6,274 results match your criteria Hypophosphatemia


An Overview of Rickets in Children.

Kidney Int Rep 2020 Jul 11;5(7):980-990. Epub 2020 Apr 11.

Akron Nephrology Associates/Cleveland Clinic Akron General, Akron, Ohio, USA.

Rickets is a common bone disease worldwide that is associated with disturbances in calcium and phosphate homeostasis and can lead to short stature and joint deformities. Rickets can be diagnosed based on history and physical examination, radiological features, and biochemical tests. It can be classified into 2 major groups based on phosphate or calcium levels: phosphopenic and calcipenic. Read More

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http://dx.doi.org/10.1016/j.ekir.2020.03.025DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7335963PMC

Rickets in Association with Skin Diseases and Conditions: a Review with Emphasis on Screening and Prevention.

Photodermatol Photoimmunol Photomed 2020 Jul 9. Epub 2020 Jul 9.

State University of New York Downstate and Veterans Affairs Medical Center, Brooklyn, NY, USA.

Background: Rickets is a common disease worldwide. In the developed world, its prevalence dramatically decreased but still diagnosed in at-risk populations. The skin plays a critical role in vitamin D synthesis. Read More

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http://dx.doi.org/10.1111/phpp.12590DOI Listing

Cabozantinib in patients with platinum-refractory metastatic urothelial carcinoma: an open-label, single-centre, phase 2 trial.

Lancet Oncol 2020 Jul 6. Epub 2020 Jul 6.

Developmental Therapeutics Branch, Magnuson Clinical Center, Bethesda, MD, USA.

Background: Cabozantinib is a multikinase inhibitor of MET, VEGFR, AXL, and RET, which also has an effect on the tumour immune microenvironment by decreasing regulatory T cells and myeloid-derived suppressor cells. In this study, we examined the activity of cabozantinib in patients with metastatic platinum-refractory urothelial carcinoma.

Methods: This study was an open-label, single-arm, three-cohort phase 2 trial done at the National Cancer Institute (Bethesda, MD, USA). Read More

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http://dx.doi.org/10.1016/S1470-2045(20)30202-3DOI Listing

A Case of Hyperparathyroidism Which Developed After Resection of a Fibroblast Growth Factor 23-producing Tumor.

Intern Med 2020 Jul 7. Epub 2020 Jul 7.

First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Japan.

A 53-year-old woman presented with bone pain and was diagnosed with osteomalacia because of hypophosphatemia, hyperphosphatasemia, bone pain, and radiographic findings. Because her intact-fibroblast growth factor 23 (FGF23) levels were high and contrast-enhanced computed tomography revealed a mass in the anterior ethmoid sinus, FGF23-related osteomalacia was diagnosed. The tumor was resected, but she developed hypercalcemia and elevated blood parathyroid hormone (PTH) levels. Read More

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http://dx.doi.org/10.2169/internalmedicine.4412-19DOI Listing

Life-threatening hypophosphatemia following intravenous iron infusion.

Nefrologia 2020 Jul 4. Epub 2020 Jul 4.

Department of Internal Medicine, Saint Louis University School of Medicine, Saint Louis, MO, United States of America.

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http://dx.doi.org/10.1016/j.nefro.2020.03.007DOI Listing

Proximal renal tubular function in HIV-infected children on tenofovir disoproxil fumarate for treatment of HIV infection at two tertiary hospitals in Harare, Zimbabwe.

PLoS One 2020 7;15(7):e0235759. Epub 2020 Jul 7.

Department of Paediatrics, University of Zimbabwe College of Health Sciences, Harare, Zimbabwe.

Background: Renal abnormalities in HIV infected children may be due to the HIV infection or treatment among other factors. Tenofovir disoproxil fumarate (TDF) is associated with proximal renal tubular dysfunction, proteinuria and decrease in glomerular function. Studies in developed countries have shown variable prevalence of proximal renal tubular dysfunction in children on TDF. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0235759PLOS

Renal Phosphate Handling in Antiretroviral-naïve HIV-infected Patients.

Infect Disord Drug Targets 2020 Jul 7. Epub 2020 Jul 7.

Department of Medicine (HIV Unit), University of Abuja Teaching Hospital, Abuja. Nigeria.

Background: Human immunodeficiency virus (HIV) infection impairs renal function, thereby affecting renal phosphate metabolism.

Objectives: We prospectively estimated the prevalence of phosphate abnormalities (mild, moderate to life-threatening hypophosphataemia, and hyperphosphataemia) before initiating antiretroviral therapy (ART).

