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https://www.readbyqxmd.com/read/29350657/an-icet-a-survey-on-hypoparathyroidism-in-patients-with-thalassaemia-major-and-intermedia-a-preliminary-report
#1
Vincenzo De Sanctis, Ashraf T Soliman, Duran Canatan, Heba Elsedfy, Mehran Karimi, Shahina Daar, Hala Rimawi, Soteroula Christou, Nicos Skordis, Ploutarchos Tzoulis, Praveen Sobti, Shruti Kakkar, Yurdanur Kilinc, Doaa Khater, Saif A Alyaarubi, Valeriya Kaleva, Su Han Lum, Mohamed A Yassin, Forough Saki, Maha Obiedat, Salvatore Anastasi, Maria Concetta Galati, Giuseppe Raiola, Saveria Campisi, Nada Soliman, Mohamed Elshinawy, Soad Al Jaouni, Salvatore Di Maio, Yasser Wali, Ihab Zaki Elhakim, Christos Kattamis
Hypoparathyroidism (HPT) is a rare disease with leading symptoms of hypocalcemia, associated with high serum phosphorus levels and absent or inappropriately low levels of parathyroid hormone (PTH). In patients with thalassemias it is mainly attributed to transfusional iron overload, and suboptimal iron chelation therapy. The main objectives of this survey were to provide data on the prevalence, demographic and clinical features of HPT in thalassemia major (TM) and intermedia (TI) patients living in different countries, and to assess its impact in clinical medical practice...
January 16, 2018: Acta Bio-medica: Atenei Parmensis
https://www.readbyqxmd.com/read/29344330/developments-in-rare-bone-diseases-and-mineral-disorders
#2
REVIEW
Siobhan Bacon, Rachel Crowley
In the last decade, there have been a number of significant advances made in the field of rare bone diseases. In this review, we discuss the expansion of the classification system for osteogenesis imperfecta (OI) and the resultant increase in therapeutic options available for management of OI. Bisphosphonates remain the most widely used intervention for OI, although the effect on fracture rate reduction is equivocal. We review the other therapies showing promising results, including denosumab, teriparatide, sclerostin, transforming growth factor β inhibition and gene targeted approaches...
January 2018: Therapeutic Advances in Chronic Disease
https://www.readbyqxmd.com/read/29340121/complications-of-transfusion-dependent-%C3%AE-thalassemia-patients-in-sistan-and-baluchistan-south-east-of-iran
#3
Maryam Yaghobi, Ebrahim Miri-Moghaddam, Naderi Majid, Ali Bazi, Ali Navidian, Asiyeh Kalkali
Background: Thalassemia syndromes are among prevalent hereditary disorders imposing high expenses on health-care system worldwide and in Iran. Organ failure represents a life-threatening challenge in transfusion- dependent β-thalassemia (TDT) patients. The purpose of the present study was to determine the frequency of organ dysfunctions among TDT patients in Sistan and Baluchistan province in South-East of Iran. Materials and Methods: Laboratory and clinical data were extracted from medical records as well as by interviews...
October 1, 2017: International Journal of Hematology-oncology and Stem Cell Research
https://www.readbyqxmd.com/read/29339573/-sup-18-sup-f-choline-pet-ct-for-parathyroid-scintigraphy-significantly-lower-radiation-exposure-of-patients-in-comparison-to-conventional-nuclear-medicine-imaging-approaches
#4
Sebastijan Rep, Marko Hocevar, Janja Vaupotič, Urban Zdesar, Katja Zaletel, Luka Lezaic
Parathyroid subtraction scintigraphy is the most commonly used imaging method for localization of hyperfunctioning parathyroid glands (HPG) in primary hyperparathyroidism, a common endocrine disorder. Hybrid (SPECT/CT) imaging with 99mTc-sestaMIBI (MIBI) at an early and delayed phase (dual-phase imaging) may be the most accurate conventional imaging approach, but includes additional radiation exposure due to added CT imaging. Recently, 18F-choline (FCH) PET/CT was introduced for HPG imaging, which can also be performed using the dual-phase approach...
January 17, 2018: Journal of Radiological Protection: Official Journal of the Society for Radiological Protection
https://www.readbyqxmd.com/read/29335796/association-between-frailty-and-bone-loss-in-patients-undergoing-maintenance-hemodialysis
#5
Kei Yoneki, Jun Kitagawa, Keika Hoshi, Manae Harada, Takaaki Watanabe, Takahiro Shimoda, Ryota Matsuzawa, Atsushi Yoshida, Yusuke Matsunaga, Yasuo Takeuchi, Kentaro Kamiya, Atsuhiko Matsunaga
Frailty is significantly associated with bone loss in the general population. However, it is unclear whether this association also exists in patients undergoing hemodialysis who have chronic kidney disease-mineral and bone disorder (CKD-MBD). This study aimed to assess the association between frailty and bone loss in patients undergoing hemodialysis. This cross-sectional study included 214 (90 women, 124 men) Japanese outpatients undergoing maintenance hemodialysis three times per week, with a mean age of 67...
