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Focal segmental glomerulosclerosis fsgs

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https://www.readbyqxmd.com/read/28098122/a-case-of-abdominal-aortic-thrombosis-associated-with-the-nephrotic-syndrome
#1
Jannet Labidi, Yosra Selmi, Yosra Ben Ariba, Zied Elloumi, Saleh Othmani
Thromboembolic disease is an important and frequent complication in patients with the nephrotic syndrome (NS), and the consequences are often severe. Usually, the venous system is affected. Arterial thrombosis has rarely been reported and occurs mainly in children. We report the case of a 27-year-old man with a history of NS due to focal and segmental glomerulosclerosis resistant to steroids and cyclosporine, admitted for bilateral pain in the calves. Aortogram revealed a suspended thrombus in the abdominal aorta just below the origin of the renal arteries with embolism into the left tibioperoneal trunk and the right anterior tibial artery...
January 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28098115/a-novel-fibrillin-1-mutation-in-an-egyptian-marfan-family-a-proband-showing-nephrotic-syndrome-due-to-focal-segmental-glomerulosclerosis
#2
Mohammad Al-Haggar, Ashraf Bakr, Yahya Wahba, Paul J Coucke, Fatma El-Hussini, Mona Hafez, Riham Eid, Abdel-Rahman Eid, Amr Sarhan, Ali Shaltout, Ayman Hammad, Sohier Yahia, Ahmad El-Rifaie, Dina Abdel-Hadi
Marfan syndrome (MFS), the founding member of connective tissue disorder, is an autosomal dominant disease; it is caused by a deficiency of the microfibrillar protein fibrillin-1 (FBN1) and characterized by involvement of three main systems; skeletal, ocular, and cardiovascular. More than one thousand mutations in FBN1 gene on chromosome 15 were found to cause MFS. Nephrotic syndrome (NS) had been described in very few patients with MFS being attributed to membranoproliferative glomerulonephritis secondary to infective endocarditis...
January 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28093933/acute-kidney-injury-in-idiopathic-nephrotic-syndrome-of-childhood-is-a-major-risk-factor-for-the-development-of-chronic-kidney-disease
#3
Afshan Yaseen, Vina Tresa, Ali Asghar Lanewala, Seema Hashmi, Irshad Ali, Sabeeta Khatri, Muhammed Mubarak
BACKGROUND: Acute kidney injury (AKI) is an important complication of idiopathic nephrotic syndrome (INS) and is associated with adverse outcomes, especially the development of chronic kidney disease (CKD). We aimed to determine the clinical profile of children with INS who developed AKI and its short-term outcome. MATERIAL AND METHODS: This prospective study was conducted from March 2014 to October 2015. A total of 119 children of INS (age: 2-18 years) fulfilling the pediatric RIFLE criteria for the diagnosis of AKI were enrolled and followed up for 3 months to determine the outcome...
November 2017: Renal Failure
https://www.readbyqxmd.com/read/28081313/clinical-and-histopathological-features-of-asymptomaticpersistent-microscopic-hematuria-in-children
#4
Serçin Güven, İbrahim Gökçe, Neslihan Çiçek Deniz, Ülger Altuntaş, Nurdan Yıldız, Harika Alpay
BACKGROUND/AIM: We analyzed the clinical and pathological features and prognosis of 106 children with persistent asymptomatic microscopic hematuria (PAMH) with or without mild proteinuria. MATERIALS AND METHODS: This was a retrospective study of 106 children who were referred to our clinics from 2000 to 2013 for evaluation of PAMH. RESULTS: Among the 106 patients, 69 (65%) were female and 37 (35%) were male. The patients were divided into two groups: 101 patients with isolated microscopic hematuria (IMH) and 5 patients with asymptomatic microscopic hematuria and mild proteinuria (AMHP)...
