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Nephrotic syndrome

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https://www.readbyqxmd.com/read/29219816/nephrotic-syndrome-due-to-lupus-like-glomerulonephritis-in-an-hiv-positive-patient
#1
J S Wiegersma, C F M Franssen, A Diepstra
Lupus nephritis, a well-known complication in systemic lupus erythematosus, is characterised by a proliferative glomerulonephritis or membranous nephropathy along with a full-house immunofluorescence pattern on renal biopsy. There are very few exceptions in which similar histopathological findings are present, but case reports show that an increasing number of HIV-positive patients (mostly black Africans, but also white patients) have HIV-immune complex disease (HIVICK), which can mimic lupus nephritis. Lupus-like HIVICK is treated differently than 'true' lupus nephritis, so distinction is warranted...
November 2017: Netherlands Journal of Medicine
https://www.readbyqxmd.com/read/29218062/preliminary-study-regarding-the-association-between-tumor-necrosis-factor-alpha-gene-polymorphisms-and-childhood-idiopathic-nephrotic-syndrome-in-romanian-pediatric-patients
#2
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
https://www.readbyqxmd.com/read/29217878/a-randomized-controlled-trial-of-intravenous-versus-oral-cyclophosphamide-in-steroid-resistant-nephrotic-syndrome-in-children
#3
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
https://www.readbyqxmd.com/read/29217769/structural-insights-into-ankyrin-repeat-mediated-recognition-of-the-kinesin-motor-protein-kif21a-by-kank1-a-scaffold-protein-in-focal-adhesion
#4
Wenfei Pan, Kang Sun, Kun Tang, Qingpin Xiao, Chenxue Ma, Cong Yu, Zhiyi Wei
Kidney ankyrin repeat-containing proteins (KANK1/2/3/4) belong to a family of scaffold proteins, playing critical roles in cytoskeleton organization, cell polarity and migration. Mutations in KANK proteins are implicated in cancers and genetic diseases, such as nephrotic syndrome. KANK proteins can bind various target proteins through different protein regions, including a highly conserved ankyrin repeat domain (ANKRD). However, the molecular basis for target recognition by the ANKRD remains elusive. In this study, we solved a high-resolution crystal structure of the ANKRD of KANK1 in complex with a short sequence of the motor protein kinesin family member 21A (KIF21A), revealing that the highly specific target-binding mode of the ANKRD involves combinatorial use of two interfaces...
December 7, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29217372/acute-peritoneal-dialysis-in-neonatal-intensive-care-unit-an-8-year-experience-of-a-referral-hospital
#5
Aslihan Kara, Metin Kaya Gurgoze, Mustafa Aydin, Erdal Taskin, Unal Bakal, Aysen Orman
BACKGROUND: The aim of present study was to evaluate the indications, complications and outcomes of acute peritoneal dialysis (APD) in neonates at a referral university hospital during the previous 8 years. METHODS: This retrospective analysis included a total of 52 newborn infants who underwent APD in a neonatal intensive care unit between January 2008 and March 2016. Demographic, clinical, laboratory and microbiological data were extracted from patients' medical files...
November 16, 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/29214407/clinical-features-and-pathogenesis-of-membranoproliferative-glomerulonephritis-a-nationwide-analysis-of-the-japan-renal-biopsy-registry-from-2007-to-2015
#6
Naoki Nakagawa, Naoyuki Hasebe, Motoshi Hattori, Michio Nagata, Hitoshi Yokoyama, Hiroshi Sato, Hitoshi Sugiyama, Akira Shimizu, Yoshitaka Isaka, Shoichi Maruyama, Ichiei Narita
BACKGROUND: The incidence and age distribution of membranoproliferative glomerulonephritis (MPGN) vary throughout the world by race and ethnicity. We sought to evaluate the clinical features, pathogenesis, and age distribution of MPGN among a large nationwide data from the Japan Renal Biopsy Registry (J-RBR). METHODS: A cross-sectional survey of 593 patients with MPGN (types I and III) registered in the J-RBR between 2007 and 2015 was conducted. Clinical parameters, and laboratory findings at diagnosis were compared between children (< 20 years), adults (20-64 years), and elderly patients (≥ 65 years)...
December 6, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/29212948/elevated-urinary-creld2-is-associated-with-endoplasmic-reticulum-stress-mediated-kidney-disease
#7
Yeawon Kim, Sun-Ji Park, Scott R Manson, Carlos Af Molina, Kendrah Kidd, Heather Thiessen-Philbrook, Rebecca J Perry, Helen Liapis, Stanislav Kmoch, Chirag R Parikh, Anthony J Bleyer, Ying Maggie Chen
ER stress has emerged as a signaling platform underlying the pathogenesis of various kidney diseases. Thus, there is an urgent need to develop ER stress biomarkers in the incipient stages of ER stress-mediated kidney disease, when a kidney biopsy is not yet clinically indicated, for early therapeutic intervention. Cysteine-rich with EGF-like domains 2 (CRELD2) is a newly identified protein that is induced and secreted under ER stress. For the first time to our knowledge, we demonstrate that CRELD2 can serve as a sensitive urinary biomarker for detecting ER stress in podocytes or renal tubular cells in murine models of podocyte ER stress-induced nephrotic syndrome and tunicamycin- or ischemia-reperfusion-induced acute kidney injury (AKI), respectively...
