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A Review of Endocrine Disorders in Thalassaemia

Open Journal of Endocrine and Metabolic Diseases 2014年第2期4卷 25-34页

作者： Parijat De Radhika Mistry Christine Wright Shivan Pancham wyn burbridge Kalyan Gangopadhayay Terence Pang Gautam Das Department of Diabetes and Endocrinology Southern General Hospital Glasgow UK Department of Diabetes Endocrinology and Lipid Metabolism City Hospital Birmingham UK Sickle Cell and Thalassaemia Unit City Hospital Birmingham UK

Endocrine dysfunction in thalassaemia is amongst the most common complication and is principally attributed to excessive iron overload and suboptimal chelation. The prevalence is quite high particularly in multiethnic populations but determining the prevalence is often difficult due to the widespread heterogeneity of the population and timing of exposure to chelation therapy. Disturbances in growth, pubertal development, abnormal gonadal functions, impaired thyroid, parathyroid and adrenal functions, diabetes and disorderly bone growth are commonly encountered. Early detection and institution of appropriate transfusion regimen and chelation therapy and treatment of complications are the keys to managing this population including regular follow. In this article, we review the literature in relation to the various endocrine complications encountered in thalassaemia.

关键词： Thalassaemia Chelation Endocrinopathies Diabetes Hypothyroidism

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Validation of the Daily Diary of Fatigue Symptoms—Fibromyalgia (DFS-Fibro)

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Open Journal of Rheumatology and Autoimmune Diseases 2013年第2期3卷 92-103页

作者： Claire burbridge Tara Symonds Louise Humphrey Rob Arbuckle Ian Hirsch Laurence Whelan Adelphi Values Ltd. Bollington UK AstraZeneca UK Limited Macclesfield UK Pfizer Global R&D Sandwich UK Pfizer Ltd. Walton Oaks UK

Introduction: Fatigue is an important fibromyalgia (FM) symptom, but existing measures of fatigue are unlikely to meet regulatory standards for clinical trial use. We describe the development and validation of the Daily Diary of Fatigue Symptoms—Fibromyalgia (DFS-Fibro), a 24-hour recall, patient-reported outcome (PRO) measure of fatigue in FM that is administered electronically (ePRO). Methods: There were 3 phases of work: 1) item generation based on concept elicitation interviews with FM patients, with clinical relevance confirmed by expert clinician review;2) pilot testing/cognitive debriefing interviews with FM patients;and 3) psychometric validation using data from a methodology study with 145 FM patients. The measure was finalised based on both qualitative and quantitative findings. Results: Twenty-three items were generated in phase 1, some minor revisions were made following the pilot testing and cognitive debriefing (phase 2) but none were deleted. All patients found the measure easy to understand and use. Item reduction was conducted taking into account both the initial psychometric data and the earlier qualitative research, resulting in a final 5 item measure of the “symptom” of fatigue. The 5-item DFS-Fibro had very high internal consistency (alpha = 0.99), and strong test-re-test reliability (r > 0.84), convergent validity and known-groups validity. Conclusion: The DFS-Fibro has strong psychometric properties and strong face and content validity for the measurement of fatigue in FM.

关键词： Fatigue Fibromyalgia Patient Reported Psychometric Validation Qualitative Questionnaire Development Diary ePRO

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Rotational assisted endoscopic retrograde cholangiopancreatography in patients with reconstructive gastrointestinal surgical anatomy

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World Journal of Gastrointestinal Endoscopy 2015年第3期7卷 278-282页

作者： Majed El Zouhairi James B Watson Svetang V Desai David K Swartz Alejandra Castillo-Roth Mahfuzul Haque Paul S Jowell Malcolm S Branch Rebecca A burbridge Division of Gastroenterology Duke University Medical Center Northern New Mexico Gastroenterology Associates

AIM: To evaluate the success rates of performing therapy utilizing a rotational assisted enteroscopy device in endoscopic retrograde cholangiopancreatography(ERCP) in surgically altered anatomy patients. METHODS: Between June 1, 2009 and November 8, 2012, we performed 42 ERCPs with the use of rotational enteroscopy for patients with altered anatomy(39 with gastric bypass Roux-en-Y, 2 with Billroth Ⅱ gastrectomy, and 1 with hepaticojejunostomy associated with liver transplant). The indications for ERCP were: choledocholithiasis: 13 of 42(30.9%), biliary obstruction suggested on imaging: 20 of 42(47.6%), suspected sphincter of Oddi dysfunction: 4 of 42(9.5%), abnormal liver enzymes: 1 of 42(2.4%), ascending cholangitis: 2 of 42(4.8%), and bile leak: 2 of 42(4.8%). All procedures were completed with the Olympus SIF-Q180 enteroscope and the Endo-Ease Discovery SB overtube produced by Spirus Medical. RESULTS: Successful visualization of the major ampulla was accomplished in 32 of 42 procedures(76.2%). Cannulation of the bile duct was successful in 26 of 32 procedures reaching the major ampulla(81.3%). Successful therapeutic intervention was completed in 24 of 26 procedures in which the bileduct was cannulated(92.3%). The overall intention to treat success rate was 64.3%. In terms of cannulation success, the intention to treat success rate was 61.5%. Ten out of forty two patients(23.8%) required admission to the hospital after procedure for abdominal pain and nausea, and 3 of those 10 patients(7.1%) had a diagnosis of post-ERCP pancreatitis. The average hospital stay was 3 ***: It is reasonable to consider an attempt at rotational assisted ERCP prior to a surgical intervention to alleviate biliary complications in patients with altered surgical anatomy.

关键词： Gastric bypass Gastrostomy Cholangiopancreatography Endoscopic retrograde Double-Balloon enteroscopy Ampulla of vater Sphincterotomy Endoscopic Pancreatitis Retrospective studies

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