5,697 results match your criteria Hypophosphatemia


Chiari I malformation and basilar invagination in fibrous dysplasia: prevalence, mechanisms, and clinical implications.

J Bone Miner Res 2018 Jun 20. Epub 2018 Jun 20.

Skeletal Disorders and Mineral Homeostasis Section, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, MD.

Fibrous dysplasia (FD) is a mosaic disorder of benign fibro-osseous lesions, which may be associated with extraskeletal features as part of McCune-Albright syndrome (MAS). Cranial base abnormalities, including Chiari I malformation (CM1), in which the cerebellum extends below the foramen magnum, and secondary basilar invagination (BI), in which the odontoid prolapses into the posterior cranial fossa, are potentially serious complications of metabolic bone disorders. The purpose of this study was to determine the prevalence, natural history, and risk factors for CM1 and BI in patients with FD/MAS, and to determine mechanisms of cranial base deformities. Read More

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Multicenter, randomized, double-blind phase 2 trial of FOLFIRI with regorafenib or placebo as second-line therapy for metastatic colorectal cancer.

Cancer 2018 Jun 15. Epub 2018 Jun 15.

Indiana University, Indianapolis, Indiana.

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. Read More

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June 2018
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Acquired hypophosphatemic osteomalacia is easily misdiagnosed or neglected by rheumatologists: A report of 9 cases.

Exp Ther Med 2018 Jun 27;15(6):5389-5393. Epub 2018 Apr 27.

Department of Rheumatology, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, Guangdong 510080, P.R. China.

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. Read More

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June 2018
2 Reads

Changing paradigms in metabolic support and nutrition therapy during critical illness.

Curr Opin Crit Care 2018 Jun 8. Epub 2018 Jun 8.

Department of Intensive Care Medicine, Gelderse Vallei Hospital, Ede, The Netherlands.

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. Read More

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Refeeding syndrome: relevance for the critically ill patient.

Curr Opin Crit Care 2018 Jun 8. Epub 2018 Jun 8.

Department of Intensive Care Medicine, Gelderse Vallei Hospital, Ede, The Netherlands.

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. Read More

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[Clinical and immunohistopathologic study of phosphaturic mesenchymal tumor].

Zhonghua Bing Li Xue Za Zhi 2018 Jun;47(6):427-431

Department of Pathology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.

To study the clinicopathological characteristics and immunohistochemical phenotype of phosphaturic mesenchymal tumor (PMT) . 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. The mean age of PMT patients was 42 years (range 13 to 70 years), with a male to female ratio of 1. Read More

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Eldecalcitol Causes FGF23 Resistance for Pi Reabsorption and Improves Rachitic Bone Phenotypes in the Male Hyp Mouse.

Endocrinology 2018 Jun 5. Epub 2018 Jun 5.

Department of Molecular Nutrition, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan.

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. Vehicle, ED71, or 1,25-dihydroxyvitamin D was subcutaneously injected 5 times/week in wild-type (WT) and Hyp mice for 4 weeks, from 4 to 8 weeks of age. Read More

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June 2018
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Legionella Pneumonia Complicated with Acquired Fanconi Syndrome: A Case Report.

Intern Med 2018 Jun 6. Epub 2018 Jun 6.

Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Science, Japan.

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. Read More

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Genetic analysis of three families with X-linked dominant hypophosphatemic rickets.

J Pediatr Endocrinol Metab 2018 Jun 2. Epub 2018 Jun 2.

Provincial Clinical Medical College, Fujian Medical University, Fuzhou, P.R. China.

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. Read More

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What a Trainee Surgeon Should Know About Refeeding Syndrome: A Literature Review.

Cureus 2018 Mar 28;10(3):e2388. Epub 2018 Mar 28.

Mayo Hospital, King Edward Medical University, Lahore, PAK.

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. Read More

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Admission serum phosphate levels predict hospital mortality.

Hosp Pract (1995) 2018 Jun 18:1-7. Epub 2018 Jun 18.

a Division of Nephrology and Hypertension, Department of Medicine , Mayo Clinic , Rochester , MN , USA.

