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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/28078835/pathological-characteristics-of-fifty-patients-with-renal-amyloidosis-in-sri-lanka
#7
D L Dasanayaka, S Wijetunge, N V Ratnatunga
Renal involvement is the most important cause of morbidity and mortality in systemic amyloidosis. This retrospective analysis was conducted to analyse the clinico-pathological characteristics of renal amyloidosis in a group of Sri Lankan patients undergoing renal biopsy. Renal amyloidosis was observed in 50/ 9712 (0.5%) renal biopsies. The underlying cause for amyloidosis was not known in most. Of the known causes multiple myeloma was the commonest. Nephrotic range proteinuria was the most common clinical outcome and most had grade I to III chronic kidney disease at the time of diagnosis...
30, 2016: Ceylon Medical Journal
https://www.readbyqxmd.com/read/28075049/src-homology-2-domain-containing-inositol-5-phosphatase-ameliorates-high-glucose-induced-extracellular-matrix-deposition-via-the-phosphatidylinositol-3-kinase-protein-kinase-b-pathway-in-renal-tubular-epithelial-cells
#8
Fan Li, Lisha Li, Jun Hao, Shuxia Liu, Huijun Duan
A typical hallmark of diabetic kidney disease (DKD) is an excessive deposition of extracellular matrix (ECM) in the glomerulus and renal tubulointerstitium, leading to glomerulosclerosis and tubular interstitial fibrosis. Src homology 2 domain-containing inositol 5'-phosphatase (SHIP) is a negative regulator of the phosphatidylinositol 3-kinase/protein kinase B (PI3K/Akt) signaling. Here, we investigated the effect of SHIP on ECM deposition in diabetic mice and high glucose-stimulated human renal tubular epithelial cells (HK2 cells)...
January 11, 2017: Journal of Cellular Biochemistry
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
#9
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
#10
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/28064215/pathophysiological-mechanisms-of-renal-fibrosis-a-review-of-animal-models-and-therapeutic-strategies
#11
REVIEW
António Nogueira, Maria João Pires, Paula Alexandra Oliveira
Chronic kidney disease (CKD) is a long-term condition in which the kidneys do not work correctly. It has a high prevalence and represents a serious hazard to human health and estimated to affects hundreds of millions of people. Diabetes and hypertension are the two principal causes of CKD. The progression of CKD is characterized by the loss of renal cells and their replacement by extracellular matrix (ECM), independently of the associated disease. Thus, one of the consequences of CKD is glomerulosclerosis and tubulointerstitial fibrosis caused by an imbalance between excessive synthesis and reduced breakdown of the ECM...
January 2017: In Vivo
https://www.readbyqxmd.com/read/28063595/the-mitochondrial-targeted-peptide-ss-31-improves-glomerular-architecture-in-mice-of-advanced-age
#12
Mariya T Sweetwyne, Jeffrey W Pippin, Diana G Eng, Kelly L Hudkins, Ying Ann Chiao, Matthew D Campbell, David J Marcinek, Charles E Alpers, Hazel H Szeto, Peter S Rabinovitch, Stuart J Shankland
Although age-associated changes in kidney glomerular architecture have been described in mice and man, the mechanisms are unknown. It is unclear if these changes can be prevented or even reversed by systemic therapies administered at advanced age. Using light microscopy and transmission electron microscopy, our results showed glomerulosclerosis with injury to mitochondria in glomerular epithelial cells in mice aged 26 months (equivalent to a 79-year-old human). To test the hypothesis that reducing mitochondrial damage in late age would result in lowered glomerulosclerosis, we administered the mitochondrial targeted peptide, SS-31, to aged mice...
