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nephrotic syndrome review

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https://www.readbyqxmd.com/read/28078995/sphingolipids-in-genetic-and-acquired-forms-of-chronic-kidney-diseases
#1
Norishi Ueda
Sphingolipids (SLs) regulate apoptosis, proliferation, and stress response. SLs, including ceramide, glycosphingolipids (glucosylceramide, lactosylceramide, and gangliosides) and sphingosine-1-phosphate (S1P), play a role in the pathogenesis and progression of genetic (lysosomal storage disease, congenital nephrotic syndrome and polycystic kidney disease) and non-genetic forms of chronic kidney diseases (CKDs). SLs metabolism defects promote complications (cardiovascular events, etc.) via oxidant stress in CKDs...
January 12, 2017: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/28072731/focal-segmental-glomerulosclerosis-lagged-behind-the-onset-of-rheumatoid-arthritis-by-7-years-a-case-report-and-literature-review
#2
Yang Liu, Hong-Yan Wen, Li-Hua Wang, Chen Wang
INTRODUCTION: The co-existence of focal segmental glomerulosclerosis (FSGS) and rheumatoid arthritis (RA), presenting either together or in succession, is very rare. A variety of histopathological features in the clinical renal disease may occur in RA. Only 8 studies have previously reported this poorly understood connection. CLINICAL FINDINGS/DIAGNOSES: A case of a 54-year-old male with RA lasting for more than 7 years developed cheirarthritis as the first signs...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28065518/membranous-nephropathy-integrating-basic-science-into-improved-clinical-management
#3
REVIEW
Daniel C Cattran, Paul E Brenchley
Idiopathic membranous nephropathy (INM) remains a common cause of the nephrotic syndrome in adults. The autoimmune nature of IMN was clearly delineated in 2009 with the identification of the glomerular-deposited IgG to be a podocyte receptor, phospholipase A2 receptor (PLA2R) in 70% to 75% of cases. This anti-PLA2R autoantibody, predominantly the IgG4 subclass, has been quantitated in serum using an enzyme-linked immunosorbent assay and has been used to aid diagnosis and monitor response to immunosuppressive therapy...
January 5, 2017: Kidney International
https://www.readbyqxmd.com/read/28056871/lung-adenocarcinoma-expressing-receptor-for-advanced-glycation-end-products-with-primary-systemic-al-amyloidosis-a-case-report-and-literature-review
#4
Shouichi Okamoto, Shinsaku Togo, Ichiro Nagata, Kazue Shimizu, Yoshika Koinuma, Yukiko Namba, Jun Ito, Toshimasa Uekusa, Kazuhisa Takahashi
BACKGROUND: Receptor for advanced glycation end-products (RAGE), a receptor for amyloids, is constitutively expressed in lungs and generally observed to be downregulated in lung cancer tissues. However, increasing levels of RAGE or serum amyloids is associated with poor outcome in lung cancer patients. We report a rare case of primary systemic amyloid light-chain (AL) amyloidosis in biopsy-proven multiple organs with early-stage non-small cell lung cancer (NSCLC) that displayed strong staining for RAGE in the tumour tissue...
January 5, 2017: BMC Cancer
https://www.readbyqxmd.com/read/27999949/anemia-in-nephrotic-syndrome-approach-to-evaluation-and-treatment
#5
Franca Iorember, Diego Aviles
Nephrotic syndrome is one of the most common glomerular diseases that affect in children. Complications may occur in nephrotic syndrome as a result of the disease itself as well as its treatment. Most of these complications result from excessive urinary protein losses, and control of proteinuria is the most effective treatment strategy. Anemia is one of the many complications seen in patients with persistent nephrotic syndrome and may occur as a result of excessive urinary losses of iron, transferrin, erythropoietin, transcobalamin and/or metals...
December 21, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27984544/bilateral-serous-macular-detachment-in-a-patient-with-nephrotic-syndrome
#6
Ayse D Bilge, Sevil A Yaylali, Sara Yavuz, İlke B Simsek
PURPOSE: The purpose of this study was to report a case of a woman with nephrotic syndrome who presented with blurred vision because of bilateral serous macular detachment. METHODS: Case report and literature review. RESULTS: A 55-year-old woman with a history of essential hypertension, diabetes, and nephrotic syndrome was presented with blurred vision in both eyes. Her fluorescein angiography revealed dye leakage in the early and subretinal pooling in the late phases, and optical coherence tomography scans confirmed the presence of subretinal fluid in the subfovel area...
