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idiopathic nephrotic syndrome

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https://www.readbyqxmd.com/read/28914167/nephrotic-syndrome-in-infants-and-children-pathophysiology-and-management
#1
Mallory L Downie, Claire Gallibois, Rulan S Parekh, Damien G Noone
Nephrotic syndrome is defined by nephrotic-range proteinuria (≥40 mg/m(2)/hour or urine protein/creatinine ratio ≥200 mg/mL or 3+ protein on urine dipstick), hypoalbuminaemia (<25 g/L) and oedema. This review focuses on the classification, epidemiology, pathophysiology, management strategies and prognosis of idiopathic nephrotic syndrome of childhood, and includes a brief overview of the congenital forms.
September 15, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28898290/comparative-effectiveness-and-tolerance-of-immunosuppressive-treatments-for-idiopathic-membranous-nephropathy-a-network-meta-analysis
#2
Song Ren, Ying Wang, Li Xian, Tadashi Toyama, Meg Jardine, Guisen Li, Vlado Perkovic, Daqing Hong
BACKGROUND: Immunosuppressive agents in general are shown to prevent renal progression and all-cause mortality in idiopathic membranous nephropathy (IMN) patients with nephrotic syndrome. However, the efficacy and safety of different immunosuppressive treatments have not been systematic assessed and compared. A network meta-analysis was performed to compare different immunosuppressive treatment in IMN. METHODS: Cochrane library, MEDLINE, EMBASE and trial register system were searched for randomized controlled trials reporting the treatments for IMN to May 3, 2016...
2017: PloS One
https://www.readbyqxmd.com/read/28891307/a-comprehensive-narrative-review-of-diagnostic-biomarkers-in-human-primary-membranous-nephropathy
#3
Shiva Kalantari, Mohsen Nafar
Membranous nephropathy (MN) is relatively major cause of nephrotic syndrome in adults which is recognized as an organ-specific autoimmune disease. The etiology of most cases is idiopathic, whereas the secondary MN is caused by systemic autoimmune diseases, infections, medications and malignancies. The idiopathic disease is developed by the formation of sub-epithelial immune complex deposits most likely due to binding the circulating auto-antibodies to intrinsic antigen on podocytes. The major auto antibody is the anti-phospholipase A2 receptor (anti-PLA2R), however, it is not enough sensitive...
September 11, 2017: Biomarkers in Medicine
https://www.readbyqxmd.com/read/28882100/low-dose-cyclosporine-in-treatment-of-membranous-nephropathy-with-nephrotic-syndrome-effectiveness-and-renal-safety
#4
Xiaojuan Yu, Lin Ruan, Zhen Qu, Zhao Cui, Yimiao Zhang, Xin Wang, Liqiang Meng, Xiaojing Liu, Fang Wang, Ying Zhang, Gang Liu, Li Yang
BACKGROUND: To observe effectiveness and renal safety of long-term low-dose cyclosporine in idiopathic membranous nephropathy (IMN). METHODS: Sixty-eight patients were enrolled in this prospective cohort study. Renal endpoint was defined as a decrease in eGFR ≥50% from baseline and a development of eGFR ≤60 ml/min/1.73m(2). RESULTS: A cyclosporine dose of 2.0 ± 0.5 mg/kg/d and a prednisone of 0.3 ± 0.2 mg/kg/d were prescribed...
November 2017: Renal Failure
https://www.readbyqxmd.com/read/28879428/difficult-to-treat-idiopathic-nephrotic-syndrome-established-drugs-open-questions-and-future-options
#5
REVIEW
Markus J Kemper, Lisa Valentin, Michael van Husen
The idiopathic nephrotic syndrome in childhood can be classified according to the International Study of Kidney Disease in Children (ISKDC) based on the response to steroids. Typically, steroid-sensitive nephrotic syndrome (SSNS) is characterised by minimal changes in disease (MCD) histology, whereas in steroid-resistant nephrotic syndrome (SRNS) focal segmental glomerulosclerosis (FSGS) is the most prevalent lesion. Patients with SSNS may develop frequent relapses and/or steroid dependency, which can be difficult to treat...
