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Pediatric Nephrology: Journal of the International Pediatric Nephrology Association

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https://www.readbyqxmd.com/read/29687195/professor-gavin-c-arneil-1923-2018
#1
EDITORIAL
Heather Maxwell, David A Hughes, T James Beattie
No abstract text is available yet for this article.
April 23, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29675809/is-cytokine-release-syndrome-the-cause-of-rituximab-treatment-related-infusion-reactions-in-children-with-nephrotic-syndrome-impact-of-anti-rituximab-antibodies
#2
LETTER
Shuichiro Fujinaga, Tomohiko Nishino
No abstract text is available yet for this article.
April 19, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29671067/vaccinations-in-pediatric-kidney-transplant-recipients
#3
REVIEW
Thomas G Fox, Corina Nailescu
Pediatric kidney transplant (KT) candidates should be fully immunized according to routine childhood schedules using age-appropriate guidelines. Unfortunately, vaccination rates in KT candidates remain suboptimal. With the exception of influenza vaccine, vaccination after transplantation should be delayed 3-6 months to maximize immunogenicity. While most vaccinations in the KT recipient are administered by primary care physicians, there are specific schedule alterations in the cases of influenza, hepatitis B, pneumococcal, and meningococcal vaccinations; consequently, these vaccines are usually administered by transplant physicians...
April 18, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29666917/measurement-of-iron-status-in-chronic-kidney-disease
#4
Wesley Hayes
Anemia is a common complication of chronic kidney disease (CKD) in children, and dysregulation of iron homeostasis plays a central role in its pathogenesis. Optimizing iron status is a prerequisite for effective treatment of anemia. Insufficient iron can lead to inappropriate escalation of the erythropoiesis-stimulating agent (ESA) dose, which is associated with adverse outcomes. Excess iron supplementation also has negative sequelae including free radical tissue damage and increased risk of systemic infection...
April 17, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29663071/cyclosporine-a-responsive-congenital-nephrotic-syndrome-with-single-heterozygous-variants-in-nphs1-nphs2-and-plce1
#5
Anna Eichinger, Sabine Ponsel, Carsten Bergmann, Roman Günthner, Julia Hoefele, Kerstin Amann, Bärbel Lange-Sperandio
BACKGROUND: Congenital nephrotic syndrome (CNS) is primarily a monogenetic disease, with the majority of cases due to changes in five different genes: the nephrin (NPHS1), podocin (NPHS2), Wilms tumor 1 (WT1), laminin ß2 (LAMB2), and phospholipase C epsilon 1 (PLCE1, NPHS3) gene. Usually CNS is not responsive to immunosuppressive therapy, but treatment with ACE inhibitors, AT1 receptor blockade and/or indomethacin can reduce proteinuria. If the disease progresses to end-stage renal disease, kidney transplantation is the therapy of choice...
April 16, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29651604/patterns-and-frequency-of-renal-abnormalities-in-fanconi-anaemia-implications-for-long-term-management
#6
Vijaya Sathyanarayana, Beth Lee, Neville B Wright, Rui Santos, Denise Bonney, Robert Wynn, Leena Patel, Kate Chandler, Ed Cheesman, Detlev Schindler, Nicholas J A Webb, Stefan Meyer
BACKGROUND: Fanconi anaemia (FA) is an inherited disease with bone marrow failure, variable congenital and developmental abnormalities, and cancer predisposition. With improved survival, non-haematological manifestations of FA become increasingly important for long-term management. While renal abnormalities are recognized, detailed data on patterns and frequency and implications for long-term management are sparse. METHODS: We reviewed clinical course and imaging findings of FA patients with respect to renal complications in our centre over a 25-year period to formulate some practical suggestions for guidelines for management of renal problems associated with FA...
April 12, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29651603/cry-of-a-dying-kidney-answers
#7
Intezar Ahmed, Sunita Singh, Farhanul Huda, Nilotpal Chowdhury, Ankit Agarwal
No abstract text is available yet for this article.
April 12, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29651602/cry-of-a-dying-kidney-questions
#8
Intezar Ahmed, Sunita Singh, Farhanul Huda, Nilotpal Chowdhury, Ankit Agarwal
No abstract text is available yet for this article.
