5,901 results match your criteria Hypophosphatemia


What are the appropriate reference limits for the diagnosis of hypophosphataemia in paediatric patients?

J Clin Pathol 2019 Apr 17. Epub 2019 Apr 17.

Pathology and Laboratory Medicine, KK Women's and Children's Hospital, Singapore, Singapore

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http://dx.doi.org/10.1136/jclinpath-2019-205736DOI Listing

Association of admission serum glucose-phosphate ratio with severity and prognosis of aneurysmal subarachnoid hemorrhage.

World Neurosurg 2019 Apr 14. Epub 2019 Apr 14.

Department of Neurosurgery, The Affiliated Drum Tower Hospital, School of Medicine, Nanjing University, Zhongshan Road 321, Nanjing, 210008 Jiangsu Province PR China. Electronic address:

Background: and Purpose Serum hyperglycemia and hypophosphatemia were reported to be common in patients with aneurysmal subarachnoid hemorrhage (aSAH). This study was aimed to explore whether admission serum glucose-phosphate ratio was associated with the severity and prognosis of aSAH.

Methods: We retrospectively analyzed 198 patients with aSAH who were admitted within 24 hours of hemorrhage occurrence to a single academic hospital from June 2016 to September 2017. Read More

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http://dx.doi.org/10.1016/j.wneu.2019.04.071DOI Listing
April 2019
1 Read

MAPK inhibition and growth hormone: a promising therapy in XLH.

FASEB J 2019 Apr 11:fj201802007R. Epub 2019 Apr 11.

Division of Pediatrics, Department of Medicine, Faculty of Medicine and Health Sciences, University of Oviedo, Oviedo, Spain.

X-linked hypophosphatemia (XLH) leads to growth retardation and bone deformities, which are not fully avoided by conventional treatment with phosphate and vitamin D analogs. Pediatric patients have been treated with growth hormone (GH), and recent findings suggest that blocking fibroblast growth factor 23 actions may be the most effective therapy, but its effects on growth are not known. We here report the effect of MAPK inhibition alone or associated with GH on growth and growth plate and bone structure of young Hyp (the XLH animal model) mice. Read More

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https://www.fasebj.org/doi/10.1096/fj.201802007R
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http://dx.doi.org/10.1096/fj.201802007RDOI Listing
April 2019
2 Reads

Refeeding Syndrome as a Possible Cause of Very Early Mortality in Acute Pancreatitis.

Gut Liver 2019 Feb 27. Epub 2019 Feb 27.

Department of Internal Medicine, Gil Medical Center, Gachon University College of Medicine, Incheon, Korea.

Background/aims: : The incidence and cause of early mortality in acute pancreatitis (AP) arenot fully understood. Refeeding syndrome (RFS) is a fatal clinical complication that can occuras a result of fluid and electrolyte shifts during early nutritional rehabilitation for malnourishedpatients. This study was conducted to determine the clinical implications of RFS in patientswith AP. Read More

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http://dx.doi.org/10.5009/gnl18458DOI Listing
February 2019
3 Reads

Safety of Oral and Intravenous Iron.

Acta Haematol 2019 Apr 10;142(1):8-12. Epub 2019 Apr 10.

Division of Hematology/Medical Oncology, Department of Pathology and Pediatrics, Oregon Health and Science University, Portland, Oregon, USA,

As the adverse effects of iron deficiency are better recognized, the use of oral and intravenous iron has increased dramatically. Oral iron is often poorly tolerated, with up to 70% or more of patients noting gastrointestinal issues; this may affect adherence to therapy. In addition, many patients will not respond to oral iron due to their underlying illness. Read More

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http://dx.doi.org/10.1159/000496966DOI Listing
April 2019
1 Read

A phase 1b study evaluating the safety and pharmacokinetics of regorafenib in combination with cetuximab in patients with advanced solid tumors.

Int J Cancer 2019 Apr 8. Epub 2019 Apr 8.

University of Southern California/Norris Comprehensive Center, Los Angeles, CA.

Regorafenib 160 mg orally once daily (QD) 3 weeks on/1 week off is approved in colorectal cancer, gastrointestinal stromal tumors, and hepatocellular carcinoma. We established the safety and pharmacokinetics (PK) of regorafenib combined with cetuximab in advanced refractory solid tumors. This was a phase 1, open-label, dose-escalation study (NCT01973868) in patients with advanced/metastatic solid tumors who progressed after standard therapy. Read More

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http://dx.doi.org/10.1002/ijc.32317DOI Listing
April 2019
3 Reads

Infantile hypercalcemia with novel compound heterozygous mutation in SLC34A1 encoding renal sodium-phosphate cotransporter 2a: a case report.

Ann Pediatr Endocrinol Metab 2019 Mar 31;24(1):64-67. Epub 2019 Mar 31.

Department of Pediatrics, Keimyung University Dongsan Medical Center, Keimyung University School of Medicine, Daegu, Korea.