September 6, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28878838/correlation-between-idiopathic-nephrotic-syndrome-and-atopy-in-children-short-review
#6
Elena Camelia Berghea, Mihaela Balgradean, Ionela-Loredana Popa
The idiopathic nephrotic syndrome is a common chronic kidney diseases in children defined by the association of massive proteinuria and hypoalbuminemia in a relapsing/remission course, with histological aspect of minimal changes (also called minimal change disease) in the majority of the cases, but its pathogenesis remains not very well known. Clinical and immunological studies have consistently shown a relationship between atopic diathesis, immunoglobulin E and cytokines involved in immunoglobulin E synthesis and idiopathic nephrotic syndrome...
January 2017: Mædica
https://www.readbyqxmd.com/read/28877834/clinicopathological-features-of-idiopathic-membranous-nephropathy-in-33-adolescents
#7
Chao Li, Hang Li, Yu-Bing Wen, Jia-Ning Li, Wei-Feng Lin, Jian-Fang Cai, Lin Duan, Yan Li, Xue-Mei Li, Xue-Wang Li
Objective To investigate the clinicopathological features and prognosis of idiopathic membranous nephropathy(IMN)in adolescents. Methods This was a retrospective study on IMN patients hospitalized between June 2012 and December 2014,and a total of 33 IMN patients aged between 13 and 24 years old were enrolled in the study.Meanwhile,33 IMN patients aged more than 24 years old were selected randomly as control group during the same period.Diagnosis was confirmed by renal biopsy,and the secondary causes of membranous nephropathy were ruled out...
August 20, 2017: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
https://www.readbyqxmd.com/read/28870123/bayesian-treatment-comparison-using-parametric-mixture-priors-computed-from-elicited-histograms
#8
Peter F Thall, Moreno Ursino, Véronique Baudouin, Corinne Alberti, Sarah Zohar
A Bayesian methodology is proposed for constructing a parametric prior on two treatment effect parameters, based on graphical information elicited from a group of expert physicians. The motivating application is a 70-patient randomized trial to compare two treatments for idiopathic nephrotic syndrome in children. The methodology relies on histograms of the treatment parameters constructed manually by each physician, applying the method of Johnson et al. (2010). For each physician, a marginal prior for each treatment parameter characterized by location and precision hyperparameters is fit to the elicited histogram...
January 1, 2017: Statistical Methods in Medical Research
https://www.readbyqxmd.com/read/28869958/management-of-nephrotic-syndrome-a-case-report-from-lao-pdr
#9
Phanekham Souvannamethy
We report the case of a 23-year-old woman with a 2-week history of swelling around the eyes and both legs, and generalized body swelling. She had a history of chronic constipation and poor diet but no fever, recent illnesses, or hematuria. Examination revealed bilateral pedal edema and mild ascites. Laboratory investigations showed low hemoglobin 79 g/L, low mean corpuscular volume 53 fL, thrombocytosis 973 × 109/L, and marked hypochromia and microcytosis, with low iron and ferritin. She had hypoalbuminemia and reduced serum protein (albumin 1...
2017: Blood Purification
https://www.readbyqxmd.com/read/28868292/hereditary-podocytopathies-in-adults-the-next-generation
#10
REVIEW
Olivia Boyer, Guillaume Dorval, Aude Servais
Idiopathic nephrotic syndrome may have two underlying mechanisms: either (1) an alteration of the immune system resulting in the production of a putative circulating factor of glomerular permeability; or (2) mutations in the structural genes of the glomerular filtration barrier in which case patients are typically multidrug resistant and do not recur after transplantation. The latter forms have been recently recognized as "hereditary podocytopathies." In the past few years, positional cloning approaches that allow the identification of gene mutations underlying diseases whose pathophysiology is unknown and animal models have helped decipher the pathophysiological mechanisms of the glomerular filtration process...