Methods: A cross-sectional analysis was performed on 170 consecutive newly diagnosed ART-naïve, HIV-infected patients attending our HIV/AIDS clinics over a period of one year. Read More

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http://dx.doi.org/10.2174/1871526520666200707115259DOI Listing

Tumor-Induced Osteomalacia Caused by a Phosphaturic Mesenchymal Tumor of the Sole Presenting as a Crippling Illness in a Postmenopausal Woman.

J Foot Ankle Surg 2020 Jul 2. Epub 2020 Jul 2.

Professor, Department of Orthopedics, West China School of Medicine/West China Hospital, Sichuan University, Chengdu, Sichuan, People's Republic of China. Electronic address:

Tumor-induced osteomalacia, a rare and intriguing paraneoplastic syndrome that is usually caused by a phosphaturic mesenchymal tumor, leads to severe pain and hypophosphatemia. However, during clinical practice, most patients suffer from significant delay of diagnosis and treatment because the symptoms are similar to those of some very common diseases, such as osteoporosis and osteoarthritis. Moreover, physical complaints from postmenopausal women usually exacerbate the possibility of such delays. Read More

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http://dx.doi.org/10.1053/j.jfas.2020.04.012DOI Listing

Alterations of bone material properties in adult patients with X-linked hypophosphatemia (XLH).

J Struct Biol 2020 Jun 30:107556. Epub 2020 Jun 30.

Ludwig Boltzmann Institute of Osteology at Hanusch Hospital of OEGK and AUVA Trauma Centre Meidling, 1(st) Medical Department Hanusch Hospital, Vienna, Austria; Medical Directorate, Hanusch Hospital, Vienna, Austria.

X-linked hypophosphatemia (XLH) caused by PHEX mutations results in elevated serum FGF23 levels, renal phosphate wasting and low 1,25-dihydroxyvitamin D. The glycophosphoprotein osteopontin, a potent inhibitor of mineralization normally degraded by PHEX, accumulates within the bone matrix. Conventional therapy consisting of supplementation with phosphate and vitamin D analogs is burdensome and the effects on bone material poorly characterized. Read More

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http://dx.doi.org/10.1016/j.jsb.2020.107556DOI Listing

Acute hemolysis crisis revealed a Wilson disease.

Ann Biol Clin (Paris) 2020 07 3. Epub 2020 Jul 3.

Service d'hématologie biologique, Institut de biologie clinique, CHU-Hôpitaux de Rouen, France.

Wilson disease is a rare inherited disorder of copper metabolism that affects liver and brain due to copper tissue accumulation. The mechanism involved is based on mutations of the ATP7B gene. Children have predominant hepatic manifestations while adult are more often diagnosed by neurological and psychiatric symptoms. Read More

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http://dx.doi.org/10.1684/abc.2020.1574DOI Listing

The international X-linked hypophosphataemia (XLH) registry (NCT03193476): rationale for and description of an international, observational study.

Orphanet J Rare Dis 2020 Jun 30;15(1):172. Epub 2020 Jun 30.

Royal Manchester Children's Hospital and Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK.

Background: X-linked hypophosphataemia (XLH) is a rare, hereditary, progressive and lifelong phosphate wasting disorder characterised by pathological elevations in fibroblast growth factor (FGF) 23 concentration and activity; XLH has an incidence of approximately 1 in 20-25,000 individuals. Excess FGF23 activity leads to increased phosphate excretion in the kidneys - mediated by downregulation of renal tubular phosphate transporters - and reduced phosphate absorption in the intestines - due to impaired vitamin D activation. This results in impaired bone growth and mineralisation, short and disproportionate stature, leg bowing, musculoskeletal pain, spontaneous dental abscesses, rickets, and osteomalacia. Read More

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http://dx.doi.org/10.1186/s13023-020-01434-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7329472PMC

Long-term outcomes for Asian patients with X-linked hypophosphataemia: rationale and design of the SUNFLOWER longitudinal, observational cohort study.

BMJ Open 2020 Jun 29;10(6):e036367. Epub 2020 Jun 29.

Department of Pediatrics, Graduate School of Medicine, Osaka University, Suita, Japan.

Introduction: X-linked hypophosphataemic rickets/osteomalacia (XLH) is a chronic, debilitating genetic disease characterised by skeletal abnormalities and growth disorder. The burden of XLH begins in childhood and continues throughout life. Conventional medical therapy with phosphate, active vitamin D and surgery do not address the underlying pathophysiology of the disease. Read More

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http://dx.doi.org/10.1136/bmjopen-2019-036367DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7328740PMC

Paroxysmal Ventricular Standstill: A Rare Cardiac Manifestation of Syncope.

Am J Case Rep 2020 Jun 17;21:e924381. Epub 2020 Jun 17.

Emergency Department, Charing Cross Hospital, Imperial College Healthcare NHS Trust, London, United Kingdom.

