Jackowski Tomasz, Petriczko Elżbieta, Horodnicka-Józwa Anita, Walczak Mieczysław
Klinika Pediatrii, Endokrynologii, Diabetologii, Chorób Metabolicznych i Kardiologii Wieku Rozwojowego PUM.
Pediatr Endocrinol Diabetes Metab. 2017;23(4):209-214. doi: 10.18544/PEDM-23.04.0095.
Thyroid hormone resistance syndrome, also known as Refetoff syndrome, is a rare disease associated with decreased reaction of body tissues to thyroid hormones (TH). Patients with Refetoff syndrome tend to have elevated free TH concentrations and normal or inadequately elevated TSH (caused by thyrotropic cells in the pituitary gland insensitivity to TH). The cause of the disease is the mutation in TR-beta receptor gene. Depending on the clinical presentation generalised and pituitary resistance to TH are described. The syndrome is often misdiagnosed as hyperthyroidism and unnecessarily treated with anti-thyroid drugs. Some patients receive l-thyroxine treatment for apparent hypothyroidism. In this report, three patients are presented with a long history in our Clinic and Outpatient Clinic.
甲状腺激素抵抗综合征,也称为Refetoff综合征,是一种罕见疾病,与身体组织对甲状腺激素(TH)反应降低有关。Refetoff综合征患者往往游离TH浓度升高,促甲状腺激素(TSH)正常或升高不足(由垂体中的促甲状腺细胞对TH不敏感引起)。该病的病因是TR-β受体基因突变。根据临床表现可分为全身性和垂体性TH抵抗。该综合征常被误诊为甲状腺功能亢进症,并被不必要地使用抗甲状腺药物治疗。一些患者因明显的甲状腺功能减退症而接受左甲状腺素治疗。在本报告中,介绍了在我们的诊所和门诊有长期病史的三名患者。