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Pediatric Nephrology: Journal of the International Pediatric Nephrology Association

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https://www.readbyqxmd.com/read/28220235/successful-discontinuation-of-eculizumab-under-immunosuppressive-therapy-in-deap-hus
#1
Agnes Hackl, Rasmus Ehren, Michael Kirschfink, Peter F Zipfel, Bodo B Beck, Lutz T Weber, Sandra Habbig
BACKGROUND: Deficiency of complement factor H-related plasma proteins and complement factor H autoantibody-positive hemolytic uremic syndrome (DEAP-HUS), which is characterized by the deficiency of complement-factor H-related (CFHR) plasma proteins and the subsequent formation of autoantibodies against complement factor H (CFH), has been reported to have an adverse outcome in one third of patients. Therapy options include prompt removal of antibodies by plasma exchange and immunosuppressive therapy...
February 20, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28213687/ofatumumab-for-the-treatment-of-childhood-nephrotic-syndrome
#2
Chia-Shi Wang, Rochelle Schmidt Liverman, Rouba Garro, Roshan Punnoose George, Anastacia Glumova, Alana Karp, Stephanie Jernigan, Barry Warshaw
BACKGROUND: Ofatumumab is a humanized anti-CD20 monoclonal antibody that has recently garnered interest as a potential therapeutic agent for nephrotic syndrome. We report our center's experience in administering ofatumumab to five pediatric patients with idiopathic nephrotic syndrome. METHODS: Between March 2015 and November 2016, five patients were treated with ofatumumab. One patient had post-transplant recurrent focal segmental glomerulosclerosis (FSGS) which had been resistant to plasmapheresis and numerous immunosuppressive agents...
February 17, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28210841/rare-genetic-variant-in-the-cfb-gene-presenting-as-atypical-hemolytic-uremic-syndrome-and-immune-complex-diffuse-membranoproliferative-glomerulonephritis-with-crescents-successfully-treated-with-eculizumab
#3
Khalid Alfakeeh, Mohammed Azar, Majid Alfadhel, Alsuayri Mansour Abdullah, Nourah Aloudah, Khaled O Alsaad
BACKGROUND: Complement factor B gene (CFB) is an important component of the alternate pathway of complement activation that provides an active subunit that associates with C3b to form the C3 convertase, which is an essential element in complement activation. Among the complement-associated disorders, mutations and pathogenic variants in the CFB gene are relatively rare phenomena. Moreover, mutated CFB affiliation with immune-complex diffuse membranoproliferative glomerulonephritis (IC-MPGN) and atypical hemolytic uremic syndrome (aHUS) are considered a highly rare occurrence...
February 16, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28210840/dietary-sources-of-energy-and-nutrient-intake-among-children-and-adolescents-with-chronic-kidney-disease
#4
Wen Chen, Kirstie Ducharme-Smith, Laura Davis, Wun Fung Hui, Bradley A Warady, Susan L Furth, Alison G Abraham, Aisha Betoko
BACKGROUND: Our purpose was to identify the main food contributors to energy and nutrient intake in children with chronic kidney disease (CKD). METHODS: In this cross-sectional study of dietary intake assessed using Food Frequency Questionnaires (FFQ) in the Chronic Kidney Disease in Children (CKiD) cohort study, we estimated energy and nutrient intake and identified the primary contributing foods within this population. RESULTS: Completed FFQs were available for 658 children...
February 16, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28210839/thrombotic-microangiopathy-caused-by-methionine-synthase-deficiency-diagnosis-and-treatment-pitfalls
#5
Maria Helena Vaisbich, Andressa Braga, Maria Gabrielle, Clarissa Bueno, Flávia Piazzon, Fernando Kok
BACKGROUND: Inborn errors of cobalamin (Cbl) metabolism form a large group of rare diseases. One of these, Cbl deficiency type C (CblC), is a well-known cause of thrombotic microangiopathy (TMA), especially in infants. However, there has only been a single published case of TMA associated to Cbl deficiency type G (CblG), also known as methionine synthase deficiency (MSD). CASE DIAGNOSIS/TREATMENT: A 21-month-old boy presented with pallor and oral ulcers during episodes of upper respiratory infection (URI)...
February 16, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28210838/urinary-ngal-deficiency-in-recurrent-urinary-tract-infections
#6
Catherine S Forster, Kathryn Johnson, Viral Patel, Rebecca Wax, Nancy Rodig, Jonathan Barasch, Richard Bachur, Richard S Lee
INTRODUCTION: Children with recurrent urinary tract infections (rUTI) often show no identifiable cause of their infections. Neutrophil gelatinase-associated lipocalin (NGAL) is known to be upregulated within the uroepithelium and kidney of patients with UTI and exhibits a localized bacteriostatic effect through iron chelation. We hypothesize that some patients with rUTI without an identifiable cause of their recurrent infections have locally deficient NGAL production. We therefore explored whether a lack of NGAL production may be a factor in the pathogenesis of rUTI...