July 2017: Kidney Diseases
https://www.readbyqxmd.com/read/28856214/the-roche-total-mycophenolic-acid%C3%A2-assay-an-application-protocol-for-the-abx-pentra-400-analyzer-and-comparison-with-lc-ms-in-children-with-idiopathic-nephrotic-syndrome
#11
François Parant, Bruno Ranchin, Marie-Claude Gagnieu
BACKGROUND: For TDM of mycophenolate acid (MPA), the Roche Total Mycophenolic Acid® assay based on the inhibition of recombinant inosine monophosphate dehydrogenase (IMPDH) has been shown to be a simple and reliable alternative to chromatographic methods. We have adapted this assay on the ABX Pentra 400 analyzer (HORIBA). OBJECTIVE: To investigate the analytical performances of the Roche Total Mycophenolic Acid® assay on the ABX Pentra 400 and to compare it to an LC-MS method using samples from children with nephrotic syndrome treated with mycophenolate mofetil (MMF)...
April 2017: Pract Lab Med
https://www.readbyqxmd.com/read/28856105/a-case-of-compartment-syndrome-in-the-scrotum-and-inguinal-area-complicating-idiopathic-nephrotic-syndrome-of-childhood
#12
Rola S Saqan, Rami S Alazab, Mohammed Allouh
Idiopathic nephrotic syndrome (INS) is a kidney disease characterized by massive proteinuria. Protein loss leads to decreased oncotic pressure shifting the fluids into the interstitial space causing edema, complications such as infections and thromboembolism occur. We report a 7-year-old, diagnosed with NS presenting with a relapse. He developed ascites and scrotal edema followed by severe scrotal pain and redness, progressing rapidly to ecchymosis in the inguinal areas not in continuity with the scrotum. Ultrasound with color Doppler was inconclusive, scrotal exploration was done along with skin incision in the inguinal areas and scrotum, which appeared to relieve the condition rapidly...
November 2017: Urology Case Reports
https://www.readbyqxmd.com/read/28852480/intravenous-cyclophosphamide-and-oral-prednisolone-is-a-safe-and-effective-treatment-option-for-idiopathic-membranous-nephropathy
#13
Vinod Mathrani, Abdulfattah Alejmi, Siân Griffin, Gareth Roberts
BACKGROUND: Idiopathic membranous nephropathy (IMN) is one of the most common causes of nephrotic syndrome in adults. A proportion of patients will experience spontaneous remission and the decision to offer immunosuppression is guided by the presence of adverse prognostic features. Data relating to the efficacy of different immunosuppressive protocols is lacking, in particular there are little data available on the efficacy or benefits of an intravenous (IV) cyclophosphamide-based regimen...
August 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28792718/mdr-1-and-cyp3a5-polymorphisms-in-pediatric-idiopathic-nephrotic-syndrome-impact-on-susceptibility-and-response-to-steroids-preliminary-results
#14
Amira Moussa, Sameh Mabrouk, Haithem Hamdouni, Maroua Ajmi, Miniar Tfifha, Asma Omezzine, Saoussen Abroug, Ali Bouslama
BACKGROUND: Oral steroid treatment is the first line of therapy for childhood nephrotic syndrome (NS). However, resistance to this treatment has been observed in some patients. Here, we investigated the association of two steroid metabolism-related genes with susceptibility to childhood NS and the steroid response. METHODS: We genotyped the single nucleotide polymorphisms (SNP) of MDR-1 [C1236T (rs1128503), G2677T/A (rs2032582), and C3435T (rs1045642)] and the CYP3A5 gene (A6986G) in 63 NS patients and 110 age and gender matched controls by PCR-RFLP...
July 1, 2017: Clinical Laboratory
https://www.readbyqxmd.com/read/28781794/a-case-of-membranous-glomerulopathy-associated-with-lung-cancer-and-review-of-the-literature
#15
Aydin Aytekin, Ahmet Ozet, Irem Bilgetekin, Betul Ogut, Aydin Ciltas, Mustafa Benekli
Membraneous nephropathy (MN) is the most commonly occurring nephrotic syndrome in adults as well as the most common paraneoplastic nephropathy associated with solid tumors, and it is mostly associated with gastrointestinal system and lung carcinomas. Accurate diagnosis is important as the treatment of paraneoplastic glomerulonephritis is very varied from that of idiopathic ones. In the current report, a case of a patient that was referred with proteinuria and edema and was diagnosed with lung cancer, and responded markedly to treatment of malignancy, with improvement of MN, is presented...
