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Nephrology and children

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https://www.readbyqxmd.com/read/29657197/adamts-13-level-in-children-with-severe-diarrhea-associated-hemolytic-uremic-syndrome-unmasking-new-association
#1
Naglaa A Khalifa, Heba H Gawish, Noora A Khalifa, Doaa M Tawfeek, Saed M Morsy
Severe deficiency of ADAMTS-13 leads to thrombotic thrombocytopenic purpura. Few studies have reported reduced activity of ADAMTS-13 in patients with atypical and typical hemolytic uremic syndrome (HUS). We hypothesized that ADAMTS-13 deficiency might play a role in the pathogenesis of severe HUS. This study aimed to evaluate the ADAMTS-13 level in severe typical HUS. This prospective case-control study was carried out in the Pediatric Nephrology Unit and Clinical Pathology Department, Faculty of Medicine, Zagazig University from February 2013 to February 2014...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29623269/cystic-kidney-diseases-from-the-adult-nephrologist-s-point-of-view
#2
REVIEW
Roman-Ulrich Müller, Thomas Benzing
Cystic kidney diseases affect patients of all age groups with the onset spanning from prenatal disease to late adulthood. Autosomal-dominant polycystic kidney disease (ADPKD) is by far the most common renal cystic disease. However, there are various cystic kidney diseases, the onset of which occurs at different times in life and depends on the type of the disease and the causative genes involved. When genetic kidney diseases are discussed in the adult setting this view is usually limited on autosomal-dominant kidney disease, the most frequent genetic disorder causing adult onset ESRD...
2018: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29617835/patient-and-transplant-outcome-in-infants-starting-renal-replacement-therapy-before-2-years-of-age
#3
Julien Hogan, Justine Bacchetta, Marina Charbit, Gwenaelle Roussey, Robert Novo, Michel Tsimaratos, Joelle Terzic, Tim Ulinski, Arnaud Garnier, Elodie Merieau, Jérôme Harambat, Isabelle Vrillon, Olivier Dunand, Denis Morin, Etienne Berard, Francois Nobili, Cécile Couchoud, Marie-Alice Macher
Background: Despite major technical improvements in the care of children requiring renal replacement therapy (RRT) before 2 years of age, the management of those patients remains challenging and transplantation is generally delayed until the child weighs 10 kg or is 2 years old. In this national cohort study, we studied patient and graft survival in children starting RRT before 2 years of age to help clinicians and parents when deciding on RRT initiation and transplantation management...
March 29, 2018: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29597214/evolution-of-technology-for-continuous-renal-replacement-therapy-forty-years-of-improvement
#4
Claudio Ronco
Continuous arteriovenous hemofiltration (CAVH) was proposed in 1977 as an alternative treatment for acute renal failure in patients in whom peritoneal dialysis or hemodialysis was clinically or technically precluded. In the mid-1980s, this technique was extended to infants and children. CAVH presented important advantages in the areas of hemodynamic stability, control of circulating volume, and nutritional support. However, there were serious shortcomings such as the need for arterial cannulation and limited solute clearance...
2018: Contributions to Nephrology
https://www.readbyqxmd.com/read/29572749/eculizumab-treatment-in-severe-pediatric-stec-hus-a-multicenter-retrospective-study
#5
Lucas Percheron, Raluca Gramada, Stéphanie Tellier, Remi Salomon, Jérôme Harambat, Brigitte Llanas, Marc Fila, Emma Allain-Launay, Anne-Laure Lapeyraque, Valerie Leroy, Anne-Laure Adra, Etienne Bérard, Guylhène Bourdat-Michel, Hassid Chehade, Philippe Eckart, Elodie Merieau, Christine Piètrement, Anne-Laure Sellier-Leclerc, Véronique Frémeaux-Bacchi, Chloe Dimeglio, Arnaud Garnier
BACKGROUND: Hemolytic uremic syndrome related to Shiga-toxin-secreting Escherichia coli infection (STEC-HUS) remains a common cause of acute kidney injury in young children. No specific treatment has been validated for this severe disease. Recently, experimental studies highlight the potential role of complement in STEC-HUS pathophysiology. Eculizumab (EC), a monoclonal antibody against terminal complement complex, has been used in severe STEC-HUS patients, mostly during the 2011 German outbreak, with conflicting results...
March 23, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29499888/alternatives-for-the-bedside-schwartz-equation-to-estimate-glomerular-filtration-rate-in-children
#6
REVIEW
Hans Pottel, Laurence Dubourg, Karolien Goffin, Pierre Delanaye
The bedside Schwartz equation has long been and still is the recommended equation to estimate glomerular filtration rate (GFR) in children. However, this equation is probably best suited to estimate GFR in children with chronic kidney disease (reduced GFR) but is not optimal for children with GFR >75 mL/min/1.73 m2 . Moreover, the Schwartz equation requires the height of the child, information that is usually not available in the clinical laboratory. This makes automatic reporting of estimated glomerular filtration rate (eGFR) along with serum creatinine impossible...