February 16, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28210837/toll-like-receptor-3-tlr-3-tlr-4-and-cd80-expression-in-peripheral-blood-mononuclear-cells-and-urinary-cd80-levels-in-children-with-idiopathic-nephrotic-syndrome
#7
Om P Mishra, Ravindra Kumar, Gopeshwar Narayan, Pradeep Srivastava, Abhishek Abhinay, Rajniti Prasad, Ankur Singh, Vineeta V Batra
BACKGROUND: The aims of this study were (1) to detect toll-like receptor (TLR)-3, TLR-4 and CD80 expression in peripheral blood mononuclear cells (PBMCs) and estimate urinary CD80 levels in children with idiopathic nephrotic syndrome and (2) to investigate the utility of these markers to differentiate between biopsy-proven minimal change disease (MCD) and focal segmental glomeruloscelerosis (FSGS). METHODS: The study included 70 patients with idiopathic nephrotic syndrome (NS), of whom 40 had steroid-sensitive NS (SSNS; 25 with active NS, 15 in remission) and 30 had steroid-resistant NS (SRNS) patients, and 23 healthy controls...
February 16, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28204946/clinical-outcomes-in-children-with-henoch-sch%C3%A3-nlein-purpura-nephritis-without-crescents
#8
Jean Daniel Delbet, Julien Hogan, Bilal Aoun, Iulia Stoica, Rémi Salomon, Stéphane Decramer, Isabelle Brocheriou, Georges Deschênes, Tim Ulinski
BACKGROUND: Henoch-Schönlein purpura is the most common vasculitis in children. Its long-term prognosis depends on renal involvement. The management of Henoch-Schönlein purpura nephritis (HSPN) remains controversial. This study reports the prognosis of children with HSPN presenting with class 2 International Study of Kidney Disease in Children (ISKDC) nephritis. METHODS: All children with HSPN class 2 diagnosed between 1995 and 2015 in four pediatric nephrology centers were included, and clinical and biological data were collected from the medical files...
February 15, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28204945/spectrum-of-mutations-in-chinese-children-with-steroid-resistant-nephrotic-syndrome
#9
Fang Wang, Yanqin Zhang, Jianhua Mao, Zihua Yu, Zhuwen Yi, Li Yu, Jun Sun, Xiuxiu Wei, Fangrui Ding, Hongwen Zhang, Huijie Xiao, Yong Yao, Weizhen Tan, Svjetlana Lovric, Jie Ding, Friedhelm Hildebrandt
BACKGROUND: The aim of this study was to elucidate whether genetic screening test results of pediatric patients with steroid-resistant nephrotic syndrome (SRNS) vary with ethnicity. METHODS: Using high-throughput DNA sequencing, 28 nephrotic syndrome-related genes were analyzed in 110 chil-dren affected by SRNS and 10 children with isolated proteinuria enrolled by 5 centers in China (67 boys, 53 girls). Their age at disease onset ranged from 1 day to 208 months (median, 48...
February 15, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28197888/stop-adding-insult-to-injury-identifying-and-managing-risk-factors-for-the-progression-of-acute-kidney-injury-in-children
#10
Wesley Hayes
Acute kidney injury (AKI) is common in children admitted to hospital. Whilst some recover normal kidney function following an acute kidney insult, a significant proportion experience long-term sequelae. The aim of this review is to summarize current understanding of the processes that can lead to sequelae following AKI. Kidney injury, repair, recovery and progression are described. Risk factors for progression are outlined, and potential strategies to stratify the risk of progression in children with AKI are discussed...
February 15, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28197887/the-iskdc-classification-and-a-new-semiquantitative-classification-for-predicting-outcomes-of-henoch-sch%C3%A3-nlein-purpura-nephritis
#11
Mikael Koskela, Elisa Ylinen, Elli-Maija Ukonmaanaho, Helena Autio-Harmainen, Päivi Heikkilä, Jouko Lohi, Outi Jauhola, Jaana Ronkainen, Timo Jahnukainen, Matti Nuutinen
BACKGROUND: Histological findings from primary kidney biopsies were correlated with patient outcomes in a national cohort of paediatric Henoch-Schönlein nephritis (HSN) patients. METHODS: Primary kidney biopsies from 53 HSN patients were re-evaluated using the ISKDC (International Study of Kidney Disease in Children) classification and a modified semiquantitative classification (SQC) that scores renal findings and also takes into account activity, chronicity and tubulointerstitial indices...
February 14, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28197886/spot-urine-protein-to-creatinine-ratio
#12
EDITORIAL
Guido Filler, Shih-Han Susan Huang
In a recent article in Pediatric Nephrology, EM Yang and colleagues (Pediatr Nephrol 2017: doi: 10.1007/s00467-016-3587-6 ) published a retrospective cross-sectional study involving a cohort of 442 children with an mean estimated glomerular filtration rate of >60 mL/min/1.73 m(2). The authors measured 24-h urine protein excretion (24-h UProt) alongside the morning spot urine protein to creatinine ratio (Prot/Cr) in this group of patients. While the Prot/Cr may be the only feasible way to routinely estimate the daily protein excretion of a young child, inter-individual variability in childrens' urinary creatinine excretion (UCr) may heavily influence the result...
