TY - JOUR
T1 - Congenital goiter and the development of metastatic follicular carcinoma with evidence for a leak of nonhormonal iodide
T2 - Clinical, pathological, kinetic, and biochemical studies and a review of the literature
AU - Cooper, David S.
AU - Axelrod, Lloyd
AU - Degroot, Leslie J.
AU - Vickery, Austin L.
AU - Maloof, Farahe
PY - 1981/2
Y1 - 1981/2
N2 - We report a large kindred of patients with congenital goiter, followed for 15 yr, in which two siblings (one male and one female) developed metastatic follicular thyroid carcinoma. These two patients were evaluated by iodine kinetic analysis. None of the classical defects of T4 biosynthesis was present in either patient. Rather, both patients had extremely rapid rates of iodine turnover, with elevated 131I uptake and excessive spillage of iodide in the urine. Serum iodoalbumin was present, probably as a nonspecific result of glandular hyperplasia. Iodine kinetic analysis after the ingestion of potassium perchlorate and methimazole was compatible with a leak of nonhormonal iodide from the thyroid. It is not possible to determine whether this iodide leak is the primary pathogenetic defect or is secondary to another unidentified abnormality.
AB - We report a large kindred of patients with congenital goiter, followed for 15 yr, in which two siblings (one male and one female) developed metastatic follicular thyroid carcinoma. These two patients were evaluated by iodine kinetic analysis. None of the classical defects of T4 biosynthesis was present in either patient. Rather, both patients had extremely rapid rates of iodine turnover, with elevated 131I uptake and excessive spillage of iodide in the urine. Serum iodoalbumin was present, probably as a nonspecific result of glandular hyperplasia. Iodine kinetic analysis after the ingestion of potassium perchlorate and methimazole was compatible with a leak of nonhormonal iodide from the thyroid. It is not possible to determine whether this iodide leak is the primary pathogenetic defect or is secondary to another unidentified abnormality.
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U2 - 10.1210/jcem-52-2-294
DO - 10.1210/jcem-52-2-294
M3 - Article
C2 - 7462393
AN - SCOPUS:0019380270
SN - 0021-972X
VL - 52
SP - 294
EP - 306
JO - Journal of Clinical Endocrinology and Metabolism
JF - Journal of Clinical Endocrinology and Metabolism
IS - 2
ER -