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Pediatric Nephrology: Journal of the International Pediatric Nephrology Association

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https://www.readbyqxmd.com/read/28083702/increased-urinary-neutrophil-gelatinase-associated-lipocalin-in-very-low-birth-weight-infants-with-oliguria-and-normal-serum-creatinine
#1
So Young Shin, Ji Yong Ha, Sang Lak Lee, Won Mok Lee, Jae Hyun Park
BACKGROUND: In infants, oliguria is defined as a urine output of <1.5 mL/kg/h. The aim of our study was to assess the impact of oliguria on urinary neutrophil gelatinase-associated lipocalin (NGAL) and serum cystatin C (CysC) levels in very-low-birth-weight infants (VLBWIs) with a normal serum creatinine (Cr) level. METHODS: Fifty-seven VLBWIs were enrolled in the study. Urinary NGAL, serum CysC and Cr levels and urinary NGAL/Cr ratios were measured. Infants with Apgar scores of >5 at 5 min and/or a serum Cr level of >1...
January 12, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28083701/disorders-of-fatty-acid-oxidation-and-autosomal-recessive-polycystic-kidney-disease-different-clinical-entities-and-comparable-perinatal-renal-abnormalities
#2
Agnes Hackl, Katrin Mehler, Ingo Gottschalk, Anne Vierzig, Marcus Eydam, Jan Hauke, Bodo B Beck, Max C Liebau, Regina Ensenauer, Lutz T Weber, Sandra Habbig
BACKGROUND: Differential diagnosis of prenatally detected hyperechogenic and enlarged kidneys can be challenging as there is a broad phenotypic overlap between several rare genetic and non-genetic disorders. Metabolic diseases are among the rarest underlying disorders, but they demand particular attention as their prognosis and postnatal management differ from those of other diseases. METHODS: We report two cases of cystic, hyperechogenic and enlarged kidneys detected on prenatal ultrasound images, resulting in the suspected diagnosis of autosomal recessive polycystic kidney disease (ARPKD)...
January 12, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28074283/diabetes-and-nephrotic-syndrome-questions
#3
Rodney D Gilbert, Edward Hind, Bhumita Vadgama
No abstract text is available yet for this article.
January 10, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28070668/what-happens-to-asymptomatic-lower-pole-kidney-stones-smaller-than-10%C3%A2-mm-in-children-during-watchful-waiting
#4
Onur Telli, Nurullah Hamidi, Uygar Bagci, Arif Demirbas, Ahmet Metin Hascicek, Tarkan Soygur, Berk Burgu
BACKGROUND: The optimal management of lower pole kidney (LPK) stones in children is controversial. The aim of this study was to determine the outcomes of children with asymptomatic isolated LPK stones smaller than 10 mm during follow-up. METHODS: A total of 242 patients with 284 stones presenting at our institution between June 2004 and December 2014 with an asymptomatic, single LPK stone with a diameter of <10 mm were enrolled in the study. All children were assigned to receive first-line therapy and then categorized according to the need for medical intervention...
January 9, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28070667/erratum-to-regional-citrate-anticoagulation-for-continuous-renal-replacement-therapy-in-children
#5
Mayerly Prada Rico, Jaime Fernández Sarmiento, Ana María Rojas Velasquez, Luz Stella González Chaparro, Ricardo Gastelbondo Amaya, Hernando Mulett Hoyos, Daniel Tibaduiza, Ana Maria Quintero Gómez
No abstract text is available yet for this article.
January 9, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28062909/paediatric-anti-neutrophil-cytoplasmic-antibody-anca-associated-vasculitis-an-update-on-renal-management
#6
REVIEW
Lucy A Plumb, Louise Oni, Stephen D Marks, Kjell Tullus
The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of disorders characterized by necrotizing inflammation of the small to medium vessels in association with autoantibodies against the cytoplasmic region of the neutrophil. Included in this definition are granulomatosis with polyangiitis (GPA, formerly known as Wegener's granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome). AAV are chronic, often relapsing diseases that can be organ or life threatening...
January 6, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28039534/hypomagnesemia-and-increased-risk-of-new-onset-diabetes-mellitus-after-transplantation-in-pediatric-renal-transplant-recipients
#7
Wesley Hayes, Sheila Boyle, Adrian Carroll, Detlef Bockenhauer, Stephen D Marks
BACKGROUND: New-onset diabetes after transplantation (NODAT) is a significant co-morbidity following kidney transplantation. Lower post-transplant serum magnesium levels have been found to be an independent risk factor for NODAT in adult kidney transplant recipients. METHODS: We undertook a retrospective analysis of risk factors for NODAT in pediatric renal transplant recipients at our institution with the aim of determining if hypomagnesemia confers a significant risk of developing NODAT in this patient population...
