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Focal segmental glomerulosclerosis fsgs

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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
#1
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
#2
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
#3
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
#4
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
https://www.readbyqxmd.com/read/29657209/spectrum-of-biopsy-proven-renal-diseases-a-single-center-experience
#5
Lakshminarayana R Gopaliah, Indu Sudakaran, Seethalekshmy Vijayan Nalumakkal, Ranjit Narayanan, Biju Meckattuparamban Vareed
Kidney biopsy is one of the most important tools in the assessment of kidney disease as histopathological diagnosis promotes evidence-based practice in Nephrology. This study included 271 consecutive percutaneous kidney biopsies (145 males and 126 females) performed at EMS Memorial Cooperative Hospital, Perinthalmanna, Kerala, India, from September 2009 to March 2016. Among the biopsy-proven renal diseases (BPRD), primary glomerular diseases (PGD) were the most common (77.78%) followed by secondary glomerular diseases (SGD) (12...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29657200/complementary-bodybuilding-a-potential-risk-for-permanent-kidney-disease
#6
Wael El-Reshaid, Kamel El-Reshaid, Shaikha Al-Bader, Ahmad Ramadan, John Patrick Madda
We report our experience of renal disease associated with bodybuilders who had been on high-protein diet, anabolic androgenic steroids (AASs), and growth hormone (GH) for years. A total of 22 adult males who volunteered information about use of high protein diet and AAS or GH were seen over a six-year period with renal disease. Kidney biopsy revealed focal segmental glomerulosclerosis (FSGS) in eight, nephroangiosclerosis in four, chronic interstitial nephritis in three, acute interstitial nephritis in two, nephrocalcinosis with chronic interstitial nephritis in two, and single patients with membranous glomerulopathy, crescentic glomerulopathy, and sclerosing glomerulonephritis...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29644057/clinical-and-pathological-phenotype-of-genetic-causes-of-focal-segmental-glomerulosclerosis-in-adults
#7
Nicola Lepori, Ladan Zand, Sanjeev Sethi, Gema Fernandez-Juarez, Fernando C Fervenza
Focal segmental glomerulosclerosis (FSGS) is a histologic lesion resulting from a variety of pathogenic processes that cause injury to the podocytes. Recently, mutations in more than 50 genes expressed in podocyte or glomerular basement membrane were identified as causing genetic forms of FSGS, the majority of which are characterized by onset in childhood. The prevalence of adult-onset genetic FSGS is likely to be underestimated and its clinical and histological features have not been clearly described. A small number of studies of adult-onset genetic FSGS showed that there is heterogeneity in clinical and histological findings, with a presentation ranging from sub-nephrotic proteinuria to full nephrotic syndrome...
April 2018: Clinical Kidney Journal
https://www.readbyqxmd.com/read/29570919/de-novo-cmv-associated-collapsing-focal-segmental-glomerulosclerosis-in-a-kidney-transplant-recipient
#8
Anup M Patel, Robert D Zenenberg, Ryan J Goldberg
Cytomegalovirus (CMV) infection in kidney transplant recipients occurs due to transmission from the donor organ, reactivation of latent infection, or a primary infection. The clinical manifestations of CMV infection range from mild viral syndrome to tissue invasive disease, including colitis, retinitis, meningitis, hepatitis, and pneumonitis. CMV infection can also cause several types of kidney manifestations including tubulointerstitial nephritis and glomerulopathy. Focal segmental glomerulosclerosis (FSGS) after kidney transplantation most often occurs as a result of recurrent disease but de novo FSGS after kidney transplantation has been described (1)...
March 23, 2018: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/29569191/urinary-cd80-excretion-is-a-predictor-of-good-outcome-in-children-with-primary-nephrotic-syndrome
#9
Chen Ling, Xiaorong Liu, Ying Shen, Zhi Chen, Jianfeng Fan, Yeping Jiang, Qun Meng
BACKGROUND: The level of urinary cluster of differentiation 80 (uCD80) is elevated in most children with minimal change disease (MCD) as opposed to focal segmental glomerulosclerosis (FSGS) during the acute phase. The objective of this follow-up study was to evaluate whether uCD80 elevation is actually associated with MCD and whether it signals better prognosis. METHODS: We evaluated uCD80 levels and a series of putative progression factors in a cohort of 64 patients with nephrotic syndrome (NS) seen between 2011 and 2016...
