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https://www.readbyqxmd.com/read/28933341/c4d-expressing-glomerulopathy-and-proteinuria-post-transplantation-of-a-%C3%A2-too-big-for-size-mismatched-kidney-allograft-an-unusual-case-with-good-outcome%C3%A2
#1
Francois Gougeon, Alexei V Mikhailov, Keisha Gibson, Tomasz Kozlowski, Harsharan K Singh, Volker Nickeleit
A 5-year-old severely growth-retarded child with tubulointerstitial, oliguric end-stage renal disease received an adult-size kidney transplant. Three years post grafting under standard triple immunosuppression (mycophenolate mofetil, tacrolimus, and prednisone) de novo nephrotic range proteinuria without the nephrotic syndrome developed. Graft function was normal (serum creatinine: 0.2 - 0.3 mg/dL), there were no donor-specific HLA antibodies (DSA), and the urine sediment was inactive. Two biopsies collected 3 and 4 years post-transplantation showed severe glomerular capillary wall remodeling and associated pseudolinear C4d staining as morphologic correlates for the proteinuria...
September 21, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28921709/kidney-allograft-failure-in-the-steroid-free-immunosuppression-era-a-matched-case-control-study
#2
Mohamad M Alkadi, Jim Kim, Meredith J Aull, Joseph E Schwartz, John R Lee, Anthony Watkins, Jun B Lee, Darshana M Dadhania, Surya V Seshan, David Serur, Sandip Kapur, Manikkam Suthanthiran, Choli Hartono, Thangamani Muthukumar
We studied the causes and predictors of death-censored kidney allograft failure among 1670 kidney recipients transplanted at our center in the corticosteroid-free maintenance immunosuppression era. As of January 1, 2012, we identified 137 recipients with allograft failure; 130 of them (cases) were matched 1-1 for recipient age, calendar year of transplant and donor type with 130 recipients with functioning grafts (controls). Median time to allograft failure was 29 months (interquartile range 18-51). Physician validated and biopsy confirmed categories of allograft failure were: acute rejection (21%), glomerular disease (19%), transplant glomerulopathy (13%), interstitial fibrosis tubular atrophy (10%), and polyomavirus-associated nephropathy (7%)...
September 18, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/28904428/collapsing-glomerulopathy-a-troublemaker-for-the-renal-allograft-lessons-learnt
#3
K V Kanodia, A V Vanikar, L K Nigam, R D Patel, K S Suthar, H V Patel, H L Trivedi
Collapsing glomerulopathy (CG) is a well-recognized distinct morphological pattern of proliferative parenchymal injury leading to rapid graft failure. We conducted a single-center retrospective study to evaluate the prevalence, clinicopathological features, and prognosis of CG in renal transplant recepient. We analyzed 2518 renal allograft biopsies performed from 2007 to 2015 and correlated their clinicopathological features. The prevalence of CG was 0.83% (21 out of 2518) of allograft biopsies with a higher prevalence of 1...
September 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28883987/obesity-and-kidney-disease-hidden-consequences-of-the-epidemic
#4
REVIEW
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. A high BMI 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...
August 2017: Future Science OA
https://www.readbyqxmd.com/read/28877681/fibronectin-glomerulopathy-complicated-with-persistent-cloaca-and-congenital-esophageal-atresia-a-case-report-and-literature-review
#5
Misaki Takii, Takaichi Suehiro, Aya Shima, Hideki Yotsueda, Satoshi Hisano, Ritsuko Katafuchi
BACKGROUND: Fibronectin glomerulopathy is a rare, inherited, autosomal dominant, glomerular disease characterized by proteinuria, microscopic hematuria, hypertension, massive glomerular deposits of fibronectin, and slow progression to end-stage renal failure. Because the incident of fibronectin glomerulopathy is extremely low, the pathophysiology, genetic abnormalities, epidemiology, and mechanisms remain to be elucidated. CASE PRESENTATION: We report a 21-year-old woman with fibronectin glomerulopathy, who had been diagnosed with persistent cloaca and congenital esophageal atresia at birth...
September 6, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28873129/association-of-serum-soluble-urokinase-receptor-levels-with-progression-of-kidney-disease-in-children
#6
Franz Schaefer, Howard Trachtman, Elke Wühl, Marietta Kirchner, Salim S Hayek, Ali Anarat, Ali Duzova, Sevgi Mir, Dusan Paripovic, Alev Yilmaz, Francesca Lugani, Klaus Arbeiter, Mieczyslaw Litwin, Jun Oh, Maria Chiara Matteucci, Jutta Gellermann, Simone Wygoda, Augustina Jankauskiene, Günter Klaus, Jiri Dusek, Sara Testa, Aleksandra Zurowska, Alberto Caldas Afonso, Melissa Tracy, Changli Wei, Sanja Sever, William Smoyer, Jochen Reiser
Importance: Conventional methods to diagnose and monitor chronic kidney disease (CKD) in children, such as creatinine level and cystatin C-derived estimated glomerular filtration rate (eGFR) and assessment of proteinuria in spot or timed urine samples, are of limited value in identifying patients at risk of progressive kidney function loss. Serum soluble urokinase receptor (suPAR) levels strongly predict incident CKD stage 3 in adults. Objective: To determine whether elevated suPAR levels are associated with renal disease progression in children with CKD...
