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https://www.readbyqxmd.com/read/28620422/special-considerations-for-vitamin-d-in-the-south-asian-population-in-the-uk
#1
REVIEW
Nicola M Lowe, Issak Bhojani
The human requirement for vitamin D is achieved primarily through the synthesis of this prehormone in the skin during exposure to ultraviolet B (UVB) radiation, with only a minor contribution from the diet, year round. Achieving optimal vitamin D status is therefore largely dependent upon adequate exposure of the skin to sunlight, however, the length of exposure required varies with latitude and season, and is also dependent upon skin pigmentation, with darker skin requiring greater exposure than fair skin due to the protective effects of melanin against UVB radiation...
June 2017: Therapeutic Advances in Musculoskeletal Disease
https://www.readbyqxmd.com/read/28614212/phosphaturic-mesenchymal-tumors-clinicopathologic-immunohistochemical-and-molecular-analysis-of-22-cases-expanding-their-morphologic-and-immunophenotypic-spectrum
#2
Abbas Agaimy, Michael Michal, Simion Chiosea, Fredrik Petersson, Ladislav Hadravsky, Glenn Kristiansen, Raymund E Horch, Jan Schmolders, Arndt Hartmann, Florian Haller, Michal Michal
Phosphaturic mesenchymal tumor (PMT) is a rare neoplasm of uncertain histogenesis that has been linked to tumor-induced osteomalacia (TIO) since 1959. The neoplastic cells produce increased amount of FGF23 which results in TIO via uncontrolled renal loss of phosphate (phosphaturia), and consequently diminished bone mineralization. To date, ∼300 cases have been reported. Although there is increasing evidence that PMT can be diagnosed by reproducible histopathologic features, firm diagnosis has been often restricted to cases associated with TIO and, hence, diagnosis of "nonphosphaturic variants" remained challenging...
June 13, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28612338/nutritional-rickets-and-osteomalacia-in-the-twenty-first-century-revised-concepts-public-health-and-prevention-strategies
#3
REVIEW
Suma Uday, Wolfgang Högler
PURPOSE OF REVIEW: Nutritional rickets and osteomalacia are common in dark-skinned and migrant populations. Their global incidence is rising due to changing population demographics, failing prevention policies and missing implementation strategies. The calcium deprivation spectrum has hypocalcaemic (seizures, tetany and dilated cardiomyopathy) and late hypophosphataemic (rickets, osteomalacia and muscle weakness) complications. This article reviews sustainable prevention strategies and identifies areas for future research...
June 13, 2017: Current Osteoporosis Reports
https://www.readbyqxmd.com/read/28609017/clinical-aspects-of-magnesium-physiology-in-patients-on-dialysis
#4
REVIEW
Paraish S Misra, Sharon J Nessim
Magnesium balance is infrequently discussed in the dialysis population, and the clinical consequences of derangements in magnesium homeostasis are incompletely understood. There is an association between hypomagnesemia and adverse outcomes including increases in cardiovascular disease and mortality, while elevated magnesium levels have also been linked with complications such as osteomalacia. In this review, we discuss the features of magnesium physiology relevant to dialysis patients and provide an updated summary of the literature linking magnesium derangements with bone disease, cardiovascular disease, sudden cardiac death, and mortality...
June 13, 2017: Seminars in Dialysis
https://www.readbyqxmd.com/read/28603080/a-novel-auditory-ossicles-membrane-and-the-development-of-conductive-hearing-loss-in-dmp1-null-mice
#5
Kun Lv, Haiyang Huang, Xing Yi, Mark E Chertoff, Chaoyuan Li, Baozhi Yuan, Robert J Hinton, Jian Q Feng
Genetic mouse models are widely used for understanding human diseases but we know much less about the anatomical structure of the auditory ossicles in the mouse than we do about human ossicles. Furthermore, current studies have mainly focused on disease conditions such as osteomalacia and rickets in patients with hypophosphatemia rickets, although the reason that these patients develop late-onset hearing loss is unknown. In this study, we first analyzed Dmp1 lac Z knock-in auditory ossicles (in which the blue reporter is used to trace DMP1 expression in osteocytes) using X-gal staining and discovered a novel bony membrane surrounding the mouse malleus...
