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Hypophosphatemia

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https://www.readbyqxmd.com/read/28620595/the-predicting-ability-of-serum-phosphorus-to-assess-the-duration-of-mechanical-ventilation-in-critically-ill-patients
#1
Reihanak Talakoub, Mahshid Bahrami, Azim Honarmand, Saeed Abbasi, Hamideh Gerami
BACKGROUND: No previous study exists to evaluate serum phosphorus (Ph) level as a predictor of the need to mechanical ventilation (MV). This study was designed to determine the predictive ability of admission serum Ph level on MV in patients admitted in Intensive Care Unit (ICU). MATERIALS AND METHODS: This prospective study was conducted on 100 patients (>16 years old), admitted to our ICU over 1-year. Patients were classified into two groups according to the days of the need to MV...
2017: Advanced Biomedical Research
https://www.readbyqxmd.com/read/28620244/specific-ablation-of-mouse-fam20c-in-cells-expressing-type-i-collagen-leads-to-skeletal-defects-and-hypophosphatemia
#2
Peihong Liu, Su Ma, Hua Zhang, Chao Liu, Yongbo Lu, Li Chen, Chunlin Qin
FAM20C mutations in humans cause Raine syndrome and our previous studies showed that global inactivation of mouse Fam20C led to bone and dental defects. By crossbreeding 2.3  kb Col 1a1-Cre mice with Fam20C (flox/flox) mice, we created 2.3  kb Col 1a1-Cre;Fam20C (foxl/flox) (cKO) mice, in which Fam20C was inactivated in cells expressing Type I collagen. This study showed that the long bones of cKO mice were shorter and had a lower level of mineralization compared to the normal mice. The collagen fibrils in Fam20C-deficient bone were disorganized and thicker while the growth plate cartilage in cKO mice was disorganized and wider compared to the normal mice...
June 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28616212/hypophosphatemia-in-critically-ill-patients-with-acute-kidney-injury-treated-with-hemodialysis-is-associated-with-adverse-events
#3
Cynthia Lim, Han Khim Tan, Manish Kaushik
Background. Hypophosphatemia in critically ill patients may be exacerbated by renal replacement therapy (RRT). We aimed to identify risk factors and adverse outcomes associated with hypophosphatemia in intensive care patients treated with RRT for acute kidney injury (AKI). Methods. This was a secondary analysis of data from a single-center prospective cohort study of medical and surgical intensive care patients with RRT for AKI between 18 December 2010 and 3 April 2013. Demographic, comorbidity, laboratory and RRT data were retrieved from patient case notes and electronic medical records...
June 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28608052/craniofacial-and-dental-characteristics-of-patients-with-vitamin-d-dependent-rickets-type-1a-compared-to-controls-and-patients-with-x-linked-hypophosphatemia
#4
Hans Gjørup, Signe Sparre Beck-Nielsen, Dorte Haubek
ᅟOBJECTIVES: Vitamin-D-dependent rickets type 1A (VDDR1A) is a rare inherited disease caused by defective activation of vitamin D. The aim of the study was to describe the craniofacial characteristics and the dental phenotype of patients with genetically confirmed VDDR1A. The VDDR1A findings were compared to findings in patients with X-linked hypophosphatemia (XLH) and healthy controls. MATERIAL AND METHODS: Ten patients with VDDR1A were identified. The reference group for the comparison of cephalometric findings was 49 adults without chronic disease...
June 12, 2017: Clinical Oral Investigations
https://www.readbyqxmd.com/read/28603080/a-novel-auditory-ossicles-membrane-and-the-development-of-conductive-hearing-loss-in-dmp1-null-mice
#5
Kun Lv, Haiyang Huang, Xing Yi, Mark E Chertoff, Chaoyuan Li, Baozhi Yuan, Robert J Hinton, Jian Q Feng
Genetic mouse models are widely used for understanding human diseases but we know much less about the anatomical structure of the auditory ossicles in the mouse than we do about human ossicles. Furthermore, current studies have mainly focused on disease conditions such as osteomalacia and rickets in patients with hypophosphatemia rickets, although the reason that these patients develop late-onset hearing loss is unknown. In this study, we first analyzed Dmp1 lac Z knock-in auditory ossicles (in which the blue reporter is used to trace DMP1 expression in osteocytes) using X-gal staining and discovered a novel bony membrane surrounding the mouse malleus...
