Improvement in the care of multiple endocrine neoplasia Type 1 through a regional multidisciplinary clinic

门1 医学 多发性内分泌肿瘤 无症状的 多学科方法 疾病 儿科 普通外科 重症监护医学 内分泌系统 内科学 社会科学 生物化学 化学 社会学 激素 基因
作者
Helen White,Blair Jc,Jonathan Pinkney,Daniel J. Cuthbertson,Ryan W. Day,Astrid Weber,I. A. MacFarlane
出处
期刊:QJM: An International Journal of Medicine [Oxford University Press]
卷期号:103 (5): 337-345 被引量:9
标识
DOI:10.1093/qjmed/hcq020
摘要

Multiple endocrine neoplasia type 1 (MEN1) is associated with significant morbidity and mortality. Timely detection of MEN1 kindred, together with treatment of associated tumours, results in an improved outcome. We describe how the development of a dedicated multidisciplinary MEN clinic has improved the diagnosis and treatment of MEN1-associated endocrinopathies.A dedicated MEN clinic was developed at Aintree University Hospital, Liverpool in 2002 for patients living in Merseyside, Cheshire and North Wales. The multidisciplinary approach adopted, aimed to improve communication and continuity of care. Patients see all clinicians involved in their care (Consultant Endocrinologist, Paediatrician, Clinical Geneticist and Endocrine Surgeon) simultaneously, allowing for a unified, clear approach and a reduction in unnecessary attendances. The clinicians adopt a proactive approach to tracing the relatives of patients, with the aim of identifying kindred with previously asymptomatic disease.In 2002, 16 patients from 5 families were diagnosed clinically with MEN1. Twenty MEN1-associated endocrinopathies had been diagnosed and 21 surgical procedures had been performed. By the end of 2008, 45 patients from 15 families had been identified, with 83 endocrinopathies diagnosed and 50 surgical procedures performed. Ninety-four known relatives are awaiting screening for MEN1.The successful identification of patients with MEN1 has resulted in an exponential increase in the number of patients attending the clinic. As relatives undergo screening, the diagnosis of MEN is likely to increase. The ever increasing numbers of patients requiring screening, surveillance and treatment has implications in the planning of future service provision.
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