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pediatric hypophosphatemia

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https://www.readbyqxmd.com/read/27889724/phenotype-of-dent-disease-in-a-cohort-of-indian-children
#1
Swati Bhardwaj, Ranjeet Thergaonkar, Aditi Sinha, Pankaj Hari, Cheong Hi, Arvind Bagga
OBJECTIVE: To describe the clinical and genotypic features of Dent disease in children diagnosed at our center over a period of 10 years. DESIGN: Case series. SETTING: Pediatric Nephrology Clinic at a referral center in Northern India. METHODS: The medical records of patients with Dent disease diagnosed and followed up at this hospital from June 2005 to April 2015 were reviewed. The diagnosis of Dent disease was based on presence of all three of the following: (i) low molecular weight proteinuria, (ii) hypercalciuria and (iii) one of the following: nephrolithiasis, hematuria, hypophosphatemia or renal insufficiency, with or without mutation in CLCN5 or OCRL1 genes...
November 15, 2016: Indian Pediatrics
https://www.readbyqxmd.com/read/27820122/tumor-induced-osteomalacia-in-a-3-year-old-with-unresectable-central-giant-cell-lesions
#2
Stephanie S Crossen, Eduardo Zambrano, Beverley Newman, Jonathan A Bernstein, Anna H Messner, Laura K Bachrach, Clare J Twist
Tumor-induced osteomalacia (TIO) is a rare cause of hypophosphatemia involving overproduction of fibroblast growth factor 23. TIO has been described largely in adults with small mesenchymal tumors. We report a case of TIO in a child who presented with knee pain and radiographic findings concerning for rickets, and was found to have maxillomandibular giant cell lesions. The patient was treated with oral phosphorus and calcitriol, surgical debulking, and intralesional corticosteroids, which resulted in tumor regression and normalization of serum fibroblast growth factor 23and phosphorus...
November 4, 2016: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27392619/hypophosphatemia-in-critically-ill-children-risk-factors-outcome-and-mechanism
#3
Satish Kumar Shah, Mohammad Irshad, Nandita Gupta, Sushil Kumar Kabra, Rakesh Lodha
OBJECTIVES: To determine the prevalence of hypophosphatemia in critically ill children and its association with clinical outcomes; to determine risk factors and mechanism of hypophosphatemia. METHODS: Levels of serum phosphate, phosphate intake, renal phosphate handling indices and blood gases were measured on days 1, 3, 7 and 10 of pediatric intensive care unit (PICU) stay. Hypophosphatemia was defined as any serum phosphorus <3.8 mg/dl for children younger than 2 y and <3...
November 2016: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/27380125/incidence-and-clinical-outcome-of-hypophosphatemia-in-pediatric-burn-patients
#4
Heitor Pons Leite, Larissa Araújo Pinheiro Nogueira, Ariane Helena Calassa Teodosio
The objective of this study is to investigate the factors associated with serum phosphate concentrations in severely burned children and whether hypophosphatemia is associated with outcome. Seventy-eight children with a total body surface area of 24% (6.0-68.5) were retrospectively analyzed for serum phosphate concentrations during the first 10 days of stay in the intensive care unit (ICU). The method of generalized estimating equations was used to evaluate the effect of the exposure variables for serum phosphate concentrations during the study period...
June 29, 2016: Journal of Burn Care & Research: Official Publication of the American Burn Association
https://www.readbyqxmd.com/read/27150561/neonatal-transient-hypophosphatemic-hypercalciuric-rickets-in-dizygous-twins-a-role-for-maternal-alendronate-therapy-before-pregnancy-or-antireflux-medications
#5
M Gerin, G Jambon, A Fouque-Aubert, C Raybaud, P Cochat, O Claris, J Bacchetta
BACKGROUND: Bisphosphonates (BP) are sometimes used in children and young women, but their use requires expertise and caution due to the relative lack of long-term efficacy and safety data. CLINICAL CASES: We report on two dizygotic male twins with a past of mild prematurity who presented at the age of 2 months with moderate clinical craniotabes, hypophosphatemia, normal circulating calcium, severe hypercalciuria, and low parathyroid hormone levels. Following supplementation with oral phosphorus and native vitamin D, the clinical and biological abnormalities disappeared within 2 months...
