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    The Predicting Ability of Serum Phosphorus to Assess the Duration of Mechanical Ventilation in Critically Ill Patients.
    Adv Biomed Res 2017 25;6:51. Epub 2017 Apr 25.
    Department of Anesthesiology, Faculty of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.
    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. Read More

    Specific ablation of mouse Fam20C in cells expressing type I collagen leads to skeletal defects and hypophosphatemia.
    Sci Rep 2017 Jun 15;7(1):3590. Epub 2017 Jun 15.
    Department of Biomedical Sciences, Texas A&M University College of Dentistry, Dallas, Texas, 75246, USA.
    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. Read More

    Hypophosphatemia in critically ill patients with acute kidney injury treated with hemodialysis is associated with adverse events.
    Clin Kidney J 2017 Jun 5;10(3):341-347. Epub 2017 Jan 5.
    Department of Renal Medicine, Singapore General Hospital, Singapore, Singapore.
    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). Read More

    Craniofacial and dental characteristics of patients with vitamin-D-dependent rickets type 1A compared to controls and patients with X-linked hypophosphatemia.
    Clin Oral Investig 2017 Jun 12. Epub 2017 Jun 12.
    Department of Dentistry and Oral Health, Aarhus University, Aarhus, Denmark.
    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. Read More

    Clinical and imaging mimickers of axial spondyloarthritis.
    Semin Arthritis Rheum 2017 May 20. Epub 2017 May 20.
    Department of Diagnostic Imaging, Sheba Medical Center, Tel Hashomer, Ramat Gan, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
    The first step in the diagnosis of a patient with suspected axial spondyloarthritis is to differentiate the signs and symptoms of the disease from other disorders, potentially manifesting with similar clinical and imaging features. This review examines diffuse idiopathic skeletal hyperostosis, osteitis condensans ilii, and other developmental and metabolic disorders that may mimic axial spondyloarthritis, highlighting the diagnostic caveats and discussing shared and distinguishing aspects of these conditions in order to improve the clinician's ability to set them apart. Read More

    A novel auditory ossicles membrane and the development of conductive hearing loss in Dmp1-null mice.
    Bone 2017 Jun 8. Epub 2017 Jun 8.
    Biomedical Sciences, Texas A&M College of Dentistry, Dallas, TX 75246, USA. Electronic address:
    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. Read More

    Therapeutic Effects of FGF23 c-tail Fc in a murine pre-clinical model of X-linked hypophosphatemia via the selective modulation of phosphate reabsorption.
    J Bone Miner Res 2017 Jun 10. Epub 2017 Jun 10.
    Center for Therapeutic Innovation, Pfizer, New York University School of Medicine.
    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. Read More

    Phase II Study of First-Line Trebananib Plus Sorafenib in Patients with Advanced Hepatocellular Carcinoma.
    Oncologist 2017 Jun 7. Epub 2017 Jun 7.
    Memorial Sloan Kettering Cancer Center, New York, New York, USA.
    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.

    Background: Ang-1 and Ang-2 are angiopoietins thought to promote neovascularization via activation of the Tie-2 angiopoietin receptor. Read More

    Clinical and genetic analysis of two Chinese families with vitamin D-dependent rickets type IA and follow-up.
    J Clin Res Pediatr Endocrinol 2017 Jun 7. Epub 2017 Jun 7.
    Objective: Vitamin D-dependent rickets type IA (VDDR-IA) is a rare autosomal recessive disorder characterized by the early onset of severe rickets. The objectives of this study were twofold: 1) to analyze the clinical characteristics and therapy of two patients with VDDR-IA from two separate Chinese family, and 2) investigate the CYP27B1 gene in two large pedigrees.