BACKGROUND Transient abrupt loss of consciousness due to sudden but pronounced decrease in cardiac output caused by a change in heart rate and rhythm is termed Stokes-Adams disease. Causes of Stokes-Adams syndrome are 1) transition from normal rhythm to high grade block, 2) slowing of idioventricular rhythm in the course of complete heart block, and 3) abnormal ventricular rhythm such as ventricular tachycardia and ventricular fibrillation. Paroxysmal ventricular standstill is one of the rarest causes of Stokes-Adams attack. Read More

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http://dx.doi.org/10.12659/AJCR.924381DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7322213PMC

Hypophosphatemic Hypovitaminosis D Induces Osteomalacia in the Adult Female Rat.

Endocrinology 2020 Jun 27. Epub 2020 Jun 27.

Department of Drug Design and Pharmacology, Faculty of Health and Medical Sciences, University of Copenhagen, Denmark.

Osteomalacia is a bone demineralizing disease of the adulthood, often caused by hypovitaminosis D. Current animal models of the disease mimic osteomalacia as a consequence of gastric bypass or toxic exposure to metals, but a relevant model of diet-induced osteomalacia is lacking. For that purpose, seven-month-old female Sprague Dawley rats were randomized into two weight-stratified groups and maintained four months on synthetic diets containing negligible or normal levels of vitamin D. Read More

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http://dx.doi.org/10.1210/endocr/bqaa100DOI Listing

Refeeding syndrome in small ruminants receiving parenteral nutrition.

J Vet Intern Med 2020 Jun 26. Epub 2020 Jun 26.

Department of Clinical Studies-New Bolton Center (Luethy, Stefanovski, Sweeney), School of Veterinary Medicine, University of Pennsylvania, Kennett Square, Pennsylvania, USA.

Background: Small ruminants presented to tertiary care facilities commonly suffer from severe protein-calorie malnutrition. Some of these patients require parenteral nutrition (PN; amino acids and dextrose with or without lipids) during hospitalization. Refeeding syndrome, a potentially fatal shift of electrolytes seen in malnourished patients during refeeding, may occur. Read More

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http://dx.doi.org/10.1111/jvim.15840DOI Listing

Severe hypophosphataemia following ferric carboxymaltose infusion in paediatric patients with inflammatory bowel disease.

Frontline Gastroenterol 2020 3;11(4):324-326. Epub 2019 Jul 3.

Department of Paediatric Gastroenterology, Hepatology and Nutrition, Royal Hospital for Children Glasgow, Glasgow, UK.

This case series describes the cases of three adolescent patients with established inflammatory bowel disease (IBD) who experienced significant hypophosphataemia following intravenous infusion of ferric carboxymaltose as treatment for iron deficiency anaemia. Hypophosphataemia may cause a diverse range of symptoms and may be difficult to diagnose clinically due to their non-specific nature. Checking a baseline phosphate (PO) prior to intravenous iron infusion may identify patients at higher risk for significant hypophosphataemia and perhaps allow the selection of an alternative iron preparation. Read More

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http://dx.doi.org/10.1136/flgastro-2019-101242DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7307048PMC

Changes in the metabolic profiles of dairy cows before and after calving that were mainly fed fodder beet ( L.) during the dry period.

N Z Vet J 2020 Jun 25:1-19. Epub 2020 Jun 25.

North Canterbury Veterinary Clinics, PO Box 58, Culverden 7391, New Zealand.

: Two commercial pasture-based farms within the North Canterbury district of New Zealand were feeding fodder beet ( L.) as a large proportion of the diet to cows during the dry period. On each farm 25 multiparous cows were blood sampled up to six times from 28 days before to 21 days after calving (Day 0). Read More

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http://dx.doi.org/10.1080/00480169.2020.1786475DOI Listing

Extreme anorexia nervosa: medical findings, outcomes, and inferences from a retrospective cohort.

J Eat Disord 2020 23;8:25. Epub 2020 Jun 23.

ACUTE Center for Eating Disorders at Denver Health, 723 Delaware St, Pav M 3rd floor, Denver, CO 80204 USA.

Background: Extreme anorexia nervosa (AN) is defined as a BMI < 15 kg/m in those meeting DSM-V diagnostic criteria for AN. This study seeks to define the frequency of medical complications in this group of patients in order to help inform the care of individuals < 65% ideal body weight who seek treatment for their extreme eating disorders.

Methods: Through retrospective chart review and computerized data collection, we obtained the baseline characteristics and medical findings of 281 adult patients, with AN restricting and binge-purge subtypes, admitted to the ACUTE unit for medical stabilization between May 2013 and August 2018. Read More

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http://dx.doi.org/10.1186/s40337-020-00303-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7310519PMC

Early postkidney transplantation hypophosphatemia.

