases. The aim of our study is to clarify whether the dysregulation of these barriers is associated with NS in proliferative LN and membranous LN. METHODS AND RESULTS: Fifty six patients with NS including minimal change NS in 15, primary membranous nephropathy (PMN) in 13, Class III/IV LN in 15 and Class V LN in 13 were enrolled in this study...
December 15, 2017: Histopathology
Ioana Tieranu, Monica I Dutescu, Constantin Bara, Cristian G Tieranu, Mihaela Balgradean, Olivia M Popa
Background: Childhood idiopathic nephrotic syndrome (INS) is one of the most common glomerular diseases, characterized by heavy proteinuria, hypoalbuminemia, dyslipidemia and generalized edema. Although some progresses were made regarding the pathogenesis of this disease, there are a lot of questions still left unanswered. Some of them involve the implications of several cytokines, including tumor necrosis factor alpha (TNF-alpha), in the development and clinical course of INS. Objective: Our objective was to analyze the role of two single nucleotide polymorphisms of TNF-alpha gene in the development of pediatric INS and their implication in the response to corticosteroid therapy...
September 2017: Mædica
K M Shah, A J Ohri, U S Ali
This is a randomized, parallel group, active-controlled trial to compare the efficacy of intravenous cyclophosphamide (IVCP) with oral cyclophosphamide (OCP) in patients with steroid-resistant nephrotic syndrome (SRNS) in children. Fifty consecutive children with idiopathic SRNS were biopsied and then randomized to receive either OCP at a dose of 2 mg/kg/day for 12 weeks or IVCP at a dose of 500 mg/m2 /month for 6 months. Both groups received tapering doses of oral steroids. The response was evaluated in terms of induction of complete remission (CR) or partial remission (PR), time to remit, and side effects...
November 2017: Indian Journal of Nephrology
Hanna Szymanik-Grzelak, Elżbieta Kuźma-Mroczkowska, Piotr Skrzypczyk, Teresa Bielecka, Iwona Kotula, Małgorzata Pańczyk-Tomaszewska
Children with nephrotic syndrome (NS) are at greater risk of infections than the general population, due to immunodeficiency in the course of the disease and the treatment. In this study we present 4 children (2 girls, 2 boys), mean age 7.6 ±5.1 years, with NS/proteinuria and latent tuberculosis in 3 children and lymph node tuberculosis in 1 child. The reasons for testing these children for tuberculosis (TB) were the evaluation of the epidemiological status before treatment with corticosteroids (GCS), leukopenia and the relapse of NS, and non-nephrotic proteinuria...
2017: Central-European Journal of Immunology
Katarzyna Popko, Elżbieta Górska, Elżbieta Kuźma-Mroczkowska
Idiopathic nephrotic syndrome (INS) is a common chronic illness characterized by massive proteinuria and hypo-albuminemia in children. Baseline treatment is 6 month-corticotherapy. In cases of steroid resistant/dependent INS several types of treatment are used, including course of methyloprednisolone "pulses", alkylating agents, cyclosporin A, levamisole and mycophenolate mofetil. It has been suggested that children with frequently relapsing nephrotic syndrome or steroid-dependent nephrotic syndrome had a significantly longer relapse-free period if rituximab (RTX) treatment was additionally applied...
2017: Central-European Journal of Immunology
Dina Abdel Razek Midan, Nesreen Gamal Elhelbawy, Mona Salah El-Din Habib, Iman Aly Ahmedy, Rasha Ibrahim Noreldin
INTRODUCTION: Idiopathic nephrotic syndrome (INS) is a glomerular disease with completely unclear pathogenesis and different responses to steroid therapy. This study aimed to investigate the role of cytokine genes promoter polymorphisms in steroid therapy responses. MATERIALS AND METHODS: One hundred children with INS and 30 healthy controls were studied. Genotyping of TNF-α-G308A single nucleotide polymorphism was done using polymerase chain reaction-restriction fragment length polymorphism method, while of IL-6-G174C single nucleotide polymorphism was done using real-time polymerase chain reaction...
November 2017: Iranian Journal of Kidney Diseases
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