September 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/26869613/hypophosphatemia-hypomagnesemia-and-hypokalemia-in-pediatric-patients-before-and-during-exclusive-individualized-parenteral-nutrition
#6
Taís Daiene Russo Hortencio, Roberto José Negrao Nogueira, Fernando Augusto de Lima Marson, Antonio Fernando Ribeiro
BACKGROUND: Hypophosphatemia, hypomagnesemia, and hypokalemia occur in patients receiving parenteral nutrition (PN), mainly when the body's stores are depleted due to fasting or inflammation. Although these disorders are potentially fatal, few studies have reported the incidence in the pediatric population. METHODS: This study evaluated, in a historical cohort of pediatric patients, the prevalence of hypophosphatemia, hypokalemia, and hypomagnesaemia until 48 hours before initiation of PN infusion (P1) and from days 1-4 (P2) and days 5-7 (P3) of PN infusion and investigated if malnutrition, calories, and protein infusion were correlated to these disorders...
April 2016: Nutrition in Clinical Practice
https://www.readbyqxmd.com/read/26774639/prophylactic-phosphate-supplementation-for-the-inpatient-treatment-of-restrictive-eating-disorders
#7
Maya Leitner, Brett Burstein, Holly Agostino
PURPOSE: The medical stabilization of adolescent patients with restrictive eating disorders can be associated with refeeding syndrome, a potentially fatal complication preceded by refeeding hypophosphatemia (RH). Whether RH can be prevented by routine prophylactic phosphate supplementation has not been previously examined. This study sought to determine the safety and efficacy of a refeeding strategy that incorporates prophylactic phosphate supplementation to prevent RH. METHODS: Retrospective chart data were collected for patients aged younger than 18 years with restrictive eating disorders admitted to a tertiary pediatric inpatient ward between January 2011 and December 2014...
June 2016: Journal of Adolescent Health: Official Publication of the Society for Adolescent Medicine
https://www.readbyqxmd.com/read/26543054/hereditary-hypophosphatemia-in-norway-a-retrospective-population-based-study-of-genotypes-phenotypes-and-treatment-complications
#8
Silje Rafaelsen, Stefan Johansson, Helge Ræder, Robert Bjerknes
OBJECTIVE: Hereditary hypophosphatemias (HH) are rare monogenic conditions characterized by decreased renal tubular phosphate reabsorption. The aim of this study was to explore the prevalence, genotypes, phenotypic spectrum, treatment response, and complications of treatment in the Norwegian population of children with HH. DESIGN: Retrospective national cohort study. METHODS: Sanger sequencing and multiplex ligand-dependent probe amplification analysis of PHEX and Sanger sequencing of FGF23, DMP1, ENPP1KL, and FAM20C were performed to assess genotype in patients with HH with or without rickets in all pediatric hospital departments across Norway...
February 2016: European Journal of Endocrinology
https://www.readbyqxmd.com/read/26540764/nephrolithiasis-and-nephrocalcinosis-in-children-metabolic-and-genetic-factors
#9
REVIEW
Velibor Tasic, Zoran Gucev
Diagnosis and management of pediatric nephrolithiasis/nephrocalcinosis is a very complex and challenging task for every pediatrician. It is based on correct. disease history taking, which may guide to the mode of inheritance (dominant, recessive, x-linked). Ethnicity and consanguinity should also be investigated since they predispose to high prevalence of certain disorders. One should always begin with cheap and available screening tests. Herein we will review clinical, biochemical, metabolic and genetic characteristics of the inherited diseases which lead to nephrolithiasis/nephrocalcinosis, such as: idiopathic hypercalciuria, renal hypophosphatemia, renal tubular acidosis, idiopathic infantile hypercalcemia, Dent disease, familial hypomagnesemia with hypercalciuria and nephrocalcinosis, hypocitraturia, cystinuria, primary hyperoxaluria and renal hypouricemia...
September 2015: Pediatric Endocrinology Reviews: PER
https://www.readbyqxmd.com/read/26511199/a-rare-case-of-acquired-fanconi-s-syndrome-with-monoclonal-gammopathy-in-an-infant
#10
Brian Castillo, Brian N Chang, Amer Wahed, Ashok Tholpady
BACKGROUND: Monoclonal gammopathies associated with acquired Fanconi's syndrome (AFS) have been reported in the adult population. AFS is characterized by renal dysfunction resulting in proteinuria, aminoaciduria, hypophosphatemia, glucosuria, and hyperchloremic metabolic acidosis. In this case report, we document the clinical and laboratory findings of a preterm infant with features of both AFS and monoclonal gammopathy in the urine. METHODS: Clinical suspicion of AFS prompted the following laboratory studies to be performed: urine protein electrophoresis (UPEP), urine immunofixation, and urine amino acid analysis with high performance liquid chromatography (HPLC)...
