Congenital Goiter with Defective Iodide Transport

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Abstract
THE TWENTY-TWO known cases of congenital goiter, with or without cretinism, that are due to defective iodide concentrating ability have been compared. The defect may be total or partial, sometimes becomes clinically manifest long after the neonatal period, shows male predominance, and frequently occurs without evidence of consanguinity or partial defects in the parents. The degree of clinical or laboratory hypothyroidism may vary but radioiodide uptake is low or absent. The diagnosis is based on the failure to concentrate iodide in salivary or gastric secretions on the one hand, and the attainment of the euthyroid state by iodide replacement alone. Limited evidence suggests that the defect is due to the absence or malfunction of the iodide carrier. The ion exchange basis of thyroidal anion selectivity dictates certain properties for the hypothetical iodide carrier which are dicussed. Introduction The concept that iodide transport is an independent step in the biosynthesis of the thyroid hormones was first proposed in the laboratories of Leblond and Chaikoff (1, 2) on the basis of experiments demonstrating accumulation of radioiodine in thyroids in which organic iodine formation had been blocked by antithyroid agents.

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