De Sanctis Vincenzo, Tanas Rita, Gamberini Maria Rita, Sprocati Monica, Govoni Maria Rita, Marsella Maria
Department of Reproduction, Paediatric and Thalassaemia Unit, St. Anna Hospital, Ferrara, Italy.
Pediatr Endocrinol Rev. 2008 Oct;6 Suppl 1:170-3.
The severity of thyroid dysfunction in patients with beta-thalassaemia major is variable in different series. An exaggerated thyrotropin response to thyrotropin-releasing-hormone (TRH) has been found in 1 out of 5 beta-thalassaemia major patients. Because it is not well known how many of these patients will develop overt or subclinical hypothyroidism, we reviewed retrospectively the thyroid status (thyroid hormones, TBG, TG basal and TSH peak after TRH test) of 24 children and adolescents with beta-thalassemia major (mean age 12.1+/-3.9 years) on periodic transfusion therapy, regularly followed in our Centre during the last 20 years. As controls we studied 30 normal subjects aged 13.4+/-2.5 years. In our group of 24 thalassaemics an exaggerated TSH response to TRH test was found in 8 (33.3%), 3 of whom developed subclinical or overt hypothyroidism from 3 to 11 years later. TSH peak values correlated directly with ferritin levels, ALT, and compliance index to chelation therapy. Our data suggest that an exaggerated TSH response to TRH test is frequent in beta-thalassemia major, and may evolve into subclinical or overt hypothyroidism as we found in 37.5% of our patients with sub-biochemical hypothyroidism.
重型β地中海贫血患者甲状腺功能障碍的严重程度在不同系列研究中有所不同。在五分之一的重型β地中海贫血患者中发现促甲状腺激素对促甲状腺激素释放激素（TRH）的反应过度。由于尚不清楚这些患者中有多少人会发展为显性或亚临床甲状腺功能减退，我们回顾性分析了24例接受定期输血治疗的重型β地中海贫血儿童和青少年（平均年龄12.1±3.9岁）的甲状腺状况（甲状腺激素、甲状腺素结合球蛋白、基础甲状腺球蛋白和TRH试验后的促甲状腺激素峰值），这些患者在过去20年中一直在我们中心定期随访。作为对照，我们研究了30名年龄在13.4±2.5岁的正常受试者。在我们的24例地中海贫血患者组中，8例（33.3%）TRH试验中促甲状腺激素反应过度，其中3例在3至11年后发展为亚临床或显性甲状腺功能减退。促甲状腺激素峰值与铁蛋白水平、谷丙转氨酶以及螯合治疗依从指数直接相关。我们的数据表明，重型β地中海贫血患者中TRH试验促甲状腺激素反应过度很常见，并且可能发展为亚临床或显性甲状腺功能减退，正如我们在37.5%亚生化甲状腺功能减退患者中所发现的那样。