December 7, 2017: JCI Insight
https://www.readbyqxmd.com/read/29212162/rituximab-treatment-in-adults-with-refractory-minimal-change-disease-or-focal-segmental-glomerulosclerosis
#8
Hong Ren, Li Lin, Pingyan Shen, Xiao Li, Jingyuan Xie, Xiaoxia Pan, Wen Zhang, Nan Chen
Rituximab (RTX) may benefit patients with glomerular disease who suffer from focal segmental glomerular sclerosis (FSGS) or minimal change disease (MCD). Here, we have described our experience treating 6 FSGS and 9 MCD patients with steroid-dependent/refractory nephrotic syndrome (NS) with RTX. Patients received RTX (375 mg/m2) intravenously on days 1, 8, 23, and 29. During a median follow-up of 8 months (range, 3-36 months) after RTX administration, all patients achieved complete or partial remission. Relapses decreased by approximately 30-fold compared with the year preceding RTX treatment, and an 89...
November 7, 2017: Oncotarget
https://www.readbyqxmd.com/read/29207898/nr3c1-gene-polymorphisms-are-associated-with-steroid-resistance-in-patients-with-primary-nephrotic-syndrome
#9
Jie Liu, Zan Wan, Qianqian Song, Zhi Li, Yijing He, Yongjun Tang, Wanying Xie, Yanyun Xie, Jun Zhang
AIM: The aim of this study was to investigate the role of SNPs of genes involved in the glucocorticoid pathway in the development of steroid resistance in patients with primary nephrotic syndrome. METHODS: Sequenom MassARRAY method was used to sequence 25 SNP genotypes in 154 patients. The frequency distribution of the genotypes was compared between patients with steroid-sensitive nephrotic syndrome and those with steroid-resistant nephrotic syndrome. RESULTS: NR3C1 rs6196 G allele carriers had a decreased risk of steroid resistance compared with that of the A allele carriers...
December 6, 2017: Pharmacogenomics
https://www.readbyqxmd.com/read/29207799/change-in-bone-mineral-density-and-role-of-vitamin-d-and-calcium-supplementation-during-treatment-of-first-episode-nephrotic-syndrome
#10
Virendra Kumar Yadav, Shobha Sharma, Pradeep Kumar Debata, Seema Patel, Bhaskar C Kabi, Kailash Chandra Aggrawal
Introduction: Nephrotic Syndrome (NS) is one of the common illnesses in childhood. Oral glucocorticoids are mainstay of treatment, which are known to cause various short term and long term adverse effects including osteoporosis. Children with first episode and infrequent relapsing NS are not prescribed Calcium (Ca) and Vitamin D (VitD) supplements routinely. Aim: To observe change in Bone Mineral Density (BMD) in children during first episode of NS and role of VitD and Ca supplementation during the treatment of first episode of NS...
September 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/29204099/tuberculosis-infection-in-children-with-proteinuria-nephrotic-syndrome
#11
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
https://www.readbyqxmd.com/read/29204098/effectiveness-of-rituximab-in-nephrotic-syndrome-treatment
#12
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
https://www.readbyqxmd.com/read/29197864/biopsy-free-prediction-of-pathologic-type-of-primary-nephrotic-syndrome-using-a-machine-learning-algorithm
#13
Cuifang Li, Zhijiang Yao, Minfeng Zhu, Ben Lu, Hui Xu
BACKGROUND/AIMS: Renal biopsy is the gold standard to determine the pathologic type of primary nephrotic syndrome, which is critical for diagnosis, choice of treatment and evaluation of prognosis. However, in some cases, renal biopsy cannot be performed. METHODS: To explore the possibility of predicting the histology type of primary nephrotic syndrome without the need for biopsy, we trained and validated a machine learning algorithm using data from 222 patients with biopsy-confirmed primary nephrotic syndrome treated at our hospital between May 2008 and January 2016...
December 4, 2017: Kidney & Blood Pressure Research
https://www.readbyqxmd.com/read/29194833/mutations-in-coq8b-adck4-found-in-patients-with-steroid-resistant-nephrotic-syndrome-alter-coq8b-function
#14
Luis Vazquez Fonseca, Mara Doimo, Cristina Calderan, Maria Andrea Desbats, Manuel J Acosta, Cristina Cerqua, Matteo Cassina, Shazia Ashraf, Friedhelm Hildebrandt, Geppo Sartori, Placido Navas, Eva Trevisson, Leonardo Salviati
Mutations in COQ8B cause steroid-resistant nephrotic syndrome with variable neurological involvement. In yeast, COQ8 encodes a protein required for coenzyme Q (CoQ) biosynthesis, whose precise role is not clear. Humans harbor two paralogue genes: COQ8A and COQ8B (previously termed ADCK3 and ADCK4). We have found that COQ8B is a mitochondrial matrix protein peripherally associated with the inner membrane. COQ8B can complement a ΔCOQ8 yeast strain when its mitochondrial targeting sequence (MTS) is replaced by a yeast MTS...