March 22, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29561839/ccr2-antagonism-leads-to-marked-reduction-in-proteinuria-and-glomerular-injury-in-murine-models-of-focal-segmental-glomerulosclerosis-fsgs
#10
Zhenhua Miao, Linda S Ertl, Dale Newland, Bin Zhao, Yu Wang, Xiaoping Zang, James J Campbell, Xiaoli Liu, Ton Dang, Shichang Miao, Antoni Krasinski, Sreenivas Punna, Yibin Zeng, Jeffrey McMahon, Penglie Zhang, Israel F Charo, Thomas J Schall, Rajinder Singh
Focal segmental glomerulosclerosis (FSGS) comprises a group of uncommon disorders that present with marked proteinuria, nephrotic syndrome, progressive renal failure and characteristic glomerular lesions on histopathology. The current standard of care for patients with FSGS include immunosuppressive drugs such as glucocorticoids followed by calcineurin inhibitors, if needed for intolerance or inadequate response to glucocorticoids. Renin-angiotensin-aldosterone (RAAS) blockers are also used to control proteinuria, an important signature of FSGS...
2018: PloS One
https://www.readbyqxmd.com/read/29556761/recent-advances-of-animal-model-of-focal-segmental-glomerulosclerosis
#11
REVIEW
Jae Won Yang, Anne Katrin Dettmar, Andreas Kronbichler, Heon Yung Gee, Moin Saleem, Seong Heon Kim, Jae Il Shin
In the last decade, great advances have been made in understanding the genetic basis for focal segmental glomerulosclerosis (FSGS). Animal models using specific gene disruption of the slit diaphragm and cytoskeleton of the foot process mirror the etiology of the human disease. Many animal models have been developed to understand the complex pathophysiology of FSGS. Therefore, we need to know the usefulness and exact methodology of creating animal models. Here, we review classic animal models and newly developed genetic animal models...
March 20, 2018: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/29540159/a-cross-sectional-study-on-the-relationship-between-hematological-data-and-quantitative-morphological-indices-from-kidney-biopsies-in-different-glomerular-diseases
#12
Michelangelo Nigro, Davide Viggiano, Vincenzo Ragone, Tiziana Trabace, Annamaria di Palma, Michele Rossini, Giovambattista Capasso, Loreto Gesualdo, Giuseppe Gigliotti
BACKGROUND: The classical approach to the analysis of kidney biopsies is based on semi-quantitative scores of the amount of sclerosis, inflammatory infiltrate, fibrosis and vascular damage. However, advanced renal lesions may be accompanied by a paucity of clinical features and, conversely, important clinical abnormalities may be accompanied by minimal histopathological changes. The objective of this study is to correlate new, semiautomatic, quantitative features of kidney biopsies (e...
March 14, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29531077/-ubd-modifies-apol1-induced-kidney-disease-risk
#13
Jia-Yue Zhang, Minxian Wang, Lei Tian, Giulio Genovese, Paul Yan, James G Wilson, Ravi Thadhani, Amy K Mottl, Gerald B Appel, Alexander G Bick, Matthew G Sampson, Seth L Alper, David J Friedman, Martin R Pollak
People of recent African ancestry develop kidney disease at much higher rates than most other groups. Two specific coding variants in the Apolipoprotein-L1 gene APOL1 termed G1 and G2 are the causal drivers of much of this difference in risk, following a recessive pattern of inheritance. However, most individuals with a high-risk APOL1 genotype do not develop overt kidney disease, prompting interest in identifying those factors that interact with APOL1 We performed an admixture mapping study to identify genetic modifiers of APOL1 -associated kidney disease...
March 27, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29521297/the-spectrum-of-biopsy-proven-glomerular-disease-in-china-a-systematic-review
#14
REVIEW
Yue Yang, Zheng Zhang, Li Zhuo, Da-Peng Chen, Wen-Ge Li
Background: Chronic kidney disease has become a leading public health concern in China, as it is associated with increased morbidity, mortality, and costs. However, the overall situation regarding common glomerular diseases in China remains unclear. Hence, the aim of this study was to assess the national profile of the common types of glomerulonephritis in China. Methods: We searched Medline, Embase, Cochrane Library, CNKI, SinoMed, VIP, and Wanfang databases for English and Chinese language articles from inception to September 2017...
March 20, 2018: Chinese Medical Journal
https://www.readbyqxmd.com/read/29515296/clinicopathological-spectrum-of-glomerular-diseases-in-adolescents-a-single-center-experience-over-4-years
#15
V Muthu, R Ramachandran, R Nada, V Kumar, M Rathi, H S Kohli, V Jha, K L Gupta, V Sakhuja
The spectrum of biopsy-proven glomerular disease was studied from a single center in Northwestern India, among adolescents aged 13-19 years. From January 2009 to December 2012, a total of 177 patients with biopsy-proven glomerular disease were studied. The same pathologist reported all the biopsy specimens after subjecting to light, immunofluorescence, and electron microscopy. The clinical profile and laboratory findings of the patients were correlated with the histopathological spectrum of glomerular diseases...
