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glomerulopathies,kidney disease

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https://www.readbyqxmd.com/read/28737134/nephrotic-syndrome-in-primary-myelofibrosis-with-renal-extramedullary-hematopoiesis-and-glomerulopathy-in-the-jak-inhibitor-era
#1
Rachele Del Sordo, Rachele Brugnano, Carla Covarelli, Gioia Fiorucci, Franca Falzetti, Giorgio Barbatelli, Emidio Nunzi, Angelo Sidoni
Primary myelofibrosis (PMF) is an uncommon form of myeloproliferative neoplasm (MPN) characterized by a proliferation of predominantly megakaryocytes and granulocytes in the bone marrow that, in fully-developed disease, is associated with reactive deposition of fibrous connective tissue, extramedullary hematopoiesis (EMH), and splenomegaly. Kidney involvement is rare and clinically presents with proteinuria, nephrotic syndrome, and renal insufficiency. Renal damage can be due to EMH and glomerulopathy. Renal EMH presents three patterns: infiltration of the interstitium with possible renal failure caused by functional damage of parenchyma and vessels, infiltration of capsule and pericapsular adipose tissue, and sclerosing mass-like lesions that can cause hydronephrosis and hydroureter with obstructive uropathy and renal failure...
July 24, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28724797/increasing-the-level-of-peroxisome-proliferator-activated-receptor-%C3%AE-coactivator-1%C3%AE-in-podocytes-results-in-collapsing-glomerulopathy
#2
Szu-Yuan Li, Jihwan Park, Chengxiang Qiu, Seung Hyeok Han, Matthew B Palmer, Zoltan Arany, Katalin Susztak
Inherited and acquired mitochondrial defects have been associated with podocyte dysfunction and chronic kidney disease (CKD). Peroxisome proliferator-activated receptor γ coactivator-1α (PGC1α) is one of the main transcriptional regulators of mitochondrial biogenesis and function. We hypothesized that increasing PGC1α expression in podocytes could protect from CKD. We found that PGC1α and mitochondrial transcript levels are lower in podocytes of patients and mouse models with diabetic kidney disease (DKD)...
July 20, 2017: JCI Insight
https://www.readbyqxmd.com/read/28716013/long-term-renal-survival-of-%C3%AE-3-heavy-chain-deposition-disease-a-case-report
#3
Takayuki Katsuno, Shige Mizuno, Masatsuna Mabuchi, Naotake Tsuboi, Atsushi Komatsuda, Shoichi Maruyama
BACKGROUND: Monoclonal immunoglobulin deposition disease (MIDD) is characterized by the non-amyloid deposition of monoclonal immunoglobulin fragments in the basement membranes. Heavy chain deposition disease (HCDD) is a type of MIDD. HCDD is an extremely rare disease, and only three cases have been reported in Japan up to the present. The prognosis of HCDD is very poor, and optimal treatment has not been established. Only a few cases of HCDD with favorable long-term renal prognosis have been reported to date...
July 17, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28700996/immunosuppressive-treatment-in-c3-glomerulopathy-is-it-really-effective
#4
Yasar Caliskan, Ege Sinan Torun, Tarik Onur Tiryaki, Aysegul Oruc, Yasemin Ozluk, Sebahat Usta Akgul, Sonay Temurhan, Nida Oztop, Isin Kilicaslan, Mehmet Sukru Sever
BACKGROUND: C3 glomerulopathy (C3GP) is a recently identified and described disease that has a high risk of progressing into end-stage renal disease. We aimed to evaluate the effects of various immunosuppressive regimens on C3GP progression because there are conflicting data on the treatment modalities. METHODS: In this retrospective study of 66 patients with C3GP, 27 patients received mycophenolate mofetil (MMF)-based treatment, 23 received non-MMF-based treatment (prednisolone or cyclophosphamide), and 16 received conservative care...