December 1, 2017: Human Mutation
https://www.readbyqxmd.com/read/29191574/-collapsing-focal-segmental-glomerulosclerosis-induced-by-cytomegalovirus-a%C3%A2-case-report
#15
Clarisse Grèze, Cyril Garrouste, Jean-Louis Kemeny, Carole Philipponnet, Julien Aniort, Anne-Élisabeth Heng
Focal segmental glomerulosclerosis (FSGS) is a common cause of nephrotic syndrome in child and adult. The collapsing forms are of poor renal prognosis and are usually secondary to viral infections with, first and foremost, the human immunodeficiency virus. Among other viral etiologies, cytomegalovirus (CMV) is an uncommon cause. We report a case of a 32years-old patient with collapsing focal segmental glomerulosclerosis induced by cytomegalovirus with initial acute renal failure and proteinuria at 12.4g/24h...
November 27, 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/29190608/seropositivity-of-rheumatoid-arthritis-specific-tests-in-a-patient-with-nephrotic-syndrome-successful-treatment-with-rituximab
#16
Alireza Mirzaei, Yousef Ataeipoor, Mojgan Asgari, Mozhdeh Zabihiyeganeh
Presentation of rheumatoid arthritis (RA) with renal complications is very rare without articular symptoms. We here report a case of a 23-year-old woman, presenting with the edema of the extremities, no relevant previous medical history, and the features of acute tubular injury in her percutaneous kidney biopsy. Following the incidental notification of a positive rheumatoid factor test, other immunologic tests including anticyclic citrullinated peptide and antimutated citrullinated vimentin were performed, the positive results of which favored the diagnosis of RA...
November 2017: Iranian Journal of Kidney Diseases
https://www.readbyqxmd.com/read/29190601/cytokine-gene-polymorphism-in-children-with-idiopathic-nephrotic-syndrome
#17
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
https://www.readbyqxmd.com/read/29187369/pharmacological-and-genetic-inhibition-of-downstream-targets-of-p38-mapk-in-experimental-nephrotic-syndrome
#18
Xiaojing Nie, Melinda A Chanley, Ruma Pengal, David B Thomas, Shipra Agrawal, William E Smoyer
The p38MAPK pathway plays a crucial role in various glomerulopathies, with activation being associated with disease and inhibition being associated with disease amelioration. We hypothesized that the downstream targets of p38MAPK, MK2 and/or MK3, play an important role in mediating injury in experimental nephrotic syndrome via their actions on their downstream substrates HSPB1 and COX-2. To test this hypothesis, the effects of both pharmacological and genetic inhibition of MK2 and MK3 were examined in mouse Adriamycin (ADR) and rat puromycin aminonucleoside (PAN) nephropathy models...
November 29, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/29185126/long-term-outcome-of-congenital-nephrotic-syndrome-after-kidney-transplantation-in-japan
#19
Yuko Hamasaki, Masaki Muramatsu, Riku Hamada, Kenji Ishikura, Hiroshi Hataya, Hiroyuki Satou, Masataka Honda, Koichi Nakanishi, Seiichiro Shishido
BACKGROUND: Congenital nephrotic syndrome is difficult to manage, particularly the Finnish type (CNF), with patients experiencing severe edema, sepsis and thrombosis before kidney transplantation. Further, nephrosis and thrombosis remain problematic after transplantation. METHODS: Of 22 CNF patients managed at our hospital, 14 who underwent kidney transplantation were retrospectively studied. CNF was diagnosed according to standard criteria. RESULTS: The study population consisted of 3 males and 11 females...
November 28, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/29184425/obesity-and-morbid-obesity-associated-with-higher-odds-of-hypoalbuminemia-in-adults-without-liver-disease-or-renal-failure
#20
Rana H Mosli, Hala H Mosli
Background and objective: Studies are needed in order to inform recommendations for interpreting albumin levels among obese individuals without known medical conditions associated with hypoalbuminemia. The objective of this study was to examine the association of obese and morbidly obese status with hypoalbuminemia, while adjusting for age, sex, diabetes, prediabetes, diabetic nephropathy, and nephrotic syndrome. Patients and methods: Retrospective data collection from adult patients presenting to the outpatient Endocrinology and Metabolism Clinic was performed between January 2015 and December 2015...
2017: Diabetes, Metabolic Syndrome and Obesity: Targets and Therapy
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