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Focal segmental glomerulosclerosis fsgs

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https://www.readbyqxmd.com/read/29924795/nephrin-is-necessary-for-podocyte-recovery-following-injury-in-an-adult-mature-glomerulus
#1
Rakesh Verma, Madhusudan Venkatareddy, Anne Kalinowski, Theodore Li, Joanna Kukla, Ashomathi Mollin, Gabriel Cara-Fuentes, Sanjeevkumar R Patel, Puneet Garg
Nephrin (Nphs1) is an adhesion protein that is expressed at the podocyte intercellular junction in the glomerulus. Nphs1 mutations in humans or deletion in animal genetic models results in a developmental failure of foot process formation. A number of studies have shown decrease in expression of nephrin in various proteinuric kidney diseases as well as in animal models of glomerular disease. Decrease in nephrin expression has been suggested to precede podocyte loss and linked to the progression of kidney disease...
2018: PloS One
https://www.readbyqxmd.com/read/29909410/what-is-the-role-of-soluble-urokinase-type-plasminogen-activator-in-renal-disease
#2
Moin A Saleem
Soluble urokinase-type plasminogen activator -(suPAR) is an inflammatory signal with pleiotropic biological effects depending on context and post-translational modifications. Recently, [Hayek, et al: Nat Med 2017; 23: 945-953] it has been found that there is a link between suPAR and renal disease in several guises, and a key question is whether it is a driver or a marker of renal disease, and if so of which types of kidney damage. Subject of Review: Circulating suPAR has been postulated to cause acute proteinuric kidney disease, specifically focal segmental glomerulosclerosis (FSGS), though both the animal models and clinical data in the original reports have been challenged...
June 15, 2018: Nephron
https://www.readbyqxmd.com/read/29894413/acth-gel-in-resistant-focal-segmental-glomerulosclerosis-after-kidney-transplantation
#3
Tarek Alhamad, John Manllo Dieck, Usman Younus, Dany Matar, Sami Alasfar, Vikas Vujjini, Devin Wall, Bilal Kanawati, Reiser Jochen, Daniel C Brennan, Nada Alachkar
BACKGROUND: Treatment of focal segmental glomerular sclerosis (FSGS) after kidney transplantation is challenging with unpredictable outcomes. The objective was to investigate the use of adrenocorticotropic hormone (ACTH) analogue gel in kidney transplant recipients with de novo or recurrent FSGS resistant to therapeutic plasma exchange and/or rituximab. METHODS: We performed a retrospective review of cases of de novo or recurrent resistant FSGS at 2 large U.S. transplant centers between April 2012 and December 2016...
June 12, 2018: Transplantation
https://www.readbyqxmd.com/read/29891232/glomerular-disease-augments-kidney-accumulation-of-synthetic-anionic-polymers
#4
Gary W Liu, Alexander N Prossnitz, Diana G Eng, Yilong Cheng, Nithya Subrahmanyam, Jeffrey W Pippin, Robert J Lamm, Chayanon Ngambenjawong, Hamidreza Ghandehari, Stuart J Shankland, Suzie H Pun
Polymeric drug carriers can alter the pharmacokinetics of their drug cargoes, thereby improving drug therapeutic index and reducing side effects. Understanding and controlling polymer properties that drive tissue-specific accumulation is critical in engineering targeted drug delivery systems. For kidney disease applications, targeted drug delivery to renal cells that reside beyond the charge- and size-selective glomerular filtration barrier could have clinical potential. However, there are limited reports on polymer properties that might enhance kidney accumulation...
June 2, 2018: Biomaterials
https://www.readbyqxmd.com/read/29873512/proteostasis-as-a-therapeutic-target-in-glomerular-injury-associated-with-mutant-alpha-actinin-4
#5
Albert Yee, Joan Papillon, Julie Guillemette, Daniel R Kaufman, Chris R J Kennedy, Andrey V Cybulsky
Mutations in alpha-actinin-4 (actinin-4) result in hereditary focal segmental glomerulosclerosis (FSGS) in humans. Actinin-4 mutants induce podocyte injury due to dysregulation of the cytoskeleton and proteotoxicity. Injury may be associated with endoplasmic reticulum (ER) stress and polyubiquitination of proteins. We assessed if the chemical chaperone, 4-phenylbutyric acid (4-PBA) can ameliorate the proteotoxicity of an actinin-4 mutant. Actinin-4 K255E, which causes FSGS in humans (K256E in the mouse), showed enhanced ubiquitination, accelerated degradation, aggregate formation, and enhanced association with F-actin in glomerular epithelial cells (GECs)...
June 6, 2018: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/29852493/protecting-podocytes-a-key-target-for-therapy-of-focal-segmental-glomerulosclerosis
#6
Kirk N Campbell, James A Tumlin
BACKGROUND: Focal segmental glomerulosclerosis (FSGS) is a histologic pattern of injury demonstrated by renal biopsy that can arise from a diverse range of causes and mechanisms. It has an estimated incidence of 7 per 1 million and is the most common primary glomerular disorder leading to end-stage renal disease in the United States. This review focuses on damage to the podocyte and the consequences of this injury in patients with FSGS, the genetics of FSGS, and approaches to treatment with a focus on the effects on podocytes...
