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Hypophosphatemia

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https://www.readbyqxmd.com/read/29905927/multicenter-randomized-double-blind-phase-2-trial-of-folfiri-with-regorafenib-or-placebo-as-second-line-therapy-for-metastatic-colorectal-cancer
#1
Hanna K Sanoff, Richard M Goldberg, Anastasia Ivanova, Seamus O'Reilly, Samer S Kasbari, Richard D Kim, Ray McDermott, Dominic T Moore, William Zamboni, William Grogan, Allen Lee Cohn, Tanios S Bekaii-Saab, Gregory Leonard, Theresa Ryan, Olugbenga O Olowokure, Nishan H Fernando, John McCaffrey, Bassel F El-Rayes, Anne M Horgan, Gary Bradley Sherrill, George Hosni Yacoub, Bert H O'Neil
BACKGROUND: Regorafenib, a multikinase inhibitor that inhibits angiogenesis, growth, and proliferation, prolongs survival as monotherapy in patients with refractory colorectal cancer. This international, double-blind, placebo-controlled, multicenter trial assessed the efficacy of regorafenib with folinic acid, fluorouracil, and irinotecan (FOLFIRI) as a second-line treatment for metastatic colorectal cancer. METHODS: Patients with metastatic colorectal cancer who progressed on first-line oxaliplatin and fluoropyrimidine enrolled at 45 sites in the United States and Ireland...
June 15, 2018: Cancer
https://www.readbyqxmd.com/read/29904418/acquired-hypophosphatemic-osteomalacia-is-easily-misdiagnosed-or-neglected-by-rheumatologists-a-report-of-9-cases
#2
Ling Li, Shu-Xia Wang, Hong-Mei Wu, Dong-Lan Luo, Guang-Fu Dong, Yuan Feng, Xiao Zhang
The aim of the present study was to assist rheumatologists in differentiating hypophosphatemic osteomalacia (HO) from mimic rheumatology diseases. Clinical data was obtained from 9 patients with acquired HO, initially misdiagnosed as mimic rheumatologic diseases. The data were retrospectively analyzed and a literature review was performed. The etiology of the cases was as follows: Adefovir dipivoxil-induced Fanconi syndrome was present in 6 of the cases, 2 were tumors and 1 case was chronic nephropathy. The chief complaint was thoracic or back pain and arthralgia, followed by progressive muscle weakness and dramatic movement limitation...
June 2018: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29901462/changing-paradigms-in-metabolic-support-and-nutrition-therapy-during-critical-illness
#3
Arthur R H van Zanten
PURPOSE OF REVIEW: To summarize the most recent advances in acute metabolic care and critical care nutrition. RECENT FINDINGS: Recent research has demonstrated unknown consequences of high protein and amino acid administration in the early phase of ICU stay associated with dysregulated glucagon release leading to hepatic amino acid breakdown and suggested adverse effects on autophagy and long-term outcome. Progress has been made to measure body composition in the ICU...
June 8, 2018: Current Opinion in Critical Care
https://www.readbyqxmd.com/read/29901461/refeeding-syndrome-relevance-for-the-critically-ill-patient
#4
Rianne Boot, Kristine W A C Koekkoek, Arthur R H van Zanten
PURPOSE OF REVIEW: To provide an overview of recent findings concerning refeeding syndrome (RFS) among critically ill patients and recommendations for daily practice. RECENT FINDINGS: Recent literature shows that RFS is common among critically ill ventilated patients. Usual risk factors for non-ICU patients addressed on ICU admission do not identify patients developing RFS. A marked drop of phosphate levels (>0.16 mmol/l) from normal levels within 72 h of commencement of feeding, selects patients that benefit from hypocaloric or restricted caloric intake for at least 48 h resulting in lower long-term mortality...
June 8, 2018: Current Opinion in Critical Care
https://www.readbyqxmd.com/read/29886586/-clinical-and-immunohistopathologic-study-of-phosphaturic-mesenchymal-tumor
#5
D M Li, H W Wu, J D Li, W B Xia, Y Jiang, D R Zhong
Objective: To study the clinicopathological characteristics and immunohistochemical phenotype of phosphaturic mesenchymal tumor (PMT) . Methods: The clinicopathological data and immunohistochemical profiles were obtained retrospectively from 206 patients diagnosed with PMT at Peking Union Medical College Hospital (PUMCH) during July 2008 to September 2017, with a review of literature. Results: The mean age of PMT patients was 42 years (range 13 to 70 years), with a male to female ratio of 1.1∶1.0. All patients presented with different degree of bone pain, muscle weakness, shorten of stature, thoracic deformity and pathological fractures, with hypophosphatemia and high serum ALP...