September 2016: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/26308295/short-term-safety-of-zoledronic-acid-in-young-patients-with-bone-disorders-an-extensive-institutional-experience
#11
Sobenna George, David R Weber, Paige Kaplan, Kelly Hummel, Heather M Monk, Michael A Levine
CONTEXT: Zoledronic acid (ZA) is increasingly used in young patients with bone disorders. However, data related to the safety of ZA administration in this population are limited. OBJECTIVE: The study aimed to characterize the short-term safety profile of ZA and identify risk factors for ZA-related adverse events (AEs) in young patients. DESIGN, SETTING, AND PARTICIPANTS: This was a retrospective chart review of inpatients and outpatients less than 21 years old who received at least one ZA infusion between July 2010 and January 2014 at The Children's Hospital of Philadelphia...
November 2015: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/26040953/contemporary-medical-and-surgical-management-of-x-linked-hypophosphatemic-rickets
#12
REVIEW
Melinda S Sharkey, Karl Grunseich, Thomas O Carpenter
X-linked hypophosphatemia is an inheritable disorder of renal phosphate wasting that clinically manifests with rachitic bone pathology. X-linked hypophosphatemia is frequently misdiagnosed and mismanaged. Optimized medical therapy is the cornerstone of treatment. Even with ideal medical management, progressive bony deformity may develop in some children and adults. Medical treatment is paramount to the success of orthopaedic surgical procedures in both children and adults with X-linked hypophosphatemia. Successful correction of complex, multiapical bone deformities found in patients with X-linked hypophosphatemia is possible with careful surgical planning and exacting surgical technique...
July 2015: Journal of the American Academy of Orthopaedic Surgeons
https://www.readbyqxmd.com/read/26013475/long-term-renal-effects-of-tenofovir-disoproxil-fumarate-in-vertically-hiv-infected-children-adolescents-and-young-adults-a-132-month-follow-up-study
#13
Vania Giacomet, Pilar Nannini, Alessandra Vigano, Paola Erba, Annarita Benincaso, Giorgio Bedogni, Dario Cattaneo, Felicia Stefania Falvella, Gian Vincenzo Zuccotti
BACKGROUND AND OBJECTIVES: The introduction of highly active anti-retroviral therapy has led to a significant decline in morbidity and mortality. Although several studies in adult populations have shown that tenofovir-disoproxil-fumarate (TDF) use is associated with a significant loss of renal function, there is still uncertainty on the long-term TDF safety profile in pediatric HIV populations, mostly in vertically HIV-infected patients. The aim of this study was to evaluate the long-term TDF renal safety profile, during a ten-year follow up...
July 2015: Clinical Drug Investigation
https://www.readbyqxmd.com/read/25710805/mineral-metabolism-in-european-children-living-with-a-renal-transplant-a-european-society-for-paediatric-nephrology-european-renal-association-european-dialysis-and-transplant-association-registry-study
#14
Marjolein Bonthuis, Marco Busutti, Karlijn J van Stralen, Kitty J Jager, Sergey Baiko, Sevcan Bakkaloğlu, Nina Battelino, Maria Gaydarova, Bruno Gianoglio, Paloma Parvex, Clara Gomes, James G Heaf, Ludmila Podracka, Dafina Kuzmanovska, Maria S Molchanova, Tatiana E Pankratenko, Fotios Papachristou, György Reusz, Maria José Sanahuja, Rukshana Shroff, Jaap W Groothoff, Franz Schaefer, Enrico Verrina
BACKGROUND AND OBJECTIVES: Data on mineral metabolism in pediatric renal transplant recipients largely arise from small single-center studies. In adult patients, abnormal mineral levels are related to a higher risk of graft failure. This study used data from the European Society for Paediatric Nephrology/European Renal Association-European Dialysis and Transplant Association Registry to study the prevalence and potential determinants of mineral abnormalities, as well as the predictive value of a disturbed mineral level on graft survival in a large cohort of European pediatric renal transplant recipients...
May 7, 2015: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/25590845/cinacalcet-in-pediatric-and-adolescent-chronic-kidney-disease-a-single-center-experience
#15
Abdulla A Alharthi, Naglaa M Kamal, Mohamed W Abukhatwah, Laila M Sherief
Cinacalcet, a calcimimetic drug, has been shown to be efficacious in adult chronic kidney disease (CKD) patients; however, it was not fully studied in pediatric CKD patients. We aimed at assessing the effect of cinacalcet on intact parathyroid hormone (iPTH) secretion in children with CKD-4/5 with iPTH consistently ≥ 300 pg/mL refractory to conventional treatment. This is a prospective cohort analysis of 28 children with uncontrolled hyper-parathyroidism secondary to stage 4 and 5 CKD admitted to a tertiary center during the period from April 2012 to April 2014...
