A Case of Hypo-Responsiveness to Thyroid Hormone
A 16-month-old male patient with severe growth and mental retardation is reported. Although his thyroid function tests showed high circulating levels of free 3, 5, 3′-triiodothyronine (T3), free thyroxin (T4) and thyrotropin (TSH), he had no clinical manifestations suggesting hyperthyroidism. Physic...
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| Published in | Clinical Pediatric Endocrinology Vol. 5; no. 1; pp. 17 - 21 |
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| Main Authors | , , , , , , , , , |
| Format | Journal Article |
| Language | English |
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The Japanese Society for Pediatric Endocrinology
1996
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| Abstract | A 16-month-old male patient with severe growth and mental retardation is reported. Although his thyroid function tests showed high circulating levels of free 3, 5, 3′-triiodothyronine (T3), free thyroxin (T4) and thyrotropin (TSH), he had no clinical manifestations suggesting hyperthyroidism. Physical responses to exogenous T3 were normal. T4 supplement (7-10μg/kg/day) dramatically improved his growth and mental development, suggesting that his clinical symptoms were due to a relative shortage of thyroid hormone. Direct sequencing confirmed that he carried a normal thyroid hormone receptor β (TRβ) gene. The mechanism causing hypo-responsiveness to thyroid hormone in this patient is unclear. |
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| AbstractList | A 16-month-old male patient with severe growth and mental retardation is reported. Although his thyroid function tests showed high circulating levels of free 3, 5, 3′-triiodothyronine (T3), free thyroxin (T4) and thyrotropin (TSH), he had no clinical manifestations suggesting hyperthyroidism. Physical responses to exogenous T3 were normal. T4 supplement (7-10μg/kg/day) dramatically improved his growth and mental development, suggesting that his clinical symptoms were due to a relative shortage of thyroid hormone. Direct sequencing confirmed that he carried a normal thyroid hormone receptor β (TRβ) gene. The mechanism causing hypo-responsiveness to thyroid hormone in this patient is unclear. A 16-month-old male patient with severe growth and mental retardation is reported. Although his thyroid function tests showed high circulating levels of free 3, 5, 3'-triiodothyronine (T3), free thyroxine (T4) and thyrotropin (TSH), he had no clinical manifestations suggesting hyperthyroidism. Physical responses to exogenous T3 were normal. T4 supplement (7-10 μg/kg/day) dramatically improved his growth and mental development, suggesting that his clinical symptoms were due to a relative shortage of thyroid hormone. Direct sequencing confirmed that he carried a normal thyroid hormone receptor β(TRβ) gene. The mechanism causing hypo-responsiveness to thyroid hormone in this patient is unclear. |
| Author | Mori, Tetsuo Wakabayashi, Yasunobu Sakurai, Akihiro Washizawa, Kazuhiko Matsuzawa, Shigeyuki Hashizume, Kiyoshi Komiyama, Atsushi Sekiguchi, Yukio Kitazawa, Yumi Baba, Atsushi |
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| References | 8. Rösler A, Litvin Y, Hage C, Gross J, Cerasi E. Familial hyperthyroidism due to inappropriate thyrotropin secretion successfully treated with triiodothyronine. J Clin Endocrinol Metab 1982; 54: 76-82. 9. Wartofsky L, Burman KD. Alterations in thyroid function in patients with systemic illness: the “euthyroid sick syndrome.” Endocr Rev 1982; 3: 164-217. 3. Fisher DA, Dussault JH, Foley TP, Klein All, LaFranchi S, Larsen PR, et al, Screening for congenital hypothyroidism: Results of screening one million North American infants. J Pediatr 1979; 94: 700-5. 11. Ruiz M, Rajatanavin R, Young RA, Taylor C, Brown R, Braverman LE et al. Familial dysalbuminemic hyperthyroxinemia: a syndrome that can be confused with thyrotoxicosis. N Engl J Med 1982; 306: 635-9. 4. Fisher DA, Polk DH, Thyroid disease in the fetus, neonate, and child. In: DeGroot LJ, editor. Endocrinology Philadelphia: WB Saunders 1994: 783-98. 10. Refetoff S. Inherited thyroxine-binding globulin abnormalities in man. Endocr Rev 1989; 10: 275-93. 16. Refetoff S, Weiss RE, Usala SJ, Hayashi Y, The syndromes of resistance to thyroid hormone: Update 1994. In: Negro-Vilar A, editor. Endocrine reviews monographs, 3: Clinical and molecular aspects of diseases of the thyroid, Bethesda: Endocr Soc, 1994: 336-43. 6. Refetoff S, Weiss RE, Usala SJ. The syndromes of resistance to thyroid hormone. Endocr Rev 1993; 14: 348-99. 1. Utiger RD, Hypothyroidism. In: DeGroot LJ, editor. Endocrinology Philadelphia: WB Saunders 1994: 752-68. 2. Fisher DA, The thyroid. In: Brook C, editor. Clinical paediatric endocrinology. Oxford: Blackwell Scientific Publications 1989: 309-37. 12. Moses C, Lawlor J, Haddow J, Jackson IMD. Familial euthyroid hyperthyroxinemia resulting from increased thyroxine binding to thyroxine-binding prealbumin. N Engl J Med 1982; 306: 966-9. 5. Stubbe P, Gatz J, Heidemann P, Muhlen A, Hesch R. Thyroxine-binding globulin, triiodothyronine, thyroxin and thyrotropin in newborn infants and children. Horm Metab Res 1978; 10: 58-61. 14. Staeheli V, Vallotton MB, Burger A. Detection of human anti-thyroxine antibodies in different thyroid conditions. J Clin Endocrinol Metab 1975; 41: 669-75. 7. Gesundheit T, Petrick PA, Nissim M, Dahlberg PA, Doppman JL, Emerson CH, et al. Thyrotropin-secreting pituitary adenomas: Clinical and biochemical heterogeneity. Case report and follow-up of nine patients. Ann Intern Med 1989; 111: 827-35. 15. Sakurai A, Miyamoto T, Refetoff S, DeGroot LJ. Dominant negative transcriptional regulation by a mutant thyroid hormone receptor-β in a family with generalized resistance to thyroid hormone. Mol Endocrinol 1990; 4: 1988-94. 13. Sakata S, Nakamura S, Miura K. Autoantibodies against thyroid hormones or iodo-thyronine. Implications in diagnosis, thyroid function, treatment and pathogenesis. Ann Intern Med 1985; 14: 123-30. |
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