Jaffiol C, Baldet L, Torresani J, Bismuth J, Papachristou C
Service d'Endocrinologie, Hôpital Lapeyronie, Montpellier, France.
J Endocrinol Invest. 1990 Nov;13(10):839-45. doi: 10.1007/BF03349636.
A 52-year-old male presented himself with tachycardia crises which appeared first during childhood, increased in frequency without goiter or exophthalmos. Cardiac and adrenergic diseases were excluded. The thyroid function was normal regarding T4, free T4 and T3, TBG, radioiodine uptake, TSH and T3 suppressibility; however the TSH response to TRH was decreased. The lymphocyte nuclear T3 receptor was found with an affinity close to that of normal volunteers (Ka: 1.42 x 10(10) M-1 vs 1.95 +/- 0.35 x 10(10) M-1) and a binding capacity markedly increased (9.9 vs 3.7 +/- 0.4 fmol T3/100 micrograms DNA). Pindolol was inefficient on the dysrhythmia which disappeared with carbimazole and relapsed after withdrawal of the antithyroid drug. Under carbimazole, the plasma T4 markedly decreased (27.7 +/- 3.6 nmol/l) but the patient remained euthyroid. The clinical course and the laboratory data suggest that the tachycardia crises are the consequence of a hypersensitivity of the heart to thyroid hormones, associated with an increased number of T3 nuclear receptor sites in lymphocytes.
一名52岁男性出现心动过速危象，该症状始于童年时期，发作频率增加，无甲状腺肿或突眼症状。已排除心脏和肾上腺素能疾病。甲状腺功能在T4、游离T4、T3、甲状腺素结合球蛋白、放射性碘摄取、促甲状腺激素（TSH）及T3抑制试验方面均正常；然而，TSH对促甲状腺激素释放激素（TRH）的反应降低。发现淋巴细胞核T3受体的亲和力接近正常志愿者（解离常数Ka：1.42×10¹⁰ M⁻¹，正常志愿者为1.95±0.35×10¹⁰ M⁻¹），且结合能力显著增加（9.9 fmol T3/100μg DNA，正常志愿者为3.7±0.4 fmol T3/100μg DNA）。吲哚洛尔对心律失常无效，心律失常在使用卡比马唑后消失，停用抗甲状腺药物后复发。在使用卡比马唑期间，血浆T4显著降低（27.7±3.6 nmol/L），但患者仍处于甲状腺功能正常状态。临床病程和实验室数据表明，心动过速危象是心脏对甲状腺激素过敏的结果，同时伴有淋巴细胞中T3核受体位点数量增加。
