Abstract
The Syndrome of inappropriate secretion of TSH is a rare disease and has been described as a heterogenous group of disorders in juvenile and adult patients who show elevated TSH levels in the presence of elevated thyroid hormone levels.
We have observed an infant at age six weeks with congenital goiter who suffered from typical symptoms of hyperthyroidism. In spite of elevated T3 and T4 levels TSH showed basal values between 2 and 8 mU/ml. After TRH-stimulation TSH ranged between 39 and 100 mU/ml. Pituitary adenoma was ruled out; there appeared no relation to Graves' disease.
A long term therapeutic trial was performed with the analogue D-thyroxine. TSH gradually returned to normal and the goiter disappeared. After 6 years of observation the physical and psycho-intellectual development and the bone age have been stated to be normal for chronological age, although laboratory data indicated elevated thyroid hormone levels for years.
CONCLUSION: Up to now there is evidently no other report on congenital hyperthyroidism due to inappropriate TSH-secretion. Partial pituitary and peripheral thyroid hormone resistance appears to be likely. The long term therapeutic trial with D-T4 has proven to be successful.
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Klett, M., Schönberg, D. CONGENITAL HYPERTHYROIDISM DUE TO INAPPROPRIATE SECRETION OF THYROID STIMULATING HORMONE. Pediatr Res 23, 134 (1988). https://doi.org/10.1203/00006450-198801000-00200
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DOI: https://doi.org/10.1203/00006450-198801000-00200