Resistance to Thyroid Hormone in a Boy with a Severe, Complex, Congenital Heart Defect (CHD) Requiring Multiple Cardiac Surgeries—Whether and How to Prepare Child for the Surgery.

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Title: Resistance to Thyroid Hormone in a Boy with a Severe, Complex, Congenital Heart Defect (CHD) Requiring Multiple Cardiac Surgeries—Whether and How to Prepare Child for the Surgery.
Authors: Fedorczak, Anna1 (AUTHOR) anna.fedorczak@outlook.com, Kruk, Beata2 (AUTHOR) b.slap@interia.pl, Mazurek-Kula, Anna2 (AUTHOR) qla@op.pl, Kępczyński, Łukasz3 (AUTHOR) lukasz.kepczynski@iczmp.edu.pl, Stawerska, Renata1,4 (AUTHOR) renata.stawerska@icloud.com
Source: Journal of Clinical Medicine. Feb2025, Vol. 14 Issue 4, p1209. 7p.
Subject Terms: *THYROID hormone receptors, *CONGENITAL heart disease, *HORMONE receptors, *THYROID hormones, *CARDIAC surgery
Abstract: Background: Resistance to thyroid hormones (RTH) is a rare, genetically determined disease characterised by reduced tissue sensitivity to thyroid hormones (THs). It is caused by mutations in genes encoding the receptors for thyroid hormones, α (THRα) or β (THRβ), the distribution of which varies between tissues. Therefore, patients present with elevated TH levels with unsuppressed TSH levels, and symptoms of both hypothyroidism and hyperthyroidism may be present. Methods: Hence, we report the case of a boy with a complex, cyanotic, congenital heart defect who was also diagnosed with TH resistance syndrome. Results: Because of the clinical features of hyperthyroidism in preparation for cardiac surgery, thiamazole was administered, resulting in the normalisation of TH effects on the α-receptor for HTs. Due to the effectiveness of the proposed treatment, it was further introduced before the further stages of cardiac surgeries. Conclusions: The management of RTH is a constant challenge for clinicians and must be individualised. [ABSTRACT FROM AUTHOR]
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  Data: Resistance to Thyroid Hormone in a Boy with a Severe, Complex, Congenital Heart Defect (CHD) Requiring Multiple Cardiac Surgeries—Whether and How to Prepare Child for the Surgery.
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  Data: <searchLink fieldCode="AR" term="%22Fedorczak%2C+Anna%22">Fedorczak, Anna</searchLink><relatesTo>1</relatesTo> (AUTHOR)<i> anna.fedorczak@outlook.com</i><br /><searchLink fieldCode="AR" term="%22Kruk%2C+Beata%22">Kruk, Beata</searchLink><relatesTo>2</relatesTo> (AUTHOR)<i> b.slap@interia.pl</i><br /><searchLink fieldCode="AR" term="%22Mazurek-Kula%2C+Anna%22">Mazurek-Kula, Anna</searchLink><relatesTo>2</relatesTo> (AUTHOR)<i> qla@op.pl</i><br /><searchLink fieldCode="AR" term="%22Kępczyński%2C+Łukasz%22">Kępczyński, Łukasz</searchLink><relatesTo>3</relatesTo> (AUTHOR)<i> lukasz.kepczynski@iczmp.edu.pl</i><br /><searchLink fieldCode="AR" term="%22Stawerska%2C+Renata%22">Stawerska, Renata</searchLink><relatesTo>1,4</relatesTo> (AUTHOR)<i> renata.stawerska@icloud.com</i>
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  Data: <searchLink fieldCode="JN" term="%22Journal+of+Clinical+Medicine%22">Journal of Clinical Medicine</searchLink>. Feb2025, Vol. 14 Issue 4, p1209. 7p.
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  Data: *<searchLink fieldCode="DE" term="%22THYROID+hormone+receptors%22">THYROID hormone receptors</searchLink><br />*<searchLink fieldCode="DE" term="%22CONGENITAL+heart+disease%22">CONGENITAL heart disease</searchLink><br />*<searchLink fieldCode="DE" term="%22HORMONE+receptors%22">HORMONE receptors</searchLink><br />*<searchLink fieldCode="DE" term="%22THYROID+hormones%22">THYROID hormones</searchLink><br />*<searchLink fieldCode="DE" term="%22CARDIAC+surgery%22">CARDIAC surgery</searchLink>
– Name: Abstract
  Label: Abstract
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  Data: Background: Resistance to thyroid hormones (RTH) is a rare, genetically determined disease characterised by reduced tissue sensitivity to thyroid hormones (THs). It is caused by mutations in genes encoding the receptors for thyroid hormones, α (THRα) or β (THRβ), the distribution of which varies between tissues. Therefore, patients present with elevated TH levels with unsuppressed TSH levels, and symptoms of both hypothyroidism and hyperthyroidism may be present. Methods: Hence, we report the case of a boy with a complex, cyanotic, congenital heart defect who was also diagnosed with TH resistance syndrome. Results: Because of the clinical features of hyperthyroidism in preparation for cardiac surgery, thiamazole was administered, resulting in the normalisation of TH effects on the α-receptor for HTs. Due to the effectiveness of the proposed treatment, it was further introduced before the further stages of cardiac surgeries. Conclusions: The management of RTH is a constant challenge for clinicians and must be individualised. [ABSTRACT FROM AUTHOR]
– Name: AbstractSuppliedCopyright
  Label:
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  Data: <i>Copyright of Journal of Clinical Medicine is the property of MDPI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.)
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        Value: 10.3390/jcm14041209
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        Text: English
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      – TitleFull: Resistance to Thyroid Hormone in a Boy with a Severe, Complex, Congenital Heart Defect (CHD) Requiring Multiple Cardiac Surgeries—Whether and How to Prepare Child for the Surgery.
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              Text: Feb2025
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