Background: The aim of this study was to assess the relationship between admission serum phosphate levels and in-hospital mortality in all hospitalized patients.

Methods: All adult hospitalized patients who had admission serum phosphate available between years 2009 and 2013 were enrolled. Admission serum phosphate was categorized based on its distribution into six groups (<2. Read More

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SLC34A3 Intronic Deletion in an Iranian Kindred with Hereditary Hypophosphatemic Rickets with Hypercalciuria and Review of Reported Cases.

J Clin Res Pediatr Endocrinol 2018 May 29. Epub 2018 May 29.

Tehran University of Medical Sciences, Endocrinology and Metabolism Clinical Sciences Institute, Endocrinology and Metabolism Research Center, Tehran, Iran.

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.

Methods: In this paper we describe the clinical examination, biochemical profile and gene analysis of Iranian kindred with a 101bp deletion in SLC34A3 gene. Read More

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May 2018
2 Reads

Burosumab Therapy in Children with X-Linked Hypophosphatemia.

N Engl J Med 2018 05;378(21):1987-1998

From Yale University School of Medicine, New Haven, CT (T.O.C.); Shriners Hospital for Children and Division of Bone and Mineral Diseases, Department of Internal Medicine, Washington University School of Medicine, St. Louis (M.P.W.); Indiana University School of Medicine, Indianapolis (E.A.I.); University of Groningen, Groningen, the Netherlands (A.M.B.); Birmingham Children's Hospital, Birmingham (W. Högler), Royal Manchester Children's Hospital, Manchester (R.P.), and Great Ormond Street Hospital, London (W. van't Hoff) - all in the United Kingdom; Assistance Publique-Hôpitaux de Paris Hôpital Bicêtre, Paris (A.L.); and Ultragenyx Pharmaceutical, Novato (M.M., C.-Y.C., A.S., E.K., J.S.M.), and University of California at San Francisco, San Francisco (A.A.P.) - both in California.

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. Read More

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Early hypophosphatemia in very low birth weight preterm infants.

Adv Clin Exp Med 2018 May 21. Epub 2018 May 21.

Department and Clinic of Neonatology, University Hospital, Wroclaw Medical University, Poland.

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. Read More

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May 2018
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Distant lung metastases caused by a histologically benign phosphaturic mesenchymal tumor.

Endocrinol Diabetes Metab Case Rep 2018 16;2018. Epub 2018 May 16.

Division of Endocrinology and Metabolism, 1st Department of Internal Medicine, AHEPA University Hospital, Thessaloniki, Greece.

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. Read More

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May 2018
2 Reads

[Recurrent urolithiasis as a symptom of primary hyperparathyroidism in a 16-year-old boy].

Pol Merkur Lekarski 2018 Apr;44(262):208-210

Medical University of Lublin, Poland: Department of Pediatric Nephrology.

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. Read More

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April 2018
2 Reads

Adefovir dipivoxil induced hypophosphatemic osteomalacia in chronic hepatitis B: a comparative study of Chinese and foreign case series.

BMC Pharmacol Toxicol 2018 May 16;19(1):23. Epub 2018 May 16.

Department of Geriatrics, Qilu Hospital of Shandong University, Jinan, Shandong, China.

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. Read More

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Hypercalcemia and hypophosphatemia among preterm infants receiving aggressive parenteral nutrition.

Arch Argent Pediatr 2018 06;116(3):e371-e377

Departamento de Pediatría, Servicio de Neonatología, Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires.

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. Read More

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Hypophosphatemia in a Malnourished Child: When Renal Fanconi Syndrome Does Not Stand for Refeeding Syndrome.

JPEN J Parenter Enteral Nutr 2018 May 10. Epub 2018 May 10.

Department of Pediatrics, University of Chicago Medical Center, Chicago, Illinois, USA.

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. Read More

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Impaired mineral quality in dentin in X-linked hypophosphatemia.

Connect Tissue Res 2018 Dec;59(sup1):91-96

a Orofacial Pathologies, Imaging and Biotherapies Laboratory EA2496, Dental School , Paris Descartes University , Paris , France.

