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focal segmental glomerulosclerosis

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https://www.readbyqxmd.com/read/29775445/the-clinical-pattern-of-nephrotic-syndrome-in-children-has-no-effect-on-the-concentration-of-soluble-urokinase-receptor-supar-in-serum-and-urine
#1
Agnieszka Ochocińska, Wioletta Jarmużek, Roman Janas
Concentration of soluble urokinase receptor (suPAR) was regarded as viable marker to differentiate the focal segmental glomerulosclerosis (FSGS) from other glomerulopathies and also as predictive parameter for progression of renal disease. AIM: The aim of this study was to evaluate serum and urine (s)(u)suPAR concentration in steroid-sensitive and steroid-resistant nephrotic children treated with different (double and triple-drug) regimens. MATERIALS AND METHODS: Overall 43 children were evaluated including 14 patients with steroid-resistant nephrotic syndrome (SRNS) aged 9±6 years and 29 with steroid-sensitive nephrotic syndrome (SSNS) aged 9±5 years, as well as control group (n=59)...
April 23, 2018: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29764427/col4a5-and-lama5-variants-co-inherited-in-familial-hematuria-digenic-inheritance-or-genetic-modifier-effect
#2
Konstantinos Voskarides, Gregory Papagregoriou, Despina Hadjipanagi, Ioanelli Petrou, Isavella Savva, Avraam Elia, Yiannis Athanasiou, Androulla Pastelli, Maria Kkolou, Michalis Hadjigavriel, Christoforos Stavrou, Alkis Pierides, Constantinos Deltas
BACKGROUND: About 40-50% of patients with familial microscopic hematuria (FMH) caused by thin basement membrane nephropathy (TBMN) inherit heterozygous mutations in collagen IV genes (COL4A3, COL4A4). On long follow-up, the full phenotypic spectrum of these patients varies a lot, ranging from isolated MH or MH plus low-grade proteinuria to chronic renal failure of variable degree, including end-stage renal disease (ESRD). METHODS: Here, we performed Whole Exome Sequencing (WES) in patients of six families, presenting with autosomal dominant FMH, with or without progression to proteinuria and loss of renal function, all previously found negative for severe collagen IV mutations...
May 16, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29761241/glucocorticoids-in-the-treatment-of-patients-with-primary-focal-segmental-glomerulosclerosis-and-moderate-proteinuria
#3
Jianni Huang, Li Lin, Jingyuan Xie, Xiao Li, Pingyan Shen, Xiaoxia Pan, Hong Ren, Nan Chen
BACKGROUND: To compare the efficacy of glucocorticoids in primary focal segmental glomerulosclerosis (pFSGS) patients with moderate proteinuria. Registered at http://www.chictr.org.cn/ , study No. ChiCTR-OPN-17012789. METHODS: pFSGS patients with urine protein between 1.0 and 3.5 g/24 h were recruited from 2006 to 2016. No decline in urine protein > 50% was observed after 2 months of run-in angiotensin-converting enzyme inhibitors/angiotensin-receptor blockers (ACEI/ARB) treatment...
May 14, 2018: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/29752403/cryo-em-structure-of-the-cytoplasmic-domain-of-murine-transient-receptor-potential-cation-channel-subfamily-c-member-6-trpc6
#4
Caleigh M Azumaya, Francisco Sierra-Valdez, Julio F Cordero-Morales, Terunaga Nakagawa
The kidney maintains the internal milieu by regulating the retention and excretion of proteins, ions, and small molecules. The glomerular podocyte forms the slit diaphragm of the ultrafiltration filter, whose damage leads to progressive kidney failure and focal segmental glomerulosclerosis (FSGS). The canonical transient receptor potential 6 (TRPC6) ion channel is expressed in the podocyte and mutations in its cytoplasmic domain cause FSGS in humans. In vitro evaluation of disease-causing mutations in TRPC6 has revealed that these genetic alterations result in abnormal ion channel gating...
May 11, 2018: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29748623/plasma-microrna-panel-is-a-novel-biomarker-for-focal-segmental-glomerulosclerosis-and-associated-with-podocyte-apoptosis
#5
Bin Xiao, Li-Na Wang, Wei Li, Li Gong, Ting Yu, Qian-Fei Zuo, Hong-Wen Zhao, Quan-Ming Zou
Focal segmental glomerulosclerosis (FSGS) is a frequent glomerular disease, and is the common cause of nephrotic syndrome. However, there is no validated diagnostic blood biomarker for FSGS. Here, we performed a real-time PCR-based high-throughput miRNA profiling to identify the plasma signature for FSGS. We found four miRNAs (miR-17, miR-451, miR-106a, and miR-19b) were significantly downregulated in the plasma of FSGS patients (n = 97) compared with healthy controls (n = 124) in the training, validation, and blinded-test phases...
