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nephrologic syndrome

Luis Palma-Garcia, Victor Velásquez-Rimachi, Armando Pezo-Pezo, Joseph Roig, Julio Perez-Villegas
BACKGROUND: Therapeutic plasma exchange (TPE) is an extracorporeal procedure which consists of removing the patient's plasma and replacing it with an appropriate replacement fluid. Plasma and blood cells are separated by a centrifugation process. Our department has used TPE for several years, and in 2013 we introduced an institutional apheresis protocol. The main objective of this report is to describe the TPE procedures performed between 2013 and 2016 in the Peruvian population. METHODS: We analyzed the technical and clinical aspects of 864 centrifugal TPE procedures as well as the associated complications...
March 14, 2018: Journal of Clinical Apheresis
Arunkumar Subbiah, Soumita Bagchi, Dipankar Bhowmik, Sandeep Mahajan, R K Yadav, Yogesh Chhabra, Sanjay Agarwal
BACKGROUND: There are annual outbreaks of Dengue infection in tropical and subtropical countries. This retrospective study aimed to assess the clinical manifestation of dengue and outcome in renal transplant recipients. METHODS: Renal transplant recipients diagnosed with dengue in the nephrology department during the outbreak from August 2015 to December 2015 were included in the study. RESULTS: Twenty patients developed dengue presenting during the outbreak...
March 7, 2018: Transplant Infectious Disease: An Official Journal of the Transplantation Society
Suheyla Apaydin
Therapeutic plasma aphresis (plasmapheresis) is one form of treatment that is frequently used in practice of Nephrology. Plasmapheresis is the most important part of the therapies for Goodpasture's syndrome and anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis which are causes of rapidly progressive glomerulonephritis. The reason why the effectiveness of plasmapheresis therapy cannot be clearly demonstrated in renal involvement in these diseases is that it does not appear to be possible to recruit an adequate number of patients and plasmapheresis is not effective in advanced disease if early treatment is not initiated...
February 20, 2018: Transfusion and Apheresis Science
Joanne M Bargman, Michael Girsberger
BACKGROUND: Peritoneal dialysis (PD) is one of the corner stones of renal replacement therapy and should be strongly considered if preemptive kidney transplantation is not available. SUMMARY: There are several initiatives that may help the growth in the use of PD around the world. First, PD is an underused and valuable option in patients with heart failure and the chronic cardiorenal syndrome, especially in those with frequent hospitalizations despite optimal medical therapy...
January 26, 2018: Blood Purification
Bedra Sharif, Moumita Barua
PURPOSE OF REVIEW: The widespread adoption of next-generation sequencing by research and clinical laboratories has begun to uncover the previously unknown genetic basis of many diseases. In nephrology, one of the best examples of this is seen in focal and segmental glomerulosclerosis (FSGS) and nephrotic syndrome. We review advances made in 2017 as a result of human and molecular genetic studies as it relates to FSGS and nephrotic syndrome. RECENT FINDINGS: There are more than 50 monogenic genes described in steroid-resistant nephrotic syndrome and FSGS, with seven reported in 2017...
February 19, 2018: Current Opinion in Nephrology and Hypertension
Joanne Reid, Helen R Noble, Gary Adamson, Andrew Davenport, Ken Farrington, Denis Fouque, Kamyar Kalantar-Zadeh, John Mallett, C McKeaveney, S Porter, David S Seres, Joanne Shields, Adrian Slee, Miles D Witham, Alexander P Maxwell
BACKGROUND: Surveys using traditional measures of nutritional status indicate that muscle wasting is common among persons with end-stage kidney disease (ESKD). Up to 75% of adults undergoing maintenance dialysis show some evidence of muscle wasting. ESKD is associated with an increase in inflammatory cytokines and can result in cachexia, with the loss of muscle and fat stores. At present, only limited data are available on the classification of wasting experienced by persons with ESKD...
February 13, 2018: BMC Nephrology
Mine Koruyucu, Figen Seymen, Genco Gencay, Koray Gencay, Elif Bahar Tuna, Teo Jeon Shin, Hong-Keun Hyun, Young-Jae Kim, Jung-Wook Kim
BACKGROUND/AIMS: Enamel-renal syndrome is characterized by nephrocalcinosis, enamel defects, gingival hyperplasia and eruption failures. It has been recently identified that recessive mutations in the FAM20A gene result in amelogenesis imperfecta (AI)-gingival fibromatosis. The aim of this research to determine whether AI patients with known -FAM20A mutations also have nephrocalcinosis. METHODS: Complete oral and radiological examinations were performed for all participating family members...