January 15, 2018: Journal of Bone and Mineral Metabolism
https://www.readbyqxmd.com/read/29316724/biochemical-and-clinical-impact-of-organic-uremic-retention-solutes-a-comprehensive-update
#6
REVIEW
Raymond Vanholder, Anneleen Pletinck, Eva Schepers, Griet Glorieux
In this narrative review, the biological/biochemical impact (toxicity) of a large array of known individual uremic retention solutes and groups of solutes is summarized. We classified these compounds along their physico-chemical characteristics as small water-soluble compounds or groups, protein bound compounds and middle molecules. All but one solute (glomerulopressin) affected at least one mechanism with the potential to contribute to the uremic syndrome. In general, several mechanisms were influenced for each individual solute or group of solutes, with some impacting up to 7 different biological systems of the 11 considered...
January 8, 2018: Toxins
https://www.readbyqxmd.com/read/29299645/transcutaneous-laryngeal-ultrasonography-for-laryngeal-immobility-diagnosis-in-patients-with-voice-disorders-after-thyroid-parathyroid-surgery
#7
Diane S Lazard, Héloïse Bergeret-Cassagne, Muriel Lefort, Laurence Leenhardt, Gilles Russ, Frédérique Frouin, Christophe Trésallet
BACKGROUND: Transcutaneous laryngeal ultrasonography (TLUS) was recently developed to assess recurrent nerve palsy after thyroid/parathyroid surgery, with variable rates of efficiency. The aim of the current study was to evaluate this technique using subjective estimation and post-processing quantitative data. METHODS: Fifty subjects presenting with a recurrent nerve palsy and 50 "controls" presenting with voice, swallowing, or breathing disorders following thyroid/parathyroid surgery were prospectively included...
January 3, 2018: World Journal of Surgery
https://www.readbyqxmd.com/read/29285441/high-dose-vitamin-d-supplementation-precipitating-hypercalcemic-crisis-in-granulomatous-disorders
#8
Vijaya Sarathi, Hareeshababu Karethimmaiah, Amit Goel
Background: Vitamin D supplementation precipitating hypercalcemic crisis is often the first manifestation in patients with granulomatous disorders. Methods: We report our experience on patients presenting with hypercalcemic crisis due to granulomatous disorder and the role of Vitamin D supplementation in the precipitation of hypercalcemic crisis in them. Results: The study included five patients with granulomatous disorders who presented with hypercalcemic crisis...
November 2017: Indian Journal of Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29285439/incidence-of-endocrine-disorders-in-indian-adult-male-population
#9
K V S H Kumar, S K Patnaik
Background: The comprehensive epidemiology of endocrine disorders is lacking from our country. Most of the available data pertain to the prevalence of diabetes and thyroid disorders only. We studied the incidence of endocrine disorders in a cohort of service personnel followed for a long duration. Materials and Methods: The data for this descriptive epidemiologic study were derived from the electronic medical records of the male service personnel enrolled between 1990 and 2015...
November 2017: Indian Journal of Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29280743/current-nomenclature-of-pseudohypoparathyroidism-inactivating-parathyroid-hormone-parathyroid-hormone-related-protein-signaling-disorder
#10
Serap Turan
Disorders related to parathyroid hormone (PTH) resistance and PTH signaling pathway impairment are historically classified under the term of pseudohypoparathyroidism (PHP). The disease was first described and named by Fuller Albright and colleagues in 1942. Albright hereditary osteodystrophy (AHO) is described as an associated clinical entity with PHP, characterized by brachydactyly, subcutaneous ossifications, round face, short stature and a stocky build. The classification of PHP is further divided into PHP-Ia, pseudo-PHP (pPHP), PHP-Ib, PHP-Ic and PHP-II according to the presence or absence of AHO, together with an in vivo response to exogenous PTH and the measurement of Gsα protein activity in peripheral erythrocyte membranes in vitro...