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. Read More

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December 2018

A Challenging Case of Tumor-Induced Osteomalacia: Pathophysiological and Clinical Implications.

Calcif Tissue Int 2018 May 7. Epub 2018 May 7.

Department of Internal Medicine and Medical Disciplines, "Sapienza" University, Viale del Policlinico 155, 00161, Rome, Italy.

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. Read More

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Increased prevalence of asymptomatic vertebral fractures in HIV-infected patients over 50 years of age.

Arch Osteoporos 2018 May 8;13(1):56. Epub 2018 May 8.

Department of Infectious Diseases, Ramon y Cajal Hospital, Madrid, Spain.

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. Read More

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Glucosuria without diabetes: key to the diagnosis of fragility fractures due to Fanconi syndrome.

BMJ Case Rep 2018 May 7;2018. Epub 2018 May 7.

Department of Orthopedics, College of Medicine and Philippine General Hospital, University of the Philippines Manila, Manila, Philippines.

A 64-year-old woman had fragility fractures which caused her to have gross deformities and confined her to bed. These were initially ascribed to vitamin D deficiency. However, despite correction of the deficiency, she did not improve. Read More

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May 2018
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The elevation of circulating fibroblast growth factor 23 without kidney disease does not increase cardiovascular disease risk.

Kidney Int 2018 May 5. Epub 2018 May 5.

Institute of Physiology, University of Zurich, and National Center for Competence in Research NCCR Kidney.CH, Zurich, Switzerland. Electronic address:

High circulating fibroblast growth factor 23 (FGF23) levels are probably a major risk factor for cardiovascular disease in chronic kidney disease. FGF23 interacts with the receptor FGFR4 in cardiomyocytes inducing left ventricular hypertrophy. Moreover, in the liver FGF23 via FGFR4 increases the risk of inflammation which is also found in chronic kidney disease. Read More

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May 2018
3 Reads
8.56 Impact Factor

Bone and mineral metabolism at 55 haemodialysis centres in Lima.

Nefrologia 2018 May - Jun;38(3):279-285

Servicio de Metabolismo Óseo y Mineral, Instituto Reina Sofía de Investigación, REDinREN del ISCIII, Hospital Universitario Central de Asturias, Universidad de Oviedo, Oviedo , Asturias, España.

Background: Mineral and bone metabolism disorders are common complications in haemodialysis patients that present significant geographical variability.

Objectives: The objective of this study was to assess these disorders for the first time in haemodialysis patients from Peru.

Methods: The study included 1551 haemodialysis patients from 55 centres affiliated with the Social Health System of Peru in the city of Lima. Read More

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Celiac crisis, a rare and profound presentation of celiac disease: a case report.

BMC Gastroenterol 2018 May 2;18(1):59. Epub 2018 May 2.

Diagnostic, Emergency and Medical Services, Gold Coast Hospital and Health Service, Gold Coast University Hospital, 1 Hospital Blvd, Southport, Gold Coast, Queensland, 4215, Australia.

Background: Celiac crisis is a life-threatening manifestation of celiac disease and is rare in adults, with only a handful of cases documented worldwide and mostly in children.

Case Presentation: A profoundly emaciated 43-year-old female presented with profuse diarrhoea, shortness of breath, left leg swelling with ulceration and immobility (Body Mass Index (BMI) = 14.7 kg/m). Read More

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May 2018
1 Read

FGF23 Regulates Wnt/β-Catenin Signaling-Mediated Osteoarthritis in Mice Overexpressing High-Molecular-Weight FGF2.

Endocrinology 2018 Jun;159(6):2386-2396

Division of Endocrinology and Metabolism, Department of Medicine, School of Medicine, UConn Health, Farmington, Connecticut.

Although humans with X-linked hypophosphatemia (XLH) and the Hyp mouse, a murine homolog of XLH, are known to develop degenerative joint disease, the exact mechanism that drives the osteoarthritis (OA) phenotype remains unclear. Mice that overexpress high-molecular-weight fibroblast growth factor (FGF) 2 isoforms (HMWTg mice) phenocopy both XLH and Hyp, including OA with increased FGF23 production in bone and serum. Because HMWTg cartilage also has increased FGF23 and there is cross-talk between FGF23-Wnt/β-catenin signaling, the purpose of this study was to determine if OA observed in HMWTg mice is due to FGF23-mediated canonical Wnt signaling in chondrocytes, given that both pathways are implicated in OA pathogenesis. Read More

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June 2018
1 Read

Heparin free dialysis in critically sick children using sustained low efficiency dialysis (SLEDD-f): A new hybrid therapy for dialysis in developing world.