June 8, 2018: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/29878089/eldecalcitol-causes-fgf23-resistance-for-pi-reabsorption-and-improves-rachitic-bone-phenotypes-in-the-male-hyp-mouse
#6
Ichiro Kaneko, Hiroko Segawa, Kayo Ikuta, Ai Hanasaki, Toru Fujii, Sawako Tatsumi, Shinsuke Kido, Tomoka Hasegawa, Norio Amizuka, Hitoshi Saito, Ken-Ichi Miyamoto
X-linked hypophosphatemia (XLH), the most common form of inheritable rickets, is caused by inactivation of PHEX (phosphate-regulating gene with homologies to endopeptidases on the X chromosome), and leads to fibroblast growth factor 23 (FGF23)-dependent renal phosphate (Pi) wasting. In the present study, we investigated whether maintaining Pi homeostasis with a novel potent vitamin D3 analog, eldecalcitol (1α,25-dihydroxy-2β-(3-hydroxypropyloxy)vitamin D3; ED71), could improve hypophosphatemic rickets in a murine model of XLH, the Hyp mouse...
June 5, 2018: Endocrinology
https://www.readbyqxmd.com/read/29877286/legionella-pneumonia-complicated-with-acquired-fanconi-syndrome-a-case-report
#7
Ryo Koda, Ryo Itoh, Masafumi Tsuchida, Kazumasa Ohashi, Noriaki Iino, Toshinori Takada, Ichiei Narita
Legionella pneumonia is occasionally accompanied by renal complications; however, the cause of this remains unknown. We herein report a 70-year-old Japanese man with Legionella pneumonia who presented with hyponatremia, hypophosphatemia, and hypouricemia. The levels of urinary β2-microglobulin and N-acetyl-β-D-glucosaminidase were remarkably high, indicating severe renal tubular damage. The presence of glycosuria and aminoaciduria as well as increased fractional excretion of uric acid and decreased tubular reabsorption of phosphate indicated that the patient's condition was complicated with Fanconi syndrome...
June 6, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29858904/genetic-analysis-of-three-families-with-x-linked-dominant-hypophosphatemic-rickets
#8
Xinfu Lin, Yaobin Zhu, Jiewei Luo, Jianbin Huang
BACKGROUND: Hypophosphatemic rickets, including familial hypophosphatemic vitamin D-resistant rickets, which commonly manifests in childhood, is generally hereditary. X-linked dominant hypophosphatemic rickets (XLH, MIM307800), caused by inactivating mutations in the PHEX gene, is the most common form. This study aimed to identify the gene mutations responsible for three cases of XLH and its clinical phenotype. METHODS: We conducted a genetic diagnosis and clinical phenotypic linkage analysis of three pedigrees with XLH...
June 2, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29850383/what-a-trainee-surgeon-should-know-about-refeeding-syndrome-a-literature-review
#9
REVIEW
Muneeba Nasir, Balakh S Zaman, Ahmad Kaleem
Refeeding syndrome (RFS) is potentially fatal, yet there is limited understanding regarding its management among general surgeons due in part to a lack of universally accepted guidelines for RFS diagnosis. The aim of this review is to equip general surgery trainees with the essentials of RFS including a review of the National Institute for Health and Care Excellence (NICE) best practice guidelines for RFS. We used the keywords "refeeding", "syndrome", and "hypophosphatemia" to search PubMed, Embase, and Medline databases...
March 28, 2018: Curēus
https://www.readbyqxmd.com/read/29809158/slc34a3-intronic-deletion-in-an-iranian-kindred-with-hereditary-hypophosphatemic-rickets-with-hypercalciuria-and-review-of-reported-cases
#10
Shirin Hasani-Ranjbar, Hanieh Sadat Ejtahed, Mahsa M Amoli, Fatemeh Bitarafan, Mostafa Qorbani, Akbar Soltani, Bahareh Yarjoo
OBJECTIVE: Hereditary Hypophosphatemic Rickets with Hypercalciuria (HHRH) is a very rare inheritable hypophosphatemic rickets/osteomalacia characterized by decreased renal phosphate reabsorption, hypophosphatemia, vitamin D refractory rickets, hyperphosphaturia, hypercalciuria, elevated circulating 1, 25-dihydroxy vitamin D levels and low serum parathyroid hormone (PTH) levels, leading to growth retardation, limb deformities, bone pain, muscle weakness, rickets and osteomalacia. Biallelic mutations in SLC34A3/NPT2c gene are responsible for the occurrence of the disease...
