Read by QxMD icon Read

Minimal change nephrotic syndrome

Pankaj Beniwal, Lalit Pursnani, Sanjeev Sharma, R K Garsa, Mohit Mathur, Prasad Dharmendra, Vinay Malhotra, Dhanajai Agarwal
Studies published from centers across India have reported different and contradicting patterns of glomerular disease. In this retrospective study, we report our experience from a Tertiary Care Center in Northwest India. A total of 702 renal biopsies performed between 2008 and 2013 were reviewed of which 80 were excluded from the study because of having insufficient records or if the biopsies were taken from an allograft. The study included 411 males (66.1 %) and 211 females (33.9%) with an age range of 12-70 years (mean 30...
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
Chang-Yien Chan, Kar-Hui Ng, Jinmiao Chen, Jinhua Lu, Caroline Guat-Lay Lee, Puay-Hoon Tan, Stanley C Jordan, He Henry Yang, Hui-Kim Yap
Our established interleukin-13 ( IL-13 ) overexpression rat model of minimal change-like nephropathy provided a platform to study the molecular signalling pathways in T-helper 2 (Th2) cytokine associated minimal change nephrotic syndrome. We hypothesized that IL-13 may act directly on podocytes, causing podocyte foot process effacement and hence proteinuria in our rat model of minimal change-like nephropathy. This study aimed firstly to delineate the glomerular "gene signature" associated with IL-13-mediated dysregulation of podocyte related proteins, and subsequently to investigate the role of the differentially regulated genes in IL-13-mediated podocyte injury...
October 5, 2016: Clinical Science (1979-)
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
Aoi Yamashiro, Takahiro Uchida, Seigo Ito, Naoki Oshima, Takashi Oda, Hiroo Kumagai
A 46-year-old woman suddenly developed peripheral edema. Her massive proteinuria, hypoproteinemia, and renal biopsy findings yielded the diagnosis of minimal change disease (MCD). In addition, lung Mycobacterium avium infection was diagnosed according to a positive culture of her bronchoalveolar lavage fluid. The lung lesion was improved by anti-nontuberculous mycobacteria therapy. Surprisingly, her proteinuria also gradually decreased and she attained complete remission of MCD without any immunosuppressive therapy...
2016: Internal Medicine
Xiujin Shen, Hong Jiang, Meike Ying, Zhoutao Xie, Xiayu Li, Haibing Wang, Jie Zhao, Chuan Lin, Yucheng Wang, Shi Feng, Jia Shen, Chunhua Weng, Weiqiang Lin, Huiping Wang, Qin Zhou, Yan Bi, Meng Li, Lingyan Wang, Tongyu Zhu, Xiaoru Huang, Hui-Yao Lan, Jing Zhou, Jianghua Chen
Podocyte injury and the appearance of proteinuria are features of minimal-change disease (MCD). Cyclosporin A (CsA) and tacrolimus (FK506) has been reported to reduce proteinuria in patients with nephrotic syndrome, but mechanisms remain unknown. We, therefore, investigated the protective mechanisms of CsA and FK506 on proteinuria in a rat model of MCD induced by puromycin aminonucleoside (PAN) and in vitro cultured mouse podocytes. Our results showed that CsA and FK506 treatment decreased proteinuria via a mechanism associated to a reduction in the foot-process fusion and desmin, and a recovery of synaptopodin and podocin...
2016: Scientific Reports
Angelika Bickel, Irene Koneth, Annette Enzler-Tschudy, Jörg Neuweiler, Lukas Flatz, Martin Früh
BACKGROUND: Pembrolizumab is an anti- Programmed Death 1 (PD-1) antibody approved in melanoma, non-small cell lung cancer and investigated in malignant pleural mesothelioma. The most frequent immunotherapy related autoimmune reactions include dermatitis, pneumonitis, colitis, hypophysitis, uveitis, hypothyreodism, hepatitis and interstitial nephritis. CASE PRESENTATION: We describe a 62-year old patient diagnosed with malignant pleural mesothelioma who experienced ten days after the second dose of third line therapy with pembrolizumab sudden onset of generalized edema including legs and eyelids and weight gain of 15 kg resulting from nephrotic syndrome and acute renal failure...