2018: American Journal of Nephrology
https://www.readbyqxmd.com/read/29848418/histopathological-spectrum-and-short-term-outcome-of-treatment-with-cyclophosphamide-in-relapsing-steroid-sensitive-nephrotic-syndrome
#7
Irshad Ali Bajeer, Sabeeta Khatri, Veena Tresa, Seema Hashmi, Mohammed Mubarak, Ali Asghar Lanewala
OBJECTIVE: To determine the short-term outcome of cyclophosphamide (CPO) course in children with relapsing steroid sensitive nephrotic syndrome (SSNS) with different histopathological lesions. STUDY DESIGN: Descriptive, observational study. PLACE AND DURATION OF STUDY: Pediatric Nephrology Department, Sindh Institute of Urology and Transplantation, Karachi, from January 2012 to December 2014. METHODOLOGY: All children with relapsing steroid-sensitive nephrotic syndrome, who underwent renal biopsy and received cyclophosphamide therapy, were included and followed up for 2 years...
June 2018: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/29808114/preoperative-low-density-lipoprotein-apheresis-for-preventing-recurrence-of-focal-segmental-glomerulosclerosis-after-kidney-transplantation
#8
Akihito Sannomiya, Toru Murakami, Ichiro Koyama, Kosaku Nitta, Ichiro Nakajima, Shohei Fuchinoue
Background: Focal segmental glomerulosclerosis (FSGS) often develops rapidly and frequently progresses to renal failure, while the recurrence rate after kidney transplantation is 20-50%. We performed low-density lipoprotein (LDL) apheresis before kidney transplantation in FSGS patients to prevent recurrence. Methods: Five adult patients with chronic renal failure due to FSGS undergoing living related donor kidney transplantation were investigated retrospectively...
2018: Journal of Transplantation
https://www.readbyqxmd.com/read/29786187/-steroid-resistant-focal-segmental-glomerulosclerosis-treated-with-cascade-plasmafiltration-and-rituximab
#9
Paola Tatangelo, Francesco Londrino, Giorgio Di Vasta, Giuliana Guido, Alessia Centi, Sara Dominijanni, Eleonora Bernabei, Maria Stella Caramiello, Damiano Di Franco, Roberto Palumbo
A 39-year man with primary steroid resistant focal segmental glomerulosclerosis (FSGS) was treated with mycophenolate mofetil and ACE-inhibitors. After six months a different therapeutics approach was mandatory due to the worsening of renal function and the relapse of proteinuria at the nephrotic range. The combination of cascade plasmafiltration and single dose of rituximab (375 mg/m²) achieved clinical remission and improved renal function in six months follow up. Cascade plasmafiltration in association with rituximab can be considered as a salvage method for primary steroid-resistant FSGS...
May 2018: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/29785489/trpc6-inactivation-confers-protection-in-a-model-of-severe-nephrosis-in-rats
#10
Eun Young Kim, Parisa Yazdizadeh Shotorbani, Stuart E Dryer
Mutations in canonical transient receptor potential-6 (TRPC6) channels give rise to rare familial forms of focal and segmental glomerulosclerosis (FSGS). Here we examined a possible role for TRPC6 in the progression of chronic puromycin aminonucleoside (PAN) nephrosis in Sprague-Dawley rats, a classic model of acquired nephrotic syndromes. We used CRISPR/Cas9 technology to delete a 239-bp region within exon 2 of the Trpc6 gene (Trpc6del allele). Trpc6del/del rats expressed detectable Trpc6 transcripts missing exon 2, and TRPC6 proteins could be detected by immunoblot of renal cortex...
May 22, 2018: Journal of Molecular Medicine: Official Organ of the "Gesellschaft Deutscher Naturforscher und Ärzte"
https://www.readbyqxmd.com/read/29779709/a-mutation-in-transcription-factor-mafb-causes-focal-segmental-glomerulosclerosis-with-duane-retraction-syndrome
#11
Yoshinori Sato, Hiroyasu Tsukaguchi, Hiroyuki Morita, Koichiro Higasa, Mai Thi Nhu Tran, Michito Hamada, Toshiaki Usui, Naoki Morito, Shoichiro Horita, Takao Hayashi, Junko Takagi, Izumi Yamaguchi, Huan Thanh Nguyen, Masayo Harada, Kiyoko Inui, Yuichi Maruta, Yoshihiko Inoue, Fumihiko Koiwa, Hiroshi Sato, Fumihiko Matsuda, Shinya Ayabe, Seiya Mizuno, Fumihiro Sugiyama, Satoru Takahashi, Ashio Yoshimura
Focal segmental glomerulosclerosis (FSGS) is a leading cause of end-stage renal disease in children and adults. Genetic factors significantly contribute to early-onset FSGS, but the etiologies of most adult cases remain unknown. Genetic studies of monogenic syndromic FSGS exhibiting extra-renal manifestations have uncovered an unexpected biological role for genes in the development of both podocytes and other cellular lineages. To help define these roles, we studied two unrelated families with FSGS associated with Duane Retraction Syndrome, characterized by impaired horizontal eye movement due to cranial nerve malformation...
May 17, 2018: Kidney International
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
#12
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/29752403/cryo-em-structure-of-the-cytoplasmic-domain-of-murine-transient-receptor-potential-cation-channel-subfamily-c-member-6-trpc6
#13
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
#14
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/29740794/focal-segmental-glomerulosclerosis-with-heterozygous-apolipoprotein-e5-glu3lys
#15
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/29717532/the-urgent-need-for-more-research-on-how-to-treat-recurrent-focal-and-segmental-glomerulosclerosis
#16
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
#17
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
#18
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
#19
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...
June 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
#20
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
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