J Res Med Sci 2020 13;25:36. Epub 2020 Apr 13.

Department of Medicine, Hasheminejad Kidney Center, Iran University of Medical Sciences, Tehran, Iran.

Background: As hypophosphatemia is a common multifactorial problem of kidney transplantation (Tx), this research aimed at studying the frequency of posttransparent hypophosphatemia in the early postkidney Tx period and investigating the risk components associated with the situation.

Materials And Methods: In this study, 50 renal transplant recipients on the day before (-1) and on days 10 (+10) and 30 (+30) days after kidney Tx were examined for the levels of serum phosphate (Pi). Levels of serum creatinine (Cr), Pi, 25-hydroxyvitamin D (25[OH] D), intact parathyroid hormone (iPTH) and fibroblast growth factor 23 (FGF-23), the 24 h urinary excretion of Pi and Cr, estimated glomerular filtration rate (eGFR), and the ratio of transport maximum of Pi (TMP) to eGFR (TMP/GFR) were evaluated on the same days. Read More

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http://dx.doi.org/10.4103/jrms.JRMS_452_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7306234PMC

Decreased Compressional Sound Velocity Is an Indicator for Compromised Bone Stiffness in X-Linked Hypophosphatemic Rickets (XLH).

Front Endocrinol (Lausanne) 2020 9;11:355. Epub 2020 Jun 9.

Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, BCRT - Berlin Institute of Health Center for Regenerative Therapies, Berlin, Germany.

To assess the diagnostic potential of bidirectional axial transmission (BDAT) ultrasound, and high-resolution peripheral quantitative computed tomography (HR-pQCT) in X-linked hypophosphatemia (XLH, OMIM #307800), a rare genetic disorder of phosphate metabolism caused by mutations in the gene. BDAT bone ultrasound was performed at the non-dominant distal radius (33% relative to distal head) and the central left tibia (50%) in eight XLH patients aged between 4.2 and 20. Read More

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http://dx.doi.org/10.3389/fendo.2020.00355DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7296046PMC

Metabolic Complications Occur More Frequently in Older Patients Receiving Parenteral Nutrition.

Nutr Clin Pract 2020 Aug 24;35(4):627-633. Epub 2020 Jun 24.

Department of Pharmacy, Cooper University Hospital, Camden, New Jersey, USA.

Background: The European Society for Clinical Nutrition and Metabolism Guidelines for Parenteral Nutrition in Geriatric Patients state metabolic complications are more frequent in elderly patients. However, literature provides limited information about metabolic complications in older patients receiving parenteral nutrition (PN). Consequently, the purpose of this study was to compare the development of metabolic complications in older vs younger patients receiving PN. Read More

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http://dx.doi.org/10.1002/ncp.10499DOI Listing
August 2020
2.058 Impact Factor

Phase 1 dose-escalation study of a novel oral PI3K/mTOR dual inhibitor, LY3023414, in patients with cancer.

Invest New Drugs 2020 Jun 23. Epub 2020 Jun 23.

Department of Gastrointestinal Oncology, National Cancer Center Hospital East, Chiba, Japan.

LY3023414 is an oral, selective adenosine triphosphate-competitive inhibitor of class I phosphatidylinositol 3-kinase isoforms, mammalian target of rapamycin, and DNA-protein kinase in clinical development. We report results of a 3 + 3 dose-escalation Phase 1 study for twice-daily (BID) dosing of LY3023414 monotherapy in Japanese patients with advanced malignancies. The primary objective was to evaluate tolerability and safety of LY3023414. Read More

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http://dx.doi.org/10.1007/s10637-020-00968-5DOI Listing

The Case | Severe hypophosphatemia in a patient with lymphoma.

Kidney Int 2020 Jul;98(1):243-244

Division of Nephrology, Department of Medicine, Jewish General Hospital, Montreal, Quebec, Canada. Electronic address:

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http://dx.doi.org/10.1016/j.kint.2020.01.012DOI Listing

CT-Measured Psoas Muscle Density as a Predictive Factor for Hypophosphatemia Associated with Refeeding.

JPEN J Parenter Enteral Nutr 2020 Jun 22. Epub 2020 Jun 22.

The Ohio State University, Division of Trauma, Critical Care, and Burn, Department of Surgery, Columbus, OH, USA.

Background: Initiation of parenteral nutrition (PN) after a period of starvation can be complicated by refeeding syndrome (RFS). RFSs is associated with electrolyte abnormalities including hypomagnesemia, hyponatremia, and most commonly hypophosphatemia. Risk factors associated with the development of RFS include recent weight loss, low BMI, and electrolyte deficiencies, however, these associations are not strong. Read More

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http://dx.doi.org/10.1002/jpen.1943DOI Listing

Intravenous iron and chronic obstructive pulmonary disease: a randomised controlled trial.

