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因肝血管肿瘤导致的甲状腺功能减退症在无甲状腺的成年患者中出现。

Consumptive hypothyroidism resulting from hepatic vascular tumors in an athyreotic adult.

机构信息

Department of Medicine, Division of Endocrinology, University of Colorado School of Medicine, Aurora, Colorado 80045, USA.

出版信息

J Clin Endocrinol Metab. 2011 Jul;96(7):1966-70. doi: 10.1210/jc.2010-2104. Epub 2011 Apr 20.

Abstract

CONTEXT

Consumptive hypothyroidism is a rare syndrome resulting from increased catabolism of T(4) and T(3) by increased type 3 iodothyronine deiodinase (D3) activity. Consumptive hypothyroidism has primarily been described as a paraneoplastic syndrome in infants as well as in two adults with D3-expressing tumors.

OBJECTIVE

The aim of the study was to report the third case of consumptive hypothyroidism in an adult and the first in an athyreotic patient.

DESIGN, SETTING, AND PATIENT: We present a 38-yr-old athyreotic female who was euthyroid on a stable therapeutic dose of thyroid hormone for many years and then developed marked hyperthyrotropinemia, coincident with the discovery of large D3-expressing hepatic vascular tumors. The patient also had low serum T(3) and elevated serum rT(3). Hyperthyrotropinemia transiently worsened after surgical resection of the vascular tumors and then persisted for 3 wk after the operation, despite further increases in levothyroxine therapy.

INTERVENTION

The patient's vascular tumor and adjacent normal liver parenchyma were probed with a polyclonal antibody directed against D3.

MAIN OUTCOME MEASURES AND RESULTS

D3 immunostaining of the patient's vascular tumor was positive, with no significant immunoreactivity in the adjacent normal hepatic tissue.

CONCLUSIONS

This is the third case report of consumptive hypothyroidism in an adult and the first in an athyreotic individual. This case demonstrates that hyperthyrotropinemia may persist after partial liver resection, possibly from the hepatic resection itself.

摘要

背景

消耗性甲状腺功能减退症是一种罕见的综合征,由 3 型甲状腺素脱碘酶 (D3) 活性增加导致 T(4) 和 T(3) 分解代谢增加所致。消耗性甲状腺功能减退症主要在婴儿和两名表达 D3 的肿瘤的成年患者中被描述为副肿瘤综合征。

目的

本研究旨在报告第三例成年消耗性甲状腺功能减退症病例,也是首例无甲状腺患者病例。

设计、设置和患者:我们报告了一位 38 岁的无甲状腺女性患者,多年来一直服用稳定剂量的甲状腺激素维持甲状腺功能正常,随后出现明显的促甲状腺激素升高,同时发现存在大量表达 D3 的肝血管肿瘤。患者的血清 T(3) 水平较低,rT(3) 水平升高。血管肿瘤手术后,促甲状腺激素短暂恶化,随后尽管进一步增加了左甲状腺素治疗,仍持续 3 周。

干预

用针对 D3 的多克隆抗体探查患者的血管肿瘤及其邻近的正常肝组织。

主要观察指标和结果

患者血管肿瘤的 D3 免疫染色阳性,而邻近的正常肝组织无明显免疫反应。

结论

这是第三例成年消耗性甲状腺功能减退症病例报告,也是首例无甲状腺个体病例报告。本病例表明,部分肝切除术后促甲状腺激素可能持续存在,可能与肝切除本身有关。

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