January 2018: Advances in Chronic Kidney Disease
https://www.readbyqxmd.com/read/29490907/parents-refusing-dialysis-for-a-3-month-old-with-renal-failure
#7
Laurel Willig, Erin Paquette, D Micah Hester, Bradley A Warady, John D Lantos
A 3-month-old boy with failure to thrive was referred to a nephrology clinic after a diagnostic workup for failure to thrive revealed a serum urea nitrogen level of 95 mg/dL and creatinine level of 3.6 mg/dL. A renal ultrasound revealed marked bilateral hydronephrosis with little remaining renal cortex in either kidney. A voiding cystourethrogram revealed evidence of posterior urethral valves. The child had no evident comorbidities. Fulguration of the valves was successfully performed but did not lead to improvement in kidney function...
February 28, 2018: Pediatrics
https://www.readbyqxmd.com/read/29485220/factors-that-influenced-undergoing-renal-replacement-therapy-and-survival-in-children-with-acute-kidney-injury
#8
Fatma Zehra Oztek-Celebi, Sare Gulfem Ozlu, Ozlem Aydog
BACKGROUND: Acute kidney injury (AKI) is an important clinical condition which is associated with increased mortality and morbidity. This study was performed to identify the factors that influence AKI stage, undergoing renal replacement therapy (RRT) and mortality. METHODS: This study was retrospectively conducted on 219 children with AKI who had been referred to paediatric nephrology division of Dr. Sami Ulus Teaching Hospital during their inpatient treatment from 2008 to 2012...
February 27, 2018: Nephrology
https://www.readbyqxmd.com/read/29460702/comparison-between-disease-onset-patterns-of-egyptian-juvenile-and-adult-systemic-lupus-erythematosus-single-centre-experience
#9
H H Abdel-Nabi, R A Abdel-Noor
Background Systemic lupus erythematosus (SLE) is a complex autoimmune disease that occurs worldwide in both children and adults, with different disease manifestations, activity and severity between them. Objectives To analyse the difference in disease onset patterns and activity in Egyptian children and adults with SLE. Methods A retrospective cohort study conducted on 298 Egyptian SLE patients, 215 adults (a-SLE) (>18 years) and 83 children (j-SLE) (≤18 years). Disease onset, clinical manifestations and laboratory investigations were recorded...
January 1, 2018: Lupus
https://www.readbyqxmd.com/read/29456205/mutational-analysis-of-agxt-gene-in-libyan-children-with-primary-hyperoxaluria-type-1-at-tripoli-children-hospital
#10
Naziha R Rhuma, Omar A Fituri, Laila T Sabei
Primary hyperoxaluria type 1 (PH1) is an inborn error of glyoxylate metabolism. It results from genetic mutation of the AGXT gene. The study objective was to verify the clinical and epidemiological patterns of PH1 in Libyan children at Tripoli Children Hospital confirmed by AGXT gene mutation. A descriptive case series study of 53 children with PH1 diagnosed between 1994 and 2015 was carried out in the Nephrology Unit at Tripoli Children Hospital. Diagnosis of PH1 was based on the clinical presentation (renal stones or nephrocalcinosis), positive family history of PH1, and high 24 h urinary oxalate...
January 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29429831/predictors-of-poor-outcome-in-children-with-steroid-sensitive-nephrotic-syndrome
#11
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
https://www.readbyqxmd.com/read/29429752/patterns-of-clinical-response-to-eculizumab-in-patients-with-c3-glomerulopathy
#12
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
https://www.readbyqxmd.com/read/29393148/acute-kidney-injury-diagnosis-and-classification-in-adults-and-children
#13
Zaccaria Ricci, Stefano Romagnoli
BACKGROUND: The acute reduction of kidney function in critically and noncritically ill patients (regardless of their age) is one of the deadliest clinical conditions ever reported in modern medicine. Acute kidney injury (AKI) symptoms are sneaky and potentially difficult to be identified at the right time at the bedside. One of the greatest efforts of the recent history of critical care nephrology has been to find a common classification for AKI definition and staging with the purpose of allowing a timely diagnosis and push forward epidemiologic research...
2018: Contributions to Nephrology
https://www.readbyqxmd.com/read/29372471/validation-of-estimated-glomerular-filtration-rate-equations-for-japanese-children
#14
Yoshimitsu Gotoh, Osamu Uemura, Kenji Ishikura, Tomoyuki Sakai, Yuko Hamasaki, Yoshinori Araki, Riku Hamda, Masataka Honda
BACKGROUND: The gold standard for evaluation of kidney function is renal inulin clearance (Cin). However, the methodology for Cin is complicated and difficult, especially for younger children and/or patients with bladder dysfunction. Therefore, we developed a simple and easier method for obtaining the estimated glomerular filtration rate (eGFR) using equations and values for several biomarkers, i.e., serum creatinine (Cr), serum cystatin C (cystC), serum beta-2 microglobulin (β2MG), and creatinine clearance (Ccr)...