2016: BMC Cancer
A R Chaudhury, T Rajarajan, R Yousuf, E Fernando, A A Kurien
Some patients with systemic lupus erythematosus (SLE) present with sudden onset of nephrotic syndrome and biopsy findings may be of minimal change disease or focal segmental glomerulosclerosis with diffuse foot process effacement on electron microscopy but without significant immune deposits. This entity is termed lupus podocytopathy. Clinicians and renal pathologists need to be aware of this condition. Though steroid sensitive, it needs follow-up to recognize flare and class change, thereby optimizing therapy...
July 2016: Indian Journal of Nephrology
Syed Sajid Hussain Shah, Farkhanda Hafeez
OBJECTIVE: To compare the efficacy of tacrolimus versus cyclosporine (Calcineurin Inhibitors) in the management of childhood steroid-resistant nephritic syndrome (SRNS). STUDY DESIGN: Quasi-experimental study. PLACE AND DURATION OF STUDY: Department of Paediatric Nephrology at The Children's Hospital and Institute of Child Health, Lahore, from August 2014 to September 2015. METHODOLOGY: Patients of either gender aged 1 - 12 years, with the diagnosis of mesangioproliferative glomerulonephritis (MesangioPGN), focal segmental glomerulosclerosis (FSGS) or minimal-change disease (MCD) were included...
July 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
Josephine M Vos, Joshua Gustine, Helmut G Rennke, Zachary Hunter, Robert J Manning, Toni E Dubeau, Kirsten Meid, Monique C Minnema, Marie-Jose Kersten, Steven P Treon, Jorge J Castillo
The incidence and prognostic impact of nephropathy related to Waldenström macroglobulinaemia (WM) is currently unknown. We performed a retrospective study to assess biopsy-confirmed WM-related nephropathy in a cohort of 1391 WM patients seen at a single academic institution. A total of 44 cases were identified, the estimated cumulative incidence was 5·1% at 15 years. There was a wide variation in kidney pathology, some directly related to the WM: amyloidosis (n = 11, 25%), monoclonal-IgM deposition disease/cryoglobulinaemia (n = 10, 23%), lymphoplasmacytic lymphoma infiltration (n = 8, 18%), light-chain deposition disease (n = 4, 9%) and light-chain cast nephropathy (n = 4, 9%), and some probably related to the WM: thrombotic microangiopathy (TMA) (n = 3, 7%), minimal change disease (n = 2, 5%), membranous nephropathy (n = 1, 2%) and crystal-storing tubulopathy (n = 1, 2%)...
July 29, 2016: British Journal of Haematology
Xiao-Ling Niu, Sheng Hao, Ping Wang, Wei Zhang, Gui-Mei Guo, Ying Wu, Xin-Yu Kuang, Guang-Hua Zhu, Wen-Yan Huang
Rituximab (RTX) can be used in children with nephrotic syndrome, particularly in those with steroid-dependent nephrotic syndrome (SDNS). However, at present there is no unified standard of how to use RTX, with regard to the amount of doses and frequency, in children with nephrotic syndrome. The study aimed to investigate the therapeutic efficacy of a single dose of RTX in children with steroid-dependent minimal change nephrotic syndrome (SD-MCNS). The patients with biopsy-proven minimal change disease (MCD) and clinical features of SDNS received a single dose of RTX (375 mg/m(2))...
August 2016: Biomedical Reports
Fahad Edrees, Robert M Black, Laszlo Leb, Helmut Rennke
Kidney injury associated with lymphoproliferative disorders is rare, and the exact pathogenetic mechanisms behind it are still poorly understood. Glomerular involvement presenting as a nephrotic syndrome has been reported, usually secondary to membranoproliferative glomerulonephritis. We report a case of a 63-year-old male who presented with bilateral leg swelling due to nephrotic syndrome and acute kidney injury. A kidney biopsy showed minimal change disease with light chain deposition; however, no circulating light chains were present...