December 30, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28035470/the-clinical-and-laboratory-features-of-chinese-han-anti-factor-h-autoantibody-associated-hemolytic-uremic-syndrome
#8
Di Song, Xiao-Rong Liu, Zhi Chen, Hui-Jie Xiao, Jie Ding, Shu-Zhen Sun, Hong-Yan Liu, Wei-Yi Guo, Su-Xia Wang, Feng Yu, Ming-Hui Zhao
BACKGROUND: Anti-complement factor H (CFH) autoantibody-associated hemolytic uremic syndrome (HUS) is a severe sub-type of HUS. METHODS: We assessed the clinical and renal pathological features, circulating complement levels, and genetic background of Chinese pediatric patients with this sub-type of HUS. Thirty-three consecutive patients with acute kidney injury who tested positive for serum anti-CFH autoantibodies were enrolled in this study. RESULTS: All of the eight patients who underwent renal biopsies presented with changes typical of thrombotic microangiopathy, especially changes in chronic characteristics...
December 29, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28028615/podocyte-number-and-density-changes-during-early-human-life
#9
Masao Kikuchi, Larysa Wickman, Raja Rabah, Roger C Wiggins
BACKGROUND: Podocyte depletion, which drives progressive glomerulosclerosis in glomerular diseases, is caused by a reduction in podocyte number, size or function in the context of increasing glomerular volume. METHODS: Kidneys obtained at autopsy from premature and mature infants who died in the first year of life (n = 24) were used to measure podometric parameters for comparison with previously reported data from older kidneys. RESULTS: Glomerular volume increased 4...
December 27, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28013382/functional-assessment-of-a-novel-col4a5-splice-region-variant-and-immunostaining-of-plucked-hair-follicles-as-an-alternative-method-of-diagnosis-in-x-linked-alport-syndrome
#10
Andrew F Malone, Steven D Funk, Tarek Alhamad, Jeffrey H Miner
BACKGROUND: Many COL4A5 splice region variants have been described in patients with X-linked Alport syndrome, but few have been confirmed by functional analysis to actually cause defective splicing. We sought to demonstrate that a novel COL4A5 splice region variant in a family with Alport syndrome is pathogenic using functional studies. We also describe an alternative method of diagnosis. METHODS: Targeted next-generation sequencing results of an individual with Alport syndrome were analyzed and the results confirmed by Sanger sequencing in family members...
December 24, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28013381/pilot-study-of-the-effect-of-cholecalciferol-supplementation-on-hepcidin-in-children-with-chronic-kidney-disease-results-of-the-d-fense-trial
#11
Meredith A Atkinson, Stephen P Juraschek, Michael S Bertenthal, Barbara Detrick, Susan L Furth, Edgar R Miller
BACKGROUND: Hepcidin is a key mediator of the anemia of chronic kidney disease (CKD). There is emerging evidence that 25-hydroxyvitamin D (25D) regulates hepcidin production. METHODS: A randomized controlled trial of daily vitamin D supplementation for 12 weeks was performed with the aim to test the effects of 4000 versus 400 IU of cholecalciferol on serum hepcidin levels in children with non-dialysis CKD recruited at a tertiary care children's hospital. Hepcidin was quantified using a validated competitive enzyme-linked immunosorbent assay...
December 24, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28012007/long-term-health-related-quality-of-life-and-psychological-adjustment-in-children-after-haemolytic-uraemic-syndrome
#12
Helene Werner, Kathrin Buder, Markus A Landolt, Thomas J Neuhaus, Guido F Laube, Giuseppina Spartà
BACKGROUND: In children after haemolytic-uraemic syndrome (HUS), little is known about long-term health-related quality of life (HRQoL) and psychological adjustment as defined by behavioural problems, depressive symptoms and post-traumatic stress symptoms. METHODS: Sixty-two paediatric patients with a history of HUS were included in this study. Medical data of the acute HUS episode were retrieved retrospectively from hospital records. Data on the clinical course at study investigation were assessed by clinical examination and laboratory evaluation...
December 23, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28012006/diabetes-and-nephrotic-syndrome-answers
#13
Rodney D Gilbert, Edward Hind, Bhumita Vadgama
No abstract text is available yet for this article.
December 23, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28012005/primary-non-surgical-management-of-unilateral-ureteropelvic-junction-obstruction-in-children-a-systematic-review
#14
REVIEW
Marcus Weitz, Maria Schmidt, Guido Laube
Ureteropelvic junction obstruction (UPJO) is the most common obstructive uropathy and its optimal management remains controversial. However, there is a current trend towards non-surgical management. We aimed to determine the effects of the non-surgical management in children with unilateral UPJO. For a systematic review, we searched MEDLINE, EMBASE, CENTRAL, clinical trials registries, and selected conference proceedings for eligible studies. Any type of study reporting the outcomes renal function, secondary surgical intervention, drainage pattern or hydronephrosis of non-surgical management in children with unilateral UPJO was included...