December 14, 2016: Retinal Cases & Brief Reports
https://www.readbyqxmd.com/read/27924730/congenital-malformations-attributed-to-prenatal-exposure-to-cyclophosphamide
#7
Padmanabhan Rengasamy
Cyclophosphamide (CPA) remains one of the most widely prescribed anticancer drugs. It is also used in the treatment of rheumatoid arthritis, childhood nephrotic syndrome and systemic lupus erythematosus. It is a potent immunosuppressive agent. It is commonly used in blood and bone marrow transplantation. With the growing trend among women postponing childbearing, the number of women who are diagnosed with breast cancer is also increasing thus escalating the chances of exposure of the unborn child to antineoplastic drugs...
December 6, 2016: Anti-cancer Agents in Medicinal Chemistry
https://www.readbyqxmd.com/read/27872827/zinc-supplementation-as-an-adjunct-to-standard-therapy-in-childhood-nephrotic-syndrome-a-systematic-review
#8
Girish Chandra Bhatt, Shikha Jain, Rashmi Ranjan Das
AIM: To evaluate the role of zinc as add on treatment to the "recommended treatment" of nephrotic syndrome (NS) in children. METHODS: All the published literature through the major databases including Medline/Pubmed, Embase, and Google Scholar were searched till 31(st) December 2015. Reference lists from the articles were reviewed to identify additional pertinent articles. Retrieved papers concerning the role of zinc in childhood NS were reviewed by the authors, and the data were extracted using a standardized data collection tool...
November 8, 2016: World Journal of Clinical Pediatrics
https://www.readbyqxmd.com/read/27814935/diuretic-resistance
#9
Ewout J Hoorn, David H Ellison
Diuretic resistance is defined as a failure to achieve the therapeutically desired reduction in edema despite a full dose of diuretic. The causes of diuretic resistance include poor adherence to drug therapy or dietary sodium restriction, pharmacokinetic issues, and compensatory increases in sodium reabsorption in nephron sites that are not blocked by the diuretic. To illustrate the pathophysiology and management of diuretic resistance, we describe a patient with nephrotic syndrome. This patient presented with generalized pitting edema and weight gain despite the use of oral loop diuretics...
January 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/27806747/children-with-steroid-resistant-nephrotic-syndrome-long-term-outcomes-of-sequential-steroid-therapy
#10
Hui Zhang, Zheng Wang, Li Qun Dong, Yan Nan Guo
OBJECTIVE: This study aimed to investigate the long-term outcomes in children with steroid-resistant nephrotic syndrome (SRNS), who received methylprednisolone pulse therapy (MPT)-based sequential steroid therapy. In particular, we aimed to observe whether these patients had a high risk of adverse events. METHODS: We conducted a retrospective study over a 5-year period. The long-term outcomes for children with SRNS receiving sequential therapy were observed. RESULTS: Sixty-three children were diagnosed with SRNS and underwent MPT-based sequential steroid therapy...
September 2016: Biomedical and Environmental Sciences: BES
https://www.readbyqxmd.com/read/27783158/steroid-resistant-nephrotic-syndrome-a-persistent-challenge-for-pediatric-nephrology
#11
Samriti Dogra, Frederick Kaskel
Steroid-resistant nephrotic syndrome remains a challenge to treat, but various efforts are underway to better understand the pathogenesis and improve patient outcomes. This review provides an update on the newer advances in understanding the molecular etiologies for a variety of podocyte abnormalities, potential circulating factors that may initiate and sustain the steroid-resistant state, genetic mutations, and precision medicine treatment modalities in this continuously perplexing disorder.