May 10, 2018: Cell Death & Disease
https://www.readbyqxmd.com/read/29747582/the-renal-manifestations-of-type-4-familial-partial-lipodystrophy-a-case-report-and-review-of-literature
#6
Ru-Xuan Chen, Lei Zhang, Wei Ye, Yu-Bing Wen, Nuo Si, Hang Li, Ming-Xi Li, Xue-Mei Li, Ke Zheng
BACKGROUND: Lipodystrophy syndromes are rare disorders of variable body fat loss associated with potentially serious metabolic complications. Familial partial lipodystrophy (FPLD) is mostly inherited as an autosomal dominant disorder. Renal involvement has only been reported in a limited number of cases of FPLD. Herein, we present a rare case of proteinuria associated with type 4 FPLD, which is characterized by a heterozygous mutation in PLIN1 and has not been reported with renal involvement until now...
May 10, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29740794/focal-segmental-glomerulosclerosis-with-heterozygous-apolipoprotein-e5-glu3lys
#7
Masaru Sasaki, Tetsuhiko Yasuno, Kenji Ito, Akira Matsunaga, Satoshi Hisano, Yasuhiro Abe, Katsuhisa Miyake, Kosuke Masutani, Hitoshi Nakashima, Takao Saito
Apolipoprotein (apo) E5 is a rare apoE isoform. The apoE5 (Glu3Lys) variant, which is caused by the substitution of lysine with glutamic acid at codon 3, has a relative frequency of 0.1% in Japan. Previous studies have reported that apoE5 (Glu3Lys) is associated with hyperlipidemia and cardiovascular diseases, but this isoform has higher LDL receptor-binding activity than that of normal apoE3. Nephropathy associated with apoE5 (Glu3Lys) alone has not yet been reported. We present a case of a 51-year-old man with nephrotic syndrome...
May 8, 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29731057/clinical-outcomes-of-kidney-transplantation-in-patients-with-biopsy-proven-glomerulonephritis
#8
H Park, W Y Park, S S Kang, S M Yeo, S Han, S B Park, K Jin
BACKGROUND: The clinical outcomes after kidney transplantation (KT) according to the types of glomerulonephritis (GN) as the cause of end-stage renal disease (ESRD) are various, but there are not many studies on this. METHODS: Among 1,253 patients who had KT between November 1982 and January 2017, 183 recipients with biopsy-proven GN as the primary cause of ESRD were enrolled. We analyzed the incidence of recurrent GN and the factors associated with recurrence and graft and patient survivals...
May 2018: Transplantation Proceedings
https://www.readbyqxmd.com/read/29728434/focal-segmental-glomerulosclerosis-in-a-patient-with-prefibrotic-primary-myelofibrosis
#9
Gopal Krishana Bohra, Durga Shankar Meena, Nitin Bajpai, Abhishek Purohit
We report a case of 56-year-old man presented to us with chief complaints of frothy urine and leg swelling. A urinalysis revealed nephrotic-range proteinuria. Haematological investigations revealed thrombocytosis, leucocytosis and peripheral blood smear showed a leucoerythroblastic picture. JAK 2 mutation was positive. To confirm the diagnosis of myeloproliferative neoplasm, bone marrow biopsy was done, which was suggestive of primary myelofibrosis. The patient underwent kidney biopsy due to rapidly declining renal function and persistent proteinuria, which was suggestive of focal segmental glomerulosclerosis...
May 4, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29726752/spectrum-of-renal-disease-in-hiv-infected-children-report-of-five-cases
#10
Karalanglin Tiewsoh, Ankur Kumar Jindal, Dhrubajyoti Sharma, Sunil Arora, Ranjana W Minz, Parimal Agrawal, Ritambhra Nada, Deepti Suri
There is a paucity of literature on renal diseases associated with HIV infection in Asian countries. Renal disease in HIV-infected children can involve the glomerulus, interstitium, tubules or blood vessels of the kidney. In this case series, five HIV-infected children with various forms of renal disease are reported. The renal pathology included HIV-associated nephropathy, collapsing focal segmental glomerulosclerosis without tubular changes, tubule-interstitial nephritis and minimal change disease (MCD). Case five fulfilled the classification criteria for childhood polyarteritis nodosa (PAN)...