    Methods: Medical history, clinical manifestations, physical examination, radiological findings and laboratory data were analyzed from two patients with VDDR-IA. Read More

    [Early hypophosphataemia in at risk newborns. Frequency and magnitude].
    An Pediatr (Barc) 2017 Jun 3. Epub 2017 Jun 3.
    Servicio de Neonatología, Hospital Universitario 12 de Octubre, Facultad de Medicina, Universidad Complutense de Madrid, Red SAMID del Instituto Carlos III, Instituto de Investigación Hospital Universitario 12 de Octubre, Madrid, España.
    Objective: To determine the frequency and magnitude of neonatal hypophosphataemia (<4mg/dL) in a neonatal Intensive Care Unit and to describe risk groups.

    Patients And Methods: Retrospective study of hospitalised newborns over a 44 month period (phase 1). Retrospective study of <1,500g/<32 weeks of gestation newborns over a 6 month period (phase 2). Read More

    Celiac Crisis: A Rare Or Rarely Recognized Disease.
    J Ayub Med Coll Abbottabad 2016 Oct-Dec;28(4):672-675
    Department of Hepatology & Nutrition, Children Hospital & Institute of child health, Lahore, Pakistan.
    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. Read More

    Our great forgotten, chronic respiratory sufferers
    Nutr Hosp 2017 05 8;34(Suppl 1):38-45. Epub 2017 May 8.
    Servicio de Medicina Intensiva. Hospital Germans Trias i Pujol. Badalona, Barcelona.
    Lung’s own properties make that nutritional support, besides covering the requirements can modulate its infl ammatory response. Lung tissue has a low glucose stock. Fatty acids are the main energy producer of type II pneumocytes, which use them in order to form phospholipids, essential for surfactant whose creation and release decrease in acute lung injury (ALI). Read More

    Pseudohypophosphatemia associated with high-dose liposomal amphotericin B therapy.
    Clin Biochem 2017 May 31. Epub 2017 May 31.
    Department of Laboratory Medicine, University of Washington School of Medicine, Seattle, WA, United States. Electronic address:
    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). Read More

    Cisplatin-Induced Renal Salt Wasting Requiring over 12 Liters of 3% Saline Replacement.
    Case Rep Nephrol 2017 10;2017:8137078. Epub 2017 May 10.
    Ronald Reagan UCLA Medical Center, Kidney Transplant, Los Angeles, CA 90095, USA.
    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. Read More

    Oncogenic Osteomalacia: An Approach to Diagnosis with a Case Report.
    J Clin Diagn Res 2017 Apr 1;11(4):ED05-ED07. Epub 2017 Apr 1.
    Assistant Professor, Department of Nuclear Medicine, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, India.
    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. Read More

    Post-Transplant Hypophosphatemia and the Risk of Death-Censored Graft Failure and Mortality after Kidney Transplantation.
    Clin J Am Soc Nephrol 2017 May 25. Epub 2017 May 25.
    Department of Internal Medicine, Division of Nephrology, University Medical Center Groningen and University of Groningen, Groningen, The Netherlands
    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. Read More

    Do Patients With a Baseline Clinical Condition Warranting the Cautious Use of Parenteral Nutrition Develop Subsequent Metabolic Complications?
    Nutr Clin Pract 2017 Jun 1;32(3):400-406. Epub 2016 Dec 1.
    1 Philadelphia College of Pharmacy, University of the Sciences, Philadelphia, Pennsylvania, USA.
    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). Read More

    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.
    Int Urol Nephrol 2017 May 19. Epub 2017 May 19.
    Department of Chemistry, University of Cape Town, Rondebosch, Cape Town, 7701, South Africa.
    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. Read More

    Analysis of Hypo- and Hyperphosphatemia in an Intensive Care Unit Cohort.
    Anesth Analg 2017 Jun;124(6):1897-1905
    From the *Skåne University Hospital, Lund, Getingevägen 4, SE-22185 Lund, Sweden; and †Science & Registry Nordic, Annedalsvägen 9, SE-22764 Lund, Sweden.
    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. Read More