PLoS One 2018 26;13(4):e0195536. Epub 2018 Apr 26.

Pediatric Nephrology, Akron Children's Hospital, Akron, Ohio, United States of America.

Background: In critically sick adults, sustained low efficiency dialysis [SLED] appears to be better tolerated hemodynamically and outcomes seem to be comparable to CRRT. However, there is paucity of data in critically sick children. In children, two recent studies from Taiwan (n = 11) and India (n = 68) showed benefits of SLED in critically sick children. Read More

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April 2018
3 Reads

Fibroblast Growth Factor 23-Induced Hypophosphatemia in Acute Leukemia.

J Endocr Soc 2018 May 6;2(5):437-443. Epub 2018 Apr 6.

Division of Metabolism, Endocrinology, and Diabetes, Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan.

Fibroblast growth factor 23 (FGF23)-induced hypophosphatemia is a rare paraneoplastic syndrome of phosphate wasting that, if unrecognized, may cause tumor-induced osteomalacia. It is classically associated with benign mesenchymal tumors but occasionally has been found in patients with other malignancies. Hypophosphatemia has been associated with acute leukemia but has not previously been reported to be due to inappropriate FGF23 secretion. Read More

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Hypophosphatemia and duration of respiratory failure and mortality in critically ill patients.

Acta Anaesthesiol Scand 2018 Apr 23. Epub 2018 Apr 23.

Department of Anaesthesiology, Nordsjaellands Hospital, Hillerød, University of Copenhagen, Hillerød, Denmark.

Background: Hypophosphatemia has been associated with prolonged duration of respiratory failure and increased mortality in critically ill patients, but there is very limited evidence supporting the negative effects of low phosphate. We examined the association between hypophosphatemia at ICU admission and time to successful weaning and 28-day mortality.

Methods: This was a cohort study that included all mechanically ventilated adult patients admitted to the ICU in 2013 at Nordsjaellands Hospital. Read More

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Vanadate-Induced Renal cAMP and Malondialdehyde Accumulation Suppresses Alpha 1 Sodium Potassium Adenosine Triphosphatase Protein Levels.

Toxicol Res 2018 Apr 15;34(2):143-150. Epub 2018 Apr 15.

Division of Nephrology, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.

It has been demonstrated that vanadate causes nephrotoxicity. Vanadate inhibits renal sodium potassium adenosine triphosphatase (Na, K-ATPase) activity and this is more pronounced in injured renal tissues. Cardiac cyclic adenosine monophosphate (cAMP) is enhanced by vanadate, while increased cAMP suppresses Na, K-ATPase action in renal tubular cells. Read More

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Multicenter Phase IB Trial of Carboxyamidotriazole Orotate and Temozolomide for Recurrent and Newly Diagnosed Glioblastoma and Other Anaplastic Gliomas.

J Clin Oncol 2018 Jun 23;36(17):1702-1709. Epub 2018 Apr 23.

Antonio Omuro, University of Miami, Miami, FL; Kathryn Beal, Robert J. Young, Thomas J. Kaley, Lisa M. DeAngelis, Mariza Daras, Igor T. Gavrilovic, Ingo Mellinghoff, Eli L. Diamond, Andrew McKeown, Malbora Manne, Andrew Caterfino, Krishna Patel, Philip Gutin, Viviane Tabar, Debyani Chakravarty, Timothy A. Chan, Cameron W. Brennan, and Elena Pentsova, Memorial Sloan Kettering Cancer Center; Rashida A. Karmali, Tactical Therapeutics, Inc, New York; Katharine McNeill, Montefiore Medical Center, Bronx, NY; Alissa Thomas, University of Vermont, Burlington, VT; Xuling Lin, National Neuroscience Institute, Singapore; Robert Terziev, University Hospital, Zurich, Switzerland; Linda Bavisotto, Porta Clinica PLLC, Seattle, WA; Greg Gorman, Samford University McWhorter School of Pharmacy, Birmingham, AL; Michael Lamson, Nuventra Pharma Sciences, Durham, NC; and Elizabeth Garrett-Mayer, Medical University of South Carolina, Charleston, SC.

