De Sanctis Vincenzo, Soliman Ashraf T, Canatan Duran, Tzoulis Ploutarchos, Daar Shahina, Di Maio Salvatore, Elsedfy Heba, Yassin Mohamed A, Filosa Aldo, Soliman Nada, Mehran Karimi, Saki Forough, Sobti Praveen, Kakkar Shruti, Christou Soteroula, Albu Alice, Christodoulides Constantinos, Kilinc Yurdanur, Al Jaouni Soad, Khater Doaa, Alyaarubi Saif A, Lum Su Han, Campisi Saveria, Anastasi Salvatore, Galati Maria Concetta, Raiola Giuseppe, Wali Yasser, Elhakim Ihab Z, Mariannis Demetris, Ladis Vassilis, Kattamis Christos
Quisisana Hospital, Ferrara.
Acta Biomed. 2019 Jan 15;89(4):481-489. doi: 10.23750/abm.v89i4.7774.
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.
在成年重型地中海贫血（TM）患者中，出现并报告了一些隐匿性和新出现的内分泌并发症，如：中枢性甲状腺功能减退（CH）、甲状腺癌、潜在性皮质醇缺乏症和生长激素缺乏症（GHD）。由于这些并发症的早期检测对于适当的治疗和随访至关重要，地中海贫血和青少年医学内分泌疾病国际临床医生网络（ICET-A）在不同国家照顾TM患者的医生（儿科医生、血液科医生和内分泌科医生）中开展了一项关于成年TM患者这些并发症的调查。本文展示了15个国家报告的数据。在3114名成年TM患者中登记的最常见内分泌并发症是CH和GHD（分别为4.6%和3.0%），其次是潜在性皮质醇缺乏症（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上的乳头状或滤泡状甲状腺癌，并进行了肺叶切除术或甲状腺切除术。在202名18岁以下的TM患者中，报告的内分泌并发症有：GHD占4.5%，潜在性皮质醇缺乏症占4.4%，中枢性甲状腺功能减退占0.5%。过渡阶段是许多临床医生感兴趣的领域，特别是随着患有复杂慢性健康状况的患者对新治疗方法产生反应，其寿命延长超出想象。总之，我们的调查有助于更好地了解医生目前在检测、预防和治疗成年TM患者中普遍存在的一些内分泌并发症方面的临床实践和观点。建议在多学科专业环境中进行定期监测、早期诊断、治疗和随访。