    KIT Inhibition by Imatinib in Patients with Severe Refractory Asthma.
    N Engl J Med 2017 05;376(20):1911-1920
    From Brigham and Women's Hospital, Harvard Medical School, Boston (K.N.C., H.R.K., J.C., J.L., A.C.-T., D.G., T.M.L., J.A.B., E.I.), and Novartis Institutes for BioMedical Research, Cambridge (S.K.) - both in Massachusetts; Washington University, St. Louis (M.C.); University of Wisconsin, Madison (N.J.); Columbia University Medical Center, New York (E.D.); Cleveland Clinic, Cleveland (S.E.); University of Alabama at Birmingham, Birmingham (J.L.T.); Temple University, Philadelphia (K.S.), and Pennsylvania State University, Hershey (V.M.C.) - both in Pennsylvania; and National Jewish Health, Denver (M.E.W.).
    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. Read More

    Growth hormone therapy for a patient with idiopathic Fanconi syndrome and growth hormone deficiency.
    CEN Case Rep 2017 May 9;6(1):85-87. Epub 2017 Feb 9.
    Department of Pediatrics, Hokkaido University Hospital, N15, W7, Sapporo, 060-8638, Japan.
    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. Read More

    Serum calcitriol levels in a patient with X-linked hypophosphatemia complicated by autosomal dominant polycystic kidney disease.
    CEN Case Rep 2017 May 22;6(1):29-35. Epub 2016 Oct 22.
    Department of Pediatrics, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita City, Osaka, 565-0871, Japan.
    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. Read More

    Cinacalcet in hyperparathyroidism management after pediatric renal transplantation.
    CEN Case Rep 2016 Nov 22;5(2):141-143. Epub 2016 Jan 22.
    Pediatric Nephrology Department, Robert Debré Hospital, 48 Boulevard Sérurier, 75019, Paris, France.
    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. Read More

    Dental management of patients with X-linked hypophosphatemia.
    Restor Dent Endod 2017 May 6;42(2):146-151. Epub 2017 Jan 6.
    Department of Conservative Dentistry, School of Dentistry and Dental Science Research Institute, Chonnam National University, Gwangju, Korea.
    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. Read More

    Cardiovascular consequences of hypophosphatemia.
    Panminerva Med 2017 May 12. Epub 2017 May 12.
    Department of Medicine, John A. Burns School of Medicine, University of Hawaii at Manoa, Honolulu, Hawaii.
    Introduction: Few studies have been conducted to evaluate the effect of hypophosphatemia on cardiovascular consequences. The goal of this review was to determine whether hypophosphatemia is associated with cardiovascular consequences and to increase its awareness as a new clinical entity and a reversible cause of cardiovascular consequences.

    Evidence Acquisition: We searched MEDLINE and PubMed through September 2016 for primary studies that reported the relationship between hypophosphatemia and cardiovascular consequences including cardiomyopathy and arrhythmia. Read More

    Hypophosphatemia after nontraumatic intracranial hemorrhage.
    Acta Anaesthesiol Scand 2017 Jul 11;61(6):641-649. Epub 2017 May 11.
    Department Anesthesiology, Division of Intensive Care Medicine, Oulu University Hospital, Medical Research Center Oulu, Research Group of Surgery, Anesthesia and Intensive Care, Medical Faculty, University of Oulu, Oulu, Finland.
    Background: The aim of this study was to assess the incidence and contributing factors of hypophosphatemia and the association with poor long-term outcome after nontraumatic intracranial hemorrhage.