Idiopathic infantile hypercalcemia is characterized by hypercalcemia, dehydration, vomiting, and failure to thrive, and it is due to mutations in 24-hydroxylase (CYP24A1). Recently, mutations in sodium-phosphate cotransporter (SLC34A1) expressed in the kidney were discovered as an additional cause of idiopathic infantile hypercalcemia. This report describes a female infant admitted for evaluation of nephrocalcinosis. Read More

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http://dx.doi.org/10.6065/apem.2019.24.1.64DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6449619PMC
March 2019
2 Reads

Doughnut Shaped Parathyroid Adenoma

Mol Imaging Radionucl Ther 2019 Mar;28(1):41-43

University of Health Sciences, Dışkapı Yıldırım Beyazıt Training and Research Hospital, Department of Pathology, Ankara, Turkey

A 52-year-old woman presented with a complaint of neck swelling. The patient showed signs of hyperparathyroidism: hypercalcemia, and hypophosphatemia. Tc-99m MIBI dual-phase parathyroid scintigraphy and SPECT revealed increased activity in a regular-bordered, “doughnut”-shaped mass on the left side of the thyroid gland with a central hypoactive area. Read More

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http://dx.doi.org/10.4274/mirt.galenos.2018.06977DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6455100PMC
March 2019
8 Reads

Ventricular bigeminy and trigeminy caused by hypophosphataemia during diabetic ketoacidosis treatment: a case report.

Ital J Pediatr 2019 Apr 2;45(1):42. Epub 2019 Apr 2.

Department of Pediatric Endocrinology and Diabetology, Medical University of Lublin, Lublin, Poland.

Background: Hypophosphatemia has many causes, and is often encountered during DKA (Diabetic Ketoacidosis) treatment. However, it rarely requires clinical intervention.

Case Presentation: Ventricular arrhythmia was observed in a 10-year-old girl with newly diagnosed type 1 diabetes mellitus and hypophosphatemia while undergoing treatment for ketoacidosis. Read More

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http://dx.doi.org/10.1186/s13052-019-0633-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6444668PMC
April 2019
3 Reads

[Tumor-induced osteomalacia caused by an FGF23-secreting myopericytoma : Case report and literature review].

Orthopade 2019 Apr 1. Epub 2019 Apr 1.

Klinik für Orthopädie und Unfallchirurgie, UKSH Campus Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland.

Background: The symptoms of muscle weakness, bone pain and fragility fractures can be an indication of osteomalacia. Phosphate is often not considered within osteologic parameters, decreased levels are therefore easily overseen. The additional test for fibroblast growth factor 23 (FGF23) as indicator for tumor-induced osteomalacia (TIO) is still largely unfamiliar. Read More

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http://dx.doi.org/10.1007/s00132-019-03719-4DOI Listing
April 2019
2 Reads

Partial Fanconi Syndrome Induced by Ifosfamide.

Cureus 2019 Jan 23;11(1):e3947. Epub 2019 Jan 23.

Internal Medicine, Kettering Medical Center, Dayton, USA.

Several commonly used chemotherapeutic agents, antibiotics, antivirals, and antiepileptic medications can cause partial or full Fanconi syndrome, disorders which can generally be described as transport defects in the proximal renal tubule, associated with non-anion gap metabolic acidosis. Fanconi syndrome is underreported and therefore often missed in the clinical setting. Herein, we present a case report that details the course of a 64-year-old female with a history of stage IV undifferentiated pleomorphic sarcoma who after her sixth chemotherapeutic cycle (adriamycin, ifosfamide, and mesna) developed severe hypokalemia, hypophosphatemia, and proteinuria without glycosuria, eventually diagnosed with partial Fanconi syndrome. Read More

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http://dx.doi.org/10.7759/cureus.3947DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6433442PMC
January 2019
1 Read

Assessment of nephrotoxicity associated with combined cisplatin and mitomycin C usage in laparoscopic hyperthermic intraperitoneal chemotherapy.

Int J Hyperthermia 2019 Apr 1:1-6. Epub 2019 Apr 1.

b Department of Surgical Oncology , The University of Texas MD Anderson Cancer Center , Houston , TX , USA.

Background: Laparoscopic hyperthermic intraperitoneal chemotherapy (HIPEC) has been used to treat various peritoneal malignancies. Cisplatin and mitomycin C (MMC) are agents commonly used in these procedures and, individually, each has been associated with acute kidney injury (AKI). There is limited literature on the complications associated with the use of both agents in HIPEC. Read More

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http://dx.doi.org/10.1080/02656736.2019.1597175DOI Listing
April 2019
2 Reads

Giant Cell Tumor of Tendon Sheath Revealed on 68Ga-DOTA-TATE PET/CT in a Patient With Suspicious Tumor-Induced Osteomalacia.

Clin Nucl Med 2019 Mar 29. Epub 2019 Mar 29.

Department of Pathology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.

Giant cell tumor of tendon sheath (GCTTS) is benign mesenchymal neoplasm, and it rarely induces hypophosphatemia. We report a 49-year-old woman with suspicious tumor-induced osteomalacia underwent Ga-DOTA-TATE PET/CT to identify potential causative tumor, which showed a hypermetabolic soft tissue nodule in the right foot. Pathology confirmed it as GCTTS following surgical resection. Read More

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http://dx.doi.org/10.1097/RLU.0000000000002550DOI Listing
March 2019
2 Reads

Dual Tracer 99mTc-Pertechnetate/99mTc-MIBI Dual-Time-Point SPECT/CT Parathyroid Gland Assessment Regarding to Parathyroid Gland Size and Biochemical Parameters - Two Years Single Imaging Centre Experience.

Acta Medica (Hradec Kralove) 2019 ;62(1):1-5

Nuclear Medicine Department, University Hospital Hradec Králové, Czech Republic.

