Abstract
Congenital goitrous hypothyroidism may be caused by a metabolic bloc in the synthesis of the thyroid hourmones or by an abnormal synthesis of iodinated proteins in the thyroid. Previously we have utilized labeling in vitro with 3H-leucine and Na125I in the study of the normal synthesis of 17-19 S thyroglobulin in the human fetal thyroid gland (Olin, Vecchio, Ekholm, and Almqvist: Endocrinology, 86: 1041, 1970). This system has been applied to 2-10 mg specimens from congenital goiters obtained by percutaneous biopsy.
This report deals with two children with a pathological percholorate-induced discharge of radioiodine. The first patient was an 18-day-old boy with diffuse goiter and hypothyroidism. The biochemical analysis revealed a normal uptake in vitro of 125I. No 125I was present in the crude extract after dialysis. Sucrose density gradient centrifugation of the soluble proteins, however, showed a distinct label of 3H in the 17-19 S region. The 3H-radioactivity was specifically precipitated by antiserum against human adult thyroglobulin.
The second patient was a 4-year-old girl with goiter and hypothyroidism since at least I year of age. At that time the perchlorate test was performed before treatment. The biochemical studies were performed while the patient was treated with thyroxine. Biopsies were obtained before and after stimulation with TSH (Actyron I IU/d for 5 days). Before TSH stimulation no iodide was accumulated in vitro and a slight 3H-peak was prescent in the 17-19 S region of the sucrose density gradient. After TSH stimulation the thyroid tissue did accumulate 125I, but only 3H was incorporated into the 17-19 S proteins.
The results indicate a normal synthesis of the protein moiety of thyroglobulin in these two congenital goiters with a defect in the organification of iodine. This is at variance with the current opinion that this type of goiter is caused by a deficient systhesis of thyroglobulin and a pathological formation of thyralbumin.
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Olin, P. 12. Studies on thyroid proteins in congenital goiter. Pediatr Res 5, 85 (1971). https://doi.org/10.1203/00006450-197102000-00017
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DOI: https://doi.org/10.1203/00006450-197102000-00017