February 13, 2018: Nephron
Kirtisudha Mishra, Sandeep Kumar Kanwal, Sushma Veeranna Sajjan, Vikram Bhaskar, Bimbadhar Rath
BACKGROUND AND OBJECTIVE: Nearly 50% of the children with steroid sensitive nephrotic syndrome (SSNS) have a frequently relapsing (FR) or steroid dependent (SD) course, experiencing steroid toxicities and complications of immunosuppression. The study aimed to compare parameters between children with infrequent relapsing (IFR) and FR/SD nephrotic syndrome and to identify the factors associated with a FR/SD course. METHODS: A retrospective analysis of medical records from 2009 to 2014, of children with SSNS attending the pediatric nephrology clinic in a tertiary care medical college and hospital...
February 8, 2018: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
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
Melih Ustaoğlu, Feyza Önder, Nilgün Solmaz, Savaş Öztürk, Mesut Ayer
A 25-year-old woman presented with acute bilateral blurred vision and history of headache, dizziness, and syncope for three days. Her visual acuity was 20/60 in both eyes. Fundoscopy revealed multiple bilateral peripapillary yellow-white patches like cotton wool spots, intraretinal hemorrhages and macular edema. The patient was diagnosed with Purtscher-like retinopathy based on the retinal findings and lack of trauma history. She was urgently admitted to the nephrology clinic due to thrombotic microangiopathy findings (hemoglobinemia, thrombocytopenia, and acute renal failure)...
December 2017: Turkish Journal of Ophthalmology
Hsien-Fu Chiu, Hung-Chun Chen, Kuo-Cheng Lu, Kuo-Hsiung Shu
BACKGROUND: Despite the development of biomarkers and noninvasive imaging tools, biopsy remains the only method for correctly diagnosing patients with unexplained hematuria, proteinuria and renal failure. Renal biopsy has been performed for several decades in Taiwan; however, a national data registry is still lacking until 2013. METHODS: The Renal Biopsy Registry Committee was established within the Taiwan Society of Nephrology in January 2013. A biopsy registry format, including basic demographic data, baseline clinical features, laboratory data, and clinical and pathological diagnosis was developed...
January 10, 2018: BMC Nephrology
Adebowale Adeyemo, Christopher Esezobor, Adaobi Solarin, Asiri Abeyagunawardena, Jameela A Kari, Sherif El Desoky, Larry A Greenbaum, Margret Kamel, Mahmoud Kallash, Cynthia Silva, Alex Young, Tracey E Hunley, Nilka de Jesus-Gonzalez, Tarak Srivastava, Rasheed Gbadegesin
BACKGROUND: Few data exist for the genetic variants underlying the risk for steroid-sensitive nephrotic syndrome (SSNS) in children. The objectives of this study were to evaluate HLA-DQA1 and APOL1 variants as risk factors for SSNS in African American children and use classic HLA antigen types and amino acid inference to refine the HLA-DQA1 association. STUDY DESIGN: Case-control study. SETTING & PARTICIPANTS: African American children with SSNS or steroid-resistant nephrotic syndrome (SRNS) were enrolled from Duke University and centers participating in the Midwest Pediatric Nephrology Consortium...
December 22, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
Andrei Niculae, Ileana Peride, Vlad Vinereanu, Daniela Rădulescu, Ovidiu Gabriel Bratu, Bogdan Florin Geavlete, Ionel Alexandru ChecheriŢă
Monoclonal gammopathy with renal significance (MGRS) is a relative new-described entity, diagnosed especially in older patients and deriving from the group with monoclonal gammopathy of undetermined significance (MGUS). Various renal lesions may arise in MGRS, according to the ultrastructural characteristics of the monoclonal immunoglobulin deposition in the kidney, from proliferative glomerulopathies and amyloidosis to light chain proximal tubulopathy and crystal-storing histiocytosis. Although both are considered premalign or non-malignant hematological conditions, kidney involvement in MGRS aggravates the prognosis of the patients and need to be treated aggressively...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
Yuqi Shen, Jingyuan Xie, Li Lin, Xiao Li, Pingyan Shen, Xiaoxia Pan, Hong Ren, Nan Chen
BACKGROUND: Steroid therapy has become an effective option for patients with primary Sjogren's syndrome with tubulointerstitial nephritis (TIN), while the use of cytotoxic agents is still debated. Our study aimed to compare the clinical outcomes of patients treated with cyclophosphamide (CTX) combined with glucocorticoids with those of patients treated with glucocorticoids alone. METHODS: All patients with primary Sjogren's syndrome with chronic TIN admitted to the Division of Nephrology, Ruijin Hospital, from January 1, 2002, to April 30, 2016, and treated with steroids alone or combined with CTX were included...