June 8, 2017: Bone
https://www.readbyqxmd.com/read/28600887/therapeutic-effects-of-fgf23-c-tail-fc-in-a-murine-pre-clinical-model-of-x-linked-hypophosphatemia-via-the-selective-modulation-of-phosphate-reabsorption
#6
Kristen Johnson, Kymberly Levine, Joseph Sergi, Jean Chamoun, Rachel Roach, Jackie Vekich, Mike Favis, Mark Horn, Xianjun Cao, Brian Miller, William Snyder, Dikran Aivazian, William Reagan, Edwin Berryman, Jennifer Colangelo, Victoria Markiewicz, Cedo Bagi, Thomas P Brown, Anthony Coyle, Moosa Mohammadi, Jeanne Magram
Fibroblast growth factor 23 (FGF23) is the causative factor of X-linked hypophosphatemia (XLH), a genetic disorder effecting 1:20,000 that is characterized by excessive phosphate excretion, elevated FGF23 levels and a rickets/osteomalacia phenotype. FGF23 inhibits phosphate reabsorption and suppresses 1α,25-dihydroxyvitamin D (1,25D) biosynthesis, analytes that differentially contribute to bone integrity and deleterious soft tissue mineralization. As inhibition of ligand broadly modulates downstream targets, balancing efficacy and unwanted toxicity is difficult when targeting the FGF23 pathway...
June 10, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28592620/phase-ii-study-of-first-line-trebananib-plus-sorafenib-in-patients-with-advanced-hepatocellular-carcinoma
#7
Ghassan K Abou-Alfa, Jean-Frederic Blanc, Steven Miles, Tom Ganten, Jörg Trojan, Jonathan Cebon, Andre K Liem, Lara Lipton, Charu Gupta, Benjamin Wu, Michael Bass, Ellen Hollywood, Jennifer Ma, Margaret Bradley, Jason Litten, Leonard B Saltz
LESSONS LEARNED: Trebananib leveraging anti-angiogenic mechanism that is distinct from the classic sorafenib anti-vascular endothelial growth factor inhibition did not demonstrate improved progression-free survival at 4 months in patients with advanced hepatocellular carcinoma (HCC).In support of previously reported high Ang-2 levels' association with poor outcome in HCC for patients, trebananib treatment with lower baseline Ang-2 at study entry was associated with improved overall survival to 22 months and may suggest future studies to be performed within the context of low baseline Ang-2...
June 7, 2017: Oncologist
https://www.readbyqxmd.com/read/28586591/celiac-crisis-a-rare-or-rarely-recognized-disease
#8
Nadia Waheed, Huma Arshad Cheema, Hassan Suleman, Zafar Fayyaz, Iqra Mushtaq, Muhammad, Almas Hashmi
OBJECTIVE: Celiac crisis is a serious life threatening complication of celiac disease characterized by profuse diarrhoea, severe dehydration and metabolic disturbances leading to neuromuscular weakness, cardiac arrhythmias and sudden death. It has been described as rare condition and not well documented in the literature. To improve awareness and facilitate diagnosis of this condition, we studied risk factors, pattern of presentation and management plans of celiac crisis. METHODS: It was a descriptive cross sectional study...
October 2016: Journal of Ayub Medical College, Abbottabad: JAMC
https://www.readbyqxmd.com/read/28578095/pseudohypophosphatemia-associated-with-high-dose-liposomal-amphotericin-b-therapy
#9
James A Mays, Dina N Greene, Anne Poon, Anna E Merrill
BACKGROUND: Hypophosphatemia is commonly observed in critically ill patients. Inorganic phosphorus is quantified by spectrophotometric measurement of a phosphomolybdate complex, a method with multiple documented interferents. Our clinical laboratory was contacted to investigate a case of asymptomatic hypophosphatemia in a patient receiving high-dose liposomal amphotericin B therapy (L-AMB). METHODS: In vitro experiments were performed by spiking L-AMB into residual plasma specimens...
May 31, 2017: Clinical Biochemistry
https://www.readbyqxmd.com/read/28573057/cisplatin-induced-renal-salt-wasting-requiring-over-12-liters-of-3-saline-replacement
#10
Phuong-Chi Pham, Pavani Reddy, Shaker Qaqish, Ashvin Kamath, Johana Rodriguez, David Bolos, Martina Zalom, Phuong-Thu Pham
Cisplatin is known to induce Fanconi syndrome and renal salt wasting (RSW). RSW typically only requires transient normal saline (NS) support. We report a severe RSW case that required 12 liters of 3% saline. A 57-year-old woman with limited stage small cell cancer was admitted for cisplatin (80 mg/m(2)) and etoposide (100 mg/m(2)) therapy. Patient's serum sodium (SNa) decreased from 138 to 133 and 125 mEq/L within 24 and 48 hours of cisplatin therapy, respectively. A diagnosis of syndrome of inappropriate antidiuretic hormone secretion (SIADH) was initially made...
