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glomerulopathies,kidney disease

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https://www.readbyqxmd.com/read/29782633/macrothrombocytopenia-renal-dysfunction-and-nephrotic-syndrome-in-a-young-male-patient-a-case-report-of-myh9-related-disease
#1
Gabriela Sevignani, Giovana Memari Pavanelli, Sibele Sauzem Milano, Bianca Ramos Ferronato, Maria Aparecida Pachaly, Hae Ii Cheong, Mauricio de Carvalho, Fellype Carvalho Barreto
MYH9-related disease is an autosomal dominant disorder caused by mutations of the MYH9 gene, which encodes the non-muscle myosin heavy chain IIA on chromosome 22q12. It is characterized by congenital macrothrombocytopenia, bleeding tendency, hearing loss, and cataracts. Nephropathy occurs in approximately 30% of MYH9-related disease in a male patient carrier of a de novo missense mutation in exon 1 of the MYH9 gene [c.287C > T; p.Ser(TCG)96(TTG)Leu]. He presented all phenotypic manifestations of the disease, but cataracts...
May 17, 2018: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/29782630/iga-nephropathy-in-salvador-brazil-clinical-and-laboratory-presentation-at-diagnosis
#2
Brenda Navarro de Souza, Maria Brandão Tavares, Maria Fernanda Sanches Soares, Washington Luis Conrado Dos Santos
INTRODUCTION: IgA nephropathy (IgAN) is the most prevalent primary glomerulopathy in the world, but great variation is reported in different countries. In Brazil, the reported prevalence is high in the Southeastern States and low in Salvador, Bahia State, Brazil. OBJECTIVES: This study investigated the clinical and histological patterns of patients with IgAN in Salvador, Brazil. METHODS: This is a descriptive study that included all patients with a diagnosis of IgAN performed in native kidney biopsies collected from referral nephrology services of public hospitals in Salvador between 2010 and 2015...
May 17, 2018: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/29775446/-chronic-kidney-disease-in-children-up-to-2-years-of-age-a-single-center-data-analysis
#3
Kinga Musiał, Marta Sałamacha, Sylwia Gralec, Anna Pers, Łukasz Jacheć, Danuta Zwolińska
Although anomalies in the urinary tract are the leading cause of chronic kidney disease (CKD) in the pediatric population, there are few studies focusing on etiological discrepancies between younger and older children. AIM: The aim of the study was to perform a comparative analysis of etiology of CKD in children hospitalized in the Department of Pediatric Nephrology at the Wrocław Medical University, with reference to the patients' age and gender. MATERIALS AND METHODS: The retrospective analysis considered medical records of 174 patients aged 0-18 years, diagnosed with CKD, hospitalized in our Department in the years 2011-2017...
April 23, 2018: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29766469/proteinuric-glomerulopathy-in-an-adolescent-with-a-distal-partial-trisomy-chromosome-1
#4
Takaya Sasaki, Masahiro Okabe, Takeshi Tosaki, Yu Honda, Masahiro Ishikawa, Nobuo Tsuboi, Takashi Yokoo
We report a case of distal partial trisomy 1 from q32.1 to 41 that have exhibited proteinuric glomerulopathy. The patient was a 17-year-old adolescent with clinical features of low birth weight, mild mental retardation and mild deafness, from the birth. He exhibited non-nephrotic range proteinuria with the mild obesity since the age of sixteen. Image studies did not reveal morphological abnormalities of the kidneys. Renal biopsy findings showed no definitive evidence of primary glomerular diseases, and were characterized by a very low glomerular density, glomerulomegaly and focal effacement of podocyte foot processes...
