The pathology of dyshormonogenetic goitre
The pathology of dyshormonogenetic goitre
复制标题
激素性甲状腺肿的病理学
作者：
J. Kennedy
近年来，由于激素合成过程中的酶缺陷，人们对甲状腺肿的临床和生化方面产生了相当大的兴趣，并有全面的综述文章（McGirr, 1960; Murray and McGirr, 1964; Stanbury, 1966; McGirr and Thomson, 1968）。生化异常尚未明确与甲状腺结构改变有关。Smith（1960）报道了一系列14例碘化物组织缺陷的病例，也有一些关于这种或其他缺陷的小群体或单个病例的简短报道。本文通过对30例甲状腺发育异常和3例家族性甲状腺病变原因不明的甲状腺切除标本的个人评估，试图将甲状腺结构变化与已建立的缺陷联系起来。生物合成缺陷有：(1)形成异常碘蛋白16例；(2)单碘酪氨酸（MIT）和二碘酪氨酸（DIT）脱碘缺陷8例；(3)碘-6组织缺陷病例。大多数病例的临床和生化细节已被报道（Hutchison and McGirr, 1956; McGirr, Hutchison and Clement, 1959b; McGirr et al., 1960; Murray et al.， 196%和b）。
THERE has been considerable recent interest in the clinical and biochemical aspects of goitre due to enzyme defects in hormone synthesis, and comprehensive review articles are available (McGirr, 1960; Murray and McGirr, 1964; Stanbury, 1966; McGirr and Thomson, 1968). The biochemical abnormalities have not yet been related definitively to structural changes in the thyroid gland. Smith (1960) reported a series of 14 cases with defective organification of iodide and there are a number of brief reports on small groups or single cases of this and other defects. The present paper is an attempt to correlate the structural changes in the gland with the established defects, based on personal assessment of thyroidectomy specimens in 30 cases of goitre due to dyshormonogenesis and 3 cases of familial goitre in which the cause of the lesion is unknown. The defects in biosynthesis are: (1) Formation of an abnormal iodoprotein-16 cases; (2) defective deiodination of monoiodotyrosine (MIT) and diiodotyrosine (DIT) -8 cases; and (3) defective organification of iodine-6 cases. Clinical and biochemical details of the majority of the cases have been reported (Hutchison and McGirr, 1956; McGirr, Hutchison and Clement, 1959b; McGirr et al., 1960; Murray et al., 196% and b).