Introduction: Preoperative parathyroid imaging is inevitable part of focused parathyroid surgery. The aim of our study was assessment of parathyroid scintigraphy diagnostic accuracy regarding to size and metabolic parameters of hyperfunctioning parathyroid tissue.

Material And Methods: Parathyroid scintigraphy for suspected primary hyperparathyroidism was performed in 95 patients during years 2015 and 2016. Read More

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https://actamedica.lfhk.cuni.cz/62/1/0001/
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http://dx.doi.org/10.14712/18059694.2019.38DOI Listing
January 2019
6 Reads

[Adefovir Dipivoxil-induced Fanconi's Syndrome and Osteomalacia Following Multiple Bone Fractures in a Patient with Chronic Hepatitis B].

Yakugaku Zasshi 2019 ;139(4):641-645

Department of Pharmacy, Okayama University Hospital.

We herein present the case of a 66-year-old Japanese man with Fanconi's syndrome. He had been receiving adefovir dipivoxil (ADV) for the treatment of entecavir (ETV)-resistant chronic hepatitis B (CHB) for four years in his 8-year treatment of hepatocellular carcinoma (HCC), but was referred to our hospital after increased levels of bone pain in his ribs, knees, and ankles. Renal dysfunction, hypophosphatemia, and increased levels of bone alkaline phosphatase were found by a hematology test after admission for treatment of HCC. Read More

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http://dx.doi.org/10.1248/yakushi.18-00170DOI Listing
April 2019
3 Reads

Diagnosis, treatment-monitoring and follow-up of children and adolescents with X-linked hypophosphatemia (XLH).

Metabolism 2019 Mar 27. Epub 2019 Mar 27.

APHP, Endocrinology and Diabetology for Children, Bicêtre Paris Sud Hospital, Le Kremlin-Bicêtre, France; APHP, Reference Center for Rare Disorders of Calcium and Phosphate Metabolism, filière OSCAR, Paris, France; APHP, Platform of Expertise for Rare Disorders Paris-Sud, Bicêtre Paris Sud Hospital, Le Kremlin-Bicêtre, France.

Early diagnosis, optimal therapeutic management and regular follow up of children with X-linked hypophosphatemia (XLH) determine their long term outcomes and future quality of life. Biochemical screening of potentially affected newborns in familial cases and improving physician's knowledge on clinical signs, symptoms and biochemical characteristics of XLH for de novo cases should lead to earlier diagnosis and treatment initiation. The follow-up of children with XLH includes clinical, biochemical and radiological monitoring of treatment (efficacy and complications) and screening for XLH-related dental, neurosurgical, rheumatological, cardiovascular, renal and ENT complications. Read More

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http://dx.doi.org/10.1016/j.metabol.2019.03.009DOI Listing
March 2019
2 Reads

Identification of a p.Trp403* nonsense variant in PHEX causing X-linked hypophosphatemia by inhibiting p38 MAPK signaling.

Hum Mutat 2019 Mar 28. Epub 2019 Mar 28.

Key Laboratory of Molecular Biophysics of the Ministry of Education, College of Life Science and Technology and Center for Human Genome Research, Huazhong University of Science and Technology, Wuhan, Hubei, China.

X-linked hypophosphatemia (XLH) is the most common hereditary rickets, caused by mutations in PHEX encoding the phosphate regulating endopeptidase homolog X-linked. Here, we report a nonsense variant in exon 11 of PHEX (c.1209G>A p. Read More

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http://dx.doi.org/10.1002/humu.23743DOI Listing
March 2019
1 Read
5.144 Impact Factor

Serum PTH reference values in an adult Brazilian population: implications for the diagnosis of hyperparathyroidism.

Arch Endocrinol Metab 2019 Mar 21. Epub 2019 Mar 21.

Santa Casa de Belo Horizonte, Belo Horizonte, MG, Brasil.

Objective: To define serum parathyroid hormone (PTH) reference values in carefully selected subjects following the recommended pre-analytical guidelines.

Subjects And Methods: First, 676 adults who would be submitted to thyroidectomy were evaluated. Patients using interfering medications or with malabsorption syndrome, hypomagnesemia, hyper- or hypophosphatemia, hypo- or hypercalcemia, 25-hydroxyvitamin D < 30 ng/dL, estimated glomerular filtration rate < 60 mL/min/1. Read More

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http://dx.doi.org/10.20945/2359-3997000000117DOI Listing
March 2019
1 Read

Phosphate supplementation for hypophosphatemia during continuous renal replacement therapy in adults.

Ren Fail 2019 Nov;41(1):72-79

a Dialysis Center , Konkuk University Medical Center , Seoul , Korea.

Background: Hypophosphatemia is common during continuous renal replacement therapy (CRRT) in critically ill patients and can cause generalized muscle weakness, prolonged respiratory failure, and myocardial dysfunction. This study aimed to investigate the efficacy and safety of adding phosphate to the dialysate and replacement solutions to treat hypophosphatemia occurring in intensive CRRT in critically ill patients.

Methods: We retrospectively analyzed 73 patients treated with intensive CRRT (effluent flow ≥35 ml/kg/hr) in the intensive care unit. Read More

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http://dx.doi.org/10.1080/0886022X.2018.1561374DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6442196PMC
November 2019
1 Read

Oral symptoms and oral health-related quality of life of individuals with x-linked hypophosphatemia.