June 8, 2017: Bone
https://www.readbyqxmd.com/read/28600887/therapeutic-effects-of-fgf23-c-tail-fc-in-a-murine-pre-clinical-model-of-x-linked-hypophosphatemia-via-the-selective-modulation-of-phosphate-reabsorption
#6
Kristen Johnson, Kymberly Levine, Joseph Sergi, Jean Chamoun, Rachel Roach, Jackie Vekich, Mike Favis, Mark Horn, Xianjun Cao, Brian Miller, William Snyder, Dikran Aivazian, William Reagan, Edwin Berryman, Jennifer Colangelo, Victoria Markiewicz, Cedo Bagi, Thomas P Brown, Anthony Coyle, Moosa Mohammadi, Jeanne Magram
Fibroblast growth factor 23 (FGF23) is the causative factor of X-linked hypophosphatemia (XLH), a genetic disorder effecting 1:20,000 that is characterized by excessive phosphate excretion, elevated FGF23 levels and a rickets/osteomalacia phenotype. FGF23 inhibits phosphate reabsorption and suppresses 1α,25-dihydroxyvitamin D (1,25D) biosynthesis, analytes that differentially contribute to bone integrity and deleterious soft tissue mineralization. As inhibition of ligand broadly modulates downstream targets, balancing efficacy and unwanted toxicity is difficult when targeting the FGF23 pathway...
June 10, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28579442/phosphaturic-mesenchymal-tumor-of-the-nasal-cavity-and-paranasal-sinuses-a-clinical-curiosity-presenting-a-diagnostic-challenge
#7
Shubhada V Kane, Aanchal Kakkar, Nikita Oza, Epari Sridhar, Prathamesh S Pai
Phosphaturic mesenchymal tumor (PMT) is a rare mesenchymal neoplasm associated with tumor-induced osteomalacia (TIO) and elevated serum FGF-23. Common in extremities, PMT rarely occurs in sinonasal region. We report a series of sinonasal PMT diagnosed at our institute over a 6-year period. Six cases of sinonasal PMT were identified during this period, of which five presented with features of TIO. Median age of patients was 45.5 years. All six tumors were composed of stellate to spindled cells, with prominent staghorn vasculature in four cases...
June 1, 2017: Auris, Nasus, Larynx
https://www.readbyqxmd.com/read/28571152/oncogenic-osteomalacia-an-approach-to-diagnosis-with-a-case-report
#8
Biswajit Dey, Debasis Gochhait, Hema Subramanian, Madhusudhanan Ponnusamy
Oncogenic osteomalacia, also known as tumour induced osteomalacia, is a rare paraneoplastic syndrome caused by mesenchymal tumours secreting Fibroblast Growth Factor-23 (FGF-23). The characteristic biochemical findings include hypophosphatemia and low 1,25-dihydroxy vitamin D. The differential diagnosis for hypophosphatemia are varied. We present a case of oncogenic osteomalacia in a 29-year-old female, who presented with complaints of generalized diffuse bone pain and walking difficulty for six months duration...
April 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28559948/bone-scintigraphy-and-tenofovir-induced-osteomalacia-in-chronic-hepatitis-b
#9
Alex Khoo Cheen Hoe, Lee Yeong Fong
Tenofovir, used in the treatment of chronic hepatitis B and HIV, is known for its side effects on the kidneys and bones. We share interesting images of a patient with tenofovir-induced osteomalacia on Technetium-99 m hydroxymethyelene (Tc-99 m HDP) bone scintigraphy. Pattern recognition of this bone scintigraphy and correlation with the clinical history is essential to avoid misdiagnosis.
June 2017: Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/28545150/total-hip-knee-arthroplasty-in-the-treatment-of-tumor-induced-osteomalacia-patients-more-than-1-year-follow-up
#10
Wei Zhu, Qi Ma, Yanyan Bian, Qianyu Zhuang, Zenan Xia, Jin Jin, Xisheng Weng
BACKGROUND: Tumor-induced osteomalacia (TIO) may result in a better prognosis after complete resection of the causative neoplasm. However, tumors located proximal to the articular surface of the metaphysis remain largely uninvestigated. METHODS: A retrospective study of sixteen patients was undertaken to evaluate treatment of tumors with joint arthroplasty and tumor resection. The bone metabolism index, hip/knee joint function, arthroplasty complications and symptoms were followed up for at least 12 months in each patient...