April 12, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29637272/coq2-nephropathy-a-treatable-cause-of-nephrotic-syndrome-in-children
#9
Michelle C Starr, Irene J Chang, Laura S Finn, Angela Sun, Austin A Larson, Jens Goebel, Coral Hanevold, Jenny Thies, Johan L K Van Hove, Sangeeta R Hingorani, Christina Lam
BACKGROUND: Nephrotic syndrome can be caused by a subgroup of mitochondrial diseases classified as primary coenzyme Q10 (CoQ10 ) deficiency. Pathogenic COQ2 variants are a cause of primary CoQ10 deficiency and present with phenotypes ranging from isolated nephrotic syndrome to fatal multisystem disease. CASE-DIAGNOSIS/TREATMENT: We report three pediatric patients with COQ2 variants presenting with nephrotic syndrome. Two of these patients had normal leukocyte CoQ10 levels prior to treatment...
April 10, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29637271/correction-to-systematic-assessment-of-urinary-hydroxy-oxo-glutarate-for-diagnosis-and-follow-up-of-primary-hyperoxaluria-type-iii
#10
Ada Ventzke, Markus Feldkötter, Andrew Wei, Jutta Becker, Bodo B Beck, Bernd Hoppe
The unit of the HOG-creatinine ratio presented in this article is calculated in μmol/mg creatinine instead of the demonstrated unit of μmol/μmol. This applies to the parameter in the text of the article and the labeling of Figs. 1, 2b and 3c.
April 10, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29626243/postnatal-imaging-of-prenatally-detected-hydronephrosis-when-is-voiding-cystourethrogram-necessary
#11
Sofia Visuri, Reetta Kivisaari, Timo Jahnukainen, Seppo Taskinen
OBJECTIVE: To evaluate whether grade 4-5 vesicoureteral reflux (VUR) can be predicted from renal ultrasound (RUS) findings and perform voiding cystourethrograms (VCUGs) only on high-risk patients. METHODS: The RUS and VCUG images of infants with prenatally detected hydronephrosis admitted to our institution between 2003 and 2013 were re-evaluated. The UTI episodes were collected retrospectively from patient journals. Patients with complex urinary tract anomalies were excluded...
April 7, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29626242/incidence-of-kidney-stone-disease-in-icelandic-children-and-adolescents-from-1985-to-2013-results-of-a-nationwide-study
#12
Vidar O Edvardsson, Solborg E Ingvarsdottir, Runolfur Palsson, Olafur S Indridason
BACKGROUND: An increase in the incidence of kidney stone disease has been reported for all age groups worldwide. To examine this trend, we conducted a nationwide study of the epidemiology of kidney stones in Icelandic children and adolescents over a 30-year period. METHODS: Computerized databases of all major hospitals and medical imaging centers in Iceland were searched for International Classification of Diseases and radiologic and surgical procedure codes indicative of kidney stones in patients aged < 18 years, followed by a thorough medical record review...
April 6, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29626241/infectious-disease-risks-in-pediatric-renal-transplantation
#13
REVIEW
Felicia A Scaggs Huang, Lara Danziger-Isakov
Renal transplantation is a vital treatment option in children with ESRD with more than 10,000 pediatric kidney transplants and survival rates of greater than 80% at 10 years post-transplant in the USA alone. Despite these advances, infection remains a significant cause of morbidity in pediatric recipients. Screening potential organ donors and recipients is imperative to identify and mitigate infectious risks in the transplant patient. Despite the unique risks of each patient, the timing of many infections post-transplant is predictable...
April 6, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29619552/using-dynamic-treatment-regimes-to-understand-erythropoietin-stimulating-agent-hyporesponsiveness
#14
Ari H Pollack, Assaf P Oron, Joseph T Flynn, Raj Munshi
BACKGROUND: Erythropoietin-stimulating agent hyporesponsiveness (ESAH) is associated with increased cardiovascular mortality in patients with end-stage renal disease (ESRD) on hemodialysis. Dynamic treatment regimes (DTR), a clinical decision support (CDS) tool that guides the prescription of specific therapies in response to variations in patient states, have been used to guide treatment for chronic illnesses that require frequent monitoring and therapy changes. Our objective is to explore the role of utilizing a DTR to reduce ESAH in pediatric hemodialysis patients...
April 4, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29616329/proliferative-glomerulonephritis-with-monoclonal-igg-deposits-in-children-and-young-adults
#15
Guolan Xing, Robert Gillespie, Badreldin Bedri, Albert Quan, Pingchuan Zhang, Xin J Zhou
BACKGROUND: Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) has been recognized as a distinct entity in recent years. To the best of our knowledge, all patients with PGNMID reported thus far were older than 20 years of age. We now report five cases of PGNMID in patients under 20 years of age. METHODS: The clinical database was searched for patients with native kidney biopsies from 9/2011 to 8/2017, and cases with a diagnosis of PGNMID were retrieved...