May 4, 2018: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/29725648/nephcure-accelerating-cures-institute-a-multidisciplinary-consortium-to-improve-care-for-nephrotic-syndrome
#11
Debbie S Gipson, David T Selewski, Susan F Massengill, Mary Margaret Modes, Hailey Desmond, Lauren Lee, Elaine Kamil, Matthew R Elliott, Sharon G Adler, Gia Oh, Richard A Lafayette, Patrick E Gipson, Aditi Sinha, Arvind Bagga, Anne Pesenson, Cheryl Courtlandt, Cathie Spino, Richard Eikstadt, Renée Pitter, Samara Attalla, Anne Waldo, Richard Winneker, Noelle E Carlozzi, Jonathan P Troost, Irving Smokler, Mark Stone
Introduction: NephCure Accelerating Cures Institute (NACI) is a collaborative organization sponsored by NephCure Kidney International and the University of Michigan. The Institute is composed of 7 cores designed to improve treatment options and outcomes for patients with glomerular disease: Clinical Trials Network, Data Warehouse, Patient-Reported Outcomes (PRO) and Endpoints Consortium, Clinical Trials Consulting Team, Quality Initiatives, Education and Engagement, and Data Coordinating Center...
March 2018: KI Reports
https://www.readbyqxmd.com/read/29717532/the-urgent-need-for-more-research-on-how-to-treat-recurrent-focal-and-segmental-glomerulosclerosis
#12
EDITORIAL
Guido Filler, Jaime M Restrepo
No abstract text is available yet for this article.
May 1, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29713631/molecular-mechanisms-of-proteinuria-in-focal-segmental-glomerulosclerosis
#13
REVIEW
Yumeng Wen, Sapna Shah, Kirk N Campbell
Focal segmental glomerulosclerosis (FSGS) is the most common primary glomerular disease resulting in end-stage renal disease in the USA and is increasing in prevalence worldwide. It is a diverse clinical entity with idiopathic, genetic, metabolic, infectious, and other causes that culminate in a characteristic histologic pattern of injury. Proteinuria is a hallmark of FSGS as well as other primary and secondary glomerular disorders. The magnitude of proteinuria at disease onset and during treatment has prognostic implications for renal survival as well as associated cardiovascular morbidity and mortality...
2018: Frontiers in Medicine
https://www.readbyqxmd.com/read/29682387/surgical-management-of-encapsulating-peritoneal-sclerosis-a-case-report-in-kidney-transplant-patient
#14
R Shahbazov, M Talanian, J L Alejo, F Azari, A Agarwal, K L Brayman
Introduction: Encapsulating peritoneal sclerosis (EPS) is a clinical syndrome of progressive fibrotic change in response to prolonged, repetitive, and typically severe insult to the peritoneal mesothelium, often occurring in the setting of peritoneal dialysis (PD). Clear guidelines for successful management remain elusive. We describe the successful surgical management of EPS in a 28-year-old male s/p deceased donor kidney transplant for end-stage renal disease (ESRD) secondary to focal segmental glomerulosclerosis (FSGS)...
2018: Case Reports in Surgery
https://www.readbyqxmd.com/read/29680473/rituximab-in-the-management-of-pediatric-steroid-resistant-nephrotic-syndrome-a-systematic-review
#15
Manel Jellouli, Rim Charfi, Bayen Maalej, Abdelmajid Mahfoud, Sameh Trabelsi, Tahar Gargah
OBJECTIVES: To evaluate the efficacy and safety of rituximab in children with steroid-resistant nephrotic syndrome. STUDY DESIGN: A systematic review evaluating the efficacy and safety of rituximab in children with steroid-resistant nephrotic syndrome was performed. Data from studies, performed before April 2017 were collected, from MEDLINE, Cochrane Library, Scopus, and Web of Science. Study eligibility criteria included clinical trials and observational studies with a minimal sample size of 5 patients, regarding treatment with rituximab in children with steroid-resistant nephrotic syndrome...
April 18, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29679356/regional-variations-in-immunosuppressive-therapy-in-patients-with-primary-nephrotic-syndrome-the-japan-nephrotic-syndrome-cohort-study
#16
Ryohei Yamamoto, Enyu Imai, Shoichi Maruyama, Hitoshi Yokoyama, Hitoshi Sugiyama, Kosaku Nitta, Tatsuo Tsukamoto, Shunya Uchida, Asami Takeda, Toshinobu Sato, Takashi Wada, Hiroki Hayashi, Yasuhiro Akai, Megumu Fukunaga, Kazuhiko Tsuruya, Kosuke Masutani, Tsuneo Konta, Tatsuya Shoji, Takeyuki Hiramatsu, Shunsuke Goto, Hirofumi Tamai, Saori Nishio, Arimasa Shirasaki, Kojiro Nagai, Kunihiro Yamagata, Hajime Hasegawa, Hidemo Yasuda, Shizunori Ichida, Tomohiko Naruse, Kei Fukami, Tomoya Nishino, Hiroshi Sobajima, Satoshi Tanaka, Toshiyuki Akahori, Takafumi Ito, Terada Yoshio, Ritsuko Katafuchi, Shouichi Fujimoto, Hirokazu Okada, Eiji Ishimura, Junichiro James Kazama, Keiju Hiromura, Tetsushi Mimura, Satashi Suzuki, Yosuke Saka, Tadashi Sofue, Yusuke Suzuki, Yugo Shibagaki, Kiyoki Kitagawa, Kunio Morozumi, Yoshiro Fujita, Makoto Mizutani, Takashi Shigematsu, Naoki Kashihara, Hiroshi Sato, Seiichi Matsuo, Ichiei Narita, Yoshitaka Isaka
BACKGROUND: The lack of high-quality clinical evidences hindered broad consensus on optimal therapies for primary nephrotic syndromes. The aim of the present study was to compare prevalence of immunosuppressive drug use in patients with primary nephrotic syndrome across 6 regions in Japan. METHODS: Between 2009 and 2010, 380 patients with primary nephrotic syndrome in 56 hospitals were enrolled in a prospective cohort study [Japan Nephrotic Syndrome Cohort Study (JNSCS)], including 141, 151, and 38 adult patients with minimal change disease (MCD), membranous nephropathy (MN), and focal segmental glomerulosclerosis (FSGS), respectively...