BMJ Open Respir Res 2020 Jun;7(1)

Department of Physiology, Anatomy and Genetics, University of Oxford, Oxford, UK

Background: Increased iron availability modifies cardiorespiratory function in healthy volunteers and improves exercise capacity and quality of life in patients with heart failure or pulmonary hypertension. We hypothesised that intravenous iron would produce improvements in oxygenation, exercise capacity and quality of life in patients with chronic obstructive pulmonary disease (COPD).

Methods: We performed a randomised, placebo-controlled, double-blind trial in 48 participants with COPD (mean±SD: age 69±8 years, haemoglobin 144. Read More

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http://dx.doi.org/10.1136/bmjresp-2020-000577DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7311010PMC

The risk of refeeding syndrome among severely malnourished tuberculosis patients in Chhattisgarh, India.

Indian J Tuberc 2020 Apr 4;67(2):152-158. Epub 2019 Apr 4.

Jan Swasthya Sahyog, India; St Louis University, St Louis, MO, USA.

Background: A secondary care hospital in rural India serving a highly tuberculosis (TB) and malnutrition endemic region.

Objective: In this study conducted on patients with chronic protein energy malnutrition (PEM) and TB, we sought to compare nurse-estimated vs. smartphone photograph analytic methods for assessing caloric intake and determine the incidence of refeeding hypophosphatemia (RH) and refeeding syndrome (RFS) in patients with TB. Read More

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http://dx.doi.org/10.1016/j.ijtb.2019.03.004DOI Listing

Clinical Evidence for the Benefits of Burosumab Therapy for X-Linked Hypophosphatemia (XLH) and Other Conditions in Adults and Children.

Front Endocrinol (Lausanne) 2020 28;11:338. Epub 2020 May 28.

Discipline of Child and Adolescent Health, University of Sydney, Sydney, NSW, Australia.

Burosumab (KRN23) is an FGF23 neutralizing antibody that has been the subject of several recent clinical trials principally focused on the treatment of hypophosphatemic rickets in patients with X-linked hypophosphatemia (XLH). Since the first publications in 2014, these trials have demonstrated efficacy with minimal safety concerns in both adult and pediatric cohorts. These studies have used dose-escalation to establish a dosing regimen that is well-tolerated in clinical use. Read More

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http://dx.doi.org/10.3389/fendo.2020.00338DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7271822PMC

The Association of Hypophosphatemia With Resistant Lactic Acidosis in Critical Care Illness.

J Investig Med High Impact Case Rep 2020 Jan-Dec;8:2324709620934963

Creighton University, Omaha, NE, USA.

Critically ill patients are known to have a variety of electrolyte abnormalities. Lactic acidosis can frequently be seen secondary to shock states and is usually treated with aggressive volume resuscitation. Interestingly, hypophosphatemia is a potential cause of resistant lactic acidosis, which may not be as commonly identified or considered. Read More

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http://dx.doi.org/10.1177/2324709620934963DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7298210PMC

Left maxillary sinus tumour-induced hypophosphataemic osteomalacia and combined with thyroid papillary carcinoma: a case report and literature review.

Intern Med J 2020 Jun;50(6):755-761

Department of Endocrinology, Baoding NO.1 Central Hospital, Baoding, China.

Tumour-induced osteomalacia (TIO) is a rare disease characterised by hypophosphataemia and clinical symptoms of osteomalacia. Herein we report the case of a 29-year-old man who was admitted to hospital with progressive bone pain and was diagnosed with TIO caused by maxillary sinus tumours. In the preoperative evaluation, it was found that the patient had thyroid malignant tumours at the same time. Read More

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http://dx.doi.org/10.1111/imj.14864DOI Listing

[Biopsychosocial care concept for children with X‑chromosomal hypophosphatemia (XLH) : Example of the multiprofessional approach of social pediatric centers].

Authors:
Dirk Schnabel

Bundesgesundheitsblatt Gesundheitsforschung Gesundheitsschutz 2020 Jul;63(7):813-820

SPZ für chronisch kranke Kinder/Pädiatrische Endokrinologie, Charité - Universitätsmedizin Berlin, Augustenburger Platz 1 (Mittelallee 7a), 13353, Berlin, Deutschland.

Patients with severe, rare and complex diseases require the multiprofessional biopsychosocial care concept of a social pediatric center for chronically ill children and adolescents. The care concept is illustrated using the example of the multiorgan disease X‑chromosomal hypophosphatemic rickets (XLH), the most common congenital form of rickets.The disease is based on inactivating mutations in the Phosphate-regulating gene with Homologies to Endopeptidases on the X‑chromosome (PHEX) gene, which leads to an increased synthesis and secretion of fibroblast growth factor 23 (FGF23). Read More

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http://dx.doi.org/10.1007/s00103-020-03171-2DOI Listing

Hypophosphataemia after ferric carboxymaltose is unrelated to symptoms, intestinal inflammation or vitamin D status.