Head Face Med 2019 Mar 23;15(1). Epub 2019 Mar 23.

Department of Cranio-Maxillofacial Surgery, Research Unit Rare Diseases with Orofacial Manifestations (RDOM), University Hospital Münster, Albert-Schweitzer-Campus 1, Gebäude W 30, 48149, Münster, Germany.

Background: The primary purpose of this study was to collect data on the oral health-related quality of life (OHRQoL) of individuals with x-linked hypophosphatemia (XLH). It was also designed to gather information on the period of diagnosis, oral symptoms, orthodontic therapy, and satisfaction with dental care and the healthcare system.

Methods: A questionnaire was developed to evaluate the OHRQoL consisting of open-ended questions and the standardised German version of the Oral Health Impact Profile-14 (OHIP-14). Read More

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http://dx.doi.org/10.1186/s13005-019-0192-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6431058PMC
March 2019
1 Read

Varied presentation of sinonasal phosphaturic mesenchymal tumour: report of a case series with follow-up.

Eur Arch Otorhinolaryngol 2019 Mar 16. Epub 2019 Mar 16.

Department of Pathology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.

Purpose: Phosphaturic mesenchymal tumour (PMT) of the paranasal sinuses is a rare tumour that is associated with oncogenous osteomalacia causing predominant musculoskeletal symptoms. We present a series of eight patients diagnosed to have PMT of the paranasal sinuses with varied presentation and highlight the appropriate evaluation required to arrive at the diagnosis.

Methods: Retrospective review of eight patients diagnosed to have PMT-causing tumour-induced osteomalacia with follow-up data. Read More

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http://dx.doi.org/10.1007/s00405-019-05341-8DOI Listing
March 2019
1 Read

Treating osteoporosis: a near miss in an unusual case of FGF-23 mediated bone loss.

BMJ Case Rep 2019 Mar 16;12(3). Epub 2019 Mar 16.

Christian Medical College, Vellore, Tamil Nadu, India.

Oncogenic osteomalacia is a potentially curable condition caused by phosphaturic mesenchymal tumours. We present the case of 54-year-old woman who presented with bony pains and muscle weakness, and was erroneously treated with bisphosphonates elsewhere on the basis of Dual Energy X-ray Absorptiometry (bone density) scan that showed 'severe osteoporosis'. Further evaluation revealed the presence of hypophosphatemia, with a 1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid avid lesion involving left mandible on PET scan. Read More

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http://dx.doi.org/10.1136/bcr-2018-228375DOI Listing
March 2019
1 Read

An integrated analysis of safety and tolerability of etelcalcetide in patients receiving hemodialysis with secondary hyperparathyroidism.

PLoS One 2019 15;14(3):e0213774. Epub 2019 Mar 15.

Amgen Inc., Thousand Oaks, California, United States of America.

Background: Calcimimetics have been shown to be effective and safe therapies for the treatment of secondary hyperparathyroidism (sHPT), a serious complication of disordered mineral metabolism associated with dialysis-dependent chronic kidney disease. Etelcalcetide, a recently approved intravenous calcimimetic, reduces serum parathyroid hormone (PTH), calcium, phosphorus, and fibroblast growth factor-23 concentrations. Here we report the first integrated safety profile of etelcalcetide using pooled data from five pivotal clinical trials. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0213774PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6420005PMC
March 2019
12 Reads
3.234 Impact Factor

Tumor-induced osteomalacia with the culprit lesion located in the palm: a case report.

J Int Med Res 2019 Mar 12:300060519833476. Epub 2019 Mar 12.

1 Department of Endocrinology, The Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, People's Republic of China, Wenzhou, China.

Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome. We herein report a rare case of TIO in a 58-year-old Chinese man who presented with a large lump in the right palm. Clinical, biochemical, and radiological assessments were performed. Read More

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http://dx.doi.org/10.1177/0300060519833476DOI Listing
March 2019
4 Reads

Tumor-induced hypophosphatemic osteomalacia caused by a mesenchymal tumor of the mandible managed by a segmental mandibulectomy and microvascular reconstruction with a free fibula flap.

Head Neck 2019 Mar 11. Epub 2019 Mar 11.

Department of Endocrine Neoplasia and Hormonal Disorders, Division of Internal Medicine, The University of Texas MD Anderson Cancer Center, Houston, Texas.

Background: Tumor-induced osteomalacia is a rare paraneoplastic syndrome in which patients develop hypophosphatemia and osteomalacia.

Methods And Results: Here, we report a unique case of a 42-year-old man who presented to our institution with a 1-year history of pain in his ribs, hips, lower back, and feet. Radiologic examination revealed a decrease in bone density and multiple insufficiency fractures. Read More

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http://dx.doi.org/10.1002/hed.25657DOI Listing
March 2019
3 Reads

Early and late adverse renal effects after potentially nephrotoxic treatment for childhood cancer.

Cochrane Database Syst Rev 2019 03 11;3:CD008944. Epub 2019 Mar 11.

Department of Pediatrics, Division of Oncology/Hematology, Amsterdam UMC, Vrije Universiteit Amsterdam, De Boelelaan 1117, Amsterdam, Netherlands, 1081 HV.