April 20, 2018: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/29676031/treatment-of-recurrent-focal-segmental-glomerulosclerosis-post-kidney-transplantation-in-australian-and-new-zealand-children-a-retrospective-cohort-study
#17
Anna Francis, Madeleine Didsbury, Hugh McCarthy, Tonya Kara
Disease recurrence affects around a third of renal transplants for children with FSGS and is associated with poor graft outcomes. Unfortunately, there are no large trials guiding treatment for recurrent FSGS. We aimed to describe current therapies and treatment response for recurrent FSGS in 4 centres in Australia and New Zealand. Data were collected on children (age <18 years) with recurrent FSGS (1990-2015). We reviewed patient charts to obtain clinical information. Ethics approval was obtained from the relevant boards...
April 19, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29672314/efficacy-of-the-combination-of-immunoadsorption-and-rituximab-for-treatment-in-a-case-of-severe-focal-and-segmental-glomerulosclerosis-recurrence-after-renal-transplantation
#18
Paloma L Martin-Moreno, Jose Rifon, Pedro Errasti
BACKGROUND/AIMS: We present a case of a male patient with severe recurrence of focal and segmental glomerulosclerosis (FSGS) after transplant. METHODS: Before the transplant he was treated with plasma exchange. Massive proteinuria was detected post-transplantation and plasma exchanges were performed without response. We administered 5 doses of Rituximab (375 mg/m2) and partial remission was achieved. Proteinuria relapse occurred 1 year post-transplant, so Immunoadsorption (IA) was started instead of plasma exchange with reduction of proteinuria...
April 19, 2018: Blood Purification
https://www.readbyqxmd.com/read/29669314/three-novel-heterozygous-col4a4-mutations-result-in-three-different-collagen-type-iv-kidney-disease-phenotypes
#19
Ang Li, Er-Zhi Gao, Ying-Xia Cui, Jian-Hong Liu, Xing Lv, Xiu-Xiu Wei, Xin-Yi Xia, Chun-Lin Gao, Feng-Xia Liu, Zheng-Kun Xia, Asan, Zhi-Hong Liu, Xiao-Jun Li
Thin basement membrane nephropathy (TBMN), autosomal dominant Alport syndrome (ADAS), and focal segmental glomerulosclerosis (FSGS) are kidney diseases that differ in clinical diagnosis, treatment, and prognosis. Nevertheless, they may result from the same causative genes. Here, we report 3 COL4A4 heterozygous mutations (p.Gly208Arg, p.Ser513Glufs*2, and p.Met1617Cysfs*39) that lead to 3 different collagen type IV kidney disease phenotypes, manifesting as TBMN, ADAS, and FSGS. Using bioinformatics analyses and pedigree verification, we show that these novel variants are pathogenetic and cosegregate with TBMN, ADAS, and FSGS...
2018: Cytogenetic and Genome Research
https://www.readbyqxmd.com/read/29661344/clinical-significance-of-urinary-biomarkers-in-patients-with-primary-focal-segmental-glomerulosclerosis
#20
Qingyan Zhang, Chunming Jiang, Tianfeng Tang, Hengjin Wang, Yangyang Xia, Qiuyuan Shao, Miao Zhang
BACKGROUND: Focal segmental glomerulosclerosis (FSGS) is often accompanied with tubulointerstitial lesion. This study aimed to assess the role of urinary biomarkers in predicting tubulointerstitial lesion and treatment response in FSGS patients. METHODS: Urinary neutrophil gelatinase-associated lipocalin (NGAL), kidney injury molecule-1 (KIM-1), N-acetyl-β-d-glucosaminidase (NAG) and retinol-binding protein (RBP) were measured in 32 FSGS patients and 22 patients with minimal change nephrotic syndrome...
April 2018: American Journal of the Medical Sciences
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