December 20, 2016: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/28072731/focal-segmental-glomerulosclerosis-lagged-behind-the-onset-of-rheumatoid-arthritis-by-7-years-a-case-report-and-literature-review
#5
Yang Liu, Hong-Yan Wen, Li-Hua Wang, Chen Wang
INTRODUCTION: The co-existence of focal segmental glomerulosclerosis (FSGS) and rheumatoid arthritis (RA), presenting either together or in succession, is very rare. A variety of histopathological features in the clinical renal disease may occur in RA. Only 8 studies have previously reported this poorly understood connection. CLINICAL FINDINGS/DIAGNOSES: A case of a 54-year-old male with RA lasting for more than 7 years developed cheirarthritis as the first signs...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28029267/the-pathological-spectrum-associated-with-the-ultrastructural-finding-of-thin-glomerular-basement-membrane-a-tertiary-medical-city-experience-and-review-of-the-literature
#6
Hala Kfoury, Maria Arafah
BACKGROUND: Thin glomerular basement membrane (GBM) has been noted in several glomerular diseases including IgA nephropathy, focal segmental glomerulosclerosis (FSGS), Fabry's disease, and Alport's syndrome. We conducted this study to investigate the pathological ultrastructural spectrum of thin GBMs, to identify associated diseases, and to measure the GBM thickness in thin GBMs in our adult population. MATERIALS AND METHODS: All renal biopsies with thin GBM, diagnosed between 2010 and 2016, were retrieved and reviewed...
December 28, 2016: Ultrastructural Pathology
https://www.readbyqxmd.com/read/28028935/renoprotection-focus-on-trpv1-trpv4-trpc6-and-trpm2
#7
REVIEW
L Markó, M Mannaa, T N Haschler, S Krämer, M Gollasch
Members of the transient receptor potential (TRP) cation channel receptor family have unique sites of regulatory function in the kidney which enables them to promote regional vasodilatation and controlled Ca(2+) influx into podocytes and tubular cells. Activated TRP vanilloid 1 receptor channels (TRPV1) have been found to elicit renoprotection in rodent models of acute kidney injury following ischaemia/reperfusion. Transient receptor potential cation channel, subfamily C, member 6 (TRPC6) in podocytes is involved in chronic proteinuric kidney disease, particularly in focal segmental glomerulosclerosis (FSGS)...
November 7, 2016: Acta Physiologica
https://www.readbyqxmd.com/read/27998979/alpha-actinin-4-actn4-regulates-glucocorticoid-receptor-mediated-transactivation-and-transrepression-in-podocytes
#8
Xuan Zhao, Simran Khurana, Sharmistha Charkraborty, Yuqian Tian, John R Sedor, Leslie A Bruggeman, Hung-Ying Kao
Glucocorticoids (GCs) are a general class of steroids that possess renoprotective activity in glomeruli through their interaction with the glucocorticoid receptor. However, the mechanisms by which the GCs ameliorate proteinuria and glomerular disease are not well understood. In this study, we demonstrated that alpha actinin 4 (ACTN4), an actin-crosslinking protein known to coordinate cytoskeletal organization, interacts with the glucocorticoid receptor (GR) in the nucleus of human podocytes (HPCs), a key cell type in the glomerulus critical for kidney filtration function...
December 20, 2016: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/27998643/activated-erk1-2-increases-cd44-in-glomerular-parietal-epithelial-cells-leading-to-matrix-expansion
#9
Sebastian S Roeder, Taylor J Barnes, Jonathan S Lee, India Kato, Diana G Eng, Natalya V Kaverina, Maria W Sunseri, Christoph Daniel, Kerstin Amann, Jeffrey W Pippin, Stuart J Shankland
The glycoprotein CD44 is barely detected in normal mouse and human glomeruli, but is increased in glomerular parietal epithelial cells following podocyte injury in focal segmental glomerulosclerosis (FSGS). To determine the biological role and regulation of CD44 in these cells, we employed an in vivo and in vitro approach. Experimental FSGS was induced in CD44 knockout and wild-type mice with a cytotoxic podocyte antibody. Albuminuria, focal and global glomerulosclerosis (periodic acid-Schiff stain), and collagen IV staining were lower in CD44 knockout compared with wild-type mice with FSGS...