BMC Gastroenterol 2020 Jun 10;20(1):183. Epub 2020 Jun 10.

Department of Gastroenterology, Northern Health, 185 Cooper St, Epping, Victoria, 3076, Australia.

Background: Intravenous iron replacement is recommended for iron-deficient patients with inflammatory bowel disease (IBD), but may be associated with hypophosphataemia, predisposing to osteomalacia and fractures. This study aimed to evaluate the incidence and risk factors for hypophosphataemia following intravenous ferric carboxymaltose (FCM) in patients with IBD.

Methods: This prospective observational study of patients with and without IBD evaluated serum phosphate for 28 days following intravenous FCM, and assessed associations with symptoms, markers of inflammation and vitamin D status. Read More

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http://dx.doi.org/10.1186/s12876-020-01298-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7288415PMC

Camidanlumab tesirine, an antibody-drug conjugate, in relapsed/refractory CD25-positive acute myeloid leukemia or acute lymphoblastic leukemia: A phase I study.

Leuk Res 2020 Aug 7;95:106385. Epub 2020 Jun 7.

Blood and Marrow Transplant Program at Northside Hospital, Atlanta, GA, USA.

There is a significant need for improved therapeutics in older patients with acute leukemia. Camidanlumab tesirine is an antibody-drug conjugate against CD25, an antigen expressed in several malignancies, including acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML). This open-label, dose-escalation and -expansion study (NCT02588092) assessed the safety, activity, pharmacokinetics (PK), and immunogenicity of camidanlumab tesirine in patients with relapsed/refractory ALL/AML. Read More

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http://dx.doi.org/10.1016/j.leukres.2020.106385DOI Listing

Ripretinib in patients with advanced gastrointestinal stromal tumours (INVICTUS): a double-blind, randomised, placebo-controlled, phase 3 trial.

Lancet Oncol 2020 07 5;21(7):923-934. Epub 2020 Jun 5.

Department of Hematology and Medical Oncology, Fox Chase Cancer Center, Philadelphia, PA, USA.

Background: Resistance to approved inhibitors of KIT proto-oncogene, receptor tyrosine kinase (KIT), and platelet-derived growth factor receptor α (PDGFRA) is a clinical challenge for patients with advanced gastrointestinal stromal tumours. We compared the efficacy and safety of ripretinib, a switch-control tyrosine kinase inhibitor active against a broad spectrum of KIT and PDGFRA mutations, with placebo in patients with previously treated, advanced gastrointestinal stromal tumours.

Methods: In this double-blind, randomised, placebo-controlled, phase 3 study, we enrolled adult patients in 29 specialised hospitals in 12 countries. Read More

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http://dx.doi.org/10.1016/S1470-2045(20)30168-6DOI Listing

New Therapies for Hypophosphatemia-Related to FGF23 Excess.

Calcif Tissue Int 2020 Jun 5. Epub 2020 Jun 5.

Department of Medicine Section of Endocrinology, Yale School of Medicine, PO Box 802080, New Haven, CT, 06520, USA.

FGF23 is a hormone produced by osteocytes in response to an elevation in the concentration of extracellular phosphate. Excess production of FGF23 by bone cells, or rarely by tumors, is the hormonal basis for several musculoskeletal syndromes characterized by hypophosphatemia due to renal phosphate wasting. FGF23-dependent chronic hypophosphatemia causes rickets and osteomalacia, as well as other skeletal complications. Read More

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http://dx.doi.org/10.1007/s00223-020-00705-3DOI Listing

Tumor-Induced Osteomalacia.

Calcif Tissue Int 2020 Jun 5. Epub 2020 Jun 5.

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

Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome caused by tumoral production of fibroblast growth factor 23 (FGF23). The hallmark biochemical features include hypophosphatemia due to renal phosphate wasting, inappropriately normal or frankly low 1,25-dihydroxy-vitamin D, and inappropriately normal or elevated FGF23. TIO is caused by typically small, slow growing, benign phosphaturic mesenchymal tumors (PMTs) that are located almost anywhere in the body from the skull to the feet, in soft tissue or bone. Read More

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http://dx.doi.org/10.1007/s00223-020-00691-6DOI Listing

Genetic ablation of osteopontin in osteomalacic Hyp mice partially rescues the deficient mineralization without correcting hypophosphatemia.

J Bone Miner Res 2020 Jun 5. Epub 2020 Jun 5.

