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Nephrology pediatrics

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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/29572749/eculizumab-treatment-in-severe-pediatric-stec-hus-a-multicenter-retrospective-study
#4
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/29546930/-acute-renal-damage-secondary-to-acute-tubulointerstitial-nephritis-drug-use-case-report
#5
Niki Oikonomopoulou, Ana Belén Martínez López, Javier Urbano Villaescusa, María Del Carmen Molina Molina, Laura Butragueño Laiseca, Daniel Barraca Nuñez, Olalla Álvarez Blanco
INTRODUCTION: Acute tubulointerstitial nephritis (ATIN) is a rare entity in the pediatric age. It is de fined by the infiltration of the renal parenchyma by mononuclear and/or polynuclear cells with se condary involvement of the tubules, without glomerular injury. It can be triggered by infections or immunological diseases, drugs like NSAIDs or be of idiopathic origin. OBJECTIVE: To raise awareness among pediatricians about the prescription of NSAIDs, especially to patients of less than a year old, since they can provoke renal damage...
December 2017: Revista Chilena de Pediatría
https://www.readbyqxmd.com/read/29543226/effectiveness-of-plasma-lyso-gb3-as-a-biomarker-for-selecting-high-risk-patients-with-fabry-disease-from-multispecialty-clinics-for-genetic-analysis
#6
Hiroki Maruyama, Kaori Miyata, Mariko Mikame, Atsumi Taguchi, Chu Guili, Masaru Shimura, Kei Murayama, Takeshi Inoue, Saori Yamamoto, Koichiro Sugimura, Koichi Tamita, Toshihiro Kawasaki, Jun Kajihara, Akifumi Onishi, Hitoshi Sugiyama, Teiko Sakai, Ichijiro Murata, Takamasa Oda, Shigeru Toyoda, Kenichiro Hanawa, Takeo Fujimura, Shigehisa Ura, Mimiko Matsumura, Hideki Takano, Satoshi Yamashita, Gaku Matsukura, Ryushi Tazawa, Tsuyoshi Shiga, Mio Ebato, Hiroshi Satoh, Satoshi Ishii
PurposePlasma globotriaosylsphingosine (lyso-Gb3) is a promising secondary screening biomarker for Fabry disease. Here, we examined its applicability as a primary screening biomarker for classic and late-onset Fabry disease in males and females.MethodsBetween 1 July 2014 and 31 December 2015, we screened 2,360 patients (1,324 males) referred from 169 Japanese specialty clinics (cardiology, nephrology, neurology, and pediatrics), based on clinical symptoms suggestive of Fabry disease. We used the plasma lyso-Gb3 concentration, α-galactosidase A (α-Gal A) activity, and analysis of the α-Gal A gene (GLA) for primary and secondary screens, respectively...
March 15, 2018: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/29511888/african-pediatric-nephrology-guidebook-coordinator-scientific-editor-amal-bourquia-ipna-2015-1st-edn-isbn-978-9954-9062-5-5
#7
Mignon Irene McCulloch
No abstract text is available yet for this article.
March 6, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29504978/lupus-erythematosus-panniculitis-in-a-10-year-old-female-child-with-severe-systemic-lupus-erythematosus-a-case-report
#8
Ruolin Zhang, Xiqiang Dang, Lanjun Shuai, Qingnan He, Xiaojie He, Zhuwen Yi
RATIONALE: Lupus erythematosus panniculitis (LEP) is a rare subset of lupus erythematosus. The incidence of LEP in systemic lupus erythematosus (SLE) ranges from 2% to 5%. In the previous literature, most LEP patients were women aged from 20 to 60 years, while pediatric cases were rare, all of whom appeared on their own without SLE.A rare LEP in a 10-year-old female child with severe SLE is presented. PATIENT CONCERNS: A 10-year-old girl was admitted to our hospital for marasmus and fatigue without other typical manifestations of SLE well before the appearance of skin lesions...
January 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29499888/alternatives-for-the-bedside-schwartz-equation-to-estimate-glomerular-filtration-rate-in-children
#9
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/29482289/pioneer-women-in-pediatric-nephrology-in-poland
#10
Ryszard Grenda, Małgorzata Pańczyk-Tomaszewska, Aleksandra Żurowska
No abstract text is available yet for this article.
February 2018: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/29464759/urine-waste-product-or-biologically-active-tissue
#11
(no author information available yet)
AIMS: Historically, urine has been viewed primarily as a waste product with little biological role in the overall health of an individual. Increasingly, data suggest that urine plays a role in human health beyond waste excretion. For example, urine might act as an irritant and contribute to symptoms through interaction with-and potential compromise of-the urothelium. METHODS: To explore the concept that urine may be a vehicle for agents with potential or occult bioactivity and to discuss existing evidence and novel research questions that may yield insight into such a role, the National Institute of Diabetes and Digestive and Kidney Disease invited experts in the fields of comparative evolutionary physiology, basic science, nephrology, urology, pediatrics, metabolomics, and proteomics (among others) to a Urinology Think Tank meeting on February 9, 2015...
February 21, 2018: Neurourology and Urodynamics
https://www.readbyqxmd.com/read/29450639/the-xlii-spanish-congress-of-pediatric-nephrology-18-20-may-2017-madrid-spain
#12
(no author information available yet)
No abstract text is available yet for this article.
February 15, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29429831/predictors-of-poor-outcome-in-children-with-steroid-sensitive-nephrotic-syndrome
#13
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
#14
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
#15
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
#16
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/29369486/utilization-trends-and-safety-of-intravenous-iron-replacement-in-pediatric-specialty-care-a-large-retrospective-cohort-study
#17
Alexander A Boucher, Amanda Pfeiffer, Ashley Bedel, Jennifer Young, Patrick T McGann
BACKGROUND: Iron deficiency is a common and clinically diverse hematologic disorder in childhood for which oral iron is often an infeasible or ineffective treatment option. Intravenous (IV) iron can be an efficient and highly successful means of iron replacement but its use has not been well-characterized on a large scale in pediatrics. PROCEDURE: All IV iron doses administered to patients for iron replacement therapy at a tertiary pediatric hospital from January 2010 through October 2016 were evaluated...
January 25, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29289551/role-of-tubulointerstitial-lesions-in-predicting-renal-outcome-among-pediatric-onset-lupus-nephritis-a-retrospective-cohort-study
#18
Chao-Yi Wu, Hui-Ping Chien, Huang-Yu Yang, Tsung-Chieh Yao, Min-Hua Tseng, Mei-Chin Yu, Kuo-Wei Yeh, Jing-Long Huang
BACKGROUND: Raising evidence suggested a prognostic utility of tubulointerstitial lesions in lupus nephritis (LN). The exact prevalence of tubulointerstitial abnormalities and its predictive value among pediatric onset systemic lupus erythematous (pSLE) cases, however, remained unknown. METHODS: Sixty-seven pSLE subjects diagnosed with LN with initial renal samples available were enrolled and followed for an average of 6.49 ± 3.06 years. Renal histology was evaluated according to the International Society of Nephrology/Renal Pathology Society classification, National Institute of Health classification and tubulointerstitial activity index (TIAI)...
December 15, 2017: Journal of Microbiology, Immunology, and Infection, Wei Mian Yu Gan Ran za Zhi
https://www.readbyqxmd.com/read/29277510/hla-dqa1-and-apol1-as-risk-loci-for-childhood-onset-steroid-sensitive-and-steroid-resistant-nephrotic-syndrome
#19
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
#20
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
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