    Methods: This was a prospective, observational study of patients with nontraumatic intracranial hemorrhage (i.e. Read More

    Fractures and Osteomalacia in a Patient Treated With Frequent Home Hemodialysis.
    Am J Kidney Dis 2017 May 9. Epub 2017 May 9.
    Department of Pediatrics, David Geffen School of Medicine at UCLA, Los Angeles, CA.
    Bone deformities and fractures are common consequences of renal osteodystrophy in the dialysis population. Persistent hypophosphatemia may be observed with more frequent home hemodialysis regimens, but the specific effects on the skeleton are unknown. We present a patient with end-stage renal disease treated with frequent home hemodialysis who developed severe bone pain and multiple fractures, including a hip fracture and a tibia-fibula fracture complicated by nonunion, rendering her nonambulatory and wheelchair bound for more than a year. Read More

    Tumour genesis syndrome: severe hypophosphatemia and hypokalemia may be ominous presenting findings in childhood acute myeloid leukaemia.
    Eur J Pediatr 2017 May 4. Epub 2017 May 4.
    Department of Paediatrics, Queen Elizabeth Hospital, Hong Kong, SAR, Hong Kong.
    We report a 16-year-old girl who was diagnosed with acute leukaemia and a marked leucocytosis >200 × 10(9)/L. She presented with marked hypophosphatemia, hypokalemia, acute renal failure and acute respiratory failure. These electrolytes disturbances may indicate rapid tumour genesis. Read More

    Effective localization in tumor-induced osteomalacia using (68)Ga-DOTATOC-PET/CT, venous sampling and 3T-MRI.
    Endocrinol Diabetes Metab Case Rep 2017 19;2017. Epub 2017 Apr 19.
    The First Department of Medicine.
    Summary: Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome characterized by renal phosphate wasting leading to hypophosphatemia due to excessive actions of fibroblast growth factor 23 (FGF23) produced by the tumors. Although the best way of curing TIO is complete resection, it is usually difficult to detect the culprit tumors by general radiological modalities owing to the size and location of the tumors. We report a case of TIO in which the identification of the tumor by conventional imaging studies was difficult. Read More

    Gonadotrophin abnormalities in an infant with Lowe syndrome.
    Endocrinol Diabetes Metab Case Rep 2017 19;2017. Epub 2017 Apr 19.
    Departments of Paediatric Endocrinology and Diabetes.
    Summary: This case, presenting with bilateral impalpable testes, illustrates the relevance of a broad differential disorders of sex development case management. It provides new insights on hypothalamic-pituitary-gonadal (HPG) axis and testicular function abnormalities in the multisystem disorder of Lowe syndrome. Lowe syndrome, also known as oculocerebrorenal syndrome, is a rare disorder characterised by eye abnormalities, central nervous system involvement and proximal renal tubular acidosis. Read More

    Osmotic Demyelination Syndrome in a Eunatremic Patient with Chronic Kidney Disease.
    J Assoc Physicians India 2017 Mar;65(3):98-99
    Junior Resident, Department of Medicine, Era's Lucknow Medical College and Hospital, Lucknow, Uttar Pradesh.
    Osmotic demyelination syndrome is classically associated with rapid correction of hyponatremia. However, it can occur in normonatremic patients with other electrolyte abnormalities. One must suspect osmotic demyelination syndrome in susceptible patients with other electrolyte abnormalities like hypokalemia and hypophosphatemia. Read More

    Severe Hypophosphatemia Following Denosumab Administration in a Hemodialysis Patient with Progressive Prostate Cancer.
    Urol Case Rep 2017 Jul 26;13:63-65. Epub 2017 Apr 26.
    Department of Urology, Teikyo University Chiba Medical Center, 3426-3 Anesaki, Ichihara, Chiba 299-0111, Japan.
    In a 68-year-old man on maintenance hemodialysis (HD), severe anemia was detected. Bone marrow biopsy was performed for investigation of pancytopenia and pathological examination revealed adenocarcinoma of the prostate. Prostate specific antigen (PSA) was 574 ng/mL. Read More