Background: Improvements in diagnostics and treatment for paediatric malignancies resulted in a major increase in survival. However, childhood cancer survivors (CCS) are at risk of developing adverse effects caused by multimodal treatment for their malignancy. Nephrotoxicity is a known side effect of several treatments, including cisplatin, carboplatin, ifosfamide, radiotherapy and nephrectomy, and can cause glomerular filtration rate (GFR) impairment, proteinuria, tubulopathy, and hypertension. Read More

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http://dx.doi.org/10.1002/14651858.CD008944.pub3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6410614PMC

C-Terminal Fibroblast Growth Factor-23 Levels in Non-Nutritional Hypophosphatemic Rickets.

Indian J Pediatr 2019 Mar 5. Epub 2019 Mar 5.

Division of Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India.

Fibroblast growth factor-23 (FGF23) is central to phosphate homeostasis. The author examined if blood levels of FGF23 allow discrimination of classic hypophosphatemic rickets from other causes of non-nutritional rickets with hypophosphatemia. Forty-two children (median age: 102 mo) with non-nutritional rickets and hypophosphatemia were clinically classified as having distal renal tubular acidosis (RTA, n = 12), Fanconi syndrome (n = 8), classic hypophosphatemic rickets (n = 11), vitamin D dependent rickets (n = 7) and Dent disease (n = 4). Read More

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http://dx.doi.org/10.1007/s12098-019-02909-4DOI Listing
March 2019
16 Reads
0.919 Impact Factor

First-in-Asian Phase I Study of the Anti-Fibroblast Growth Factor 23 Monoclonal Antibody, Burosumab: Safety and Pharmacodynamics in Adults With X-linked Hypophosphatemia.

JBMR Plus 2019 Feb 14;3(2):e10074. Epub 2018 Sep 14.

Department of Pediatrics Osaka Hospital Japan Community Healthcare Organization (JCHO) Osaka Japan.

X-linked hypophosphatemia (XLH) is a disease caused by abnormally elevated FGF23 levels, which cause persistent hypophosphatemia accompanied by subsequent reduction in bone mineralization that presents as rickets or osteomalacia. Burosumab is a fully human monoclonal antibody targeting FGF23 that is under development for the treatment of FGF23-related hypophosphatemia including XLH. The safety, tolerability, and proof of concept of burosumab have been evaluated in patients with XLH in previous studies conducted in countries outside of Asia. Read More

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http://doi.wiley.com/10.1002/jbm4.10074
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http://dx.doi.org/10.1002/jbm4.10074DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6383703PMC
February 2019
7 Reads

Phosphorus content of muscle tissue and muscle function in dairy cows fed a phosphorus-deficient diet during the transition period.

J Dairy Sci 2019 May 1;102(5):4072-4093. Epub 2019 Mar 1.

Department of Equine Sciences, Utrecht University, 3584 CM Utrecht, the Netherlands.

Phosphorus (P) deficiency and hypophosphatemia are believed to be associated with muscle function disturbances in dairy cows, particularly around parturition. The objective of this study was to determine the effect of dietary P deprivation during late gestation and early lactation on muscle P homeostasis and muscle function in periparturient dairy cows. Thirty-six multiparous dairy cows in late gestation were randomly assigned either to undergo dietary P depletion or to be offered a diet with adequate P content from 4 wk before to 4 wk after parturition. Read More

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http://dx.doi.org/10.3168/jds.2018-15727DOI Listing
May 2019
2 Reads

Phase 1 Open-Label, Multicenter Study of First-in-Class RORγ Agonist LYC-55716: Safety, Tolerability, and Preliminary Evidence of Antitumor Activity.

Clin Cancer Res 2019 Feb 28. Epub 2019 Feb 28.

Clinical Development, Lycera Corp.

Purpose: Transcription factor retinoic acid receptor-related orphan receptor g (RORg) regulates type 17 effector T cell differentiation and function and is key to immune cell regulation. Synthetic RORg agonists modulate immune cell gene expression to increase effector T cell activity and decrease immune suppression. A Phase 1 study evaluated the safety and tolerability of LYC-55716, a first-in-class, oral, small-molecule RORg agonist in adults with relapsed/refractory metastatic cancer. Read More

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http://dx.doi.org/10.1158/1078-0432.CCR-18-3185DOI Listing
February 2019
2 Reads

Hypophosphatemia before endoscopic gastrostomy predicts higher mortality during the first week and first month post-gastrostomy: a risk marker of refeeding syndrome in gastrostomy-fed patients.

Nutr Hosp 2019 Apr;36(2):247-252

Gastroenterology Department. GENE - Artificial Feeding Team. Hospital Garcia de Orta.

Introduction: Introduction: starvation is usual in patients referred for endoscopic gastrostomy (PEG). A high risk of refeeding syndrome (RS) may contribute to poor prognosis. Objectives: this study aims to: a) evaluate serum phosphorus and magnesium when patients underwent PEG; b) determine the mortality rate during the first week and first month of enteral nutrition; and c) assess if hypophosphatemia or hypomagnesemia are associated with early mortality. Read More

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http://dx.doi.org/10.20960/nh.2251DOI Listing
April 2019
1 Read

FGF23 and its role in X-linked hypophosphatemia-related morbidity.

Orphanet J Rare Dis 2019 Feb 26;14(1):58. Epub 2019 Feb 26.

Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.