Faculty of Dentistry, McGill University, Montreal, QC, Canada.

PHEX is predominantly expressed by bone- and tooth-forming cells, and its inactivating mutations in X-linked hypophosphatemia (XLH) lead to renal phosphate wasting and severe hypomineralization of bones and teeth. Also present in XLH are hallmark hypomineralized peri-osteocytic lesions (POLs, halos) that persist despite stable correction of serum phosphate (P ) that improves bulk bone mineralization. In XLH, mineralization-inhibiting osteopontin (OPN, a substrate for PHEX) accumulates in the extracellular matrix of bone. Read More

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http://dx.doi.org/10.1002/jbmr.4101DOI Listing

Osteopetrorickets Presenting with Failure to Thrive and Hypophosphatemia.

J Endocr Soc 2020 Jun 28;4(6):bvaa044. Epub 2020 Apr 28.

Division of Pediatric Endocrinology and Metabolism, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, Alabama.

Osteopetrosis is a rare group of bone disorders characterized by defective osteoclast bone resorption causing high bone mineral density. A high bone mineral density in combination with defective skeletal mineralization results in a phenotype of osteopetrorickets. We present a rare presentation of infantile osteopetrorickets in an 8-week-old female who presented with failure to thrive, hypophosphatemia, anemia, and thrombocytopenia. Read More

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http://dx.doi.org/10.1210/jendso/bvaa044DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7255499PMC

The Game Is Afoot.

N Engl J Med 2020 06;382(23):2249-2255

From the Departments of Medicine (N.Y., A.L.M., J.L.), Pathology (J.L.H.), and Orthopedic Surgery (C.P.C.), Brigham and Women's Hospital, Boston.

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http://dx.doi.org/10.1056/NEJMcps1913599DOI Listing

Lactic Acidosis in the Setting of Severe Hypophosphatemia After High-Dose Insulin Infusion.

J Cardiothorac Vasc Anesth 2020 Apr 29. Epub 2020 Apr 29.

Department of Anesthesiology & Pain Medicine, University of Washington Medical Center, Seattle, WA.

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http://dx.doi.org/10.1053/j.jvca.2020.04.029DOI Listing

A Phase II Trial of Cabozantinib in Hormone Receptor-Positive Breast Cancer with Bone Metastases.

Oncologist 2020 May 28. Epub 2020 May 28.

Massachusetts General Hospital Cancer Center, Boston, Massachusetts, USA.

Background: We assessed the antitumor activity of cabozantinib, a potent multireceptor oral tyrosine kinase inhibitor, in patients with hormone receptor-positive breast cancer with bone metastases.

Patients And Methods: In this single-arm multicenter phase II study, patients received an initial starting dose of 100 mg, later reduced to 60 mg, per day. The primary endpoint was the bone scan response rate. Read More

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http://dx.doi.org/10.1634/theoncologist.2020-0127DOI Listing

Neonatal Refeeding Syndrome and Clinical Outcome in Extremely Low-Birth-Weight Babies: Secondary Cohort Analysis From the ProVIDe Trial.

JPEN J Parenter Enteral Nutr 2020 May 26. Epub 2020 May 26.

Liggins Institute, University of Auckland, Auckland, New Zealand.

Background: Refeeding syndrome (RS) following preterm birth has been linked to high intravenous (IV) protein intake in the presence of low electrolyte supply. In extremely low-birth-weight (ELBW) babies, we aimed to determine the incidence of RS and associations with birth characteristics and clinical outcomes.

Method: Prospective cohort study of ELBW ProVIDe Trial participants in 6 New Zealand neonatal intensive care units. Read More

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http://dx.doi.org/10.1002/jpen.1934DOI Listing

Lessons learnt from delayed diagnosis of FGF-23-producing tumour-induced osteomalacia and post-operative hungry bone syndrome.

Authors:
S Kumar T Diamond

Bone Rep 2020 Jun 6;12:100276. Epub 2020 May 6.

St George Public Hospital, Kogarah, NSW 2217, Australia.

Tumour-induced osteomalacia (TIO) is a rare paraneoplastic syndrome caused by a fibroblast growth-factor-23 (FGF-23)-secreting phosphaturic mesenchymal tumour (PMT) and is characterised by hypophosphataemic osteomalacia. We present a 36-year-old man initially presenting with diffuse bone and joint pain who was inappropriately treated for presumed ankylosing spondylitis for 2 years. Whole-body bone scan suggested metabolic bone disease, prompting referral to our endocrine institution. Read More

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http://dx.doi.org/10.1016/j.bonr.2020.100276DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7235936PMC

Biological stenciling of mineralization in the skeleton: Local enzymatic removal of inhibitors in the extracellular matrix.