May 16, 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29728434/focal-segmental-glomerulosclerosis-in-a-patient-with-prefibrotic-primary-myelofibrosis
#5
Gopal Krishana Bohra, Durga Shankar Meena, Nitin Bajpai, Abhishek Purohit
We report a case of 56-year-old man presented to us with chief complaints of frothy urine and leg swelling. A urinalysis revealed nephrotic-range proteinuria. Haematological investigations revealed thrombocytosis, leucocytosis and peripheral blood smear showed a leucoerythroblastic picture. JAK 2 mutation was positive. To confirm the diagnosis of myeloproliferative neoplasm, bone marrow biopsy was done, which was suggestive of primary myelofibrosis. The patient underwent kidney biopsy due to rapidly declining renal function and persistent proteinuria, which was suggestive of focal segmental glomerulosclerosis...
May 4, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29724174/eculizumab-as-salvage-therapy-for-recurrent-monoclonal-gammopathy-induced-c3-glomerulopathy-in-a-kidney-allograft
#6
Philipp Moog, Philipp J Jost, Maike Büttner-Herold
BACKGROUND: Monoclonal gammopathy causes several kinds of renal pathology. A rare and special form is monoclonal gammopathy-induced C3 glomerulopathy (MG-C3G). Like idiopathic C3G, MG-C3G frequently leads to end-stage renal disease. MG-C3G frequently recurs after renal transplantation, leading to graft failure in most of the patients. While there is some evidence for successful treatment of recurrent idiopathic C3 glomerulopathy with eculizumab after renal transplantation, nothing is known about its efficacy in the setting of recurrent MG-C3G...
May 3, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29706017/distribution-of-exogenous-complement-factor-h-in-mice-in-vivo
#7
Aino R Koskinen, Zhu-Zhu Cheng, Matthew C Pickering, Kalevi Kairemo, Taru Meri, H Terence Cook, Seppo Meri, T Sakari Jokiranta
Factor H is an important regulator of complement activation in plasma and on cell surfaces in both humans and mice. If FH function is compromised, inappropriate complement activation on self-surfaces can have disastrous effects as seen in the kidney diseases atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy. Since FH constructs have been proposed to be used in treatment for these diseases, we studied the distribution of exogenous FH fragments in mice. Full-length mFH, mFH1-5 and mFH18-20 fragments were radiolabeled and their distribution was examined in WT, FH-/- and FH-/- C3-/- mice in vivo...
April 29, 2018: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/29657200/complementary-bodybuilding-a-potential-risk-for-permanent-kidney-disease
#8
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/29602395/the-role-of-peroxisome-proliferator-activated-receptor-%C3%AE-coactivator-1%C3%AE-pgc-1%C3%AE-in-kidney-disease
#9
REVIEW
Szu-Yuan Li, Katalin Susztak
Peroxisome proliferator-activated receptor γ coactivator 1α (PGC-1α) is a key transcriptional regulator of mitochondrial biogenesis and function. Several recent studies have evaluated the role of PGC-1α in various renal cell types in healthy and disease conditions. Renal tubule cells mostly depend on mitochondrial fatty acid oxidation for energy generation. A decrease in PGC-1α expression and fatty acid oxidation is commonly observed in patient samples and mouse models with acute and chronic kidney disease...
March 2018: Seminars in Nephrology
https://www.readbyqxmd.com/read/29602372/-the-pathology-of-renal-transplants
#10
Eduardo Vazquez Martul
In order to make an objective assessment of the histopathology of a renal biopsy during a kidney transplant, all the various elements involved in the process must be understood. It is important to know the characteristics of the donor organ, especially if the donor is older than 65. The histopathological features of the donor biopsy, especially its vascular status, are often related to an initial poor function of the transplanted kidney. The T lymphocyte inflammatory response is characteristic in acute cellular rejection; the degree of tubulitis, together with the amount of affected parenchyme, are important factors...
April 2018: Revista Española de Patología
https://www.readbyqxmd.com/read/29597189/the-spectrum-of-glomerulopathies-in-kashmir-a-tertiary-care-experience
#11
Irshad Sirwal, Ibrahim Masoodi
BACKGROUND: There is a paucity of data on the spectrum of renal diseases prevalent in the Jammu and Kashmir region. OBJECTIVES: To determine the spectrum of glomerulopathies in adult patients attending a tertiary care centre. METHODS: Two hundred and twenty-one adequate biopsies from native kidneys of adult patients who presented with features suggestive of glomerular diseases at Sheri Kashmir Institute of medical sciences Srinagar Kashmir for the last 8 years were studied in a retrospective manner...