October 26, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27761562/diverse-etiology-of-hyperlipidemia-among-hospitalized-children-in-western-region-of-saudi-arabia
#12
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
https://www.readbyqxmd.com/read/27752010/a-clinicopathologic-study-of-glomerular-disease-a-single-center-five-year-retrospective-study-from-northwest-india
#13
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
https://www.readbyqxmd.com/read/27738599/henoch-schonlein-purpura-in-children-hospitalized-at-a-tertiary-hospital-during-2004-2015-in-korea-epidemiology-and-clinical-management
#14
Yong Hee Lee, Yu Bin Kim, Ja Wook Koo, Ju-Young Chung
PURPOSE: To investigate the epidemiology, clinical manifestations, investigations and management, and prognosis of patients with Henoch-Schonlein purpura (HSP). METHODS: We performed a retrospective review of 212 HSP patients under the age of 18 years who were admitted to Inje University Sanggye Paik Hospital between 2004 and 2015. RESULTS: The mean age of the HSP patients was 6.93 years, and the ratio of boys to girls was 1.23:1. HSP occurred most frequently in the winter (33...
September 2016: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/27726125/interventions-for-idiopathic-steroid-resistant-nephrotic-syndrome-in-children
#15
REVIEW
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
https://www.readbyqxmd.com/read/27699035/membranous-nephropathy-associated-with-malignant-pleural-mesothelioma-in-an-adult-patient-a-case-report
#16
Xinyu Pu, Yanna Dou, Dong Liu, Shan Lu, Songxia Quan, Xiaoxue Zhang, Shuang Ma, Zhanzheng Zhao
A 23-year-old man presented to our hospital with membranous nephropathy and received a detailed examination, including pleural biopsy, due to a feeling of chest oppression. The result of the pleural biopsy was malignant pleural mesothelioma. However, the patient did not have a history of asbestos or tobacco exposure. A review of the English literature identified only 7 reported cases of concomitant malignant mesothelioma and nephrotic syndrome. Furthermore, among the 7 cases reviewed, 6 had a history of asbestos exposure, 1 had a history of prolonged tobacco exposure and in only 1 case the renal pathology results revealed the presence of membranous nephropathy...
October 2016: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/27682967/idiopathic-nephrotic-syndrome-the-ebv-hypothesis
#17
REVIEW
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 Epstein-Barr Virus (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...
January 2017: Pediatric Research
https://www.readbyqxmd.com/read/27677978/use-of-a-low-dose-prednisolone-regimen-to-treat-a-relapse-of-steroid-sensitive-nephrotic-syndrome-in-children
#18
Karnika Raja, Ami Parikh, Hazel Webb, Daljit Hothi
BACKGROUND: Relapses of nephrotic syndrome are common and are treated with a course of prednisolone (2 mg/kg/day or 60 mg/m(2)/day). This is associated with major adverse effects including diabetes, weight gain, hypertension and behavioural problems. This study is a retrospective review examining the success of treating relapses in steroid-sensitive nephrotic syndrome (SSNS) with low-dose prednisolone and the consequences on subsequent relapse rates. Furthermore, a follow-up study looked at the side-effect profile during treatment with high- versus low-dose prednisolone...
January 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27651302/primary-focal-segmental-glomerulosclerosis-mirnas-and-targeted-therapies
#19
REVIEW
Johannes Leierer, Gert Mayer, Andreas Kronbichler
BACKGROUND: Primary focal segmental glomerulosclerosis (FSGS) is a common cause of nephrotic syndrome. AIMS: The pathogenic steps leading to primary FSGS are still obscure, although evidence suggests that circulatory factor(s) are involved in the onset of disease. RESULTS: Recent technical advances allow the analysis of miRNA expression in tissues and body fluids, leading to reports of miRNAs involved in the molecular mechanisms of FSGS-aetiopathogenesis...
November 2016: European Journal of Clinical Investigation
https://www.readbyqxmd.com/read/27651195/-clinical-characteristics-and-etiology-of-children-with-hypereosinophilia
#20
Chen Xiaohong, X U Yiping, L U Meiping
OBJECTIVE: To analyze the clinical characteristics and etiology of hypereosinophilia in children. METHODS: Clinical data of 88 children with hypereosinophilia admitted in Children's Hospital of Zhejiang University School of Medicine during April 2009 and May 2015 were retrospectively reviewed. The clinical manifestations, etiologies, and the correlation of disease severity with different etiologies were analyzed. RESULTS: The main clinical manifestations were fever, abdominal pain, cough and/or tachypnea, skin rash, hemafecia and diarrhea, which were observed in 19 (21...
May 25, 2016: Zhejiang da Xue Xue Bao. Yi Xue Ban, Journal of Zhejiang University. Medical Sciences
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