December 23, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27999949/anemia-in-nephrotic-syndrome-approach-to-evaluation-and-treatment
#15
Franca Iorember, Diego Aviles
Nephrotic syndrome is one of the most common glomerular diseases that affect in children. Complications may occur in nephrotic syndrome as a result of the disease itself as well as its treatment. Most of these complications result from excessive urinary protein losses, and control of proteinuria is the most effective treatment strategy. Anemia is one of the many complications seen in patients with persistent nephrotic syndrome and may occur as a result of excessive urinary losses of iron, transferrin, erythropoietin, transcobalamin and/or metals...
December 21, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27995324/hla-epitope-matching-in-pediatric-renal-transplantation
#16
Matthew P Sypek, Peter Hughes, Joshua Y Kausman
Chronic graft loss due to antibody-mediated rejection (AMR) and the difficulty of re-transplanting highly sensitized patients are two of the major long-term challenges in pediatric renal transplantation. Treatments for AMR are often ineffective and desensitization protocols can be a high risk, making prevention a highly appealing strategy. Insights into the structural determinants of humoral alloantigenicity present an exciting opportunity to reassess our current paradigm of tissue matching and potentially preventing these complications...
December 19, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27988804/monosymptomatic-nocturnal-enuresis-in-pediatric-patients-multidisciplinary-assessment-and-effects-of-therapeutic-intervention
#17
Simone N Fagundes, Adrienne Surri Lebl, Leticia Azevedo Soster, Guilherme Jorge Sousa E Silva, Edwiges Ferreira de Mattos Silvares, Vera H Koch
BACKGROUND: Few studies manage patients with isolated monosymptomatic enuresis (MNE) with multidisciplinary evaluation and pre- and long-term post-intervention monitoring. METHODS: This was a prospective study of MNE patients, aged 6-16 years, diagnosed by multidisciplinary assessment. Of the 140 initial applicants (58.6%) with MNE, 82 were included in the study and randomized for therapeutic intervention in three treatment groups, namely: alarm, desmopressin and alarm + desmopressin...
December 17, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27942854/altered-expression-of-crb2-in-podocytes-expands-a-variation-of-crb2-mutations-in-steroid-resistant-nephrotic-syndrome
#18
Tomohiro Udagawa, Tohaku Jo, Takeshi Yanagihara, Akira Shimizu, Jun Mitsui, Shoji Tsuji, Shinichi Morishita, Reiko Onai, Kenichiro Miura, Shoichiro Kanda, Yuko Kajiho, Haruko Tsurumi, Akira Oka, Motoshi Hattori, Yutaka Harita
BACKGROUND: Steroid-resistant nephrotic syndrome (SRNS) is a genetically heterogeneous disorder for which more than 25 single-gene hereditary causes have been identified. METHODS: Whole exome sequencing was performed in a 3-year-old girl with SRNS. We analyzed the expression of Crb2 and slit diaphragm molecules in the patient's glomeruli, and compared it with that of controls or other nephrotic patients. RESULTS: Whole-exome analysis identified novel compound heterozygous mutations in exons 10 and 12 of CRB2 (p...
December 10, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27942956/building-on-evidence-to-improve-patient-care
#19
REVIEW
Evelien Snauwaert, Johan VandeWalle, Evi V Nagler, Wim Van Biesen
Evidence-based medicine (EBM) is gaining importance in the current paediatric healthcare landscape. Improvement of paediatric health status is its major aim. However, for EBM to be successful, all stakeholders involved should understand what EBM really is, why and how EBM should or should not be practiced, and have the necessary skills to distinguish methodologically sound papers from biased opinion papers, and understand how and why guidelines are different from systematic reviews. Improving patient outcome requires attention to high-quality evidence and understanding of the processes of medical decision-making...
December 9, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27942955/management-dilemmas-in-pediatric-nephrology-time-limited-trials-of-dialysis-therapy
#20
Aaron Wightman
BACKGROUND: Time-limited trials of dialysis have been proposed as a third option in addition to initiation of treatment and comfort-care only in the setting of high uncertainty or discordance between the treating team and child/family or among the treating team. CASE-DIAGNOSIS/TREATMENT: The index case was noted antenatally to have severe kidney disease and pulmonary hypoplasia. In light of the guarded, but uncertain prognosis and a lack of consensus among the treating team, as well as between the treating team and the family, a time-limited trial of dialysis was initiated...
December 9, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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