2017: American Journal of Nephrology
Yeawon Kim, Sun-Ji Park, Scott R Manson, Carlos Af Molina, Kendrah Kidd, Heather Thiessen-Philbrook, Rebecca J Perry, Helen Liapis, Stanislav Kmoch, Chirag R Parikh, Anthony J Bleyer, Ying Maggie Chen
ER stress has emerged as a signaling platform underlying the pathogenesis of various kidney diseases. Thus, there is an urgent need to develop ER stress biomarkers in the incipient stages of ER stress-mediated kidney disease, when a kidney biopsy is not yet clinically indicated, for early therapeutic intervention. Cysteine-rich with EGF-like domains 2 (CRELD2) is a newly identified protein that is induced and secreted under ER stress. For the first time to our knowledge, we demonstrate that CRELD2 can serve as a sensitive urinary biomarker for detecting ER stress in podocytes or renal tubular cells in murine models of podocyte ER stress-induced nephrotic syndrome and tunicamycin- or ischemia-reperfusion-induced acute kidney injury (AKI), respectively...
December 7, 2017: JCI Insight
Sila Ulus, Gulhan Ertan Akan, Cengiz Erol
Aim: A case of asymptomatic aneurysm of spontaneous portosystemic venous fistula (SPVF) with the radiologic findings is described. Background: Although advances and more widespread use of ultrasound (US) and computed tomog -raphy angiography (CTA) have enabled more detection of SPVF in the liver, it is a rare entity. Case report: A 49-year-old male was referred to our hospital's nephrology outpatient clinic due to hypertension. Abdominal sonography examination detected a well-defined cystic lesion adjacent to the middle hepatic vein in the liver...
July 2017: Euroasian Journal of Hepato-Gastroenterology
Demet Alaygut, Meral Torun Bayram, Belde Kasap, Alper Soylu, Mehmet Türkmen, Salih Kavukcu
AIM: To investigate different etiologies and management of the rhabdomyolysis in children. METHODS: Eight pediatric rhabdomyolysis cases who applied to the Dokuz Eylul University Faculty of Medicine Department of Pediatric Nephrology with different etiologies between January 2004 and January 2012 were evaluated in terms of age, gender, admission symptoms, physical examination findings, factors provoking rhabdomyolysis, number of rhabdomyolysis attacks, laboratory results, family history and the final diagnosis received after the treatment...
November 8, 2017: World Journal of Clinical Pediatrics
Claire Hentzen, Nicolas Turmel, Camille Chesnel, Frederique Le Breton, Samer Sheikh Ismael, Gérard Amarenco
Marfan syndrome is a genetic disease responsible for causing cardiovascular, eye and musculoskeletal damages. Urinary disorders are not common. We present 4 cases of chronic urinary tract symptoms, with 2 different pathophysiological processes. Three patients presented with spinal cord infarct following aortic dissection surgery. They were affected by an overactive bladder with detrusor overactivity and detrusor-sphincter dyssynergia. One patient complained of voiding dysfunction, possibly related to dural ectasia...
November 24, 2017: Urologia Internationalis
Abdelaali Bahadi, Mohammed Reda El Farouki, Yassir Zajjari, Driss El Kabbaj
INTRODUCTION: End-stage renal disease (ESRD) is a major public health concern in Morocco with an incidence in constant progression according to MAGREDIAL "Morocco Dialysis Registry". Patients are often sent late to nephrologists, which is a source of complications recognized in several countries. For these reasons, we tried to evaluate, in our context, the prevalence and factors of this late referral (LR). METHODS: This is a retrospective study which included all patients initiating hemodialysis between January 2007 and December 2015...
December 2017: Néphrologie & Thérapeutique
Joanna Walczak-Sztulpa, Anna Wawrocka, Anna Swiader-Lesniak, Magdalena Socha, Aleksander Jamsheer, Dorota Drozdz, Anna Latos-Bielenska, Katarzyna Zachwieja
BACKGROUND: Sensenbrenner syndrome (cranioectodermal dysplasia, CED) is a very rare autosomal recessive ciliopathy first described by Judith Sensenbrenner in 1975. CED is a complex disorder characterized by craniofacial, skeletal, and ectodermal abnormalities. The clinical symptoms are variable and the CED phenotype may present intrafamilial and interfamilial differences. Sensenbrenner syndrome belongs to a group of ciliary chondrodysplasias and is a genetically heterogeneous disease...
November 14, 2017: Birth Defects Research
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