Bone 2020 May 23;138:115447. Epub 2020 May 23.

Faculty of Dentistry, McGill University, 3640 University St., Montreal, Quebec H3A 0C7, Canada; Department of Anatomy and Cell Biology, McGill University, 3640 University St., Montreal, Quebec H3A 0C7, Canada. Electronic address:

Biomineralization is remarkably diverse and provides myriad functions across many organismal systems. Biomineralization processes typically produce hardened, hierarchically organized structures usually having nanostructured mineral assemblies that are formed through inorganic-organic (usually protein) interactions. Calcium‑carbonate biomineral predominates in structures of small invertebrate organisms abundant in marine environments, particularly in shells (remarkably it is also found in the inner ear otoconia of vertebrates), whereas calcium-phosphate biomineral predominates in the skeletons and dentitions of both marine and terrestrial vertebrates, including humans. Read More

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http://dx.doi.org/10.1016/j.bone.2020.115447DOI Listing

Surgical Treatment for Severe Fixed Hyperkyphosis in an Adult Patient Suffering From Ankylosing Spondylitis and Hereditary Hypophosphatemia With Vertebral Osteopetrosis.

Clin Spine Surg 2020 May 22. Epub 2020 May 22.

Department of Orthopedics and Trauma-Surgery, Krankenhaus Nord-Klinik Floridsdorf, Vienna.

Purpose: Ankylosing spondylitis and hereditary hypophosphatemia with long-term high dose supplementation of phosphorous and calcitriol can both lead to severe structural abnormalities of the vertebrae. Impairment of spinal mobility and spinal deformity may ultimately necessitate surgical treatment. A severe fixed hyperkyphosis in a patient with ankylosing spondylitis is a surgically demanding condition, therefore, the indication for surgical treatment should be thoroughly considered and chosen individually. Read More

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http://dx.doi.org/10.1097/BSD.0000000000001011DOI Listing

Alternative causes of ankle pain in a patient with enthesopathy and X-linked hypophosphataemia.

Lancet 2020 05;395(10237):e97

Department of Rheumatology, Princess Alexandra Hospital, Brisbane, QLD, Australia; Faculty of Medicine, University of Queensland, Brisbane, QLD, Australia.

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http://dx.doi.org/10.1016/S0140-6736(20)30935-1DOI Listing

Intravenous iron supplementation therapy.

Mol Aspects Med 2020 May 19:100862. Epub 2020 May 19.

Medical University of Innsbruck, Department of Medicine I, Gastroenterology, Hepatology and Endocrinology, Anichstrasse 35, A-6020, Innsbruck, Austria. Electronic address:

Intravenous infusions of iron have evolved from a poorly effective and dangerous intervention to a safe cornerstone in the treatment of iron deficiency. Modern iron formulations are composite nanoparticles composed of carbohydrate ferric oxy-hydroxides. Iron dextran, iron derisomaltose (formely known as iron isomaltoside 1000), ferric carboxymaltose, ferrumoxytol, iron sucrose and sodium ferric gluconate can be infused at different doses and allow correction of total iron deficit with single or repeated doses in 1-2 weeks depending on the specific formulation. Read More

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http://dx.doi.org/10.1016/j.mam.2020.100862DOI Listing

Rare musculoskeletal diseases in adults: a research priority setting partnership with the James Lind Alliance.

Orphanet J Rare Dis 2020 May 19;15(1):117. Epub 2020 May 19.

The Botnar Research Centre, Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, OX3 7LD, UK.

Background: Osteogenesis imperfecta, fibrous dysplasia/McCune-Albright syndrome and X-linked hypophosphatemia are three rare musculoskeletal diseases characterised by bone deformities, frequent fractures and pain. Little high-quality research exists on appropriate treatment and long-term management of these conditions in adults. This is further worsened by limited research funding in rare diseases and a general mismatch between the existing research priorities and those of the patients. Read More

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http://dx.doi.org/10.1186/s13023-020-01398-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7238497PMC

Fibroblast growth factor receptor as a potential candidate for phosphate sensing.

Curr Opin Nephrol Hypertens 2020 Jul;29(4):446-452

Department of Molecular Endocrinology, Fujii Memorial Institute of Medical Sciences, Institute of Advanced Medical Sciences, Tokushima University, Tokushima, Japan.

Purpose Of Review: Phosphate plays essential roles in many biological processes. Serum phosphate level needs to be regulated because hypophosphatemia and hyperphosphatemia cause rickets/osteomalacia and ectopic calcification, respectively. Fibroblast growth factor (FGF) 23 is the principal hormone to regulate serum phosphate level. Read More

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http://dx.doi.org/10.1097/MNH.0000000000000618DOI Listing