2017: PloS One
https://www.readbyqxmd.com/read/28515613/secondary-sjogren-s-syndrome-presenting-with-distal-tubular-acidosis-and-quadriparesis
#11
Kartik Munta, Manimala Rao Surath, K Seshikiran
A 52-year-old female patient was admitted to Intensive Care Unit with complaints of quadriparesis. Investigations revealed distal renal tubular acidosis (DRTA) secondary to Sjogren's syndrome with involvement of the parotid and thyroid glands. Laboratory investigations showed hyperchloremic metabolic acidosis and an alkaline urine pH with clinical signs of sicca syndrome. Sjogren's syndrome is associated with DRTA and occurrences of quadriparetic hypokalemia, nephrolithiasis, and osteomalacia can be prevented with early diagnosis and lifelong treatment with potassium and alkali replacement...
April 2017: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28495360/fractures-and-osteomalacia-in-a-patient-treated-with-frequent-home-hemodialysis
#12
Mark R Hanudel, Larry Froch, Barbara Gales, Harald Jüppner, Isidro B Salusky
Bone deformities and fractures are common consequences of renal osteodystrophy in the dialysis population. Persistent hypophosphatemia may be observed with more frequent home hemodialysis regimens, but the specific effects on the skeleton are unknown. We present a patient with end-stage renal disease treated with frequent home hemodialysis who developed severe bone pain and multiple fractures, including a hip fracture and a tibia-fibula fracture complicated by nonunion, rendering her nonambulatory and wheelchair bound for more than a year...
May 9, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28469928/effective-localization-in-tumor-induced-osteomalacia-using-68-ga-dotatoc-pet-ct-venous-sampling-and-3t-mri
#13
Shintaro Kawai, Hiroyuki Ariyasu, Yasushi Furukawa, Reika Yamamoto, Shinsuke Uraki, Ken Takeshima, Kenji Warigaya, Yuji Nakamoto, Takashi Akamizu
SUMMARY: Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome characterized by renal phosphate wasting leading to hypophosphatemia due to excessive actions of fibroblast growth factor 23 (FGF23) produced by the tumors. Although the best way of curing TIO is complete resection, it is usually difficult to detect the culprit tumors by general radiological modalities owing to the size and location of the tumors. We report a case of TIO in which the identification of the tumor by conventional imaging studies was difficult...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28459498/octreotide-is-ineffective-in-treating-tumor-induced-osteomalacia-results-of-a-short-term-therapy
#14
Diana Ovejero, Diala El-Maouche, Beth A Brillante, Azar Khosravi, Rachel I Gafni, Michael T Collins
Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome in which unregulated hypersecretion of fibroblast growth factor 23 (FGF23) by phosphaturic mesenchymal tumors (PMT) causes renal phosphate wasting, hypophosphatemia, and osteomalacia. The resulting mineral homeostasis abnormalities and skeletal manifestations can be reversed with surgical resection of the tumor. Unfortunately, PMTs are often difficult to locate, and medical treatment with oral phosphate and vitamin D analogues is either insufficient to manage the disease or not tolerated...
April 29, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28457494/vitamin-d-status-and-the-host-resistance-to-infections-what-it-is-currently-not-understood
#15
Pierre Olivier Lang, Richard Aspinall
PURPOSE: Vitamin D is increasingly thought to play a role in regulating immunity. This comprehensive review updates the current understanding regarding ways in which we believe that vitamin D regulates responsiveness of the immune system and how serum status modulates the host defense against pathogens. METHODS: The literature was searched by using PubMed and Scopus with the following key words: vitamin D, immunity, innate and adaptive immunity, infectious disease, and vaccine response...
