Skip to main content
NCBI home page
Archives of Disease in Childhood logoLink to Archives of Disease in Childhood
. 1980 Mar;55(3):207–212. doi: 10.1136/adc.55.3.207

Peripheral insensitivity to thyroid hormones in a euthyroid girl with goitre.

J Mäenpää, K Liewendahl
PMCID: PMC1626762  PMID: 6247978

Abstract

A 9-year-old girl was euthyroid with a small goitre, exophthalmos, scaphocephalic skull, minor sketelal abnormalities, and raised serum thyroid hormone concentrations. Other members of the family did not have goitres and their thyroid hormone levels were normal. From age 3 years the patient was treated for Graves's disease, but after 4 years treatment was stopped because of enlargement of the goitre. Despite increased serum thyroxine (T4), free T4 (FT4), and triiodothyronine (T3), basal serum TSH, and the TSH response to thyrotropin-releasing hormone (TRH) were normal. Pituitary refractoriness was present because full suppression of the TSH response to TRH was achieved only after daily administration of 500 micrograms thyroxine. Urinary excretion of hydroxyproline, and the activity of red cell glucose-6-phosphate dehydrogenase remained normal when excess T4 was administered, demonstrating the tissue resistance to thyroid hormones. Peripheral lymphocytes were found to have nuclear receptors for T3 with normal affinity, but the relatively low binding capacity indicated that the biochemical defect might be a deficiency of nuclear receptor protein. The findings in this patient differ somewhat from previously reported cases of peripheral resistance to thyroid hormones.