Purpose Carboxyamidotriazole orotate (CTO) is a novel oral inhibitor of non-voltage-dependent calcium channels with modulatory effects in multiple cell-signaling pathways and synergistic effects with temozolomide (TMZ) in glioblastoma (GBM) models. We conducted a phase IB study combining CTO with two standard TMZ schedules in GBM. Methods In cohort 1, patients with recurrent anaplastic gliomas or GBM received escalating doses of CTO (219 to 812. Read More

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June 2018
2 Reads

Respiratory subtype of panic disorder: Can serum phosphate levels be a possible outcome to group cognitive-behavior therapy?

J Affect Disord 2018 Aug 12;235:474-479. Epub 2018 Apr 12.

Universidade Federal do Rio Grande do Sul, Brazil; Universidade Federal de Ciências da Saúde de Porto Alegre, Brazil.

Objective: Panic disorder (PD) respiratory subtype (RS) was described in order to cluster patients according to their symptoms. These patients are characterized by experiencing a relatively high number of noticeable respiratory symptoms during a panic attack (PA) and a higher reactivity to CO. In this study, we aimed to evaluate the clinical relevance of this diagnostic category, evaluating if there are different responses to cognitive-behavioral therapy in patients with panic disorder RS as compared to those with the non-respiratory subtype (NRS), using serum phosphate as a biological marker. Read More

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August 2018
1 Read

Burosumab: First Global Approval.

Authors:
Yvette N Lamb

Drugs 2018 Apr;78(6):707-714

Springer, Private Bag 65901, Mairangi Bay, Auckland, 0754, New Zealand.

Burosumab (Crysvita; Kyowa Hakko Kirin Co., Ltd. and Ultragenyx Pharmaceutical Inc. Read More

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April 2018
1 Read

Early versus standard initiation of renal replacement therapy in furosemide stress test non-responsive acute kidney injury patients (the FST trial).

Crit Care 2018 Apr 19;22(1):101. Epub 2018 Apr 19.

Division of Nephrology, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.

Background: The timing of initiation of renal replacement therapy (RRT) in severe acute kidney injury (AKI) remains controversial, with early initiation resulting in unnecessary therapy for some patients while expectant therapy may delay RRT for other patients. The furosemide stress test (FST) has been shown to predict the need for RRT and therefore could be used to exclude low-risk patients from enrollment in trials of RRT timing. We conducted this multicenter pilot study to determine whether FST could be used to screen patients at high risk for RRT and to determine the feasibility of incorporating FST into a trial of early initiation of RRT. Read More

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Scoliosis in Fibrous Dysplasia/McCune-Albright Syndrome: Factors Associated With Curve Progression and Effects of Bisphosphonates.

J Bone Miner Res 2018 Apr 18. Epub 2018 Apr 18.

Skeletal Disorders and Mineral Homeostasis Section, National Institute of Dental and Craniofacial Research (NIDCR), National Institutes of Health (NIH), Bethesda, MD, USA.

Scoliosis is a complication of fibrous dysplasia/McCune-Albright syndrome (FD/MAS); however, risk factors and long-term outcomes are unknown. Bisphosphonates are commonly used; however, it is unknown whether their use decrease the risk of progressive scoliosis. Clinical data from the National Institutes of Health (NIH) cohort study was reviewed. Read More

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April 2018
5 Reads

[Hypophosphatemia in preterm infants: a bimodal disorder].

Rev Chil Pediatr 2018 Feb;89(1):10-17

Complejo Asistencial Dr. Sótero del Río, Santiago, Chile.