December 27, 2017: Journal of Clinical Research in Pediatric Endocrinology
https://www.readbyqxmd.com/read/29262409/early-markers-of-cardiovascular-risk-in-autosomal-dominant-polycystic-kidney-disease
#11
Silvia Lai, Daniela Mastroluca, Silvia Matino, Valeria Panebianco, Antonio Vitarelli, Lidia Capotosto, Irene Turinese, Paolo Marinelli, Marco Rossetti, Alessandro Galani, Pia Baiocchi, Anna R D'Angelo, Paolo Palange
BACKGROUND/AIMS: Cardiovascular disease is the most frequent cause of morbidity and mortality in autosomal dominant polycystic kidney disease (ADPKD) patients, often before the onset of renal failure, and the pathogenetic mechanism is not yet well elucidated. The aim of the study was to identify early and noninvasive markers of cardiovascular risk in young ADPKD patients, in the early stages of disease. METHODS: A total of 26 patients with ADPKD and 24 control group, matched for age and sex, were enrolled, and we have assessed inflammatory indexes, mineral metabolism, metabolic state and markers of atherosclerosis and endothelial dysfunction (carotid intima media thickness (IMT), ankle brachial index (ABI), flow mediated dilation (FMD), renal resistive index (RRI), left ventricular mass index (LVMI)) and cardiopulmonary exercise testing (CPET), maximal O2 uptake (V'O2max), and O2 uptake at lactic acid threshold (V'O2@LT)...
December 15, 2017: Kidney & Blood Pressure Research
https://www.readbyqxmd.com/read/29243442/-regression-analysis-of-serum-bone-metabolic-markers-and-traditional-chinese-medicine-syndromes-in-patients-with-ckd-mbd
#12
Hai-Ming Yang, Xian-Jie Meng, Wei Wu, Ying-Lu Liu, Xiao-Juan Zhai
To analyze the interdependent relationship between serum bone metabolic markers and traditional Chinese medicine (TCM) syndromes in patients with chronic kidney disease (stages 3 and 4)-related mineral and bone disorder (CKD-MBD), in order to provide the objective basis for exploring the rules of TCM syndrome differentiation in patients with CKD-MBD. The retrospective survey was conducted to collect 105 cases with CKD (stages 3 and 4)-MBD. General clinical indexes, frequency of TCM syndromes and distribution of TCM syndrome type were investigated...
October 2017: Zhongguo Zhong Yao za Zhi, Zhongguo Zhongyao Zazhi, China Journal of Chinese Materia Medica
https://www.readbyqxmd.com/read/29239351/association-of-serum-uric-acid-levels-with-osteoporosis-and-bone-turnover-markers-in-a-chinese-population
#13
Dan-Dan Yan, Jie Wang, Xu-Hong Hou, Yu-Qian Bao, Zhen-Lin Zhang, Cheng Hu, Wei-Ping Jia
Recent evidence shows that uric acid is protective against some neurological diseases, but can be detrimental in many metabolic and cardiovascular disorders. In this study, we examined the association between serum uric acid levels and bone metabolism in Chinese males and postmenopausal females. A total of 943 males and 4256 postmenopausal females were recruited in Shanghai. The levels of serum uric acid and bone turnover markers (BTMs) were detected along with other biochemical traits. In addition, the fat distribution was calculated through MRI and image analysis software, and bone mineral density (BMD) was determined using dual-energy X-ray absorptiometry...
December 14, 2017: Acta Pharmacologica Sinica
https://www.readbyqxmd.com/read/29239035/heritable-forms-of-primary-hyperparathyroidism-a-current-perspective
#14
REVIEW
Ronald A DeLellis, Shamlal Mangray
Primary hyperparathyroidism (PHPT) is one of the most common of all endocrine disorders encountered by the practising histopathologist. The vast majority of lesions are sporadic in nature, approximately 85% of which are parathyroid adenomas, while hyperplasia and carcinoma account for 10-15% and fewer than 1%, of cases, respectively. Heritable forms of PHPT are much less common and present challenges both to clinicians and pathologists, particularly when they are the presenting feature of an endocrine syndrome...
January 2018: Histopathology
https://www.readbyqxmd.com/read/29238762/etelcalcetide-injectable-calcimimetic-for-the-treatment-of-secondary-hyperparathyroidism-in-hemodialysis-dependent-patients
#15
P A Ureña Torres, J Bover, M Cohen-Solal
Chronic kidney disease is associated with mineral and bone disorders that are now considered as a syndrome. One of the major complications of this syndrome is secondary hyperparathyroidism (SHPT). SHPT increases bone turnover and the risk of fracture. SHPT is also associated with cardiovascular calcification and high mortality risk. The classical medical therapies of SHPT lack long-term efficacy and have undesirable effects on serum calcium and phosphate levels. Surgical parathyroidectomy is a radical therapeutic solution potentially exposing patients to a permanent state of hypoparathyroidism among other complications...