January 25, 2018: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/29345400/fludrocortisone-a-treatment-for-tubulopathy-post-paediatric-renal-transplantation-a-national-paediatric-nephrology-unit-experience
#15
S R Ali, I Shaheen, D Young, I Ramage, H Maxwell, D A Hughes, D Athavale, M G Shaikh
Calcineurin inhibitors post-renal transplantation are recognized to cause tubulopathies in the form of hyponatremia, hyperkalemia, and acidosis. Sodium supplementation may be required, increasing medication burden and potentially resulting in poor compliance. Fludrocortisone has been beneficial in addressing tubulopathies in adult studies, with limited paediatric data available. A retrospective review of data from an electronic renal database from December 2014 to January 2016 was carried out. Forty-seven post-transplant patients were reviewed with 23 (49%) patients on sodium chloride or bicarbonate...
January 18, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29277510/hla-dqa1-and-apol1-as-risk-loci-for-childhood-onset-steroid-sensitive-and-steroid-resistant-nephrotic-syndrome
#16
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...
March 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29273970/renal-replacement-therapy-for-children-throughout-the-world-the-need-for-a-global-registry
#17
Sophie Ploos van Amstel, Marlies Noordzij, Bradley A Warady, Francisco Cano, Jonathan C Craig, Jaap W Groothoff, Kenji Ishikura, Alicia Neu, Hesham Safouh, Hong Xu, Kitty J Jager, Franz Schaefer
BACKGROUND: To describe the factors affecting the incidence of renal replacement therapy (RRT) among children, information from RRT registries is required. We aimed to give an overview of existing pediatric RRT registries worldwide, identify regions with a need to commence or increase data collection on pediatric RRT, and provide a rationale for developing a global RRT registry. METHODS: A survey assessing pediatric RRT registry status was sent to International Pediatric Nephrology Associateion (IPNA) members in 127 countries in January 2016...
May 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29244791/management-and-outcomes-of-childhood-goodpasture-s-disease
#18
Carmela P Menzi, Barbara S Bucher, Mario G Bianchetti, Gianluigi Ardissino, Giacomo D Simonetti
BACKGROUND: In an attempt to improve knowledge about childhood Goodpasture's disease, we performed a retrospective analysis of patients with Goodpasture's disease from several pediatric nephrology centers. METHODS: We analyzed the responses to 27 questions that elicited information about the following: incidence, demographics, patient history and clinical presentation, diagnostics performed, acute and chronic therapy, course of disease and outcome. RESULTS: Goodpasture's disease, which is extremely rare in this age group, may manifest in 2-year-old toddlers and does not typically present with pulmonary findings before puberty...
December 15, 2017: Pediatric Research
https://www.readbyqxmd.com/read/29232664/vaccination-practices-in-pediatric-dialysis-patients-across-europe-a-european-pediatric-dialysis-working-group-and-european-society-for-pediatric-nephrology-dialysis-working-group-study
#19
Sevcan A Bakkaloğlu, Yesim Özdemir Atikel, Fabio Paglialonga, Constantinos J Stefanidis, Varvara Askiti, Enrico Vidal, Gema Ariceta, Engin Melek, Enrico Verrina, Nikoleta Printza, Karel Vondrak, Aleksandra Zurowska, Ilona Zagozdzon, Mesiha Ekim, Elif Nursel Özmert, Stephanie Dufek, Augustina Jankauskiene, Claus Peter Schmitt, Eszter Lévai, Johan Vande Walle, Nur Canpolat, Tuula Holtta, Michel Fischbach, Guenter Klaus, Christoph Aufricht, Rukshana Shroff, Alberto Edefonti
BACKGROUND: Data on the immunization practices in pediatric chronic kidney disease (CKD) patients are scarce. The purpose of this study was to evaluate current vaccination practices for children on dialysis across European pediatric nephrology centers. METHODS: A total of 18 tertiary pediatric nephrology centers from 12 European countries were included in the study. The data on universal national immunization programs and immunization practices for children with chronic disease or risk were recorded from European Center for Disease Prevention and Control and the World Health Organization...
2018: Nephron
https://www.readbyqxmd.com/read/29220099/supportive-palliative-care-should-be-integrated-into-routine-care-for-paediatric-patients-with-life-limiting-kidney-disease
#20
REVIEW
Julia Thumfart, Tobias Reindl, Cornelia Rheinlaender, Dominik Müller
Paediatric palliative care is no longer restricted to patients with cancer and has been extended to patients with other chronic conditions, such as cystic fibrosis or neuromuscular disorders. This review focused on the current state of palliative care for children and adolescents with chronic kidney disease (CKD). We assessed the literature on CKD published up to August 2017. All the papers, except one from 1996, were published this century. This review discusses the role that palliative care plays in the process of decision-making and explores the possibilities of implementing palliative care into the routine therapy of affected patients and providing support for their families...
March 2018: Acta Paediatrica
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