Background: X-linked hypophosphatemia (XLH) is an inherited disease of phosphate metabolism in which inactivating mutations of the Phosphate Regulating Endopeptidase Homolog, X-Linked (PHEX) gene lead to local and systemic effects including impaired growth, rickets, osteomalacia, bone abnormalities, bone pain, spontaneous dental abscesses, hearing difficulties, enthesopathy, osteoarthritis, and muscular dysfunction. Patients with XLH present with elevated levels of fibroblast growth factor 23 (FGF23), which is thought to mediate many of the aforementioned manifestations of the disease. Elevated FGF23 has also been observed in many other diseases of hypophosphatemia, and a range of animal models have been developed to study these diseases, yet the role of FGF23 in the pathophysiology of XLH is incompletely understood. Read More

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http://dx.doi.org/10.1186/s13023-019-1014-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6390548PMC
February 2019
1 Read

Evaluating pathogenicity of SLC34A3-Ser192Leu, a frequent European missense variant in disorders of renal phosphate wasting.

Urolithiasis 2019 Feb 23. Epub 2019 Feb 23.

Division of Nephrology, Department of Internal Medicine, University Hospital Leipzig, Liebigstr. 20, 04103, Leipzig, Germany.

Loss-of-function mutations of SLC34A3 represent an established cause of a distinct renal phosphate wasting disorder termed hereditary hypophosphatemic rickets with hypercalciuria (HHRH). SLC34A3 encodes the renal phosphate transporter NaPi2c expressed at the apical brush border of proximal renal tubules. Substitution of p. Read More

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http://dx.doi.org/10.1007/s00240-019-01116-2DOI Listing
February 2019
1 Read

Reliability of calcium-phosphorus (Ca/P) ratio as a new, accurate and inexpensive tool in the diagnosis of some Ca-P disorders.

J Endocrinol Invest 2019 Feb 22. Epub 2019 Feb 22.

Unit of Endocrinology, Department of Medical Specialties, Azienda Ospedaliero-Universitaria of Modena, Ospedale Civile di Baggiovara, Modena, Italy.

Purpose: The serum calcium/phosphorus (Ca/P) ratio is an accurate tool to differentiate patients with primary hyperparathyroidism (PHPT) from healthy subjects. However, other disorders of the Ca-P metabolism might impair the Ca/P ratio, such as hypophosphatemia (HypoP) not PHPT related. The aim of this study is to examine the diagnostic value of Ca/P ratio in the diagnosis of PHPT and HypoP not PHPT related. Read More

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http://dx.doi.org/10.1007/s40618-019-01025-6DOI Listing
February 2019
1 Read

Addition of Zoledronate to Chemotherapy in Patients with Osteosarcoma Treated with Limb-Sparing Surgery: A Phase III Clinical Trial.

Med Sci Monit 2019 Feb 23;25:1429-1438. Epub 2019 Feb 23.

Department of Bone and Soft Tissue Tumor Surgery, Cancer Hospital of China Medical University, Liaoning Cancer Hospital and Institute, Shenyang, Liaoning, China (mainland).

BACKGROUND Zoledronate has anti-bone resorption activity and is reported to reduce skeletal-related events. The objective of this study was to test the hypothesis that addition of zoledronate in chemotherapy is safe and effective in osteosarcoma. MATERIAL AND METHODS A total of 798 patients, age 25 years and above, with newly diagnosed high-grade surgically salvageable malignant osteosarcoma, were included in the trial. Read More

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http://dx.doi.org/10.12659/MSM.913236DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6397617PMC
February 2019
1 Read

A genetic variant of CYP2R1 identified in a cat with type 1B vitamin D-dependent rickets: a case report.

BMC Vet Res 2019 Feb 18;15(1):62. Epub 2019 Feb 18.

Laboratory of Veterinary Internal Medicine, Department of Veterinary Clinical Medicine, School of Veterinary Medicine, Faculty of Veterinary Science, Nippon Veterinary and Life Science University, 1-7-1 Kyonan-cho, Musashino, Tokyo, 180-8602, Japan.

Background: Vitamin D-dependent rickets is rare in animals and humans. Several types of this condition are associated with genetic variants related to vitamin D metabolism. This is the first report of type 1B vitamin D-dependent rickets in a cat. Read More

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http://dx.doi.org/10.1186/s12917-019-1784-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6378717PMC
February 2019
1 Read

Tumor-induced osteomalacia.

Osteoporos Sarcopenia 2018 Dec 12;4(4):119-127. Epub 2018 Dec 12.

Department of Endocrinology, Key Laboratory of Endocrinology, The National Commission of Health, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China.

Tumor-induced osteomalacia (TIO), also known as oncogenic osteomalacia, is a rare paraneoplastic syndrome characterized by hypophosphatemia resulting from decreased tubular phosphate reabsorption, with a low or inappropriately normal level of active vitamin D. The culprit tumors of TIO could produce fibroblast growth factor 23 which plays a role in regulating renal Pi handling and 25-hydroxyvitamin D 1α-hydroxylase activity. Chronic hypophosphatemia could eventually lead to inadequate bone mineralization, presenting as osteomalacia. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S24055255183009
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http://dx.doi.org/10.1016/j.afos.2018.12.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6372818PMC
December 2018
5 Reads

Rickets severity predicts clinical outcomes in children with X-linked hypophosphatemia: Utility of the radiographic Rickets Severity Score.

