SPORADIC HYPOTHYROIDISM ASSOCIATED WITH GOITER*

Abstract

THE occasional association of goiter with hypothyroidism in nonendemic areas has been known for many years. However, it was not until 1943 that Hamilton, Soley, Reilly and Eichorn (1) first reported functional studies using radioactive iodine in 2 hypothyroid patients with goiter. In 1946 Lerman, Jones and Calkins (2) reported studies on 2 sporadic cretinous brothers with goiters. In 1950 appeared Stanbury and Hedge's (3) now classic studies on 3 of 4 goitrous cretins in a single family. Subsequently Stanbury and his associates (4–7) have contributed much to the understanding of the disturbance in thyroid synthesis in these patients. Hubble (8), McGirr and Hutchison (9, 10), Wilkins et al. (11), Jackson (12), Zondek et al. (13), Burrell and Gairdner (14), and Levy et al. (15) have also reported various clinical features and functional studies in patients with thyroid enlargement associated with hypothyroidism. It is now generally accepted that these patients have a defect in synthesis of thyroid hormone. In some of the reports this has been documented beyond any reasonable doubt (3–7). Since the defect may be in any one step of the complex chain of reactions which lead to the final elaboration and discharge of the thyroid hormone, one would predict that many different functional types of goitrous cretins would occur. And indeed, this has been amply demonstrated by the investigations of Stanbury and associates (3–7).