May 29, 2018: Journal of Clinical Research in Pediatric Endocrinology
https://www.readbyqxmd.com/read/29791829/burosumab-therapy-in-children-with-x-linked-hypophosphatemia
#11
RANDOMIZED CONTROLLED TRIAL
Thomas O Carpenter, Michael P Whyte, Erik A Imel, Annemieke M Boot, Wolfgang Högler, Agnès Linglart, Raja Padidela, William Van't Hoff, Meng Mao, Chao-Yin Chen, Alison Skrinar, Emil Kakkis, Javier San Martin, Anthony A Portale
BACKGROUND: X-linked hypophosphatemia is characterized by increased secretion of fibroblast growth factor 23 (FGF-23), which leads to hypophosphatemia and consequently rickets, osteomalacia, and skeletal deformities. We investigated burosumab, a monoclonal antibody that targets FGF-23, in patients with X-linked hypophosphatemia. METHODS: In an open-label, phase 2 trial, we randomly assigned 52 children with X-linked hypophosphatemia, in a 1:1 ratio, to receive subcutaneous burosumab either every 2 weeks or every 4 weeks; the dose was adjusted to achieve a serum phosphorus level at the low end of the normal range...
May 24, 2018: New England Journal of Medicine
https://www.readbyqxmd.com/read/29790700/early-hypophosphatemia-in-very-low-birth-weight-preterm-infants
#12
REVIEW
Agata Pająk, Barbara Królak-Olejnik, Agnieszka Szafrańska
Refeeding Syndrome (RFS) is a well-known group of symptoms which occur after the introduction of enteral or parenteral nutrition in undernourished patients. Intrauterine growth restriction (IUGR) is the equivalent of postnatal RFS following the beginning of feeding. The aggressive parenteral nutrition of neonates with very low birth weight (VLBW) resulting from the termination of intrauterine transplacental nutrition is a source of biochemical disorders. The aim of this study was to analyze metabolic disorders in preterm infants during the 1st week of life and to determine the hypophosphatemia risk factors in low birth weight neonates receiving parenteral nutrition...
May 21, 2018: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
https://www.readbyqxmd.com/read/29785270/distant-lung-metastases-caused-by-a-histologically-benign-phosphaturic-mesenchymal-tumor
#13
Maria P Yavropoulou, Christos Poulios, Christoforos Foroulis, Symeon Tournis, Prodromos Hytiroglou, Kalliopi Kotsa, Isaak Kessisoglou, Pantelis Zebekakis
Tumor-induced osteomalacia (TIO) is a rare form of hypophosphatemia usually caused by phosphaturic mesenchymal tumors (PMTs); the biologic behavior of PMTs is under investigation. Herein we present a case of TIO with a protracted course over 12 years leading to a fatal outcome. A 39-year-old man presented with weakness in 2004 and was found to have decreased serum phosphorus, phosphaturia and low levels of 1,25-dihydroxyvitamin D3. Four years later he developed a painful left calf mass. The lesion was resected, but recurred causing extreme pain and dysfunction...
2018: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/29775451/-recurrent-urolithiasis-as-a-symptom-of-primary-hyperparathyroidism-in-a-16-year-old-boy
#14
Monika Wojciechowska, Beata Bieniaś, Aleksandra Sobieszczańska-Droździel, Anna Wieczorkiewicz-Płaza, Iwona Beń-Skowronek, Przemysław Sikora
Primary hyperparathyroidism is one of the most common endocrine diseases, however, it is rare in children. In most cases, it is caused by adenoma of these organs. Its most common complications include urolithiasis, nephrocalcinosis and osteoporosis. CASE REPORT: A 16-year-old patient was admitted to our Clinic because of his first-ever renal colic. The ultrasound examination revealed rightsided hydronephrosis caused by the presence of 9 mm stone in the upper part of the right ureter. In addition, the presence of 8 mm stone in the middle calyx of the left kidney was found...
April 23, 2018: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29769119/adefovir-dipivoxil-induced-hypophosphatemic-osteomalacia-in-chronic-hepatitis-b-a-comparative-study-of-chinese-and-foreign-case-series
#15
Nan Chen, Jian-Bo Zhang, Qiujie Zhang, Yun-Peng Zhao, Li-Yan Li, Li-Wei Liu, Fei Yu, Xin Yu, Tao Peng, Kuan-Xiao Tang
BACKGROUND: Adefovir dipivoxil (ADV)-induced renal tubular dysfunction and hypophosphatemic osteomalacia (HO) have been given great consideration in the past few years. However, no standard guidance is available due to a lack of powerful evidence from appropriate long-term prospective case-control studies and variations in the definition of renal adverse events. The aim of this study is to clarify clinical features of ADV-related HO in Chinese chronic hepatitis B patients with long-term ADV treatment in Chinese and non-Chinese comparative case series...