Bone 2019 May 14;122:76-81. Epub 2019 Feb 14.

Yale University School of Medicine, New Haven, CT, USA.

The Rickets Severity Score (RSS) was used to evaluate X-linked hypophosphatemic rickets (XLH), a genetic disorder mediated by increased circulating FGF23. The reliability of the RSS was assessed using data from a randomized, phase 2 clinical trial that evaluated the effects of burosumab, a fully human anti-FGF23 monoclonal antibody, in 52 children with XLH ages 5 to 12 years. Bilateral knee and wrist radiographs were obtained at baseline, week 40, and week 64. Read More

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http://dx.doi.org/10.1016/j.bone.2019.02.010DOI Listing

Hypocalcemia and hypophosphatemia after treatment with zoledronic acid in a patient with AL amyloidosis.

Intern Emerg Med 2019 Apr 15;14(3):447-449. Epub 2019 Feb 15.

Regional Reference Centre for Coagulation Disorders, Department of Clinical Medicine and Surgery, "Federico II" University of Naples, Via S. Pansini, 5, 80131, Naples, Italy.

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http://dx.doi.org/10.1007/s11739-019-02048-wDOI Listing
April 2019
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Hypercalcaemic Crisis Due to Primary Hyperparathyroidism: Report of Two Cases.

J Crit Care Med (Targu Mures) 2019 Jan 4;5(1):34-39. Epub 2019 Feb 4.

University of Medicine, Pharmacy, Sciences and Technology of Târgu Mureş, Romania.

Introduction: A hypercalcaemic crisis, also called para thyrotoxicosis, hyper parathyroid crisis or parathyroid storm, is a complication of primary hyperparathyroidism (PHPT) and an endocrinology emergency that can have dramatic or even fatal consequences if it is not recognised and treated in time.

Case Presentation: Two cases presented in the emergency department with critical hypercalcaemic symptoms and severe elevation of serum calcium and parathyroid hormone levels, consistent with a hypercalcaemic crisis. The first case, a 16-year-old female patient, had imaging data that highlighted a single right inferior parathyroid adenoma and a targeted surgical approach was used. Read More

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http://dx.doi.org/10.2478/jccm-2019-0004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6369571PMC
January 2019
2 Reads

Fluconazole as a New Therapeutic Tool to Manage Patients With NPTIIc (SLC34A3) Mutation: A Case Report.

Am J Kidney Dis 2019 Feb 11. Epub 2019 Feb 11.

Centre de Référence des Maladies Rénales Rares, filière ORKID, Service de Néphrologie, Rhumatologie et Dermatologie Pédiatriques; Centre de Référence des Maladies Rares du Calcium et du Phosphate, filière OSCAR; Faculté de Médecine Lyon Est, Université Lyon 1, Lyon; INSERM 1033, Prévention des Maladies Osseuses, Lyon, France. Electronic address:

Mutations in the SLC34A3 gene, encoding the sodium/phosphate cotransporter 2C (NPTIIc), induce decreased renal phosphate reabsorption, hypophosphatemia, decreased fibroblast growth factor 23 and parathyroid hormone, and increased 1,25-dihydroxyvitamin D (1,25[OH]D) levels. The complete phenotype is characterized by hypophosphatemia, hypercalciuria, and nephrolithiasis/nephrocalcinosis, leading to chronic kidney disease and osteoporosis in adults. We report a 15-year-old boy referred for nephrocalcinosis. Read More

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http://dx.doi.org/10.1053/j.ajkd.2018.12.026DOI Listing
February 2019
1 Read

A Familial Case of a Whole Germline CDC73 Deletion Discordant for Primary Hyperparathyroidism.

Horm Res Paediatr 2019 Feb 8:1-8. Epub 2019 Feb 8.

Department of Pediatrics, National Hospital Organization Tokyo Medical Center, Tokyo, Japan,

Introduction: Primary hyperparathyroidism (PHPT) occurs as part of familial syndromes, including CDC73-related disorders caused by germline pathogenic variants of the CDC73 gene, particularly in early adulthood. Herein, we report a familial case of a whole germline CDC73 deletion discordant for PHPT.

Case Description: A 15-year-old boy was admitted to our hospital because of persistent nausea and vomiting. Read More

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http://dx.doi.org/10.1159/000495800DOI Listing
February 2019
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Tumor-induced osteomalacia: experience from three tertiary care centres In India.

Endocr Connect 2019 Feb 1. Epub 2019 Feb 1.

V Agashe, Orthopaedics, PD Hinduja National Hospital and Medical Research Centre, Mumbai, India.

Tumour-induced osteomalacia (TIO) is a rare para-neoplastic syndrome characterized by recalcitrant hypophosphatemia. Reports from the Indian subcontinent are scarce, with most being single center experiences involving few patients. Herein, we conducted a retrospective analysis of 30 patients of TIO diagnosed at three tertiary care hospitals in India. Read More

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http://dx.doi.org/10.1530/EC-18-0552DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6410764PMC
February 2019
3 Reads

Vibrational spectroscopic analysis of hydroxyapatite in HYP mice and individuals with X-linked hypophosphatemia.

Ther Adv Chronic Dis 2018 11;9(12):268-281. Epub 2018 Oct 11.

Frank H. Netter MD School of Medicine, Quinnipiac University, 275 Mt. Carmel Avenue, NH-MED MNH-311H, Hamden, CT 06518, USA.