July 2016: Saudi Journal of Kidney Diseases and Transplantation
Edward J Filippone, Shirley J Dopson, Denise M Rivers, Rebeca D Monk, Suneel M Udani, Golriz Jafari, Solomon C Huang, Arafat Melhem, Bassim Assioun, Paul G Schmitz
BACKGROUND: Adrenocorticotropic hormone is being increasingly studied for treatment of various glomerulopathies, most notably membranous nephropathy. Less data are available regarding its use in idiopathic nephrotic syndrome (INS) secondary to minimal change disease (MCD) or focal segmental glomerulosclerosis (FSGS). We report here our experience with H.P. Acthar(®) Gel (repository corticotropin injection) as first-line or subsequent therapy in patients with INS. METHODS: Data were taken from three patients with MCD and ten patients with FSGS from around the US, who were treated with Acthar Gel as initial or subsequent therapy...
2016: International Medical Case Reports Journal
Shohei Fukunaga, Kei Matsumoto, Yudo Tanno, Ko Sudo, Genta Uehara, Hitoshi Miyasato, Nobuo Tsuboi, Makoto Ogura, Takashi Yokoo
A 37-year-old man diagnosed with minimal change nephrotic syndrome at the age of 17, was admitted to hospital with leg pain. He had relapsed nephrotic syndrome and thromboembolism of the right femoral and middle cerebral arteries. He was treated with steroid pulse therapy and thrombectomy. Right lower extremity necrosis developed and a below-knee amputation was performed. His nephrotic syndrome and the amputated lower extremity necrosis subsequently improved. Arterial thrombosis is a rare complication of nephrotic syndrome; however, it is a severe complication...
2016: Nihon Jinzo Gakkai Shi
Gabriel Cara-Fuentes, William L Clapp, Richard J Johnson, Eduardo H Garin
Minimal change disease (MCD) is the most common type of nephrotic syndrome in children and adolescents. The pathogenesis of proteinuria in this condition is currently being reassessed. Following the Shalhoub hypothesis, most efforts have been placed on identifying the putative circulating factor, but recent advancement in podocyte biology has focused attention on the molecular changes at the glomerular capillary wall, which could explain the mechanism of proteinuria in MCD. This report critically reviews current knowledge on the different postulated mechanisms at the glomerular capillary wall level for increased permeability to plasma proteins in MCD...
July 6, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Massimiliano Bergallo, Elisa Loiacono, Stefano Gambarino, Paola Montanari, Ilaria Galliano, Rosanna Coppo
BACKGROUND: Idiopathic nephrotic syndrome (INS) in children is a continuum of clinical disorders characterized by severe proteinuria, dyslipidemia, hypoalbuminemia, and hypercoagulability. It has been hypothesized that TLRs in Peripheral blood mononuclear cell might be involved in INS disease. Infections appear to be involved in the onset of INS. The aim of this study was to investigate the expression and function of TLRs in PBMC of INS patients and the relation with two Human endogenous retrovirus (HERV), K and W expression...
June 29, 2016: Minerva Urologica e Nefrologica, the Italian Journal of Urology and Nephrology
Evangéline Pillebout, Jérôme Vérine
IgA nephropathy is the most common form of primary glomerulonephritis worldwide and an important cause of chronic kidney disease and end-stage kidney failure. Its pathophysiology remains in part unsolved but it is recognized as an immune complex disease. Recent years have brought progress in the field through the discovery of several genetic susceptibility loci and the formulation of the multi-hit pathogenesis model. Presentation, clinical course and histology can be extremely variable, making any histological classification still difficult...
July 2016: Néphrologie & Thérapeutique
Seong Heon Kim, Se Jin Park, Kyoung Hee Han, Andreas Kronbichler, Moin A Saleem, Jun Oh, Beom Jin Lim, Jae Il Shin
Idiopathic nephrotic syndrome (INS) in children is characterized by massive proteinuria and hypoalbuminemia. Minimal change nephrotic syndrome (MCNS) is the most common form of INS in children. The pathogenesis of MCNS still remains unclear, however, several hypotheses have been recently proposed. For several decades, MCNS has been considered a T-cell disorder, which causes the impairment of the glomerular filtration barrier with the release of different circulating factors. Increased levels of several cytokines are also suggested...
May 2016: Korean Journal of Pediatrics
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"