    Octreotide Is Ineffective in Treating Tumor-Induced Osteomalacia: Results of a Short-Term Therapy.
    J Bone Miner Res 2017 Apr 29. Epub 2017 Apr 29.
    Skeletal Clinical Studies Unit, Craniofacial and Skeletal Disease Branch, National Institute of Dental and Craniofacial Research (NIDCR), National Institutes of Health (NIH), Bethesda, MD, USA.
    Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome in which unregulated hypersecretion of fibroblast growth factor 23 (FGF23) by phosphaturic mesenchymal tumors (PMT) causes renal phosphate wasting, hypophosphatemia, and osteomalacia. The resulting mineral homeostasis abnormalities and skeletal manifestations can be reversed with surgical resection of the tumor. Unfortunately, PMTs are often difficult to locate, and medical treatment with oral phosphate and vitamin D analogues is either insufficient to manage the disease or not tolerated. Read More

    Clinical and Biochemical Characteristics of Exertional Heat Stroke among Paratroopers in Agra, India.
    J Assoc Physicians India 2017 Feb;65(2):57-61
    Consultant Pathologist, Department of Internal Medicine, Military Hospital, Agra Cantt., Uttar Pradesh.
    Objective: The purpose of this study was to assess the clinical profile, biochemical parameters and outcome in a series of 78 patients of heat stroke admitted and treated in a military hospital in India.

    Methods: This was a prospective study in a military hospital. A total of 78 patients of exertional heat stroke admitted and treated over more than 2 years are reported. Read More

    Severe hypercalcaemia and hypophosphataemia with an optimised preterm parenteral nutrition formulation in two epochs of differing phosphate supplementation.
    Arch Dis Child Fetal Neonatal Ed 2017 Apr 29. Epub 2017 Apr 29.
    Neonatal Intensive Care Unit, Jenny Lind Children's Hospital, Norfolk and Norwich University Hospitals NHS Foundation Trust, Norwich, UK.
    Objective: To compare in two epochs of differing phosphate provision serum calcium, phosphate, potassium, and sodium concentrations and the frequency of abnormality of these electrolytes and of sepsis in preterm infants who received an optimised higher amino acid-content formulation.

    Design And Setting: Retrospective cohort study at a single tertiary-level neonatal unit.

    Patients: Preterm infants given parenteral nutrition (PN) in the first postnatal week during two discrete 6-month epochs in 2013-2014. Read More

    Phase 1b study of galunisertib in combination with gemcitabine in Japanese patients with metastatic or locally advanced pancreatic cancer.
    Cancer Chemother Pharmacol 2017 Jun 27;79(6):1169-1177. Epub 2017 Apr 27.
    National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-Ku, Tokyo, 104-0045, Japan.
    Purpose: Transforming growth factor-beta inhibitors may enhance the antitumor activity of gemcitabine with acceptable safety and tolerability. This open-label, multicenter, non-randomized phase 1b study assessed the safety/tolerability, pharmacokinetics, and tumor response of galunisertib plus gemcitabine in Japanese patients with advanced or metastatic pancreatic cancer.

    Methods: During each 28-day cycle, galunisertib 150 mg was administered orally twice daily (300 mg/day) for 14 days, followed by 14 days of rest. Read More

    The diagnostic dilemma of tumor induced osteomalacia: a retrospective analysis of 144 cases.
    Endocr J 2017 Apr 26. Epub 2017 Apr 26.
    Department of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.
    Diagnostic delay of tumor induced osteomalacia (TIO) is common in clinic practice. To investigate the diagnostic condition of TIO in China and raise clinicians' awareness of TIO, we retrospectively analyzed clinical manifestations, biochemical features, and specially evaluated missed diagnoses and misdiagnoses among 144 TIO patients from Peking Union Medical College Hospital during December 1982 to December 2014. Clinical presentations of TIO mainly included bone pain, difficulty in walking, pathological fractures, muscle weakness, and height loss. Read More