New nutritional approaches to treat extreme premature babies have demonstrated relevant eviden ce of metabolic disturbances with early hypophosphatemia, especially in patients with intrauterine growth restriction (IUGR). They have shown late hypophosphatemia, as well, which is characteristic in the metabolic bone disease. A sytematic search of literature describing metabolic disturbances of phosphorus in preterm newborns is presented, related to the use of early parenteral nutrition and also in the context of metabolic bone disease. Read More

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February 2018
1 Read

Clinical findings and diagnostic procedures in 270 small ruminants with obstructive urolithiasis.

J Vet Intern Med 2018 May 16;32(3):1274-1282. Epub 2018 Apr 16.

Clinic for Ruminants, Vetsuisse Faculty, University of Bern, Bremgartenstrasse 109a, Bern 3012, Switzerland.

Background: Details of the clinical signs of obstructive urolithiasis in male small ruminants have not been documented in a large population.

Objective: To describe the clinical presentation and diagnostic procedures in a large group of small ruminants with urolithiasis.

Animals: Two hundred and seventy small ruminants (158 sheep and 112 goats). Read More

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A multi-arm phase I dose escalating study of an oral NOTCH inhibitor BMS-986115 in patients with advanced solid tumours.

Invest New Drugs 2018 Apr 10. Epub 2018 Apr 10.

Peter MacCallum Cancer Centre and Royal Melbourne Hospital, University of Melbourne, 305 Grattan St, Melbourne, VIC, 3000, Australia.

Background Inhibiting Notch is a promising anti-cancer strategy as it plays a critical role in cancer stem cells maintenance and tumour angiogenesis. BMS-986115 is an orally active, selective inhibitor of gamma-secretase mediated Notch signalling. Method Two dose escalation schedules (Arm-A continuous daily schedule and Arm-B intermittent 2 times weekly schedule) of BMS-986115 were evaluated in advanced solid tumour patients. Read More

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April 2018
3 Reads

A First-in-Human Phase 1 Study of LY3023414, an Oral PI3K/mTOR Dual Inhibitor, in Patients with Advanced Cancer.

Clin Cancer Res 2018 Apr 10. Epub 2018 Apr 10.

Stephenson Oklahoma Cancer Center/Sarah Cannon Research Institute, Oklahoma City, Oklahoma.

The PI3K/mTOR pathway is frequently aberrated in cancer. LY3023414 is a potent and selective ATP-competitive inhibitor of class I PI3K isoforms, mTOR, and DNA-PK. Here we report the dose-escalation results of the first-in-human phase I study of LY3023414. Read More

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Increased Circulating FGF23 Does Not Lead to Cardiac Hypertrophy in the Male Hyp Mouse Model of XLH.

Endocrinology 2018 May;159(5):2165-2172

Endocrine Unit, Massachusetts General Hospital, Boston, Massachusetts.

Serum levels of fibroblast growth factor 23 (FGF23) markedly increase with renal impairment, with FGF23 levels correlating with the presence of left ventricular hypertrophy (LVH) and mortality in patients with chronic kidney disease (CKD). FGF23 activates calcineurin/nuclear factor of activated T cell (NFAT) signaling and induces hypertrophy in murine cardiomyocytes. X-linked hypophosphatemia (XLH) is characterized by high circulating levels of FGF23 but, in contrast to CKD, is associated with hypophosphatemia. Read More

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May 2018
1 Read

Progress and Problems in Bone and Mineral Disorders.

Authors:
Neil Gittoes

Eur Endocrinol 2017 Apr 3;13(1):19-20. Epub 2017 Apr 3.

Centre for Endocrinology, Diabetes and Metabolism, Queen Elizabeth Hospital, University Hospitals Birmingham and University of Birmingham, UK.

A number of new drugs are moving through the osteoporosis therapy pipeline. Some show great promise for patients while one has fallen by the wayside at the last hurdle. New, effective therapies are warmly welcomed but there are still uncertainties around management of osteoporosis with currently available drugs that are contributing to what is commonly being referred to as the 'treatment gap'; a differential between those patients who would benefit from treatment versus those who actually are receiving it. Read More

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April 2017
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Clinical and laboratory features of patients with osteomalacia initially presenting with neurological manifestations.