Background: X-linked hypophosphatemia (XLH) is the most common form of familial phosphate-wasting disorders, due to an inactivating mutation in the phosphate-regulating neutral endopeptidase, X-linked gene. Persistent osteomalacia, enthesophytes, osteophytes, degenerative arthritis and dental abscesses/periodontal disease dominate the adult disorder. However, the impact of insufficient phosphate on hydroxyapatite composition, the major inorganic component of bone and teeth, is unknown in individuals with XLH. Read More

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http://dx.doi.org/10.1177/2040622318804753DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6348532PMC
October 2018
3 Reads

Editorial: Endocrine and Paracrine Role of FGF23 and Klotho in Health and Disease.

Authors:
Reinhold G Erben

Front Endocrinol (Lausanne) 2019 21;10. Epub 2019 Jan 21.

Department of Biomedical Sciences, University of Veterinary Medicine Vienna, Vienna, Austria.

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http://dx.doi.org/10.3389/fendo.2019.00002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6348246PMC
January 2019
2 Reads

X-linked hypophosphatemia: Management and treatment prospects.

Joint Bone Spine 2019 Jan 31. Epub 2019 Jan 31.

Centre de référence des maladies rares du métabolisme du calcium et du phosphate, filière OSCAR and plateforme d'expertise maladies rares Paris-Sud, hôpital Bicêtre Paris Sud, AP-HP, 94270 Le Kremlin Bicêtre, France; Endocrinologie et diabète de l'enfant, hôpital Bicêtre Paris Sud, AP-HP, 94270 Le Kremlin Bicêtre, France; Inserm U1185 et université Paris Sud Paris-Saclay, Hôpital Bicêtre Paris Sud, 94270 Le Kremlin Bicêtre, France.

X-linked hypophosphatemia (XLH), due to a PHEX gene mutation, is the most common genetic form of rickets and osteomalacia. Manifestations in children consist of rickets, lower-limb bone deformities, bone pain, failure to thrive, dental abscesses, and/or craniostenosis. Adults may present with persistent bone pain, early osteoarthritis, hairline fractures and Looser zones, enthesopathy, and/or periodontitis. Read More

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http://dx.doi.org/10.1016/j.jbspin.2019.01.012DOI Listing
January 2019
2 Reads

Elevated Bone Remodeling Markers of CTX and P1NP in Addition to Sclerostin in Patients with X-linked Hypophosphatemia: A Cross-Sectional Controlled Study.

Calcif Tissue Int 2019 Feb 1. Epub 2019 Feb 1.

Department of Pediatrics, Kolding Hospital at Lillebaelt Hospital, Kolding, Denmark.

Aspects of bone remodeling have only been scarcely studied in X-linked hypophosphatemia (XLH). In this cross-sectional controlled study, we assessed biochemical indices of bone remodeling and sclerostin in 27 adult patients (median age 47 [range 24-79] years, 19 women, 8 men) with XLH matched with 81 healthy control subjects (1:3) with respect to age-, sex-, and menopausal status. Markers of bone resorption (carboxyterminal cross-linked telopeptide of type 1 collagen, CTX) and formation (N-terminal propeptide of type 1 procollagen, P1NP) were higher in XLH patients compared to controls (median [IQR] 810 [500-1340] vs 485 [265-715] ng/l and 90 [57-136] vs 49 [39-65] ug/l, respectively, both p < 0. Read More

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http://dx.doi.org/10.1007/s00223-019-00526-zDOI Listing
February 2019
2 Reads

Eltrombopag treatment during induction chemotherapy for acute myeloid leukaemia: a randomised, double-blind, phase 2 study.

Lancet Haematol 2019 Mar 29;6(3):e122-e131. Epub 2019 Jan 29.

Vanderbilt-Ingram Cancer Center, Nashville, TN, USA.

Background: Patients with acute myeloid leukaemia frequently have thrombocytopenia during induction chemotherapy. Eltrombopag, an oral thrombopoietin receptor agonist, stimulates platelet production by a similar mechanism to endogenous thrombopoietin. This study investigated safety and efficacy of eltrombopag versus placebo during anthracycline-based induction treatment of patients with acute myeloid leukaemia. Read More

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http://dx.doi.org/10.1016/S2352-3026(18)30231-XDOI Listing
March 2019
12 Reads

Hyperemesis gravidarum followed by refeeding syndrome causes electrolyte abnormalities induced rhabdomyolysis and diabetes insipidus.

Endocr J 2019 Mar 29;66(3):253-258. Epub 2019 Jan 29.

Department of Metabolic Medicine, Faculty of Life Sciences, Kumamoto University, Kumamoto 860-8556, Japan.

Although hyperemesis gravidarum (HG), an extreme form of morning sickness, is a common complication during pregnancy, HG associated simultaneous onset of rhabdomyolysis and diabetes insipidus due to electrolyte abnormalities are rare. A 34-year-old woman with severe HG at 17 weeks of gestation complicated with appetite loss, weight reduction by 17 kg, general fatigue, myalgia, weakness and polyuria was identified to have simultaneous hypophosphatemia (1.6 mg/dL) and hypokalemia (2. Read More

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http://dx.doi.org/10.1507/endocrj.EJ18-0496DOI Listing
March 2019
2 Reads