WordNet

any mechanical force that tends to retard or oppose motion
the capacity of an organism to defend itself against harmful environmental agents; "these trees are widely planted because of their resistance to salt and smog"
the action of opposing something that you disapprove or disagree with; "he encountered a general feeling of resistance from many citizens"; "despite opposition from the newspapers he went ahead" (同)opposition
(psychiatry) an unwillingness to bring repressed feelings into conscious awareness
group action in opposition to those in power
the military action of resisting the enemys advance; "the enemy offered little resistance"
the degree of unresponsiveness of a disease-causing microorganism to antibiotics or other drugs (as in penicillin-resistant bacteria)
suggestive of a thyroid disorder; "thyroid personality"
of or relating to the thyroid gland; "thyroid deficiency"; "thyroidal uptake" (同)thyroidal
the secretion of an endocrine gland that is transmitted by the blood to the tissue on which it has a specific effect (同)endocrine, internal_secretion

PrepTutorEJDIC

〈U〉〈C〉(…に)『抵抗』(『反抗』)『すること』;(…を)『こらえること』《+『to』(『against』)+『名』(do『ing』)》 / 〈U〉(病気などに対する)『抵抗力』《+『against』(『to』)+『名』》 / 〈U〉〈C〉(またresistance movement)(権力・圧制に対する)抵抗運動,レジスタンス / 〈U〉(空気の)抵抗;(電気の)抵抗
甲状腺 / 甲状腺の
ホルモン

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/06/23 05:48:15」(JST)

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Thyroid hormone resistance (sometimes Refetoff syndrome) describes a rare syndrome in which the thyroid hormone levels are elevated but the thyroid stimulating hormone (TSH) level is not suppressed, or not completely suppressed as would be expected. The first report of the condition appeared in 1967.^[1] Essentially this is decreased end organ responsiveness to thyroid hormones.^[2] A new term "impaired sensitivity to thyroid hormone" has been suggested in March 2014 by Refetoff et al.^[3]

Regulation of thyroid hormone secretion

Hypothalamus secretes a hormone called thyrotropin releasing hormone (TRH) which in turn release thyroid stimulating hormone (TSH). TSH signals thyroid to secrete thyroid hormones thyroxine (T4) and triiodothyronine (T3). T4 gets converted to active T3 in peripheral tissues with the help of deiodinase enzymes. T3 negatively feedback on the pituitary and decreases TSH secretion.

Regulation of thyroid hormone

Causes

For normal thyroid hormone action you need normal thyroid hormone transport across cell membrane, appropriate deiodination, thyroid hormone nuclear receptor, thyroid hormone response elements, co-activators, co-repressors and normal histone acetylation. Any abnormalities in the above chain can result in thyroid hormone resistance.

The most common cause of the syndrome are mutations of the β (beta) form (THRB gene) of the thyroid hormone receptor, of which over 100 different mutations have been documented.^[4]

Mutations in MCT8 and SECISBP2 have also been associated with this condition.^[5]

Incidence

Thyroid hormone resistance syndrome is rare, incidence is variously quoted as 1 in 50,000 or 1 in 40,000 live births.^[6] More than 1000 individuals have been identified with thyroid hormone resistance, of which 85% had thyroid hormone beta receptor mutation.^[5]

Presentation

The syndrome can present with variable symptoms, even between members of the same family harboring the same mutation.^[1] Typically most or all tissues are resistant to thyroid hormone, so despite raised measures of serum thyroid hormone the individual may appear euthyroid (have no symptoms of over- or underactivity of the thyroid gland). The most common symptoms are goiter and tachycardia. It has also been linked to some cases of attention deficit hyperactivity disorder (ADHD), although the majority of people with that diagnosis have no thyroid problems.^[7]

An association with depression has been proposed.^[8]

Diagnosis

The characteristic blood test results for this disorder can also be found in other disorders (for example TSH-oma (pituitary adenoma), or other pituitary disorders). The diagnosis may involve identifying a mutation of the thyroid receptor, which is present in approximately 85% of cases.^[9]

Yet, since discovery of resistance to thyroid hormones in the absence of thyroid hormone receptor beta mutations, lack of a mutation in a patient does not rule out resistance.^[10]

