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Satoshi Higuchi, Hideki Ota, Takuya Ueda, Yuta Tezuka, Kei Omata, Yoshikiyo Ono, Ryo Morimoto, Masataka Kudo, Fumitoshi Satoh, and Kei Takase

. Cardiovascular manifestations of pheochromocytoma . Cardiology in Review 2017 25 215 – 222 . ( https://doi.org/10.1097/CRD.0000000000000141 ) 10.1097/CRD.0000000000000141 28786897 4 Yu R Nissen NN Bannykh SI . Cardiac complications as initial

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K G Samsom, L M van Veenendaal, G D Valk, M R Vriens, M E T Tesselaar, and J G van den Berg

genomic landscape of SI-NETs remains poorly elucidated and biomarkers have not yet been identified. Moreover, the genetic constitution of SI-NETs has been shown to differ compared to pancreatic NETs ( 11 ). With this review we aim to provide the clinician

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Guido Zavatta and Bart L Clarke

). This review will focus on practical issues regarding the current treatment of chronic hypoparathyroidism, and discuss common challenges in the management of this rare disease within the context of the published guidelines. Conventional therapy

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Zeming Liu, Di Hu, Yihui Huang, Sichao Chen, Wen Zeng, Ling Zhou, Wei Zhou, Min Wang, Haifeng Feng, Wei Wei, Chao Zhang, Danyang Chen, and Liang Guo

undertook the research, Y H H, S C C and W Z performed the analyses, Z M L and D H wrote the main manuscript text and prepared figures. All authors reviewed the manuscript. References 1 Pacini F Schlumberger M Dralle H Elisei R Smit JW Wiersinga W

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Benjamin G Challis, Andrew S Powlson, Ruth T Casey, Carla Pearson, Brian Y Lam, Marcella Ma, Deborah Pitfield, Giles S H Yeo, Edmund Godfrey, Heok K Cheow, V Krishna Chatterjee, Nicholas R Carroll, Ashley Shaw, John R Buscombe, and Helen L Simpson

involved in tumour development and autonomous insulin secretion. Patients and methods Study population We retrospectively reviewed medical records of patients presenting to our institution between 2003 and 2016 with a diagnosis of

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Nese Cinar and Alper Gurlek

Adipose tissue secretes a variety of active biological substances, called adipocytokines, that act in an autocrine, paracrine, and endocrine manner. They have roles in appetite control, thermogenesis, and thyroid and reproductive functions. All these molecules may lead to local and generalized inflammation, mediating obesity-associated vascular disorders including hypertension, diabetes, atherosclerosis, and insulin resistance. Thyroid dysfunction is associated with changes in body weight, thermogenesis, and energy expenditure. The connections between cardiovascular risk factors such as dyslipidemia, impaired glucose tolerance, insulin resistance, atherosclerosis, and thyroid dysfunction have been reported in several studies. The adipocytokines serve as causative or protective factors in the development of these disorders in the states of thyroid dysfunction. Abnormal levels of adipocytokines (adiponectin (ADP), leptin, resistin, vaspin, and visfatin) in hypo- and hyperthyroidism have been reported with controversial results. This review aims to update the implication of novel adipokines ADP, vaspin, and visfatin in thyroid dysfunction.

Open access

Malgorzata Oczko-Wojciechowska, Agnieszka Czarniecka, Tomasz Gawlik, Barbara Jarzab, and Jolanta Krajewska

Medullary thyroid cancer (MTC) is a rare thyroid malignancy, which arises from parafollicular C-cells. It occurs in the hereditary or sporadic form. Hereditary type is a consequence of activation of the RET proto-oncogene by germline mutations, whereas about 80% of sporadic MTC tumors harbor somatic, mainly RET or rarely RAS mutations. According to the current ATA guidelines, a postoperative MTC risk stratification and long-term follow-up are mainly based on histopathological data, including tumor stage, the presence of lymph node and/or distant metastases (TNM classification), and serum concentration of two biomarkers: calcitonin (Ctn) and carcinoembryonic antigen (CEA). The type of RET germline mutation also correlates with MTC clinical characteristics. The most common and the best known RET mutation in sporadic MTC, localized at codon 918, is related to a more aggressive MTC course and poorer survival. However, even if histopathological or clinical features allow to predict a long-term prognosis, they are not sufficient to select the patients showing aggressive MTC courses requiring immediate treatment or those, who are refractory to different therapeutic methods. Besides the RET gene mutations, there are currently no other reliable molecular prognostic markers. This review summarizes the present data of genomic investigation on molecular prognostic factors in medullary thyroid cancer.

