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THU230 Consumptive Hypothyroidism Secondary To Hemangioma

Disclosure: R. Thirunagari: None. D. Taha: None. Background: Peripheral Hypothyroidism also known as consumptive hypothyroidism has been rarely described in association with vascular tumors such as hemangiomas. It is due to excessive production of type 3 iodothyronine deiodinase (D3) enzyme by the h...

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Autores principales: Thirunagari, Rajeev, Taha, Doris R
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10554050/
http://dx.doi.org/10.1210/jendso/bvad114.1479
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author Thirunagari, Rajeev
Taha, Doris R
author_facet Thirunagari, Rajeev
Taha, Doris R
author_sort Thirunagari, Rajeev
collection PubMed
description Disclosure: R. Thirunagari: None. D. Taha: None. Background: Peripheral Hypothyroidism also known as consumptive hypothyroidism has been rarely described in association with vascular tumors such as hemangiomas. It is due to excessive production of type 3 iodothyronine deiodinase (D3) enzyme by the hemangioma leading to increased rate of deiodination of T4 and T3 to inactive forms. Clinical case: We report a 4-year-old girl who was diagnosed with consumptive hypothyroidism at the age of 2 months. She was born at full term via scheduled C-section. Mother has hypothyroidism. She was admitted to NICU for respiratory distress and needed oxygen supplementation. She had a large hemangioma covering most of her back on exam. An ultrasound of the head/neck soft tissue showed an ill-defined lobular heterogeneous mass measuring 3.4 x 1.2 x 4.5 cm, markedly hypervascular with both arterial and venous flow likely consistent with hemangioma. At follow up plastic surgery clinic she had respiratory distress which necessitated readmission to the NICU for further workup and possible initiation of Propranolol. An abdominal ultrasound showed enlargement of the right adrenal and on further dedicated ultrasound a right suprarenal lobulated mass as well as intrathoracic soft tissue mass were found. MRI of the abdomen and thorax, revealed multiple large bilateral paraspinal masses throughout the thoracic and lower cervical spine(largest measuring 3.3 x 7 x 15 cm AP) invading the neural foramen at multiple levels, and causing mass effect on the spinal cord which is displaced to the left. There was mass effect on adjacent vascular structures without significant vascular compromise. Biopsy was consistent with hemangioma. Thyroid Newborn screening result was normal. Thyroid evaluation done at 3 months age revealed, free T4 level of 1.04 ng/dL and TSH level of 11.3 µIU/mL. She was started on Levothyroxine 50 mcg daily. Reverse T3 was elevated at 139.2 ng/dl (reference range 13-107 ng/dl) 2 weeks after treatment initiation. She was initiated on propranolol and was followed up by plastic surgery and oncology. She had a very good response to Propranolol. Follow up MRI at 4 years shows almost complete resolution of the mass. She continues to be on Levothyroxine but is requiring smaller dose. Conclusion: Infantile hemangiomas can express high levels of type 3 iodothyronine deiodinase (D3), an enzyme that catalyzes the inner-ring deiodination of T4 to reverse T3 (rT3) and of T3 to 3,3'-diiiodothyronine (T2), both of which are biologically inactive. The hypothyroidism is a result of degeneration of thyroid hormones at rates that exceed the synthetic capacity of the infant's thyroid gland. Our case illustrates the importance of closely monitoring thyroid function tests in infants with large hemangiomas even if newborn thyroid screening result is normal. Some infants may require higher Levothyroxine doses than usual. Hypothyroidism usually resolves after resolution of the mass. Presentation: Thursday, June 15, 2023