January 2015: Medicine (Baltimore)
https://www.readbyqxmd.com/read/25257509/phase-i-dose-escalation-and-pharmacokinetic-study-of-oral-gefitinib-and-irinotecan-in-children-with-refractory-solid-tumors
#16
R C Brennan, W Furman, S Mao, J Wu, D C Turner, C F Stewart, V Santana, L M McGregor
PURPOSE: This phase I study endeavored to estimate the maximum tolerated dose and describe the dose-limiting toxicities (DLTs) of oral irinotecan with gefitinib in children with refractory solid tumors. METHODS: Oral irinotecan was administered on days 1-5 and 8-12 with oral gefitinib (fixed dose, 150 mg/m(2)/day) on days 1-12 of a 21-day course. The escalation with overdose control method guided irinotecan dose escalation (7 dose levels, range 5-40 mg/m(2)/day)...
December 2014: Cancer Chemotherapy and Pharmacology
https://www.readbyqxmd.com/read/25157005/restrictive-eating-disorders-among-adolescent-inpatients
#17
Melissa Whitelaw, Heather Gilbertson, Katherine J Lee, Susan M Sawyer
BACKGROUND AND OBJECTIVES: Clinicians are increasingly observing adolescents who have lost large amounts of weight, experience typical cognitions and acute medical complications of anorexia nervosa (AN), yet do not meet diagnostic criteria for AN owing to weight. We refer to this category of Eating Disorder Not Otherwise Specified as EDNOS-Wt. We set out to describe the changing incidence of EDNOS-Wt compared with AN, and to compare the characteristics of these 2 groups in a cohort that required hospitalization after weight loss...
September 2014: Pediatrics
https://www.readbyqxmd.com/read/24605319/optimization-of-bone-health-in-children-before-and-after-renal-transplantation-current-perspectives-and-future-directions
#18
REVIEW
Kristen Sgambat, Asha Moudgil
The accrual of healthy bone during the critical period of childhood and adolescence sets the stage for lifelong skeletal health. However, in children with chronic kidney disease (CKD), disturbances in mineral metabolism and endocrine homeostasis begin early on, leading to alterations in bone turnover, mineralization, and volume, and impairing growth. Risk factors for CKD-mineral and bone disorder (CKD-MBD) include nutritional vitamin D deficiency, secondary hyperparathyroidism, increased fibroblast growth factor 23 (FGF-23), altered growth hormone and insulin-like growth factor-1 axis, delayed puberty, malnutrition, and metabolic acidosis...
2014: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/23966755/treatment-of-hypophosphatemic-rickets-with-phosphate-and-active-vitamin-d-in-japan-a-questionnaire-based-survey
#19
Makoto Fujiwara, Noriyuki Namba, Keiichi Ozono, Osamu Arisaka, Susumu Yokoya
Hereditary hypophosphatemic rickets represented by X-linked hypophosphatemic rickets (XLH) is a rare disorder characterized by hypophosphatemia, elevated alkaline phosphatase (ALP) and undermineralization of bone. Active vitamin D and phosphate are administered to correct hypophosphatemia and elevation of ALP. Overtreatment with phosphate leads to secondary hyperparathyroidism, and a large dose of active vitamin D has a risk of hypercalciuria. To understand the situation concerning treatment of patients with hereditary hypophosphatemic rickets in Japan, we conducted a questionnaire survey of pediatric endocrinologists...
January 2013: Clinical Pediatric Endocrinology: Case Reports and Clinical Investigations: Official Journal of the Japanese Society for Pediatric Endocrinology
https://www.readbyqxmd.com/read/23636577/hypophosphatemic-rickets-due-to-perturbations-in-renal-tubular-function
#20
REVIEW
Maria Goretti M G Penido, Uri S Alon
The common denominator for all types of rickets is hypophosphatemia, leading to inadequate supply of the mineral to the growing bone. Hypophosphatemia can result from insufficient uptake of the mineral from the gut or its disproportionate losses in the kidney, the latter being caused by either tubular abnormalities per se or the effect on the tubule of circulating factors like fibroblast growth factor-23 and parathyroid hormone (PTH). High serum levels of the latter result in most cases from abnormalities in vitamin D metabolism which lead to decreased calcium absorption in the gut and hypocalcemia, triggering PTH secretion...
March 2014: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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