December 17, 2016: Kidney International
https://www.readbyqxmd.com/read/27988512/urine-epidermal-growth-factor-monocyte-chemoattractant-protein-1-or-their-ratio-as-biomarkers-for-interstitial-fibrosis-and-tubular-atrophy-in-primary-glomerulonephritis
#10
Supanat Worawichawong, Suchin Worawichawong, Piyanuch Radinahamed, Dittapol Muntham, Nuankanya Sathirapongsasuti, Arkom Nongnuch, Montira Assanatham, Chagriya Kitiyakara
BACKGROUND/AIMS: The degree of tubular atrophy and interstitial fibrosis (IFTA) is an important prognostic factor in glomerulonephritis. Imbalance between pro-inflammatory cytokines such as monocyte chemoattractant protein- 1 (MCP-1) and protective cytokines such as epidermal growth factor (EGF) likely determine IFTA severity. In separate studies, elevated MCP-1 and decreased EGF have been shown to be associated with IFTA severity. In this study, we aim to evaluate the predictive value of urinary EGF/MCP-1 ratio compared to each biomarker individually for moderate to severe IFTA in primary glomerulonephritis (GN)...
2016: Kidney & Blood Pressure Research
https://www.readbyqxmd.com/read/27941791/bone-marrow-derived-immature-myeloid-cells-are-a-main-source-of-circulating-supar-contributing-to-proteinuric-kidney-disease
#11
Eunsil Hahm, Changli Wei, Isabel Fernandez, Jing Li, Nicholas J Tardi, Melissa Tracy, Shikha Wadhwani, Yanxia Cao, Vasil Peev, Andrew Zloza, Jevgenijs Lusciks, Salim S Hayek, Christopher O'Connor, Markus Bitzer, Vineet Gupta, Sanja Sever, David B Sykes, David T Scadden, Jochen Reiser
Excess levels of protein in urine (proteinuria) is a hallmark of kidney disease that typically occurs in conjunction with diabetes, hypertension, gene mutations, toxins or infections but may also be of unknown cause (idiopathic). Systemic soluble urokinase plasminogen activator receptor (suPAR) is a circulating factor implicated in the onset and progression of chronic kidney disease (CKD), such as focal segmental glomerulosclerosis (FSGS). The cellular source(s) of elevated suPAR associated with future and progressing kidney disease is unclear, but is likely extra-renal, as the pathological uPAR is circulating and FSGS can recur even after a damaged kidney is replaced with a healthy donor organ...
January 2017: Nature Medicine
https://www.readbyqxmd.com/read/27938596/-focal-segmental-glomerulosclerosis-after-renal-transplantation-in-a-child-with-anca-associated-glomerulonephritis-case-report-and-literature-review
#12
L P Rong, L Z Chen, Q Fu, W F Chen, L Z Sun, X Y Jiang, Y Mo
Objective: To study the clinical features and treatment of focal segmental glomerulosclerosis (FSGS) after renal transplantation in a child with ANCA-associated glomerulonephritis. Method: The clinical and pathological data of the patient treated in the Department of Pediatrics as well as in the Department of Organ Transplantation in November 2015 in the First Affiliated Hospital of Sun Yat-sen University, who was diagnosed with de novo FSGS after renal transplantation with a primary disease ANCA-associated glomerulonephritis, was analyzed retrospectively...
December 2, 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/27934809/wilms-tumour-1-gene-mutations-in-south-indian-children-with-steroid-resistant-nephrotic-syndrome
#13
Aravind Selvin Kumar, R Srilakshmi, Smk Karthickeyan, K Balakrishnan, R Padmaraj, Prabha Senguttuvan
BACKGROUND & OBJECTIVES: Clinically, nephrotic syndrome (NS) is a diverse group of symptoms; about 20 per cent of NS cases are resistant to steroid treatment, and within ten years they progress to end-stage renal disease. The present study was undertaken to identify the mutations of Wilms' tumour 1 (WT1) gene in steroid-resistant NS (SRNS) children. METHODS: A total of 173 children with SRNS and 100 children in the control group were enrolled in the study. DNA extraction was done, screened for WT1 (exons 8 and 9) gene amplified by polymerase chain reaction and direct sequencing...