March 29, 2018: Nephron
https://www.readbyqxmd.com/read/29592796/c3-glomerulopathy-in-cystic-fibrosis-a-case-report
#12
Domenico Santoro, Rossella Siligato, Carmela Vadalà, Mariacristina Lucanto, Simona Cristadoro, Giovanni Conti, Michele Buemi, Stefano Costa, Ettore Sabadini, Giuseppe Magazzù
BACKGROUND: C3 glomerulonephritis is a rare glomerulopathy characterized at renal biopsy by C3 deposition, alone or with scanty immunoglobulins, as well as by an electron-dense material in mesangium, subendothelial and subepithelial space. An abnormal systemic activation of the alternative pathway of the complement cascade is responsible for the development of the disease if triggered by several possible environmental conditions. We report the first case in literature of a patient affected by cystic fibrosis and C3GN...
March 28, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29588430/an-engineered-complement-factor-h-construct-for-treatment-of-c3-glomerulopathy
#13
Yi Yang, Harriet Denton, Owen R Davies, Kate Smith-Jackson, Heather Kerr, Andrew P Herbert, Paul N Barlow, Matthew C Pickering, Kevin J Marchbank
Background C3 glomerulopathy (C3G) is associated with dysregulation of the alternative pathway of complement activation, and treatment options for C3G remain limited. Complement factor H (FH) is a potent regulator of the alternative pathway and might offer a solution, but the mass and complexity of FH makes generation of full-length FH far from trivial. We previously generated a mini-FH construct, with FH short consensus repeats 1-5 linked to repeats 18-20 (FH1-5^18-20 ), that was effective in experimental C3G...
March 27, 2018: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29570919/de-novo-cmv-associated-collapsing-focal-segmental-glomerulosclerosis-in-a-kidney-transplant-recipient
#14
Anup M Patel, Robert D Zenenberg, Ryan J Goldberg
Collapsing focal segmental glomerulosclerosis (FSGS) is a variant of FSGS and is associated with severe nephrotic syndrome and acute kidney injury and can occur after kidney transplantation. The exact mechanism of collapsing FSGS after kidney transplantation is unknown, but potential causes include autoimmune diseases, certain malignancies, bisphosphonates, m-TOR inhibitors, interferon-alpha, HIV infection, and other viruses. We describe a case of de novo Cytomegalovirus (CMV)-associated collapsing FSGS in a kidney transplant recipient with a UL97 phosphotransferase mutation that was successfully treated with intravenous ganciclovir, intravenous immunoglobulin, and steroids...
March 23, 2018: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/29517501/evolving-complexity-of-complement-related-diseases-c3-glomerulopathy-and-atypical-haemolytic-uremic-syndrome
#15
H Terence Cook
PURPOSE OF REVIEW: The current review will discuss recent advances in our understanding of the pathology of C3 glomerulopathy and atypical haemolytic uremic syndrome (aHUS). RECENT FINDINGS: C3 glomerulopathy and aHUS are associated with abnormalities of control of the alternative pathway of complement. Recent articles have provided new insights into the classification of C3 glomerulopathy and its relationship to idiopathic immune complex-mediated glomerulonephritis...
May 2018: Current Opinion in Nephrology and Hypertension
https://www.readbyqxmd.com/read/29489695/a-case-report-of-poems-syndrome-with-renal-involvement-as-immunotactoid-glomerulopathy
#16
Yan Wang, Yu Yan, Zhun Sui, Bao Dong, Li Zuo
RATIONALE: POEMS syndrome is a rare multi-system disorder, which sometimes involves the kidney. Immunotactoid glomerulopathy (ITG) is an uncommon glomerular disease resulted from deposits of immunoglobulins and its derivatives. ITG caused by POEMS syndrome is rarely reported. PATIENT CONCERNS: A 63-year-old man was presented with acute kidney injury. In addition, it's found that he had abnormal serum free κ /λ ratio, polyneuropathy, Castleman's disease, organomegaly, endocrinopathy and skin changes...