Open access

Raja Padidela, Nadine Sawoky, Moira S. Cheung, Vrinda Saraff, and Poonam Dharmaraj

X-linked hypophosphataemia (XLH) is caused by a pathogenic variant in the PHEX gene, which leads to elevated circulating FGF23. High FGF23 causes hypophosphataemia, reduced active vitamin D concentration and clinically manifests as rickets in children, and osteomalacia in children and adults. Conventional therapy for XLH includes oral phosphate and active vitamin D analogues but does not specifically treat the underlying pathophysiology of elevated FGF23-induced hypophosphataemia. In addition, adherence to conventional therapy is limited by frequent daily dosing and side effects such as gastrointestinal symptoms, secondary hyperparathyroidism and nephrocalcinosis. Burosumab, a recombinant human IgG1 monoclonal antibody that binds to and inhibits the activity of FGF23, is administered subcutaneously every 2 weeks. In clinical trials (phase 2 and 3) burosumab was shown to improve phosphate homeostasis, that consequently resolves the skeletal/non-skeletal manifestations of XLH. Burosumab was licensed in Europe (February 2018) with NICE approving use within its marketing authorisation in October 2018. In this publication, the British Paediatric and Adolescent Bone Group (BPABG), reviewed current evidence and provides expert recommendations for care pathway and management of XLH with burosumab.

Open access

Jing Zhang, Zhiyong Zhao, Li Dong, Tao Han, Guojin Zhang, Yuntai Cao, and Junlin Zhou

Introduction and aim: It is difficult to distinguish between non-functioning pituitary macroadenomas (NFPMAs) and sellar meningiomas because of their overlapping imaging manifestations on routine magnetic resonance imaging (MRI), especially in cases of meningiomas growing into the saddle. Here, we aimed to differentiate between these two tumors using apparent diffusion coefficient (ADC) values and MRI characteristics.

Methods: A total of 60 NFPMA and 52 sellar meningioma cases confirmed by pathological analysis were retrospectively reviewed. All patients were examined via routine MRI and diffusion weighted imaging (DWI) before undergoing surgery. The clinical, MRI characteristics, and max ADC (ADCmax), average ADC (ADCmean), and minimum ADC (ADCmin) values were compared between the two tumors via Chi-square test and two sample t-tests. Receiver operating characteristic (ROC) curve and binary logistic regression analyses were conducted to determine the discrimination ability.

Results: The ADCmax, ADCmean, and ADCmin values were significantly higher in NFPMAs compared to sellar meningiomas (p < 0.001 for all). Among ADC values, ADCmax demonstrated good performance with an AUC of 0.896 (95% CI, 0.823-0.969) and accuracy of 88.7%. A cut-off value of 0.97 × 10−3 mm2/s was used for ADCmax for differentiation between tumors. A combination of ADCmax values and clinicoradiological features showed the best discrimination ability for differential diagnosis between the two tumors, with an AUC of 0.981 (95% CI, 0.958-1.000) and accuracy of 96.9%.

Conclusion: A combination of ADCmax and clinicoradiological features demonstrates good discrimination ability and high accuracy for differentiation between NFPMAs and sellar meningiomas, and is a potential quantitative tool to aid in the selection of surgical techniques.

Open access

Josef Köhrle

issues per year; fast review and processing times continue to be combined with an ambitious acceptance rate. Endocrine Connections covers original high-quality basic, translational and clinical research or review articles, covering all aspects of