July 13, 2017: American Journal of Nephrology
https://www.readbyqxmd.com/read/28690757/minimal-change-disease-in-horseshoe-kidney
#5
Yosr Chaabouni, Rahma Guesmi, Yosr Hentati, Khaoula Kammoun, Mohamed Ben Hmida, Zeineb Mnif, Tahya Boudawara, Jamil Hachicha
The horseshoe kidney is a frequent urological birth defect. The most frequent complications are urinary tract infections, stones and hydronephrosis. The occurrence of glomerular disease in horseshoe kidney is rare. Therefore, we report the first case of minimal change disease occurring in a patient with horseshoe kidney in literature. A 22-year-old Caucasian man without personal or family medical history admitted to the pneumology department for a pulmonary artery embolism. In presence of a generalized oedema, a biological assessment was performed yielding intense nephrotic syndrome with urine protein excretion 22g/day...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28682562/-renal-diseases-related-to-myh9-disorders
#6
Dario Galeano, Luca Zanoli, Vincenzo L'Imperio, Pasquale Fatuzzo, Antonio Granata
Mutations in MYH9 gene encoding the nonmuscle myosin heavy chain IIA (NMMHC-IIA) are related to a number of rare autosomal-dominant disorders which has been known as May-Hegglin disease, Sebastian syndrome, Fechtner syndrome and Epstein syndrome. Their common clinical features are congenital macrothrombocytopaenia and polymorphonuclear inclusion bodies, in addition to a variable risk of developing proteinuria, chronic kidney disease progressing toward end stage, sensorineural deafness and presenile cataracts...
April 2017: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/28638599/obesity-and-kidney-disease-hidden-consequences-of-the-epidemic
#7
Csaba P Kovesdy, Susan L Furth, Carmine Zoccali
Obesity has become a worldwide epidemic, and its prevalence has been projected to grow by 40% in the next decade. This increasing prevalence has implications for the risk of diabetes, cardiovascular disease and also for chronic kidney disease (CKD). A high body mass index is one of the strongest risk factors for new-onset CKD. In individuals affected by obesity, a compensatory hyperfiltration occurs to meet the heightened metabolic demands of the increased body weight. The increase in intraglomerular pressure can damage the kidneys and raise the risk of developing CKD in the long term...
February 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28615647/differential-synchrotron-x-ray-imaging-markers-based-on-the-renal-microvasculature-for-tubulointerstitial-lesions-and-glomerulopathy
#8
Yu-Chuan Lin, Yeukuang Hwu, Guo-Shu Huang, Michael Hsiao, Tsung-Tse Lee, Shun-Min Yang, Ting-Kuo Lee, Nan-Yow Chen, Sung-Sen Yang, Ann Chen, Shuk-Man Ka
High resolution synchrotron microtomography capable of revealing microvessels in three dimensional (3D) establishes distinct imaging markers of mouse kidney disease strongly associated to renal tubulointerstitial (TI) lesions and glomerulopathy. Two complementary mouse models of chronic kidney disease (CKD), unilateral ureteral obstruction (UUO) and focal segmental glomerulosclerosis (FSGS), were used and five candidates of unique 3D imaging markers were identified. Our characterization to differentially reflect the altered microvasculature of renal TI lesions and/or glomerulopathy demonstrated these image features can be used to differentiate the disease status and the possible cause therefore qualified as image markers...
June 14, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28614766/simple-obesity-and-renal-function
#9
D Sikorska, M Grzymislawska, M Roszak, P Gulbicka, K Korybalska, J Witowski
Increasing evidence accumulate to suggest that obesity increases the risk of chronic kidney disease independently of dyslipidemia, diabetes, and hypertension. This so-called obesity-related glomerulopathy is characterized at early stages by glomerular hypertrophy with or without secondary focal segmental glomerulosclerosis. Since, however, kidney biopsies are usually not performed at this phase, an early diagnosis of the disease is often difficult. Here, we review new developments in the pathophysiology of obesity-associated kidney dysfunction and discuss the potential of appropriate monitoring of glomerular filtration rate and albuminuria for early detection of the disease...
April 2017: Journal of Physiology and Pharmacology: An Official Journal of the Polish Physiological Society
https://www.readbyqxmd.com/read/28612263/tight-metabolic-control-plus-ace-inhibitor-therapy-improves-gsd-i-nephropathy
#10
Gyongyi O Okechuku, Lawrence R Shoemaker, Monika Dambska, Laurie M Brown, Justin Mathew, David A Weinstein
The onset of microalbuminuria (MA) heralds the onset of glomerulopathy in patients with glycogen storage disease (GSD) type I. Unlike tubulopathy, which responds to improved metabolic control, glomerulopathy in GSD I is considered refractory to medical intervention, and it is thought to inexorably progress to overt proteinuria and renal failure. Recent reports of reduced microalbuminuria following strict adherence to therapy counter this view. In contrast to type Ia, little is known regarding the prevalence of kidney disease in GSD Ib, 0, III, VI, and IX...