References

^ ^a ^b Refetoff S, DeWind LT, DeGroot LJ (1967). "Familial syndrome combining deaf-mutism, stuppled epiphyses, goiter and abnormally high PBI: possible target organ refractoriness to thyroid hormone". J. Clin. Endocrinol. Metab. 27 (2): 279–94. doi:10.1210/jcem-27-2-279. PMID 4163616.
^ http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2928892/
^ Refetoff, Samuel; Bassett, J.H. Duncan; Beck-Peccoz, Paolo; Bernal, Juan; Brent, Gregory; Chatterjee, Krishna; De Groot, Leslie J.; Dumitrescu, Alexandra M.; Jameson, J. Larry; Kopp, Peter A.; Murata, Yoshiharu; Persani, Luca; Samarut, Jacques; Weiss, Roy E.; Williams, Graham R.; Yen, Paul M. (March 2014). "Classification and Proposed Nomenclature for Inherited Defects of Thyroid Hormone Action, Cell Transport, and Metabolism". Eur Thyroid J 24 (3): 407–409. doi:10.1159/000358180.
^ "Thyroid hormone resistance caused by a novel deleterious variant of the thyroid hormone receptor beta gene". European Journal of Obstetrics 167: 118–119. doi:10.1016/j.ejogrb.2012.11.001.
^ ^a ^b Refetoff S, Dumitrescu AM (2007). "Syndromes of reduced sensitivity to thyroid hormone: genetic defects in hormone receptors, cell transporters and deiodination". Best Pract. Res. Clin. Endocrinol. Metab. 21 (2): 277–305. doi:10.1016/j.beem.2007.03.005. PMID 17574009.
^ LaFranchi SH et al. (2003). "Follow-up of newborns with elevated screening T4 concentrations". Pediatr. 143 (3): 296–301. doi:10.1067/S0022-3476(03)00184-7. PMID 14517508.
^ Hauser P, Zametkin AJ, Martinez P et al. (1993). "Attention deficit-hyperactivity disorder in people with generalized resistance to thyroid hormone". N. Engl. J. Med. 328 (14): 997–1001. doi:10.1056/NEJM199304083281403. PMID 8450877.
^ Fardella CE, Artigas RA, Gloger S et al. (2007). "Refractory depression in a patient with peripheral resistance to thyroid hormone (RTH) and the effect of triiodothyronine treatment". Endocrine 31 (3): 272–8. doi:10.1007/s12020-007-0042-7. PMID 17906375.
^ Bottcher Y, Paufler T, Stehr T, Bertschat FL, Paschke R, Koch CA (2007). "Thyroid hormone resistance without mutations in thyroid hormone receptor beta". Med. Sci. Monit. 13 (6): CS67–70. PMID 17534237.
^ Tjørve E, Tjørve KM, Olsen JO, Senum R, Oftebro H (2007). "On commonness and rarity of thyroid hormone resistance: a discussion based on mechanisms of reduced sensitivity in peripheral tissues". Med. Hypotheses 69 (4): 913–21. doi:10.1016/j.mehy.2006.12.056. PMID 17383828.

UpToDate Contents

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1. 甲状腺ホルモンに対する感受性の傷害 impaired sensitivity to thyroid hormone
2. TSH分泌下垂体腺腫 tsh secreting pituitary adenomas
3. 甲状腺機能低下症の原因となる疾患 disorders that cause hypothyroidism
4. 甲状腺機能正常型高サイロキシン血症および低サイロキシン血症 euthyroid hyperthyroxinemia and hypothyroxinemia
5. 小児および思春期における後天性甲状腺機能低下症 acquired hypothyroidism in childhood and adolescence

English Journal

Thyrotoropin receptor knockout changes monoaminergic neuronal system and produces methylphenidate-sensitive emotional and cognitive dysfunction.

Mouri A1, Hoshino Y2, Narusawa S3, Ikegami K2, Mizoguchi H4, Murata Y5, Yoshimura T2, Nabeshima T6.
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Attention deficit/hyperactivity disorder (ADHD) has been reported in association with resistance to thyroid hormone, a disease caused by a mutation in the thyroid hormone receptor β (TRβ) gene. TRβ is a key protein mediating down-regulation of thyrotropin (TSH) expression by 3,3',5-tri-iodothyron
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