2017: Case Reports in Nephrology
https://www.readbyqxmd.com/read/28571152/oncogenic-osteomalacia-an-approach-to-diagnosis-with-a-case-report
#11
Biswajit Dey, Debasis Gochhait, Hema Subramanian, Madhusudhanan Ponnusamy
Oncogenic osteomalacia, also known as tumour induced osteomalacia, is a rare paraneoplastic syndrome caused by mesenchymal tumours secreting Fibroblast Growth Factor-23 (FGF-23). The characteristic biochemical findings include hypophosphatemia and low 1,25-dihydroxy vitamin D. The differential diagnosis for hypophosphatemia are varied. We present a case of oncogenic osteomalacia in a 29-year-old female, who presented with complaints of generalized diffuse bone pain and walking difficulty for six months duration...
April 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28546442/post-transplant-hypophosphatemia-and-the-risk-of-death-censored-graft-failure-and-mortality-after-kidney-transplantation
#12
Marco van Londen, Brigitte M Aarts, Petronella E Deetman, Jessica van der Weijden, Michele F Eisenga, Gerjan Navis, Stephan J L Bakker, Martin H de Borst
BACKGROUND AND OBJECTIVES: Hypophosphatemia is common in the first year after kidney transplantation, but its clinical implications are unclear. We investigated the relationship between the severity of post-transplant hypophosphatemia and mortality or death-censored graft failure in a large cohort of renal transplant recipients with long-term follow-up. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We performed a longitudinal cohort study in 957 renal transplant recipients who were transplanted between 1993 and 2008 at a single center...
May 25, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/28537512/do-patients-with-a-baseline-clinical-condition-warranting-the-cautious-use-of-parenteral-nutrition-develop-subsequent-metabolic-complications
#13
Diana M Solomon, James M Hollands, Laura A Siemianowski, Colleen E Smith, Richard J Song, Angela L Bingham
BACKGROUND: The American Society for Parenteral and Enteral Nutrition Adult Nutrition Support Core Curriculum describes clinical conditions that warrant cautious use of parenteral nutrition (CCWCPN). The Core Curriculum authors acknowledge there is no evidence for specific criteria suggested for the clinical conditions. Consequently, the purpose of this study was to determine the impact of a baseline CCWCPN on the development of subsequent metabolic complications in patients receiving parenteral nutrition (PN)...
June 2017: Nutrition in Clinical Practice
https://www.readbyqxmd.com/read/28526969/glycaemia-and-phosphatemia-after-oral-glucose-and-maltitol-ingestion-in-subjects-from-two-different-race-groups-preliminary-evidence-of-inter-race-differences-in-metabolism-and-possible-implications-for-urinary-stone-disease
#14
Takalani Theka, Allen Rodgers
PURPOSE: Glucose (Glu) and maltitol (Mal) ingestion affect calciuria and phosphaturia. Renal phosphate leak involving hypophosphatemia is thought to be a mechanism. Inter-race differences in carbohydrate metabolism are known. We investigated the effects of Glu and Mal ingestion on glycaemia and phosphatemia in subjects from two race groups to better understand potential implications for nephrolithiasis. METHODS: Healthy black (B) (n = 8) and white (W) (n = 8) males followed a self-selected standardized diet for 7 days and a strictly controlled standardized diet on Day 8...
May 19, 2017: International Urology and Nephrology
https://www.readbyqxmd.com/read/28525508/analysis-of-hypo-and-hyperphosphatemia-in-an-intensive-care-unit-cohort
#15
Marcus Broman, Amanda M J Wilsson, Fredrik Hansson, Bengt Klarin
BACKGROUND: Blood phosphate levels are vulnerable to fluctuations and changes in phosphate levels are often neglected. The aim of this study was to evaluate whether deviations in phosphate levels correlate to higher 180-day overall mortality or morbidity. METHODS: Four thousand six hundred fifty-six patients with 19,467 phosphate values treated at the adult intensive care unit at Skåne University Hospital, Lund, Sweden during 2006-2014 were retrospectively divided into a control group and 3 study groups: hypophosphatemia, hyperphosphatemia, and a mixed group showing both hypo/hyperphosphatemia...