April 3, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29610995/extra-renal-manifestations-of-atypical-hemolytic-uremic-syndrome-in-children
#16
Kibriya Fidan, Nilüfer Göknar, Bora Gülhan, Engin Melek, Zeynep Y Yıldırım, Esra Baskın, Mutlu Hayran, Kaan Gülleroglu, Zeynep B Özçakar, Fatih Ozaltin, Oguz Soylemezoglu
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a chronic disease characterized by thrombotic microangiopathy and a high risk of end-stage kidney disease. Dysregulation and/or excessive activation of the complement system results in thrombotic microangiopathy. Interest in extrarenal manifestations of aHUS is increasing. This study aimed to determine the clinical characteristics of patients with extrarenal manifestations of aHUS in childhood. METHODS: This study included 70 children with extrarenal manifestations of HUS from the national Turkish aHUS Registry...
April 2, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29603016/ischaemia-reperfusion-injury-mechanisms-of-progression-to-chronic-graft-dysfunction
#17
REVIEW
Gerhard R Situmorang, Neil S Sheerin
The increasing use of extended criteria organs to meet the demand for kidney transplantation raises an important question of how the severity of early ischaemic injury influences long-term outcomes. Significant acute ischaemic kidney injury is associated with delayed graft function, increased immune-associated events and, ultimately, earlier deterioration of graft function. A comprehensive understanding of immediate molecular events that ensue post-ischaemia and their potential long-term consequences are key to the discovery of novel therapeutic targets...
March 30, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29594505/urinary-stone-risk-factors-in-the-descendants-of-patients-with-kidney-stone-disease
#18
Thasinas Dissayabutra, Nuttiya Kalpongkul, Jakkhaphan Rattanaphan, Chanchai Boonla, Monpicha Srisa-Art, Wattanachai Ungjaroenwathana, Piyaratana Tosukhowong
BACKGROUND: Evidence has indicated that immediate family members of nephrolithiasis patients had high opportunity to develop stones. However, they are usually not regarded to be at risk, since it is unclear if there are any lithogenic abnormalities found in non-stone-forming nephrolithiasis relatives. Our aim was to investigate urinary metabolic abnormalities in the children of nephrolithiasis patients, compared with the general population. METHODS: The 24-h urinary metabolic profile was studied for 28 calcium oxalate nephrolithiasis patients (NL) and 46 of their descendants (ND), as well as 40 non-stone-forming volunteers (V) and 34 of their descendants (VD)...
March 28, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29594504/management-of-high-blood-pressure-in-children-similarities-and-differences-between-us-and-european-guidelines
#19
Tammy M Brady, Amalia Stefani-Glücksberg, Giacomo D Simonetti
Over the last several decades, many seminal longitudinal cohort studies have clearly shown that the antecedents to adult disease have their origins in childhood. Hypertension (HTN), which has become increasingly prevalent in childhood, represents one of the most important risk factors for cardiovascular diseases (CVD) such as heart disease and stroke. With the risk of adult HTN much greater when HTN is manifest in childhood, the future burden of CVD worldwide is therefore concerning. In an effort to slow the current trajectory, professional societies have called for more rigorous, evidence-based guideline development to aid primary care providers and subspecialists in improving recognition, diagnosis, evaluation, and treatment of pediatric HTN...
March 28, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29594503/hyperphosphatemic-tumoral-calcinosis-caused-by-fgf23-compound-heterozygous-mutations-what-are-the-therapeutic-options-for-a-better-control-of-phosphatemia
#20
Debora Claramunt-Taberner, Aurélia Bertholet-Thomas, Marie-Christine Carlier, Frédérique Dijoud, Franck Chotel, Caroline Silve, Justine Bacchetta
BACKGROUND: Hyperphosphatemic familial tumoral calcinosis (HFTC) is a rare autosomal recessive disease caused by mutations in genes encoding FGF23 or its regulators, and leading to functional deficiency or resistance to fibroblast growth factor 23 (FGF23). Subsequent biochemical features include hyperphosphatemia due to increased renal phosphate reabsorption, and increased or inappropriately normal 1,25-dihydroxyvitamin D (1,25-D) levels. CASE-DIAGNOSIS/TREATMENT: A 15-year-old girl was referred for a 1...
March 28, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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