Stewart M F, Ratcliffe W A, Roberts I
Ann Clin Biochem. 1986 Jan;23 ( Pt 1):59-64. doi: 10.1177/000456328602300107.
Two patients with familial dysalbuminaemic hyperthyroxinaemia (FDH) are described in whom the albumin variant resulted in raised total T4 levels, and artefactually raised free T4 using a 'single-step' technique employing an analogue of T4 as tracer. The first patient was clinically euthyroid and presented with relapse of schizophrenia and abnormal thyroid function tests (total T4 336 nmol/L, total T3 4.2 nmol/L, TSH 1.8 mU/L, free T4 73 pmol/L). These results led to diagnostic confusion and the patient was treated with a short course of anti-thyroid drugs. The second patient had signs and symptoms of thyrotoxicosis at her first visit but was clinically euthyroid 5 months later when she was 10 weeks pregnant. Thyroid function tests were total T4 259 nmol/L, total T3 3.6 nmol/L, TSH 3.8 mU/L, free T4 46 pmol/L. Further studies showed both patients to be biochemically euthyroid. A variant albumin was confirmed in both patients by a screening test for FDH and by reverse-flow electrophoresis. Family studies on 10 relatives of the first patient identified eight with FDH. A simple screening procedure for the indentification of FDH is described and the use of laboratory tests in suspected cases is discussed.
本文描述了两名患有家族性异常白蛋白血症性甲状腺素血症(FDH)的患者,其白蛋白变体导致总T4水平升高,并使用以T4类似物作为示踪剂的“单步法”技术人为地提高了游离T4水平。第一名患者临床甲状腺功能正常,因精神分裂症复发和甲状腺功能检查异常就诊(总T4 336 nmol/L,总T3 4.2 nmol/L,促甲状腺激素[TSH] 1.8 mU/L,游离T4 73 pmol/L)。这些结果导致诊断混乱,该患者接受了短期抗甲状腺药物治疗。第二名患者初诊时有甲状腺毒症的体征和症状,但在怀孕10周、5个月后临床甲状腺功能正常。甲状腺功能检查结果为总T4 259 nmol/L,总T3 3.6 nmol/L,TSH 3.8 mU/L,游离T4 46 pmol/L。进一步研究表明两名患者生化检查甲状腺功能均正常。通过FDH筛查试验和反流电泳在两名患者中均证实存在白蛋白变体。对第一名患者的10名亲属进行的家族研究发现其中8人患有FDH。本文描述了一种用于识别FDH的简单筛查程序,并讨论了在疑似病例中实验室检查的应用。