August 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28752734/elevated-baseline-adiponectin-level-predicting-an-increased-risk-of-disease-activity-in-idiopathic-nephrotic-syndrome-and-chronic-kidney-disease-in-children
#16
Wafaa M Abo El Fotoh, Ghada M El Mashad
BACKGROUND: Adiponectin, an adipocyte-derived protein, is present abundantly in circulation and has anti-atherogenic and anti-inflammatory effects, so we aimed to assess the role of adiponectin in idiopathic nephrotic syndrome and chronic kidney disease in children. METHODS: Overall of 290 children, 95 patients with the steroid-responsive idiopathic nephrotic syndrome (50 in remission and 45 in relapse).Another 95 patients with chronic kidney disease (50 on hemodialysis and 45 on conservative therapies) and 100 apparently healthy matched children were enrolled into the study...
July 27, 2017: Minerva Pediatrica
https://www.readbyqxmd.com/read/28752288/application-of-next-generation-sequencing-technology-to-diagnosis-and-treatment-of-focal-segmental-glomerulosclerosis
#17
REVIEW
Yutaka Harita
A broad range of genetic and non-genetic factors can lead to kidney injury that manifests as focal segmental glomerulosclerosis (FSGS), which can be classified into primary (idiopathic) and secondary forms. Previous genetic approaches to familial or sporadic cases of FSGS or steroid-resistant nephrotic syndrome identified causal mutations in a subset of genes. Recently, next-generation sequencing (NGS) approaches are becoming a part of a standard assessment in medical genetics. Current knowledge of the comprehensive genomic information is changing the way we think about FSGS and draws attention not only to identification of novel causal genes, but also to potential roles for combinations of mutations in multiple genes, mutations with complex inheritance, and susceptibility genes with variable penetrance carrying relatively minor but significant effects...
July 27, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28748888/childhood-idiopathic-steroid-resistant-nephrotic-syndrome-at-a-single-center-in-khartoum
#18
El-Tigani M A Ali, Hanna F K Makki, Mohamed B Abdelraheem, Salwa O Makke, Rashid A Allidir
Prevalence, clinicopathological features, and outcome of childhood idiopathic steroid-resistant nephrotic syndrome (ISRNS) vary in different countries. We report on these parameters in a single center in Khartoum. We retrospectively reviewed all the records of children with idiopathic nephrotic syndrome (INS) followed up in the pediatric renal unit, Soba Hospital, Khartoum between 2001 and 2012. ISRNS was defined as no remission within four weeks of daily prednisolone at a dose of 60 mg/m2. In 430 children with INS 130 (28%) had SRNS with a mean age of 7...
July 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28717938/rituximab-in-steroid-sensitive-nephrotic-syndrome-lessons-from-clinical-trials
#19
REVIEW
Kazumoto Iijima, Mayumi Sako, Koichi Kamei, Kandai Nozu
Idiopathic nephrotic syndrome is the most common chronic glomerular disease in children. A total of 80-90% of patients with childhood idiopathic nephrotic syndrome achieve remission with steroid therapy [steroid-sensitive nephrotic syndrome (SSNS)]. However, approximately 50% of children with SSNS develop frequently relapsing nephrotic syndrome (FRNS) or steroid-dependent nephrotic syndrome (SDNS). Children with FRNS or SDNS are usually treated with immunosuppressive agents, but 10-20% of children receiving immunosuppressive agents still show frequent relapses or steroid dependence during or after treatment, defined as complicated FRNS or SDNS...
July 17, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28680821/a-unified-pathogenesis-for-kidney-diseases-including-genetic-diseases-and-cancers-by-the-protein-homeostasis-system-hypothesis
#20
REVIEW
Kyung-Yil Lee
Every cell of an organism is separated and protected by a cell membrane. It is proposed that harmony between intercellular communication and the health of an organism is controlled by a system, designated the protein-homeostasis-system (PHS). Kidneys consist of a variety of types of renal cells, each with its own characteristic cell-receptor interactions and producing characteristic proteins. A functional union of these renal cells can be determined by various renal function tests, and harmonious intercellular communication is essential for the healthy state of the host...
June 2017: Kidney Research and Clinical Practice
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