September 5, 2017: JAMA Pediatrics
https://www.readbyqxmd.com/read/28868299/tubuloreticular-inclusions-in-the-absence-of-systemic-lupus-erythematosus-and-hiv-infection-a-report-of-three-pediatric-cases
#7
Ayah Elmaghrabi, Elizabeth Brown, Ei Khin, Jared Hassler, Allen R Hendricks
Tubuloreticular inclusions (TRIs) are subcellular structures located within the cisternae of endoplasmic reticulum. Formation of TRIs has been linked to the exposure of excess interferon (IFN), either from endogenous or exogenous sources. In renal disease, TRIs have been most commonly associated with systemic lupus erythematosus (SLE), and human immunodeficiency virus-associated nephropathy (HIVAN). Case reports of patients with renal biopsies showing TRIs without underlying SLE or HIV are infrequent in adults, and to our knowledge none have been reported in children...
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28865030/drebrin-in-renal-glomeruli
#8
Wiebke K Ludwig-Peitsch
The central function of renal glomeruli is plasma ultrafiltration for primary urine production. The glomerular filtration barrier consists of a fenestrated endothelium, the glomerular basement membrane and podocytes, mesenchymal-like cells with actin filament-rich protrusions, the "foot processes." Their architecture and function are maintained and regulated by actin and several actin-binding proteins, mutations of which can be causative of glomerular diseases. Since initial immunostaining experiments had demonstrated intense drebrin reactions in renal glomeruli, the distribution of this protein was studied in detail in the kidneys of diverse mammalian species...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28864093/biopsy-proven-renal-involvement-and-prognosis-in-13-hispanic-patients-with-primary-sj%C3%A3-gren-syndrome
#9
Diego Luis Carrillo-Pérez, Javier Tejeda-Maldonado, Carlos Garza-García, Virgilia Soto-Abraham, Gabriela Hernández-Molina, Giovanni Arnoldo Molina-Paredes, Norma O Uribe-Uribe, Luis E Morales-Buenrostro
BACKGROUND: The aim of this study was to describe a case series of 13 Hispanic patients with primary Sjögren syndrome (pSS) and biopsy-proven renal involvement. METHODS: We describe the clinical, serological and histological characteristics as well as the prognosis in a group of patients with pSS and biopsy-proven renal involvement, treated in 2 referral nephrology units in Mexico City. RESULTS: Thirteen patients with pSS underwent kidney biopsy (KB) over a period of 27 years...
August 29, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28852479/collapsing-glomerulopathy-a-30-year-perspective-and-single-large-center-experience
#10
L Nicholas Cossey, Christopher P Larsen, Helen Liapis
Collapsing glomerulopathy (CGP) is a pattern of kidney injury seen on renal biopsy with multiple associations and etiologies. It is most commonly described in African-Americans and others with recent African ancestry. The disease is rapidly progressive and often presents with abrupt onset of renal failure and nephrotic-range proteinuria. Since its description 30 years ago, this entity has transformed from a morphologic diagnosis typically seen in the setting of HIV infection to a complicated diagnosis with numerous etiologies, many of which are associated with underlying apolipoprotein L1 (APOL1)-risk variants or other genetic disorders...
August 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28842605/deficient-insulin-mediated-upregulation-of-the-equilibrative-nucleoside-transporter-2-contributes-to-chronically-increased-adenosine-in-diabetic-glomerulopathy
#11
Sebastián Alarcón, Wallys Garrido, Claudio Cappelli, Raibel Suárez, Carlos Oyarzún, Claudia Quezada, Rody San Martín
Deficient insulin signaling is a key event mediating diabetic glomerulopathy. Additionally, diabetic kidney disease has been related to increased levels of adenosine. Therefore, we tested a link between insulin deficiency and dysregulated activity of the equilibrative nucleoside transporters (ENTs) responsible for controlling extracellular levels of adenosine. In ex vivo glomeruli, high D-glucose decreased nucleoside uptake mediated by ENT1 and ENT2 transporters, resulting in augmented extracellular levels of adenosine...
August 25, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28838767/c4-nephritic-factors-in-c3-glomerulopathy-a-case-series
#12
Yuzhou Zhang, Nicole C Meyer, Fernando C Fervenza, Winnie Lau, Adam Keenan, Gabriel Cara-Fuentes, Dingwu Shao, Aalia Akber, Veronique Fremeaux-Bacchi, Sanjeev Sethi, Carla M Nester, Richard J H Smith
BACKGROUND: C3 glomerulopathy (C3G) defines a group of rare complement-mediated kidney diseases with a shared underlying pathophysiology: dysregulation of complement in the fluid phase and glomerular microenvironment. Dysregulation can be driven by autoantibodies to C3 and C5 convertases. STUDY DESIGN: Case series. SETTING & PARTICIPANTS: 168 patients with C3G (dense deposit disease, 68; C3 glumerulonephritis, 100) selected from our C3G biobank...