    Malignant phosphaturic mesenchymal tumor with pulmonary metastasis: A case report.
    Medicine (Baltimore) 2017 Apr;96(17):e6750
    aDepartment of Orthopedic Surgery bDepartment of Medical Oncology cDepartment of Ultrasonography, the First Affiliated Hospital of China Medical University, Shenyang dDepartment of Pathology, Fushun Hospital of Traditional Chinese Medicine, Fushun eDepartment of Radiology and Key Laboratory of Diagnostic Imaging and Interventional Radiology, the First Affiliated Hospital of China Medical University fDepartment of Pathology, the First Affiliated Hospital and College of Basic Medical Sciences of China Medical University, Shenyang, PR China.
    Rationale: Phosphaturic mesenchymal tumor (PMT) is a new tumor entity of soft tissue and bone tumor recently accepted by the World Health Organization, which typically causes the paraneoplastic syndrome of tumor-induced osteomalacia (TIO). The majority of PMTs follow a benign clinical course and local recurrence occurs in < 10% of cases, malignant PMTs with distant organ metastasis are extremely uncommon.

    Patient Concerns: We reported a 41-year-old woman who was diagnosed with PMT 10 years ago with a repeated recurrence and pulmonary metastasis. Read More

    Phase 1 dose-escalation study of mirvetuximab soravtansine (IMGN853), a folate receptor α-targeting antibody-drug conjugate, in patients with solid tumors.
    Cancer 2017 Apr 25. Epub 2017 Apr 25.
    Gillette Center for Gynecologic Oncology, Massachusetts General Hospital, Boston, Massachusetts.
    Background: Mirvetuximab soravtansine (IMGN853) is an antibody-drug conjugate that selectively targets folate receptor α (FRα). In this phase 1 dose-escalation study, the authors investigated IMGN853 in patients with FRα-positive solid tumors.

    Methods: Patients received IMGN853 on day 1 of a 21-day cycle (once every 3 weeks dosing), with cycles repeated until patients experienced dose-limiting toxicity or progression. Read More

    Phosphate imbalance in patients with heart failure.
    Heart Fail Rev 2017 May;22(3):349-356
    Department of Internal Medicine, School of Medicine, University of Ioannina, 451 10, Ioannina, Greece.
    Patients with heart failure often exhibit electrolyte abnormalities, such as hyponatremia or hypokalemia/hyperkalemia. Although not as common as the other electrolyte disturbances observed in patients with heart failure, phosphate imbalance is also of high importance in this population. The aim of this review is to present the mechanisms of low or high phosphate serum levels in patients with heart failure and its role in the pathogenesis and progression of heart dysfunction. Read More

    Management of mineral and bone disorders in renal transplant recipients.
    Nephrology (Carlton) 2017 Mar;22 Suppl 2:65-69
    Department of Endocrinology, Monash Health, Melbourne, Australia.
    The management of post-transplantation bone disease is a complex problem that remains under-appreciated in clinical practice. In these patients, pre-existing metabolic bone disorder is further impacted by the use of immunosuppressive medications (glucocorticoids and calcineurin-inhibitors), variable post-transplantation renal allograft function and post-transplantation diabetes mellitus. The treatment of post-transplantation bone loss should begin pre-transplantation. Read More

    Iron-induced hypophosphatemia: an emerging complication.
    Curr Opin Nephrol Hypertens 2017 Jul;26(4):266-275
    aDepartment of Medicine I, Gastroenterology, Hepatology, Endocrinology and Metabolism bDepartment of Radiology, Medical University of Innsbruck, Innsbruck, Austria.
    Purpose Of Review: Iron-induced hypophosphatemia is a well documented side-effect but associated complications are largely neglected, because the results from single dosing studies suggest that transient decreases in plasma phosphate concentrations are asymptomatic and fully reversible. However, an increasing number of case reports and case series suggest that some patients develop severe and symptomatic hypophosphatemia. Long-term complications from hypophosphatemia include osteomalacia and bone fractures, which can result from repeated intravenous administration of certain high-dose iron preparations. Read More