April 27, 2017: Clinical Therapeutics
https://www.readbyqxmd.com/read/28450684/the-diagnostic-dilemma-of-tumor-induced-osteomalacia-a-retrospective-analysis-of-144-cases
#16
Juan Feng, Yan Jiang, Ou Wang, Mei Li, Xiaoping Xing, Li Huo, Fang Li, Wei Yu, Ding-Rong Zhong, Jin Jin, Yong Liu, Fang Qi, Wei Lv, Lian Zhou, Xun-Wu Meng, Wei-Bo Xia
Diagnostic delay of tumor induced osteomalacia (TIO) is common in clinic practice. To investigate the diagnostic condition of TIO in China and raise clinicians' awareness of TIO, we retrospectively analyzed clinical manifestations, biochemical features, and specially evaluated missed diagnoses and misdiagnoses among 144 TIO patients from Peking Union Medical College Hospital during December 1982 to December 2014. Clinical presentations of TIO mainly included bone pain, difficulty in walking, pathological fractures, muscle weakness, and height loss...
April 26, 2017: Endocrine Journal
https://www.readbyqxmd.com/read/28445300/malignant-phosphaturic-mesenchymal-tumor-with-pulmonary-metastasis-a-case-report
#17
Shui Qiu, Li-Li Cao, Yue Qiu, Ping Yan, Zi-Xuan Li, Jiang Du, Li-Mei Sun, Qing-Fu Zhang
RATIONALE: Phosphaturic mesenchymal tumor (PMT) is a new tumor entity of soft tissue and bone tumor recently accepted by the World Health Organization, which typically causes the paraneoplastic syndrome of tumor-induced osteomalacia (TIO). The majority of PMTs follow a benign clinical course and local recurrence occurs in < 10% of cases, malignant PMTs with distant organ metastasis are extremely uncommon. PATIENT CONCERNS: We reported a 41-year-old woman who was diagnosed with PMT 10 years ago with a repeated recurrence and pulmonary metastasis...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28431446/-vitamin-d-in-clinic-and-practice
#18
Armin Zittermann, Stefan Pilz
Vitamin D is unique among the vitamins, since its major source is skin synthesis by solar UVB exposure, whereas dietary intake plays only a minor role. In the general population, cutaneous vitamin D synthesis is clearly below the recommended amount. Dietary vitamin D does not fill this gap. Severe vitamin D deficiency results in rickets in infants and osteomalacia in adults. In the absence of endogenous vitamin D synthesis (e. g. during winter), oral supplements should ensure adequate daily vitamin D intake, where appropriate...
April 2017: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/28425622/targeted-disruption-of-nf1-in-osteocytes-increases-fgf23-and-osteoid-with-osteomalacia-like-bone-phenotype
#19
Nobuhiro Kamiya, Ryosuke Yamaguchi, Olumide Aruwajoye, Audrey Kim, Gen Kuroyanagi, Matthew Phipps, Naga Suresh Adapala, Jian Q Feng, Harry Kw Kim
Neurofibromatosis type 1 (NF1, OMIM 162200), caused by NF1 gene mutations, exhibits multi-system abnormalities, including skeletal deformities in humans. Osteocytes play critical roles in controlling bone modeling and remodeling. However, the role of neurofibromin, the protein product of the NF1 gene, in osteocytes is largely unknown. This study investigated the role of neurofibromin in osteocytes by disrupting Nf1 under the Dmp1-promoter. The conditional knockout (Nf1 cKO) mice displayed serum profile of a metabolic bone disorder with an osteomalacia-like bone phenotype...
April 20, 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28399017/iron-induced-hypophosphatemia-an-emerging-complication
#20
Heinz Zoller, Benedikt Schaefer, Bernhard Glodny
PURPOSE OF REVIEW: Iron-induced hypophosphatemia is a well documented side-effect but associated complications are largely neglected, because the results from single dosing studies suggest that transient decreases in plasma phosphate concentrations are asymptomatic and fully reversible. However, an increasing number of case reports and case series suggest that some patients develop severe and symptomatic hypophosphatemia. Long-term complications from hypophosphatemia include osteomalacia and bone fractures, which can result from repeated intravenous administration of certain high-dose iron preparations...
July 2017: Current Opinion in Nephrology and Hypertension
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