May 16, 2018: BMC Pharmacology & Toxicology
https://www.readbyqxmd.com/read/29756708/hypercalcemia-and-hypophosphatemia-among-preterm-infants-receiving-aggressive-parenteral-nutrition
#16
Pablo H Brener Dik, María F Galletti, Leticia T Bacigalupo, Silvia Fernández Jonusas, Gonzalo L Mariani
INTRODUCTION: Aggressive parenteral nutrition is the standard of care among very-low-birth weight preterm infants. However, in recent studies, its impact on short-term outcomes, has been evaluated. The objective was to compare the prevalence of hypercalcemia and hypophosphatemia among preterm infants receiving aggressive or standard parenteral nutrition. METHODS: Observational, retrospective study comparing a group of preterm infants weighing less than 1250 grams who received aggressive parenteral nutrition with a historical control group...
June 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29746006/hypophosphatemia-in-a-malnourished-child-when-renal-fanconi-syndrome-does-not-stand-for-refeeding-syndrome
#17
Joseph Runde, Edgardo Rivera-Rivera, Cecelia Pompeii-Wolfe, Christopher Clardy, Timothy Sentongo
Refeeding syndrome is diagnosed based on the onset of multiple laboratory abnormalities (most commonly hypophosphatemia) and clinical signs in the setting of nutrition rehabilitation of malnourished patients. Because definitions are not uniform, a broad differential diagnosis should always include renal tubular dysfunction. Our report details a 3 year-old child with undiagnosed renal tubular dysfunction who presented with the clinical picture of refeeding syndrome with refractory electrolyte abnormalities...
May 10, 2018: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/29745817/impaired-mineral-quality-in-dentin-in-x-linked-hypophosphatemia
#18
Benjamin R Coyac, Guillaume Falgayrac, Guillaume Penel, Alain Schmitt, Thorsten Schinke, Agnès Linglart, Marc D McKee, Catherine Chaussain, Claire Bardet
X-linked hypophosphatemia (XLH) is a skeletal disorder arising from mutations in the PHEX gene, transmitted in most cases as an X-linked dominant trait. PHEX deficiency leads to renal phosphate wasting and hypophosphatemia, as well as impaired mineralization of bone and dentin, resulting in severe skeletal and dental complications. Dentin mineralization defects appear as characteristic, large interglobular spaces resulting from the lack of fusion of calculospherites in the circumpulpal region during the mineralization process...
December 2018: Connective Tissue Research
https://www.readbyqxmd.com/read/29736882/a-challenging-case-of-tumor-induced-osteomalacia-pathophysiological-and-clinical-implications
#19
Luciano Colangelo, Cristiana Cipriani, Jessica Pepe, Alessandro Corsi, Chiara Sonato, Giulia Follacchio, Mirella Cilli, Walter Gianni, Federica Ferrone, Oreste Moreschini, Lorraine A Fitzpatrick, Salvatore Minisola
We investigated the usefulness of fibroblast growth factor 23 (FGF23) intraoperative assay to monitor tumor resection in patients with oncogenic osteomalacia. A 33-year-old man with 5 years' history of lumbar and pelvis pain together with multiple vertebral fractures was admitted to our hospital. He was diagnosed with ankylosing spondylitis 1 year before. Laboratory investigation showed low tubular reabsorption of phosphate (0.41 mmol/L) despite chronic hypophosphatemia (0.39/L). Increased plasma values of FGF23 (673 pg/mL; n...
May 7, 2018: Calcified Tissue International
https://www.readbyqxmd.com/read/29736771/increased-prevalence-of-asymptomatic-vertebral-fractures-in-hiv-infected-patients-over-50-years-of-age
#20
Maria Llop, W A Sifuentes, S Bañón, C Macia-Villa, M J Perez-Elías, M Rosillo, S Moreno, M Vázquez, J L Casado
The prevalence of asymptomatic vertebral fracture in HIV-infected patients over 50 was 20%, associated with older age, male sex, longer time since HIV diagnosis, and tubular renal alterations. Vertebral fractures were independent of osteoporosis at lumbar spine, and were not predicted by the use of the FRAX equation. PURPOSE: Vertebral fractures (VF) are the hallmark of osteoporotic fractures. Our objective was to determine the prevalence of asymptomatic VF and associated factors in HIV-infected patients over 50 years, and the role of FRAX equation...
May 8, 2018: Archives of Osteoporosis
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