Full text

PDF
207

Images in this article

209Fig. 1
on p.209
209Fig. 2
on p.209

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Bernal J., Refetoff S., DeGroot L. J. Abnormalities of triiodothyronine binding to lymphocyte and fibroblast nuclei from a patient with peripheral tissue resistance to thyroid hormone action. J Clin Endocrinol Metab. 1978 Dec;47(6):1266–1272. doi: 10.1210/jcem-47-6-1266. [DOI] [PubMed] [Google Scholar]
  2. Bode H. H., Danon M., Weintraub B. D., Maloof F., Crawford J. D. Partial target organ resistance to thyroid hormone. J Clin Invest. 1973 Apr;52(4):776–782. doi: 10.1172/JCI107240. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Csaba G., Sudár F., Dobozy O. Triiodothyronine receptors in lymphocytes of newborn and adult rats. Horm Metab Res. 1977 Nov;9(6):499–501. doi: 10.1055/s-0028-1093509. [DOI] [PubMed] [Google Scholar]
  4. DIGIULIO W. D., MICHALAK Z., WEINHOLD P. A., HAMILTON J. R., THOMA G. E. USE OF AGAR GEL ELECTROPHORESIS AND AUTORADIOGRAPHY TO MEASURE THYROXINE-BINDING PROTEIN CAPACITIES. J Lab Clin Med. 1964 Aug;64:349–354. [PubMed] [Google Scholar]
  5. Elewaut A., Mussche M., Vermeulen A. Familial partial target organ resistance to thyroid hormones. J Clin Endocrinol Metab. 1976 Sep;43(3):575–581. doi: 10.1210/jcem-43-3-575. [DOI] [PubMed] [Google Scholar]
  6. Gershengorn M. C., Weintraub B. D. Thyrotropin-induced hyperthyroidism caused by selective pituitary resistance to thyroid hormone. A new syndrome of "inappropriate secretion of TSH". J Clin Invest. 1975 Sep;56(3):633–642. doi: 10.1172/JCI108133. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Ginsberg J., Segal D., Ehrlich R. M., Walfish P. G. Inappropriate triiodothyronine (T3) and thyroxine (T4) radioimmunoassay levels secondary to circulating thyroid hormone autoantibodies. Clin Endocrinol (Oxf) 1978 Feb;8(2):133–139. doi: 10.1111/j.1365-2265.1978.tb02161.x. [DOI] [PubMed] [Google Scholar]
  8. Gordin A., Saarinen P. Methodological study of the radioimmunoassay of human thyrotrophin. Acta Endocrinol (Copenh) 1972 Sep;71(1):24–36. doi: 10.1530/acta.0.0710024. [DOI] [PubMed] [Google Scholar]
  9. Helenius T., Liewendahl K. Abnormal thyroid function tests in devere non-thyroidal illness: diagnostic and pathophysiologic aspects. Scand J Clin Lab Invest. 1979 Jun;39(4):389–397. doi: 10.3109/00365517909106123. [DOI] [PubMed] [Google Scholar]
  10. Kivirikko K. I., Laitinen O., Prockop D. J. Modifications of a specific assay for hydroxyproline in urine. Anal Biochem. 1967 May;19(2):249–255. doi: 10.1016/0003-2697(67)90160-1. [DOI] [PubMed] [Google Scholar]
  11. Lamberg B. A. Congenital euthyroid goitre and partial peripheral resistance to thyroid hormones. Lancet. 1973 Apr 21;1(7808):854–857. doi: 10.1016/s0140-6736(73)91421-9. [DOI] [PubMed] [Google Scholar]
  12. Lamberg B. A., Gordin A., Viherkoski M., Kvist G. Long-acting thyroid stimulator (LATS) in toxic nodular goitre, toxic adenoma and Graves' disease. Acta Endocrinol (Copenh) 1969 Oct;62(2):199–209. doi: 10.1530/acta.0.0620199. [DOI] [PubMed] [Google Scholar]
  13. Lamberg B. A., Rosengård S., Liewendahl K., Saarinen P., Evered D. C. Familial partial peripheral resistance to thyroid hormones. Acta Endocrinol (Copenh) 1978 Feb;87(2):303–312. doi: 10.1530/acta.0.0870303. [DOI] [PubMed] [Google Scholar]
  14. Lemarchand-Béraud T. H., Holm A. C., Scazziga B. R. Triodothyronine and thyroxine nuclear receptors in lyphocytes from normal, hyper- and hypothyroid subjects. Acta Endocrinol (Copenh) 1977 May;85(1):44–54. doi: 10.1530/acta.0.0850044. [DOI] [PubMed] [Google Scholar]
  15. Liewendahl K., Helenius T. Comparison of serum free thyroxine indices and "corrected" thyroxine tests. Clin Chim Acta. 1975 Nov 3;64(3):263–272. doi: 10.1016/0009-8981(75)90353-8. [DOI] [PubMed] [Google Scholar]
  16. Liewendahl K., Rosengård S., Lamberg B. A. Nuclear binding of triiodothyronine and thyroxine in lymphocytes from subjects with hyperthyroidism, hypothyroidism and resistance to thyroid hormones. Clin Chim Acta. 1978 Feb 1;83(1-2):41–48. doi: 10.1016/0009-8981(78)90204-8. [DOI] [PubMed] [Google Scholar]
  17. Liewendahl K. Triiodothyronine binding to lymphocytes from euthyroid subjects and a patient with peripheral resistance to thyroid hormone. Acta Endocrinol (Copenh) 1976 Sep;83(1):64–70. doi: 10.1530/acta.0.0830064. [DOI] [PubMed] [Google Scholar]
  18. Refetoff S., DeGroot L. J., Benard B., DeWind L. T. Studies of a sibship with apparent hereditary resistance to the intracellular action of thyroid hormone. Metabolism. 1972 Aug;21(8):723–756. doi: 10.1016/0026-0495(72)90121-7. [DOI] [PubMed] [Google Scholar]
  19. Schneider G., Keiser H. R., Bardin C. W. Peripheral resistance to thyroxine: a cause of short stature in a boy without goitre. Clin Endocrinol (Oxf) 1975 Mar;4(2):111–118. doi: 10.1111/j.1365-2265.1975.tb01519.x. [DOI] [PubMed] [Google Scholar]
  20. Staeheli V., Vallotton M. B., Burger A. Detection of human anti-thyroxine and anti-triiodothyronine antibodies in different thyroid conditions. J Clin Endocrinol Metab. 1975 Oct;41(4):669–675. doi: 10.1210/jcem-41-4-669. [DOI] [PubMed] [Google Scholar]

Articles from Archives of Disease in Childhood are provided here courtesy of BMJ Publishing Group

RESOURCES