August 2016: Indian Journal of Medical Research
https://www.readbyqxmd.com/read/27932154/successful-preemptive-kidney-transplantation-with-rituximab-induction-in-a-patient-with-focal-segmental-glomerulosclerosis-and-massive-nephrotic-syndrome-a-case-report
#14
A Kolonko, G Piecha, A Więcek
Primary focal segmental glomerulosclerosis (FSGS) recurs in 30% of patients receiving their first kidney transplant and often leads to graft loss. In the past, patients with FSGS and overt nephrotic syndrome rarely underwent transplantation. Rituximab (RTX), an anti-CD20-specific monoclonal antibody, was previously reported to be a valuable option in treating relapsing FSGS after kidney transplantation. We report here the first successful kidney transplantation in a young patient with primary FSGS and massive nephrotic syndrome treated with RTX induction...
November 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27914703/evidence-from-the-oxford-classification-cohort-supports-the-clinical-value-of-subclassification-of%C3%A2-focal-segmental-glomerulosclerosis-in-iga%C3%A2-nephropathy
#15
Shubha S Bellur, Fanny Lepeytre, Olga Vorobyeva, Stéphan Troyanov, H Terence Cook, Ian S D Roberts
Focal segmental glomerulosclerosis (FSGS) is a common finding in IgA nephropathy (IgAN). Here we assessed FSGS lesions in the Oxford Classification patient cohort and correlated histology with clinical presentation and outcome to determine whether subclassification of the S score in IgAN is reproducible and of clinical value. Our subclassification of lesions in 137 individuals with segmental glomerulosclerosis or adhesion (S1) identified 38% with podocyte hypertrophy, 10% with hyalinosis, 9% with resorption droplets within podocytes, 7% with tip lesions, 3% with perihilar sclerosis, and 2% with endocapillary foam cells...
January 2017: Kidney International
https://www.readbyqxmd.com/read/27900977/unconventional-strategies-in-the-battle-of-focal-and-segmental-glomerulosclerosis
#16
Satish Mendonca, R S V Kumar, Devika Gupta, Pooja Gupta, Satish Barki, M L Sharma
A 24-year-old male presented with classic features of the nephrotic syndrome. An initial renal biopsy revealed minimal change disease and thereafter, a second biopsy showed features of focal and segmental glomerulosclerosis. There was no response to conventional immunosuppression, and the patient had to be given rituximab; in spite of this, he went on to develop end-stage renal disease. He continued to have heavy proteinuria leading to severe hypoalbuminemia, thrombosis, infections, and malnutrition, placing the patient in a life-threatening situation...
November 2016: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/27900314/renal-and-cardiovascular-morbidities-associated-with-apol1-status-among-african-american-and-non-african-american-children-with-focal-segmental-glomerulosclerosis
#17
Robert P Woroniecki, Derek K Ng, Sophie Limou, Cheryl A Winkler, Kimberly J Reidy, Mark Mitsnefes, Matthew G Sampson, Craig S Wong, Bradley A Warady, Susan L Furth, Jeffrey B Kopp, Frederick J Kaskel
BACKGROUND AND OBJECTIVES: African-American (AA) children with focal segmental glomerulosclerosis (FSGS) have later onset disease that progresses more rapidly than in non-AA children. It is unclear how APOL1 genotypes contribute to kidney disease risk, progression, and cardiovascular morbidity in children. DESIGN SETTING PARTICIPANTS AND MEASUREMENTS: We examined the prevalence of APOL1 genotypes and associated cardiovascular phenotypes among children with FSGS in the Chronic Kidney Disease in Children (CKiD) study; an ongoing multicenter prospective cohort study of children aged 1-16 years with mild to moderate kidney disease...
