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focal segmental glomerulosclerosis

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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/28095803/increased-glomerulonephritis-recurrence-after-living-related-donation
#3
A L Kennard, S H Jiang, G D Walters
BACKGROUND: Kidney transplantation confers superior outcomes for patients with end stage kidney disease, and live donor kidneys associate with superior outcomes compared to deceased donor kidneys. Modern immunosuppression has improved rejection rates and transplant survival and, as a result, recurrence of glomerulonephritis has emerged as a major cause of allograft loss. However, many glomerulonephritides have significant genetic risk which may manifest through kidney intrinsic or systemic mechanisms...
January 17, 2017: BMC Nephrology
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
#4
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/28089478/the-clinical-course-of-minimal-change-nephrotic-syndrome-with-onset-in-adulthood-or-late-adolescence-a-case-series
#5
Rutger J Maas, Jeroen K Deegens, Johan R Beukhof, Louis J Reichert, Marc A Ten Dam, Jaap J Beutler, A Warmold L van den Wall Bake, Pieter L Rensma, Constantijn J Konings, Daniel A Geerse, Geert W Feith, Willi H Van Kuijk, Jack F Wetzels
BACKGROUND: Few studies have examined the treatment and outcome of adult-onset minimal change nephrotic syndrome (MCNS). We retrospectively studied 125 patients who had MCNS with onset in either adulthood or late adolescence. Presenting characteristics, duration of initial treatment and response to treatment, relapse patterns, complications, and long-term outcome were studied. STUDY DESIGN: Case series. SETTING & PARTICIPANTS: Patients with new-onset nephrotic syndrome 16 years or older and a histologic diagnosis of MCNS in 1985 to 2011 were identified from pathology records of 10 participating centers...
January 12, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28081313/clinical-and-histopathological-features-of-asymptomaticpersistent-microscopic-hematuria-in-children
#6
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
#7
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/28072685/nephrotic-syndrome-associated-with-metastatic-thymoma-treated-with-chemotherapy
#8
Shin Hye Yoo, Hyean-Ji Kim, Jeong-Han Kim, Gyeong-Won Lee, Jeong Hee Lee, Se Hyun Kim, Ji-Won Kim, Jin Won Kim, Jeong-Ok Lee, Yu Jung Kim, Keun-Wook Lee, Jee Hyun Kim, Soo-Mee Bang, Jong Seok Lee
RATIONALE: Nephropathy with concurrent invasive thymoma is a type of paraneoplastic syndrome. PATIENT CONCERNS AND DIAGNOSES: We report a 32-year-old female with nephrotic syndrome that was first diagnosed along with invasive thymoma and treated by means of cisplatin-based chemotherapy for the thymoma. The patient initially presented with dyspnea and generalized edema. Chest radiography and computed tomography scans revealed right pleural effusion and a mass in the right middle lung field, which were confirmed by a percutaneous lung biopsy as metastatic invasive thymoma...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28063418/focal-segmental-glomerulosclerosis-in-atypical-polycystic-kidney-disease
#9
Eun Hui Bae, Hyunsuk Kim, Sung Sun Kim, Seong Kwon Ma, Soo Wan Kim
No abstract text is available yet for this article.
January 5, 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/28059119/dysregulation-of-wti-kts-is-associated-with-the-kidney-specific-effects-of-the-lmx1b-r246q-mutation
#10
Gentzon Hall, Brandon Lane, Megan Chryst-Ladd, Guanghong Wu, Jen-Jar Lin, XueJun Qin, Elizabeth R Hauser, Rasheed Gbadegesin
Mutations in the LIM homeobox transcription factor 1-beta (LMX1B) are a cause of nail patellar syndrome, a condition characterized by skeletal changes, glaucoma and focal segmental glomerulosclerosis. Recently, a missense mutation (R246Q) in LMX1B was reported as a cause of glomerular pathologies without extra-renal manifestations, otherwise known as nail patella-like renal disease (NPLRD). We have identified two additional NPLRD families with the R246Q mutation, though the mechanisms by which LMX1BR246Q causes a renal-specific phenotype is unknown...
January 6, 2017: Scientific Reports
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
#11
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
#12
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/28024929/kidney-involvement-in-systemic-calcitonin-amyloidosis-associated-with-medullary-thyroid-carcinoma
#13
Timco Koopman, Cindy Niedlich-den Herder, Coen A Stegeman, Thera P Links, Johan Bijzet, Bouke P C Hazenberg, Arjan Diepstra
A 52-year-old woman with widely disseminated medullary thyroid carcinoma developed nephrotic syndrome and slowly decreasing kidney function. A kidney biopsy was performed to differentiate between malignancy-associated membranous glomerulopathy and tyrosine kinase inhibitor-induced focal segmental glomerulosclerosis. Surprisingly, the biopsy specimen revealed diffuse glomerular deposition of amyloid that was proved to be derived from the calcitonin hormone (Acal), produced by the medullary thyroid carcinoma...
December 23, 2016: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/27998979/alpha-actinin-4-actn4-regulates-glucocorticoid-receptor-mediated-transactivation-and-transrepression-in-podocytes
#14
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
#15
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/27994854/consequences-of-morbid-obesity-on-the-kidney-where-are-we-going
#16
Maruja Navarro Díaz
Obesity and morbid obesity are modifiable risk factors for the development and progression of kidney disease. Obesity has reached epidemic proportions and is currently an important health problem in Europe, so it is necessary to develop therapeutic and preventive strategies. The obesity-related glomerulopathy has been defined as a secondary form of focal segmental glomerulosclerosis, and its most characteristic feature is glomerulomegaly. The renal evolution of patients with obesity-related glomerulopathy (ORG) who have not been treated is unfavourable...
December 2016: Clinical Kidney Journal
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
#17
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/27942854/altered-expression-of-crb2-in-podocytes-expands-a-variation-of-crb2-mutations-in-steroid-resistant-nephrotic-syndrome
#18
Tomohiro Udagawa, Tohaku Jo, Takeshi Yanagihara, Akira Shimizu, Jun Mitsui, Shoji Tsuji, Shinichi Morishita, Reiko Onai, Kenichiro Miura, Shoichiro Kanda, Yuko Kajiho, Haruko Tsurumi, Akira Oka, Motoshi Hattori, Yutaka Harita
BACKGROUND: Steroid-resistant nephrotic syndrome (SRNS) is a genetically heterogeneous disorder for which more than 25 single-gene hereditary causes have been identified. METHODS: Whole exome sequencing was performed in a 3-year-old girl with SRNS. We analyzed the expression of Crb2 and slit diaphragm molecules in the patient's glomeruli, and compared it with that of controls or other nephrotic patients. RESULTS: Whole-exome analysis identified novel compound heterozygous mutations in exons 10 and 12 of CRB2 (p...
December 10, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27941791/bone-marrow-derived-immature-myeloid-cells-are-a-main-source-of-circulating-supar-contributing-to-proteinuric-kidney-disease
#19
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
#20
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
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