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首页> 美国卫生研究院文献>Acta Bio Medica : Atenei Parmensis >An ICET-A survey on occult and emerging endocrine complications in patients with β-thalassemia major: Conclusions and recommendations
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An ICET-A survey on occult and emerging endocrine complications in patients with β-thalassemia major: Conclusions and recommendations

机译：ICET-A调查重型β地中海贫血患者的隐匿性和新兴内分泌并发症：结论和建议

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In adult thalassemia major (TM) patients, a number of occult and emerging endocrine complications, such as: central hypothyroidism (CH), thyroid cancer, latent hypocortisolism, and growth hormone deficiency (GHD) have emerged and been reported. As the early detection of these complications is essential for appropriate treatment and follow-up, the International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescent Medicine (ICET-A) promoted a survey on these complications in adult TM patients, among physicians (pediatricians, hematologists and endocrinologists) caring for TM patients in different countries. The data reported by 15 countries are presented. The commonest endocrine complications registered in 3.114 TM adults are CH and GHD (4.6 % and 3.0 %, respectively), followed by latent hypocortisolism (1.2%). In 13 patients (0.41%) a cytological papillary or follicular thyroid carcinoma was diagnosed in 11 and 2 patients, respectively, and a lobectomy or thyroidectomy was carried out. Of 202 TM patients below the age of 18 years, the reported endocrine complications were: GHD in 4.5%, latent hypocortisolism in 4.4% and central hypothyrodisim in 0.5%. Transition phase was an area of interest for many clinicians, especially as patients with complex chronic health conditions are responding to new treatments extending their lifespan beyond imagination.. In conclusion, our survey provides a better understanding of physicians’ current clinical practices and beliefs in the detection, prevention and treatment of some endocrine complications prevailing in adult TM patients. Regular surveillance, early diagnosis, treatment and follow-up in a multi-disciplinary specialized setting are recommended. ()
机译：在成人重型地中海贫血（TM）患者中，已经出现并报道了许多隐匿性和新兴的内分泌并发症，例如：中枢性甲状腺功能减退症（CH），甲状腺癌，潜在的皮质醇减退和生长激素缺乏症（GHD）。由于这些并发症的早期发现对于适当的治疗和随访至关重要，国际地中海贫血症和青少年内分泌病临床医生网络（ICET-A）推动了对成年TM患者中这些并发症的调查，其中包括医师（儿科医生，血液学家和内分泌学家）在不同国家照顾TM患者。列出了15个国家/地区报告的数据。在3.114 TM成年人中，最常见的内分泌并发症是CH和GHD（分别为4.6％和3.0％），其次是潜在的皮质醇减退（1.2％）。在13名患者（0.41％）中，分别在11名和2名患者中诊断出细胞学性乳头状或滤泡性甲状腺癌，并进行了肺叶切除或甲状腺切除。在202岁以下18岁以下的TM患者中，报告的内分泌并发症为：GHD为4.5％，潜在性皮质醇减退为4.4％，中枢甲状腺功能减退为0.5％。过渡期是许多临床医生感兴趣的领域，尤其是当患有复杂慢性健康状况的患者对新疗法的反应延长了他们的寿命，超出了他们的想象力时。总而言之，我们的调查可以更好地了解医生目前的临床实践和对治疗的信念。检测，预防和治疗成人TM患者中普遍存在的一些内分泌并发症。建议在多学科的专业环境中进行定期监测，早期诊断，治疗和随访。 （）

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