2016: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/27884116/kidney-disease-in-the-elderly-biopsy-based-data-from-14-renal-centers-in-poland
#18
Agnieszka Perkowska-Ptasinska, Dominika Deborska-Materkowska, Artur Bartczak, Tomasz Stompor, Tomasz Liberek, Barbara Bullo-Piontecka, Anna Wasinska, Agnieszka Serwacka, Marian Klinger, Jolanta Chyl, Michal Kuriga, Robert Malecki, Krzysztof Marczewski, Bogdan Hryniewicz, Tadeusz Gregorczyk, Monika Wieliczko, Stanislaw Niemczyk, Olga Rostkowska, Leszek Paczek, Magdalena Durlik
BACKGROUND: Longer life expectancy is associated with an increasing prevalence of kidney disease. Aging itself may cause renal damage, but the spectrum of kidney disorders that affect elderly patients is diverse. Few studies, mostly form US, Asia and West Europe found differences in the prevalence of some types of kidney diseases between elderly and younger patients based on renal biopsy findings, with varied proportion between glomerulopathies and arterionephrosclerosis as a dominant injury found...
November 25, 2016: BMC Nephrology
https://www.readbyqxmd.com/read/27847659/interferon-induced-focal-segmental-glomerulosclerosis
#19
Yusuf Kayar, Nuket Bayram Kayar, Nadir Alpay, Jamshid Hamdard, Iskender Ekinci, Sebnem Emegil, Rabia Bag Soydas, Birol Baysal
Behçet's disease is an inflammatory disease of unknown etiology which involves recurring oral and genital aphthous ulcers and ocular lesions as well as articular, vascular, and nervous system involvement. Focal segmental glomerulosclerosis (FSGS) is usually seen in viral infections, immune deficiency syndrome, sickle cell anemia, and hyperfiltration and secondary to interferon therapy. Here, we present a case of FSGS identified with kidney biopsy in a patient who had been diagnosed with Behçet's disease and received interferon-alpha treatment for uveitis and presented with acute renal failure and nephrotic syndrome associated with interferon...
2016: Case Reports in Nephrology
https://www.readbyqxmd.com/read/27816064/increased-wnt-and-notch-signaling-a-clue-to-the-renal-disease-in-schimke-immuno-osseous-dysplasia
#20
Marie Morimoto, Clara Myung, Kimberly Beirnes, Kunho Choi, Yumi Asakura, Arend Bokenkamp, Dominique Bonneau, Milena Brugnara, Joel Charrow, Estelle Colin, Amira Davis, Georges Deschenes, Mattia Gentile, Mario Giordano, Andrew K Gormley, Rajeshree Govender, Mark Joseph, Kory Keller, Evelyne Lerut, Elena Levtchenko, Laura Massella, Christy Mayfield, Behzad Najafian, David Parham, Jurgen Spranger, Peter Stenzel, Uluc Yis, Zhongxin Yu, Jonathan Zonana, Glenda Hendson, Cornelius F Boerkoel
BACKGROUND: Schimke immuno-osseous dysplasia (SIOD) is a multisystemic disorder caused by biallelic mutations in the SWI/SNF-related matrix-associated actin-dependent regulator of chromatin, subfamily A-like 1 (SMARCAL1) gene. Changes in gene expression underlie the arteriosclerosis and T-cell immunodeficiency of SIOD; therefore, we hypothesized that SMARCAL1 deficiency causes the focal segmental glomerulosclerosis (FSGS) of SIOD by altering renal gene expression. We tested this hypothesis by gene expression analysis of an SIOD patient kidney and verified these findings through immunofluorescent analysis in additional SIOD patients and a genetic interaction analysis in Drosophila...
November 5, 2016: Orphanet Journal of Rare Diseases
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