June 13, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28593446/c3-glomerulonephritis-with-a-severe-crescentic-phenotype
#11
Aishwarya Ravindran, Fernando C Fervenza, Richard J H Smith, Sanjeev Sethi
BACKGROUND: C3 glomerulopathy (C3G) is rare type of glomerulonephritis resulting from the glomerular deposition of C3 due to dysregulation of the alternative pathway of complement. It is further subdivided into C3 glomerulonephritis (C3GN) and dense deposit disease (DDD), depending on the ultrastructural features. C3GN usually presents with a membranoproliferative pattern of injury. Crescents may or may not be present. However, we have noted a severe necrotizing and crescentic glomerulonephritis in a small subset of C3GN patients...
June 7, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28577739/mitochondrial-cytopathies-and-the-kidney
#12
REVIEW
Francesco Emma, Leonardo Salviati
Mitochondrial cytopathies include a heterogeneous group of diseases that are characterized by impaired oxidative phosphorylation. Current evidence suggests that renal involvement is probably more frequent than originally suspected but remains subclinical in a significant number of patients or is underestimated due to the severity of other clinical manifestations. Until recently, these diseases were thought to develop primarily in pediatric patients but patients that become symptomatic only in adulthood are now well recognized...
April 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/28566477/long-term-outcome-of-steroid-resistant-nephrotic-syndrome-in-children
#13
Agnes Trautmann, Sven Schnaidt, Beata S Lipska-Ziętkiewicz, Monica Bodria, Fatih Ozaltin, Francesco Emma, Ali Anarat, Anette Melk, Marta Azocar, Jun Oh, Bassam Saeed, Alaleh Gheisari, Salim Caliskan, Jutta Gellermann, Lina Maria Serna Higuita, Augustina Jankauskiene, Dorota Drozdz, Sevgi Mir, Ayse Balat, Maria Szczepanska, Dusan Paripovic, Alexandra Zurowska, Radovan Bogdanovic, Alev Yilmaz, Bruno Ranchin, Esra Baskin, Ozlem Erdogan, Giuseppe Remuzzi, Agnieszka Firszt-Adamczyk, Elzbieta Kuzma-Mroczkowska, Mieczyslaw Litwin, Luisa Murer, Marcin Tkaczyk, Helena Jardim, Anna Wasilewska, Nikoleta Printza, Kibriya Fidan, Eva Simkova, Halina Borzecka, Hagen Staude, Katharina Hees, Franz Schaefer
We investigated the value of genetic, histopathologic, and early treatment response information in prognosing long-term renal outcome in children with primary steroid-resistant nephrotic syndrome. From the PodoNet Registry, we obtained longitudinal clinical information for 1354 patients (disease onset at >3 months and <20 years of age): 612 had documented responsiveness to intensified immunosuppression (IIS), 1155 had kidney biopsy results, and 212 had an established genetic diagnosis. We assessed risk factors for ESRD using multivariate Cox regression models...
May 31, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28554737/podocyte-specific-jak2-overexpression-worsens-diabetic-kidney-disease-in-mice
#14
Hongyu Zhang, Viji Nair, Jharna Saha, Kevin B Atkins, Jeffrey B Hodgin, Thomas L Saunders, Martin G Myers, Thomas Werner, Matthias Kretzler, Frank C Brosius
Activation of JAK-STAT signaling has been implicated in the pathogenesis of diabetic kidney disease. An increased expression of JAK-STAT genes was found in kidney glomerular cells, including podocytes, in patients with early diabetic kidney disease. However, it is not known whether increased expression of JAK or STAT isoforms in glomerular cells can lead to worsening nephropathy in the setting of diabetes. Therefore, we overexpressed JAK2 mRNA specifically in glomerular podocytes of 129S6 mice to determine whether this change alone could worsen diabetic kidney disease...
May 26, 2017: Kidney International
https://www.readbyqxmd.com/read/28548184/obesity-and-kidney-disease-hidden-consequences-of-the-epidemic
#15
Csaba P Kovesdy, Susan Furth, Carmine Zoccali
Obesity has become a worldwide epidemic, and its prevalence has been projected to grow by 40% in the next decade. This increasing prevalence has implications for the risk of diabetes, cardiovascular disease and also for Chronic kidney disease. A high body mass index is one of the strongest risk factors for new-onset Chronic kidney disease. In individuals affected by obesity, a compensatory hyperfiltration occurs to meet the heightened metabolic demands of the increased body weight. The increase in intraglomerular pressure can damage the kidneys and raise the risk of developing Chronic kidney disease in the long-term...