January 2018: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/29473663/long-term-clinicopathologic-observation-in-a-case-of-steroid-resistant-nephrotic-syndrome-caused-by-a-novel-crumbs-homolog-2-mutation
#16
Shojiro Watanabe, Tomomi Aizawa, Hiroyasu Tsukaguchi, Koji Tsugawa, Kazushi Tsuruga, Akemi Shono, Kandai Nozu, Kazumoto Iijima, Kensuke Joh, Hiroshi Tanaka
Recent advances in high-throughput sequencing for clinical genetic testing have revealed novel disease-causing genes, such as Crumbs homolog 2 (CRB2) for early-onset steroid-resistant nephrotic syndrome (SRNS). We report the long-term clinicopathologic observation of a Japanese female patient with SRNS caused by a newly identified compound heterozygous mutation of CRB2 (p.Arg628Cys and p.Gly839Trp located in the 10th and 11th epidermal growth factor-like domains, respectively). She was initially examined during a mass urinary mass screening for 3...
February 23, 2018: Nephrology
https://www.readbyqxmd.com/read/29465426/advances-in-molecular-diagnosis-and-therapeutics-in-nephrotic-syndrome-and-focal-and-segmental-glomerulosclerosis
#17
Bedra Sharif, Moumita Barua
PURPOSE OF REVIEW: The widespread adoption of next-generation sequencing by research and clinical laboratories has begun to uncover the previously unknown genetic basis of many diseases. In nephrology, one of the best examples of this is seen in focal and segmental glomerulosclerosis (FSGS) and nephrotic syndrome. We review advances made in 2017 as a result of human and molecular genetic studies as it relates to FSGS and nephrotic syndrome. RECENT FINDINGS: There are more than 50 monogenic genes described in steroid-resistant nephrotic syndrome and FSGS, with seven reported in 2017...
February 19, 2018: Current Opinion in Nephrology and Hypertension
https://www.readbyqxmd.com/read/29456224/alport-s-syndrome-with-focal-segmental-glomerulosclerosis-lesion-pattern-to-recognize
#18
Afnan A Alsahli, Sara I Alshahwan, Amal O Alotaibi, Khaled O Alsaad, Nourah Aloudah, Mahfooz Farooqui, Abdullah A Al Sayyari
The association between Alport's syndrome (AS) and focal segmental glomerulosclerosis (FSGS) in the same patient is complex and rarely reported. We report a case of a 42-year-old male presenting with proteinuria, microscopic hematuria, elevated serum creatinine and hypertension with unremarkable physical examination apart from obesity. The renal biopsy showed well-established FSGS pattern of injury with mild interstitial fibrosis and tubular atrophy, while the electron microscopic examination demonstrated glomerular basement membranes (GBM) changes compatible with AS...
January 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29456219/descriptive-analysis-of-glomerulonephritis-by-histological-type-and-their-progression-among-adults-in-a-tertiary-care-center-in-sri-lanka
#19
Kawmadi W Gunawardena, Eranga S Wijewickrama, Carukshi Arambepola, Rushika D Lanerolle
Prevalence of different glomerulonephritides and their clinical course vary geographically. Our objectives are to assess the prevalence of different histological types of glomerulonephritis (GN) based on the light microscopic histology and to assess their progression according to histological type. A retrospective cross-sectional study was carried out among adult patients (>18 years) with a histological diagnosis of GN at the University Professorial Unit over a period of six months. Information including demographic data, renal biopsy findings, and progression of the disease through serum creatinine (SCr) level were collected through existing clinic records of consenting patients...
January 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29395351/end-stage-renal-disease-and-mortality-outcomes-across-different-glomerulonephropathies-in-a-large-diverse-us-population
#20
John J Sim, Simran K Bhandari, Michael Batech, Aviv Hever, Teresa N Harrison, Yu-Hsiang Shu, Dean A Kujubu, Tracy Y Jonelis, Michael H Kanter, Steven J Jacobsen
OBJECTIVE: To compare renal function decline, incident end-stage renal disease (ESRD), and mortality among patients with 5 common glomerular diseases in a large diverse population. PATIENTS AND METHODS: A retrospective cohort study (between January 1, 2000, and December 31, 2011) of patients with glomerulonephropathy using the electronic health record of an integrated health system was performed. Estimated glomerular filtration rate (eGFR) change, incident ESRD, and mortality were compared among patients with biopsy-proven focal segmental glomerulosclerosis (FSGS), membranous glomerulonephritis (MN), minimal change disease (MCD), immunoglobulin A nephropathy (IgAN), and lupus nephritis (LN)...
January 9, 2018: Mayo Clinic Proceedings
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