February 14, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28197885/c4d-deposits-in-iga-nephropathy-where-does-complement-activation-come-from
#13
EDITORIAL
Rosanna Coppo
No abstract text is available yet for this article.
February 14, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28194575/outcome-of-extremely-low-birth-weight-infants-with-a-history-of-neonatal-acute-kidney-injury
#14
Syeda Maqsood, Nicholas Fung, Vikas Chowdhary, Rupesh Raina, Maroun J Mhanna
OBJECTIVE: To study the outcome of extremely low birth weight (ELBW) infants with a history of acute kidney injury (AKI). METHOD: In a retrospective, case control study, medical records of all ELBW infants admitted to the neonatal intensive care unit (NICU) between Jan 2002 and Dec 2011 were reviewed. Medical records were reviewed for infants' demographics, blood pressure (BP) at NICU discharge and at ≥3 years, and estimated glomerular filtration rate (eGFR) at ≥2 years...
February 14, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28194574/tolvaptan-treatment-for-severe-neonatal-autosomal-dominant-polycystic-kidney-disease
#15
Rodney D Gilbert, Hazel Evans, Kazeem Olalekan, Arvind Nagra, Mushfequr R Haq, Mark Griffiths
BACKGROUND: Severe neonatal autosomal-dominant polycystic kidney disease (ADPKD) is rare and easily confused with recessive PKD. Managing such infants is difficult and often unsuccessful. CASE DIAGNOSIS/TREATMENT: A female infant with massive renal enlargement, respiratory compromise and hyponatraemia was treated with the arginine vasopressin receptor 2 antagonist tolvaptan. This resolved hyponatraemia, and there was no further increase in renal size. CONCLUSION: Tolvaptan may be a useful treatment for severe neonatal PKD...
February 13, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28194573/hyponatremia-and-cyst-growth-in-neonatal-polycystic-kidney-disease-a-case-for-aquaretics
#16
EDITORIAL
Detlef Bockenhauer
Hyponatremia is a common complication in neonatal polycystic kidney disease and is thought to be due to water retention. Aquaretics are drugs that promote free water excretion by blocking the arginine vasopressin receptor type 2 (AVPR2) in the collecting duct and thus impair urinary concentration. AVPR2 is also a key stimulant for cyclic AMP production in the collecting duct and in this way promotes cyst proliferation and pathologic kidney growth in autosomal dominant polycystic kidney disease (ADPKD). Consequently, the aquaretic tolvaptan is now used to slow down progression of ADPKD in adult patients...
February 13, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28194572/repetitive-reddish-discoloration-of-the-urine-in-an-adolescent-female-following-short-distance-walking-on-a-smooth-road-answers
#17
Ekaterini Siomou, Maria Baziou, Evagelos Premetis, Cristina Vercellati, Nikolaos Chaliasos, Alexandros Makis
No abstract text is available yet for this article.
February 13, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28194571/repetitive-reddish-discoloration-of-urine-in-a-female-adolescent-following-short-distance-walking-on-a-smooth-road-questions
#18
Ekaterini Siomou, Maria Baziou, Evagelos Premetis, Cristina Vercellati, Nikolaos Chaliasos, Alexandros Makis
A previously healthy 15-year-old girl was evaluated following five episodes of reddish urine discoloration after walking for approximately 30 min on a smooth roadway. In each episode, the discoloration lasted for four to five urinations and followed by normal urine dipstick tests. No other exercise-produced urine discoloration and no other symptoms were reported. Laboratory evaluation during the episodes revealed a reddish urine sample with 3+ hemoglobin/myoglobin and absence of hematuria. Full blood count, serum creatinine, liver function tests, and electrolyte levels were all within normal limits...
February 13, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28191596/efficacy-and-safety-of-rituximab-in-comparison-with-common-induction-therapies-in-pediatric-active-lupus-nephritis
#19
Biswanath Basu, Birendranath Roy, Binu George Babu
BACKGROUND: Childhood-onset lupus nephritis (LN) is one of the most severe manifestations of systemic lupus erythematosus (SLE). Despite treatment-related toxicities, cyclophosphamide (CYC) and glucocorticoid-based treatment protocols are still considered standard therapy in managing this multisystem disorder. An effective and safe alternative induction regimen is needed. METHODS: Forty-four pediatric patients with active LN aged 3.5-13.8 (median 8.4) years, of whom 32 entered the study at diagnosis of SLE, were followed over 36 months...
February 12, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28190246/blood-pressure-and-heart-rate-during-stress-in-children-born-small-for-gestational-age
#20
Kwanchai Pirojsakul, Apinya Thanapinyo, Pracha Nuntnarumit
BACKGROUND: Increased sympathetic nervous system activity has been proposed as a potential mechanism for the blood pressure (BP) elevation seen in individuals born small for gestational age (SGA). This study was carried out to detect the changes in BP and heart rate (HR) in children born SGA during exposure to stress and to assess for changes in urinary catecholamine excretion. METHODS: Nineteen children aged 6-14 years born SGA and 17 age- and gender-matched healthy controls were included in the study...
February 11, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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