June 2017: Anesthesia and Analgesia
https://www.readbyqxmd.com/read/28514613/kit-inhibition-by-imatinib-in-patients-with-severe-refractory-asthma
#16
RANDOMIZED CONTROLLED TRIAL
Katherine N Cahill, Howard R Katz, Jing Cui, Juying Lai, Shamsah Kazani, Allison Crosby-Thompson, Denise Garofalo, Mario Castro, Nizar Jarjour, Emily DiMango, Serpil Erzurum, Jennifer L Trevor, Kartik Shenoy, Vernon M Chinchilli, Michael E Wechsler, Tanya M Laidlaw, Joshua A Boyce, Elliot Israel
BACKGROUND: Mast cells are present in the airways of patients who have severe asthma despite glucocorticoid treatment; these cells are associated with disease characteristics including poor quality of life and inadequate asthma control. Stem cell factor and its receptor, KIT, are central to mast-cell homeostasis. We conducted a proof-of-principle trial to evaluate the effect of imatinib, a KIT inhibitor, on airway hyperresponsiveness, a physiological marker of severe asthma, as well as on airway mast-cell numbers and activation in patients with severe asthma...
May 18, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28509131/growth-hormone-therapy-for-a-patient-with-idiopathic-fanconi-syndrome-and-growth-hormone-deficiency
#17
Takayuki Okamoto, Yasuyuki Sato, Takeshi Yamazaki, Asako Hayashi, Toshiyuki Takahashi
Idiopathic Fanconi syndrome (FS) is characterized by a generalized dysfunction of the renal proximal tubules. Patients with FS often exhibit growth retardation due to complex factors, such as hypophosphatemia, metabolic acidosis, disturbed vitamin D metabolism and hypokalemia. To date, one FS patient has been reported to exhibit growth failure due to growth hormone deficiency (GHD), but the long-term clinical course of recombinant human GH (rhGH) therapy has not been reported. At 10 months of age, the patient was admitted to our hospital due to growth failure...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28509123/serum-calcitriol-levels-in-a-patient-with-x-linked-hypophosphatemia-complicated-by-autosomal-dominant-polycystic-kidney-disease
#18
Satomi Kajita, Takehisa Yamamoto, Naoko Tsugawa, Hirohumi Nakayama, Takuo Kubota, Toshimi Michigami, Keiichi Ozono
Serum calcitriol [1,25(OH)2D] levels are low normal in the presence of hypophosphatemia in X-linked dominant hypophosphatemic rickets (XLH) due to elevated serum fibroblast growth factor 23 (FGF23) levels. We report a peculiar finding of markedly elevated serum 1,25(OH)2D levels in a patient with XLH complicated by autosomal dominant polycystic kidney disease (ADPKD) and retinitis pigmentosa (RP). She was diagnosed with XLH, ADPKD and RP at the age of 5, 13 and 15 years, respectively. After the diagnosis of ADPKD, the 1,25(OH)2D levels increased up to 282 pg/mL treated with a pharmacological dose of 1αOHD3 (1αOHD3)...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28508965/cinacalcet-in-hyperparathyroidism-management-after-pediatric-renal-transplantation
#19
Olivier Niel, Anne Maisin, Marie-Alice Macher, Michel Peuchmaur, Georges Deschênes
Secondary hyperparathyroidism is often associated with end stage renal disease; even after renal transplantation, hyperparathyroidism may persist, and is responsible for hypercalcemia, hypophosphatemia and elevated parathyroid hormone (iPTH) levels. Parathyroid hyperplasia is frequently associated with persistent hyperparathyroidism, and may require a surgical treatment. Here, we report hyperparathyroidism along with parathyroid hyperplasia in a 7-year-old child, which persisted after renal transplant. Calcitonin and pamidronate failed to decrease serum calcium levels; clodronate was also inefficient...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28503481/dental-management-of-patients-with-x-linked-hypophosphatemia
#20
Bin-Na Lee, Hye-Yoon Jung, Hoon-Sang Chang, Yun-Chan Hwang, Won-Mann Oh
X-linked hypophosphatemia (XLH) is a hereditary metabolic disease caused by the loss of phosphate through the renal tubules into the urine, and an associated decrease in serum calcium and potassium phosphate. Its dental features include spontaneous dental abscesses that occur in the absence of trauma or dental caries. The aim of this case report was to describe the dental problems of XLH patients and to evaluate limitations in their treatment. A 14 year old male and a 38 year old female with XLH were referred to the Department of Conservative Dentistry for endodontic treatment...
May 2017: Restorative Dentistry & Endodontics
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