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Generalized resistance to thyroid hormone(RTSH)

MedGen UID:
1654700
Concept ID:
C4722330
Disease or Syndrome
Synonyms: Generalized thyroid hormone resistance; Impaired sensitivity to thyroid hormone; Thyroid hormone receptor BETA
SNOMED CT: General resistance to thyrotropin (237560005); Generalized thyroid hormone resistance (237560005)
 
HPO: HP:0002930
Monarch Initiative: MONDO:0009043
OMIM®: 190160; 218700
Orphanet: ORPHA3221

Definition

Reduced sensitivity of end organs to thyroid hormone characterized by elevated serum levels of free thyroid hormone with nonsuppressed thyroid stimulating hormone. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Conditions with this feature

Selective pituitary resistance to thyroid hormone
MedGen UID:
333543
Concept ID:
C1840364
Disease or Syndrome
Selective pituitary resistance to thyroid hormone (PRTH) results in continued thyroid-stimulating hormone (TSH) production driving hypersecretion of T3 and T4 to establish a new equilibrium, with high serum levels of free thyroid hormones together with a nonsuppressed TSH. The presence of a variety of thyrotoxic features, including palpitations, anxiety, tremor, heat intolerance, insomnia, weight loss, and increased stool frequency, suggests that peripheral tissues are less refractory to thyroid hormones than the pituitary (summary by Adams et al., 1994).
Thyroid hormone resistance, generalized, autosomal dominant
MedGen UID:
424846
Concept ID:
C2937288
Disease or Syndrome
Generalized thyroid hormone resistance (GRTH) is characterized by elevated serum levels of free thyroid hormones with inappropriately elevated thyroid-stimulating hormone (TSH) as well as clinical and biochemical evidence of decreased thyroid hormone action. Affected individuals also show unresponsiveness to large doses of exogenous thyroid hormones (summary by Parrilla et al., 1991).
Congenital nongoitrous hypothyroidism 6
MedGen UID:
482447
Concept ID:
C3280817
Disease or Syndrome
Any hypothyroidism, congenital, nongoitrous in which the cause of the disease is a mutation in the THRA gene.
Thyroid hormone resistance, generalized, autosomal recessive
MedGen UID:
483749
Concept ID:
C3489796
Disease or Syndrome
A rare, autosomal recessive inherited disorder usually caused by mutations in the THRB gene. It is characterized by a defective physiological resistance to thyroid hormones, resulting in the elevation of thyroxin and triiodothyronine in the serum.

Professional guidelines

Recent clinical studies

Etiology

Apriletti JW, Ribeiro RC, Wagner RL, Feng W, Webb P, Kushner PJ, West BL, Nilsson S, Scanlan TS, Fletterick RJ, Baxter JD
Clin Exp Pharmacol Physiol Suppl 1998 Nov;25:S2-11. doi: 10.1111/j.1440-1681.1998.tb02293.x. PMID: 9809185
Weiss RE, Weinberg M, Refetoff S
J Clin Invest 1993 Jun;91(6):2408-15. doi: 10.1172/JCI116474. PMID: 8514853Free PMC Article
Hauser P, Zametkin AJ, Martinez P, Vitiello B, Matochik JA, Mixson AJ, Weintraub BD
N Engl J Med 1993 Apr 8;328(14):997-1001. doi: 10.1056/NEJM199304083281403. PMID: 8450877
Weiss RE, Stein MA, Trommer B, Refetoff S
J Pediatr 1993 Oct;123(4):539-45. doi: 10.1016/s0022-3476(05)80947-3. PMID: 8410504
Weiss RE, Refetoff S
Annu Rev Med 1992;43:363-75. doi: 10.1146/annurev.me.43.020192.002051. PMID: 1580595