    [Identification of a novel splicing mutation of PHEX gene in a pedigree affected with X-linked hypophosphatemia].
    Zhonghua Yi Xue Yi Chuan Xue Za Zhi 2017 Apr;34(2):216-219
    Center for Reproductive Medicine, Shandong University, National Research Center for Assisted Reproductive Technology and Reproductive Genetics, The Key Laboratory for Reproductive Endocrinology of Ministry of Education, Jinan, Shandong 250000, China.
    Objective: To identify potential mutation of PHEX gene in two patients from a family affected with X-linked hypophosphatemia (XLH).

    Methods: PCR and Sanger sequencing were performed on blood samples from the patients and 100 healthy controls. Reverse transcription-PCR (RT-PCR) was used to determine the mRNA expression in patient samples. Read More

    Mutational analysis of PHEX, FGF23 and CLCN5 in patients with hypophosphataemic rickets.
    Clin Endocrinol (Oxf) 2017 Jul 11;87(1):103-112. Epub 2017 May 11.
    Department of Genetics, King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia.
    Context: Hypophosphataemic rickets (HR) is a group of rare hereditary renal phosphate wasting disorders caused by mutations in PHEX, FGF23, DMP1, ENPP1, CLCN5 or SLC34A3.

    Objective: To investigate underlying genetic defects in patients with hypophosphataemic rickets.

    Methods: We analysed genomic DNA from nine unrelated families for mutations in the entire coding region of PHEX, FGF23, DMP1, ENPP1, CLCN5 or SLC34A3 by PCR sequencing and copy number analysis. Read More

    Serum 25-Hydroxyvitamin D Insufficiency in Search of a Bone Disease.
    J Clin Endocrinol Metab 2017 Mar 30. Epub 2017 Mar 30.
    Department of Endocrinology, Austin Health, University of Melbourne.
    Introduction: Vitamin D 'insufficiency' and 'deficiency' are defined as a serum 25-hydroxyvitamin D (25(OH)D) below 75 and 30 nmol/L respectively. We aimed to determine whether these values signal hypocalcaemia and hypophosphatemia, secondary hyperparathyroidism, high bone remodeling, low areal bone mineral density (aBMD), microstructural deterioration, or reduced matric mineralization density (MMD), and so suggest whether bone fragility is present.

    Method: Concentrations of 25(OH)D, calcium, phosphate, creatinine and parathyroid hormone (PTH) were measured in 11,855 subjects. Read More

    The metabolic bone disease associated with the Hyp mutation is independent of osteoblastic HIF1α expression.
    Bone Rep 2017 Jun 17;6:38-43. Epub 2017 Jan 17.
    Department of Medical and Molecular Genetics, Indiana University School of Medicine, Indianapolis, IN 46202, United States.
    Fibroblast growth factor-23 (FGF23) controls key responses to systemic phosphate increases through its phosphaturic actions on the kidney. In addition to stimulation by phosphate, FGF23 positively responds to iron deficiency anemia and hypoxia in rodent models and in humans. The disorder X-linked hypophosphatemia (XLH) is characterized by elevated FGF23 in concert with an intrinsic bone mineralization defect. Read More

    Hungry bone syndrome.
    Curr Opin Nephrol Hypertens 2017 Jul;26(4):250-255
    aDivision of Nephrology, University of Arkansas for Medical Sciences bCentral Arkansas Veterans Affairs Healthcare System, Little Rock, Arkansas cOffice of Education, Central Alabama Veterans Healthcare System, Montgomery, Alabama, USA.
    Purpose Of Review: In the United States, the number of parathyroidectomies among patients with chronic dialysis has remained stable in the last decade. A fall in serum calcium concentration is common postparathyroidectomy in patients with hyperparathyroidism, which usually resolves in 2-4 days. A severe drop in serum total calcium concentration less than 2. Read More

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