March 2017: Revista Médica de Chile
https://www.readbyqxmd.com/read/28540902/primary-focal-segmental-glomerulosclerosis-recurring-rapidly-as-collapsing-glomerulopathy-in-a-renal-allograft-recipient
#16
Vinita Agrawal, Narayan Prasad, Pritpal Singh
Recurrent focal segmental glomerulosclerosis (FSGS) develops in about 30%-40% of patients of FSGS undergoing renal transplantation. We report a patient who received a live- related renal transplant for end-stage renal disease due to a primary FSGS (not otherwise specified) in the native kidney and presented with graft dysfunction in the immediate posttransplant period. The first and the second biopsy showed no evidence of rejection or glomerular lesion. A repeat biopsy done on the 30th day revealed recurrent FSGS morphologically presenting as collapsing variant...
May 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28540059/obesity-and-kidney-disease-hidden-consequences-of-the-epidemic
#17
EDITORIAL
Csaba P Kovesdy, Susan L Furth, Carmine Zoccali
Obesity has become a worldwide epidemic, and its prevalence has been projected to grow by 40% in the next decade. This increasing prevalence has implications for the risk of diabetes, cardiovascular disease, and also for chronic kidney disease (CKD). A high body mass index is one of the strongest risk factors for new-onset CKD. In individuals affected by obesity, a compensatory hyperfiltration occurs to meet the heightened metabolic demands of the increased body weight. The increase in intraglomerular pressure can damage the kidneys and raise the risk of developing CKD in the long term...
2017: Canadian Journal of Kidney Health and Disease
https://www.readbyqxmd.com/read/28457424/what-can-we-do-when-all-collapses-fatal-outcome-of-collapsing-glomerulopathy-and-systemic-lupus-erythematosus-with-diffuse-alveolar-hemorrhage-case-report
#18
H Pinto, R Leal, L Rodrigues, L Santos, C Romãozinho, F Macário, R Alves, J Pratas, V Sousa, C Marinho, L Prado E Castro, F Costa, M Campos, A Mota, A Figueiredo
INTRODUCTION: Collapsing glomerulopathy (CG) is a rare form of glomerular injury. Although commonly associated with human immunodeficiency virus (HIV) infection, it can occur in association with systemic lupus erythematosus (SLE). CASE REPORT: We present the case of a 50-year-old man, with chronic kidney disease secondary to focal and segmental glomerulosclerosis, who received a cadaveric kidney transplant in 2007. There were no relevant intercurrences until May 2015, when he presented with nephrotic range proteinuria (± 4 g/d)...
May 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28457396/nephrotic-range-proteinuria-in-renal-transplantation-clinical-and-histologic-correlates-in-a-10-year-retrospective-study
#19
R Leal, H Pinto, A Galvão, L Santos, C Romãozinho, F Macário, R Alves, J Pratas, V Sousa, C Marinho, L Prado E Castro, M Campos, A Mota, A Figueiredo
INTRODUCTION: There is a high incidence of nephrotic proteinuria in renal transplant recipients, which is an accurate predictor of graft loss. Despite this, its histologic correlates and prognostic implications are still not well characterized. We assessed the clinical and histological correlates of kidney transplantation patients with nephrotic range proteinuria. METHODS: We have retrospectively analyzed clinical and histological data from 50 kidney transplantation biopsy specimens from 44 renal transplant recipients with nephrotic range proteinuria between 2006 and 2015...
May 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28442280/kidney-disease-in-patients-with-obesity-it-is-not-always-obesity-related-glomerulopathy-alone
#20
Steven P Salvatore, James M Chevalier, Sheng F Kuo, Pat F Audia, Surya V Seshan
OBJECTIVE: Patients with obesity are at risk for chronic kidney disease. The aim is to characterize the spectrum of kidney disease in these patients, which may be related to obesity, termed obesity-related glomerulopathy (ORG), or may have other diseases secondary to associated or unassociated medical conditions. METHODS: Native kidney biopsies from 2000 to 2012 were retrospectively reviewed from all patients with body mass index >30kg/m(2). Glomerular diameter was measured using a standard micrometer and clinicopathologic characteristics were analyzed...
April 22, 2017: Obesity Research & Clinical Practice
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