Diagnosis

Florkowski CM, Brownlie BE, Croxson MS, Manning P, Farrand S, Smith G, Potter HC, George PM
Intern Med J 2006 Nov;36(11):738-41. doi: 10.1111/j.1445-5994.2006.01189.x. PMID: 17040361
Refetoff S
Acta Paediatr Jpn 1994 Feb;36(1):1-15. doi: 10.1111/j.1442-200x.1994.tb03121.x. PMID: 8165897
McDermott MT, Ridgway EC
Am J Med 1993 Apr;94(4):424-32. doi: 10.1016/0002-9343(93)90155-i. PMID: 8475937
Hauser P, Zametkin AJ, Martinez P, Vitiello B, Matochik JA, Mixson AJ, Weintraub BD
N Engl J Med 1993 Apr 8;328(14):997-1001. doi: 10.1056/NEJM199304083281403. PMID: 8450877
Weiss RE, Refetoff S
Annu Rev Med 1992;43:363-75. doi: 10.1146/annurev.me.43.020192.002051. PMID: 1580595

Therapy

Taniyama M, Kusano S, Miyoshi Y, Nakamura H, Kaihara M, Tobe T, Tomita M, Katagiri T, Ban Y
Exp Clin Endocrinol Diabetes 1996;104(4):339-43. doi: 10.1055/s-0029-1211464. PMID: 8886752
Refetoff S
Acta Paediatr Jpn 1994 Feb;36(1):1-15. doi: 10.1111/j.1442-200x.1994.tb03121.x. PMID: 8165897
Elia J, Gulotta C, Rose SR, Marin G, Rapoport JL
J Am Acad Child Adolesc Psychiatry 1994 Feb;33(2):169-72. doi: 10.1097/00004583-199402000-00003. PMID: 8150787
McDermott MT, Ridgway EC
Am J Med 1993 Apr;94(4):424-32. doi: 10.1016/0002-9343(93)90155-i. PMID: 8475937
Weiss RE, Balzano S, Scherberg NH, Refetoff S
JAMA 1990 Nov 7;264(17):2245-50. PMID: 2120481

Prognosis

Alberto G, Novi RF, Scalabrino E, Trombetta A, Seardo MA, Maurino M, Brossa C
Minerva Cardioangiol 2002 Apr;50(2):157-60. PMID: 12032470
Macchia E, Gurnell M, Agostini M, Giorgilli G, Marcocci C, Valenti TM, Martino E, Chatterjee KK, Pinchera A
Eur J Endocrinol 1997 Oct;137(4):370-6. doi: 10.1530/eje.0.1370370. PMID: 9368505
Groenhout EG, Dorin RI
Mol Cell Endocrinol 1994 Feb;99(1):81-8. doi: 10.1016/0303-7207(94)90149-x. PMID: 8187964
Hiramatsu R, Abe M, Morita M, Noguchi S, Suzuki T
Jpn J Hum Genet 1994 Dec;39(4):365-77. doi: 10.1007/BF01892382. PMID: 7873749

Clinical prediction guides

Spencer T, Biederman J, Wilens T, Guite J, Harding M
J Child Psychol Psychiatry 1995 Jul;36(5):879-85. doi: 10.1111/j.1469-7610.1995.tb01335.x. PMID: 7559851
Groenhout EG, Dorin RI
Mol Cell Endocrinol 1994 Feb;99(1):81-8. doi: 10.1016/0303-7207(94)90149-x. PMID: 8187964
Hauser P, Zametkin AJ, Martinez P, Vitiello B, Matochik JA, Mixson AJ, Weintraub BD
N Engl J Med 1993 Apr 8;328(14):997-1001. doi: 10.1056/NEJM199304083281403. PMID: 8450877
Weiss RE, Stein MA, Trommer B, Refetoff S
J Pediatr 1993 Oct;123(4):539-45. doi: 10.1016/s0022-3476(05)80947-3. PMID: 8410504
Takeda K, Sakurai A, DeGroot LJ, Refetoff S
J Clin Endocrinol Metab 1992 Jan;74(1):49-55. doi: 10.1210/jcem.74.1.1727829. PMID: 1727829

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