March 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29477240/increased-podocyte-sirtuin-1-function-attenuates-diabetic-kidney-injury
#17
Quan Hong, Lu Zhang, Bhaskar Das, Zhengzhe Li, Bohan Liu, Guangyan Cai, Xiangmei Chen, Peter Y Chuang, John Cijiang He, Kyung Lee
Podocyte injury and loss contribute to the progression of glomerular diseases, including diabetic kidney disease. We previously found that the glomerular expression of Sirtuin-1 (SIRT1) is reduced in human diabetic glomeruli and that the podocyte-specific loss of SIRT1 aggravated albuminuria and worsened kidney disease progression in diabetic mice. SIRT1 encodes an NAD-dependent deacetylase that modifies the activity of key transcriptional regulators affected in diabetic kidneys, including NF-κB, STAT3, p53, FOXO4, and PGC1-α...
February 22, 2018: Kidney International
https://www.readbyqxmd.com/read/29457859/post-infectious-acute-glomerulonephritis-with-podocytopathy-induced-by-parvovirus-b19-infection
#18
Satoshi Hara, Masayoshi Hirata, Kiyoaki Ito, Ichiro Mizushima, Hiroshi Fujii, Kazunori Yamada, Michio Nagata, Mitsuhiro Kawano
Human parvovirus B19 infection causes a variety of glomerular diseases such as post-infectious acute glomerulonephritis and collapsing glomerulopathy. Although each of these appears independently, it has not been fully determined why parvovirus B19 provokes such a variety of different glomerular phenotypes. Here, we report a 68-year-old Japanese man who showed endocapillary proliferative glomerulonephritis admixed with podocytopathy in association with parvovirus B19 infection. The patient showed acute onset of heavy proteinuria, microscopic hematuria and kidney dysfunction with arthralgia and oliguria after close contact with a person suffering from erythema infectiosum...
March 2018: Pathology International
https://www.readbyqxmd.com/read/29450785/the-role-of-the-alternative-pathway-of-complement-activation-in-glomerular-diseases
#19
REVIEW
Emilia Łukawska, Magdalena Polcyn-Adamczak, Zofia I Niemir
The complement system (CS) has recently been recognized as a bridge between innate and adaptive immunity that constitutes a very complex mechanism controlling the clearance of pathogens, cellular debris, and immune complexes. Out of three known pathways of complement activation, the alternative pathway (AP) plays a critical role in host defense by amplifying the complement response, independently of initiation pathway and continuously maintaining low-level activity in a process called 'thick-over.' A key molecule of the CS is C3, in which the AP is constantly activated...
February 15, 2018: Clinical and Experimental Medicine
https://www.readbyqxmd.com/read/29429752/patterns-of-clinical-response-to-eculizumab-in-patients-with-c3-glomerulopathy
#20
Moglie Le Quintrec, Anne-Laure Lapeyraque, Arnaud Lionet, Anne-Laure Sellier-Leclerc, Yahsou Delmas, Véronique Baudouin, Eric Daugas, Stéphane Decramer, Leila Tricot, Mathilde Cailliez, Philippe Dubot, Aude Servais, Catherine Mourey-Epron, Franck Pourcine, Chantal Loirat, Véronique Frémeaux-Bacchi, Fadi Fakhouri
BACKGROUND: Cases reports and small series of patients with C3 glomerulopathy have reported variable efficacy of eculizumab. STUDY DESIGN: Case series of C3 glomerulopathy. SETTING & PARTICIPANTS: Pediatric and adult patients with C3 glomerulopathy treated with eculizumab between 2010 and 2016 were identified through the C3 glomerulopathy French registry database, and a questionnaire was sent to participating French pediatric and adult nephrology centers, as well as one pediatric referral center in Québec, Canada...
February 8, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
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