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spelling pubmed-105540502023-10-06 THU230 Consumptive Hypothyroidism Secondary To Hemangioma Thirunagari, Rajeev Taha, Doris R J Endocr Soc Pediatric Endocrinology Disclosure: R. Thirunagari: None. D. Taha: None. Background: Peripheral Hypothyroidism also known as consumptive hypothyroidism has been rarely described in association with vascular tumors such as hemangiomas. It is due to excessive production of type 3 iodothyronine deiodinase (D3) enzyme by the hemangioma leading to increased rate of deiodination of T4 and T3 to inactive forms. Clinical case: We report a 4-year-old girl who was diagnosed with consumptive hypothyroidism at the age of 2 months. She was born at full term via scheduled C-section. Mother has hypothyroidism. She was admitted to NICU for respiratory distress and needed oxygen supplementation. She had a large hemangioma covering most of her back on exam. An ultrasound of the head/neck soft tissue showed an ill-defined lobular heterogeneous mass measuring 3.4 x 1.2 x 4.5 cm, markedly hypervascular with both arterial and venous flow likely consistent with hemangioma. At follow up plastic surgery clinic she had respiratory distress which necessitated readmission to the NICU for further workup and possible initiation of Propranolol. An abdominal ultrasound showed enlargement of the right adrenal and on further dedicated ultrasound a right suprarenal lobulated mass as well as intrathoracic soft tissue mass were found. MRI of the abdomen and thorax, revealed multiple large bilateral paraspinal masses throughout the thoracic and lower cervical spine(largest measuring 3.3 x 7 x 15 cm AP) invading the neural foramen at multiple levels, and causing mass effect on the spinal cord which is displaced to the left. There was mass effect on adjacent vascular structures without significant vascular compromise. Biopsy was consistent with hemangioma. Thyroid Newborn screening result was normal. Thyroid evaluation done at 3 months age revealed, free T4 level of 1.04 ng/dL and TSH level of 11.3 µIU/mL. She was started on Levothyroxine 50 mcg daily. Reverse T3 was elevated at 139.2 ng/dl (reference range 13-107 ng/dl) 2 weeks after treatment initiation. She was initiated on propranolol and was followed up by plastic surgery and oncology. She had a very good response to Propranolol. Follow up MRI at 4 years shows almost complete resolution of the mass. She continues to be on Levothyroxine but is requiring smaller dose. Conclusion: Infantile hemangiomas can express high levels of type 3 iodothyronine deiodinase (D3), an enzyme that catalyzes the inner-ring deiodination of T4 to reverse T3 (rT3) and of T3 to 3,3'-diiiodothyronine (T2), both of which are biologically inactive. The hypothyroidism is a result of degeneration of thyroid hormones at rates that exceed the synthetic capacity of the infant's thyroid gland. Our case illustrates the importance of closely monitoring thyroid function tests in infants with large hemangiomas even if newborn thyroid screening result is normal. Some infants may require higher Levothyroxine doses than usual. Hypothyroidism usually resolves after resolution of the mass. Presentation: Thursday, June 15, 2023 Oxford University Press 2023-10-05 /pmc/articles/PMC10554050/ http://dx.doi.org/10.1210/jendso/bvad114.1479 Text en © The Author(s) 2023. Published by Oxford University Press on behalf of the Endocrine Society. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs licence (https://creativecommons.org/licenses/by-nc-nd/4.0/), which permits non-commercial reproduction and distribution of the work, in any medium, provided the original work is not altered or transformed in any way, and that the work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Pediatric Endocrinology
Thirunagari, Rajeev
Taha, Doris R
THU230 Consumptive Hypothyroidism Secondary To Hemangioma
title THU230 Consumptive Hypothyroidism Secondary To Hemangioma
title_full THU230 Consumptive Hypothyroidism Secondary To Hemangioma
title_fullStr THU230 Consumptive Hypothyroidism Secondary To Hemangioma
title_full_unstemmed THU230 Consumptive Hypothyroidism Secondary To Hemangioma
title_short THU230 Consumptive Hypothyroidism Secondary To Hemangioma
title_sort thu230 consumptive hypothyroidism secondary to hemangioma
topic Pediatric Endocrinology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10554050/
http://dx.doi.org/10.1210/jendso/bvad114.1479
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