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甲状腺激素的部分外周抵抗

Partial peripheral resistance to thyroid hormone.

作者信息

Kaplan M M, Swartz S L, Larsen P R

出版信息

Am J Med. 1981 May;70(5):1115-21. doi: 10.1016/0002-9343(81)90885-8.

Abstract

A 33 year old partially thyroidectomized woman was euthyroid when ingesting 500 microgram of L-triiodothyronine (T3) daily. Her condition was evaluated during therapy with daily T3 doses between 50 and 500 microgram. She was hypothyroid and had a markedly subnormal oxygen consumption rate when taking 50 to 100 microgram T3 daily, and oxygen consumption did not increase greatly above predicted normal values despite serum T3 concentrations up to 3,200 ng/dl. Her pulse rate, blood pressure, systolic time intervals and exercise tolerance changed minimally and remained within the normal range during the different dosage schedules. Urinary creatine and hydroxyproline, indices of muscle and skeletal protein catabolism, increased normally with higher T3 doses, but serum cholesterol, creatine phosphokinase, calcium and alkaline phosphatase did not change substantially. Basal and thyrotropin-releasing hormone (TRH) stimulated thyrotropin secretion were suppressed during all T3 doses. The prolactin response to TRH was normal at 50 microgram T3/day and was reduced by higher doses of T3. Absorption of T3, serum T3 protein binding and T3 metabolic clearance rates were all within normal limits. The findings in this patient are compared to clinical and biochemical findings in 17 previously described patients. The manifestations of peripheral thyroid hormone resistance are quite variable in the organ systems involved and in the degree of involvement. The molecular basis of the abnormality in our patient remains undefined.

摘要

一名33岁的女性,曾接受部分甲状腺切除术,在每日摄入500微克左旋三碘甲状腺原氨酸(T3)时甲状腺功能正常。在每日T3剂量为50至500微克的治疗期间对其病情进行了评估。当每日服用50至100微克T3时,她处于甲状腺功能减退状态,氧消耗率明显低于正常水平,尽管血清T3浓度高达3200纳克/分升,但氧消耗并未比预测的正常值大幅增加。在不同的给药方案期间,她的脉搏率、血压、收缩期时间间期和运动耐量变化极小,仍保持在正常范围内。尿肌酸和羟脯氨酸是肌肉和骨骼蛋白分解代谢的指标,随着T3剂量增加而正常升高,但血清胆固醇、肌酸磷酸激酶、钙和碱性磷酸酶没有明显变化。在所有T3剂量下,基础促甲状腺激素分泌和促甲状腺激素释放激素(TRH)刺激的促甲状腺激素分泌均受到抑制。在每日50微克T3时,催乳素对TRH的反应正常,而更高剂量的T3会使其降低。T3的吸收、血清T3蛋白结合和T3代谢清除率均在正常范围内。将该患者的研究结果与之前描述的17例患者的临床和生化结果进行了比较。外周甲状腺激素抵抗的表现在所涉及的器官系统和受累程度方面差异很大。我们患者异常的分子基础仍不明确。

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