January 4, 2017: Kidney International
https://www.readbyqxmd.com/read/28063418/focal-segmental-glomerulosclerosis-in-atypical-polycystic-kidney-disease
#13
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
#14
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/28050001/light-chain-deposition-disease-diagnosed-with-laser-micro-dissection-liquid-chromatography-and-tandem-mass-spectrometry-of-nodular-glomerular-lesions
#15
Tomomichi Kasagi, Hironobu Nobata, Keisuke Suzuki, Naoto Miura, Shogo Banno, Akiyoshi Takami, Taro Yamashita, Yukio Ando, Hirokazu Imai
A 42-year-old man developed nephrotic syndrome and rapidly progressive renal failure. Kidney biopsy demonstrated nodular glomerulosclerosis, negative Congo red staining, and no deposition of light or heavy chains. Laser micro-dissection and liquid chromatography with tandem mass spectrometry of nodular lesions revealed the presence of a kappa chain constant region and kappa III variable region, which signified light chain deposition disease. Dexamethasone and thalidomide were effective in decreasing the serum levels of free kappa light chain from 147...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28045061/feature-selection-and-classification-of-urinary-mrna-microarray-data-by-iterative-random-forest-to-diagnose-renal-fibrosis-a-two-stage-study
#16
Le-Ting Zhou, Yu-Han Cao, Lin-Li Lv, Kun-Ling Ma, Ping-Sheng Chen, Hai-Feng Ni, Xiang-Dong Lei, Bi-Cheng Liu
Renal fibrosis is a common pathological pathway of progressive chronic kidney disease (CKD). However, kidney function parameters are suboptimal for detecting early fibrosis, and therefore, novel biomarkers are urgently needed. We designed a 2-stage study and constructed a targeted microarray to detect urinary mRNAs of CKD patients with renal biopsy and healthy participants. We analysed the microarray data by an iterative random forest method to select candidate biomarkers and produce a more accurate classifier of renal fibrosis...
January 3, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28040266/angiotensin-ii-at1-receptor-signaling-is-necessary-for-cyclooxygenase-2-dependent-postnatal-nephron-generation
#17
Stefanie Frölich, Patrick Slattery, Dominique Thomas, Itamar Goren, Nerea Ferreiros, Boye L Jensen, Rolf M Nüsing
Deletion of cyclooxygenase-2 (COX-2) causes impairment of postnatal kidney development. Here we tested whether the renin angiotensin system contributes to COX-2-dependent nephrogenesis in mice after birth and whether a rescue of impaired renal development and function in COX-2(-/-) mice was achievable. Plasma renin concentration in mouse pups showed a birth peak and a second peak around day P8 during the first 10 days post birth. Administration of the angiotensin II receptor AT1 antagonist telmisartan from day P1 to P3 did not result in cortical damage...
December 28, 2016: Kidney International
https://www.readbyqxmd.com/read/28033650/tetrahydrobiopterin-contributes-to-the-proliferation-of-mesangial-cells-and-accumulation-of-extracellular-matrix-in-early-stage-diabetic-nephropathy
#18
Jianyun Wang, Qianqian Yang, Yaxing Nie, Hao Guo, Fan Zhang, Xueyan Zhou, Xiaoxing Yin
OBJECTIVES: Nitric oxide (NO) plays an important role in the progression of early-stage diabetic nephropathy (DN), which is found to contribute to extracellular matrix (ECM) accumulation in mesangial cells (MCs). As a cofactor for NO production, tetrahydrobiopterin (BH4 ), a folacin analogue, may be responsible for the ECM accumulation and proliferation of MCs. This study was to investigate the effects of BH4 on glomerulosclerosis in early-stage DN. METHODS: In in vitro studies with cultured mesangial cells and in vivo studies with streptozotocin-induced diabetic rats, BH4 levels were assayed by HPLC; NO was determined by Griess agents; laminin and collagen IV were determined by enzyme-linked immunosorbent assay; the inducible NO synthase protein was determined by immunofluorescence staining and Western blot; and mesangial matrix expansion and MC proliferation in the renal cortex were observed by periodic acid-schiff staining and transmission electron microscopy, respectively...
February 2017: Journal of Pharmacy and Pharmacology
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
#19
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
#20
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
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