September 2017: Drugs of Today
https://www.readbyqxmd.com/read/29227640/raman-spectroscopy-applied-to-parathyroid-tissues-a-new-diagnostic-tool-to-discriminate-normal-tissue-from-adenoma
#16
Andrea Palermo, Marco Fosca, Gaia Tabacco, Federico Marini, Valerio Graziani, Maria Carla Santarsia, Filippo Longo, Angelo Lauria, Roberto Cesareo, Isabella Giovannoni, Chiara Taffon, Massimiliano Rocchia, Silvia Manfrini, Pierfilippo Crucitti, Paolo Pozzilli, Anna Crescenzi, Julietta V Rau
Primary hyperparathyroidism is an endocrine disorder characterized by autonomous production of parathyroid hormone. Patients with the symptomatic disease should be referred for parathyroidectomy. However, the distinction between the pathological condition and the benign one is very challenging in the surgical setting; therefore, accurate recognition is important to ensure success during minimally invasive surgery. At present, all intraoperative techniques significantly increase surgical time and, consequently, cost...
December 11, 2017: Analytical Chemistry
https://www.readbyqxmd.com/read/29217931/hyper-parathyroidisim-jaw-tumor-syndrome-a-rare-condition-of-incongruous-features
#17
Manchil P Redwin Dhas, Kannan S Karthiga, Joy E Tatu, Sherubin J Eugenia
Background: Hyperparathyroidism-Jaw Tumor (HPT-JT) syndrome is a rare genetic disorder bearing both a germline and a somatic CDC73 mutation (formerly known as HRPT2), which has been mapped to chromosome 1q25-q31. The association of jaw ossifying fibroma with primary hyperparathyroidisim (PHPT) is typical of HPT-JT. It may also include cystic and neoplastic renal abnormalities and uterine tumors. Case Details: Here, we report a case of HPT-JT with an initial presentation of declination in reproductive fitness...
May 2017: Ethiopian Journal of Health Sciences
https://www.readbyqxmd.com/read/29181658/effect-of-ferric-citrate-hydrate-on-fgf23-and-pth-levels-in-patients-with-non-dialysis-dependent-chronic-kidney-disease-with-normophosphatemia-and-iron-deficiency
#18
Akira Iguchi, Suguru Yamamoto, Mihoko Yamazaki, Kazuyuki Tasaki, Yasushi Suzuki, Junichiro James Kazama, Ichiei Narita
BACKGROUND: In patients with normophosphatemia with chronic kidney disease (CKD), fibroblast growth factor 23 (FGF23) and parathyroid hormone (PTH) increase urinary phosphate excretion while maintaining serum phosphate within the normal range. Recent reports have shown that, in this stage, phosphate binders do not decrease serum FGF23 and PTH levels. Iron deficiency promotes transcription of FGF23 and iron-supplementation for iron deficiency decreases serum FGF23 levels. We hypothesized that ferric citrate hydrate, an iron-based phosphate binder, will decrease serum FGF23 levels in patients with non-dialysis-dependent CKD with normophosphatemia and iron deficiency...
November 27, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/29175269/sclerostin-deficiency-modifies-the-development-of-ckd-mbd-in-mice
#19
Nadine Kaesler, Anja Verhulst, Annelies De Maré, Annika Deck, Geert J Behets, Ayshe Hyusein, Pieter Evenepoel, Jürgen Floege, Nikolaus Marx, Anne Babler, Ina Kramer, Michaela Kneissel, Rafael Kramann, Daniel Weis, Patrick C D'Haese, Vincent M Brandenburg
Sclerostin is a soluble antagonist of canonical Wnt signaling and a strong inhibitor of bone formation. We present experimental data on the role of sclerostin in chronic kidney disease - bone mineral disorder (CKD-MBD). METHODS: We performed 5/6 nephrectomies in 36-week-old sclerostin-deficient (SOST-/-) B6-mice and in C57BL/6J wildtype (WT) mice. Animals received a high phosphate diet for 11weeks. The bones were analyzed by high-resolution micro-computed tomography (μCT) and quantitative bone histomorphometry...
November 21, 2017: Bone
https://www.readbyqxmd.com/read/29171476/-recombinant-human-parathyroid-hormone-in-the-therapy-of-hypoparathyroidism
#20
A K Eremkina, N G Mokrysheva, E V Kovaleva, Yu A Krupinova
Hypoparathyroidism is an endocrine disease that results from deficiency or complete absence of parathyroid hormone (PTH), a biologically active 84-amino acid polypeptide. Standard therapy for chronic hypoparathyroidism includes oral calcium salts and active vitamin D metabolites and is aimed at maintaining a balance between optimal near-normal serum calcium concentration and normocalcuria. Traditional treatment regimens not always lead to the compensation for calcium and phosphorus metabolism. Until recently, hypoparathyroidism is the only endocrine disorder that has not been treated with the recombinant hormone...
2017: Terapevticheskiĭ Arkhiv
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