August 22, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28832357/recurrent-glomerular-disease-after-kidney-transplantation
#13
Christopher D Blosser, Roy D Bloom
PURPOSE OF REVIEW: With improving short-term kidney transplant outcomes, recurrent glomerular disease is being increasingly recognized as an important cause of chronic allograft failure. Further understanding of the risks and pathogenesis of recurrent glomerular disease enable informed transplant decisions, along with the development of preventive and treatment strategies. RECENT FINDINGS: Multiple observational studies have highlighted differences in rates and outcomes for various recurrent glomerular diseases, although these rates have not markedly improved over the last decade...
August 21, 2017: Current Opinion in Nephrology and Hypertension
https://www.readbyqxmd.com/read/28798244/atypical-haemolytic-uremic-syndrome-ahus-and-membranoproliferative-glomerulonephritis-mpgn-different-diseases-or-a-spectrum-of-complement-mediated-glomerular-diseases
#14
Ghada A Ankawi, William F Clark
Historically, patients with kidney diseases caused by genetic or acquired dysregulation of the complement alternative pathway have been grouped into clinical syndromes, C3 glomerulopathy (C3GN/DDD) and thrombotic microangiopathy (TMA), specifically atypical haemolytic uremic syndrome (aHUS). Recent data suggested that these diseases share a common pathophysiology and that patients can transition between glomerulopathies in this spectrum. Histopathologically, the main difference cited is the immunofluorescence (IF) findings, with C3 predominance in C3 glomerulopathy (compared with immunoglobulins and complements in immune complex-mediated membranoproliferative glomerulonephritis (MPGN)) and negative IF in TMA...
August 10, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28791668/obesity-related-glomerulopathy-pathogenesis-pathologic-clinical-characteristics-and-treatment
#15
REVIEW
Tianhua Xu, Zitong Sheng, Li Yao
In light of the rapid increase in the number of obesity incidences worldwide, obesity has become an independent risk factor for chronic kidney disease. Obesity-related glomerulopathy (ORG) is characterized by glomerulomegaly in the presence or absence of focal and segmental glomerulosclerosis lesions. IgM and complement 3 (C3) nonspecifically deposit in lesions without immune-complex-type deposits during ORG immunofluorescence. ORG-associated glomerulomegaly and focal and segmental glomerulosclerosis can superimpose on other renal pathologies...
September 2017: Frontiers of Medicine
https://www.readbyqxmd.com/read/28754557/accelerated-podocyte-detachment-and-progressive%C3%A2-podocyte-loss-from-glomeruli-with%C3%A2-age%C3%A2-in-alport-syndrome
#16
Fangrui Ding, Larysa Wickman, Su Q Wang, Yanqin Zhang, Fang Wang, Farsad Afshinnia, Jeffrey Hodgin, Jie Ding, Roger C Wiggins
Podocyte depletion is a common mechanism driving progression in glomerular diseases. Alport Syndrome glomerulopathy, caused by defective α3α4α5 (IV) collagen heterotrimer production by podocytes, is associated with an increased rate of podocyte detachment detectable in urine and reduced glomerular podocyte number suggesting that defective podocyte adherence to the glomerular basement membrane might play a role in driving progression. Here a genetically phenotyped Alport Syndrome cohort of 95 individuals [urine study] and 41 archived biopsies [biopsy study] were used to test this hypothesis...
July 26, 2017: Kidney International
https://www.readbyqxmd.com/read/28748184/complement-in-non-antibody-mediated-kidney-diseases
#17
REVIEW
Andrea Angeletti, Joselyn Reyes-Bahamonde, Paolo Cravedi, Kirk N Campbell
The complement system is part of the innate immune response that plays important roles in protecting the host from foreign pathogens. The complement components and relative fragment deposition have long been recognized to be strongly involved also in the pathogenesis of autoantibody-related kidney glomerulopathies, leading to direct glomerular injury and recruitment of infiltrating inflammation pathways. More recently, unregulated complement activation has been shown to be associated with progression of non-antibody-mediated kidney diseases, including focal segmental glomerulosclerosis, C3 glomerular disease, thrombotic microangiopathies, or general fibrosis generation in progressive chronic kidney diseases...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/28745696/-obesity-related-glomerulopathy-mechanisms-of-development-clinical-course
#18
REVIEW
I M Kutyrina
Obesity and overweight are recognized as epidemics of non-communicable diseases in the 21st century. The kidneys are a target organ for obesity, damage to which is considered to be an independent risk factor for the development of renal failure. Obesity-related glomerulopathy (OGP) is one of the types of renal injury in obesity, which is characterized by the development of proteinuria in patients with a body mass index (BMI) of >30 kg/m2 in the absence of other causes of kidney damage. The pathogenesis of OGP is multifactorial...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28737134/nephrotic-syndrome-in-primary-myelofibrosis-with-renal-extramedullary-hematopoiesis-and-glomerulopathy-in-the-jak-inhibitor-era
#19
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
#20
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
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