Osteoporos Int 2018 Apr 5. Epub 2018 Apr 5.

Department of Neurology, Yonsei University College of Medicine, Seoul, South Korea.

Patients with osteomalacia often visit the neurology department with conditions mimicking other myopathies. We analyzed clinical features of osteomalacia patients who visited the neurology department. These patients frequently presented with hypocalcemia, hypovitaminosis D, and pain with less severe weakness. Read More

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[Relationship between continuous renal replacement therapy and hypophosphatemia in critically ill children].

Zhonghua Er Ke Za Zhi 2018 Apr;56(4):284-288

Department of Critical Care Medicine, Children's Hospital Affiliated to Shanghai Jiao Tong University, Shanghai 200040, China.

To investigate the incidence and prognosis of hypophosphatemia in critically ill children treated with continuous blood purification (CBP). The medical records of the critically ill patients, who were treated with CBP, admitted to pediatric intensive care unit (PICU) of Shanghai Children's Hospital from May 2014 to April 2017 were retrospectively analyzed. The serum phosphorus levels were tested before CBP, at 48-72 h during CBP, at the end of CBP and on the next day after CBP finished. Read More

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Sjögren's Syndrome Associated with Fanconi's Syndrome and Osteomalacia.

Am J Case Rep 2018 Apr 3;19:392-396. Epub 2018 Apr 3.

Department of Rheumatology, Unidad de Enfermedades Reumáticas y Autoinmunes [UNERA] (Unit of Rheumatology and Autoimmune Diseases), Guayaquil, Ecuador.

BACKGROUND Sjögren's syndrome is a chronic inflammatory autoimmune disease, which is also known as sicca syndrome, due to the symptoms of dry eyes and dry mouth, and is associated with other connective tissue diseases and autoimmune diseases. Sjögren's syndrome can also be associated with renal involvement. Fanconi's syndrome is associated with impaired reabsorption in the proximal renal tubule associated with tubulointerstitial nephritis and is associated with renal tubular acidosis and hypophosphatemia. Read More

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April 2018
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Phase I trial of the mTOR inhibitor everolimus in combination with multi-agent chemotherapy in relapsed childhood acute lymphoblastic leukemia.

Pediatr Blood Cancer 2018 Jul 30;65(7):e27062. Epub 2018 Mar 30.

Department of Pediatric Oncology, Dana-Farber Cancer Institute and Boston Children's Hospital, Boston, Massachusetts.

Background: We sought to determine the feasibility of co-administering everolimus with a four-drug reinduction in children and adolescents with acute lymphoblastic leukemia (ALL) experiencing a first marrow relapse.

Procedure: This phase I study tested everolimus with vincristine, prednisone, pegaspargase and doxorubicin in patients with marrow relapse occurring >18 months after first complete remission (CR). The primary aim was to identify the maximum tolerated dose of everolimus. Read More

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July 2018
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Polyostotic Fibrous Dysplasia With and Without McCune-Albright Syndrome-Clinical Features in a Nordic Pediatric Cohort.

Front Endocrinol (Lausanne) 2018 15;9:96. Epub 2018 Mar 15.

Department of Women's and Children's Health, Karolinska Institute, Stockholm, Sweden.

Objective: Fibrous dysplasia (FD) presents as skeletal lesions in which normal bone is replaced by abnormal fibrous tissue due to mosaic mutation. McCune-Albright syndrome (MAS) refers to FD combined with skin (café-au-lait) and endocrine manifestations. This study describes the clinical childhood manifestations of polyostotic FD and MAS in a Nordic cohort. Read More

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Hypophosphatemia occurs with insulin administration during refeeding by total parenteral nutrition in rats.

J Med Invest 2018 ;65(1.2):50-55

Graduate school of Human Science and Environment.

Refeeding syndrome (RFS) is characterized by the metabolic and clinical changes that occur following aggressive nutritional supplementation in malnourished patients. Hypophosphatemia is the hallmark of RFS and is key to its prevention and treatment in clinical practice. However, the mechanism of hypophosphatemia during RFS is unclear because of the lack of an animal model. Read More

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January 2018
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