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SUN-580 Pituitary Hyperplasia Secondary to Primary Hypothyroidism in Pediatrics: A Diagnosis Not to Be Forgotten

Background: Pathologic pituitary hyperplasia (PH) in Pediatrics may be observed in several clinical settings. In PH secondary to primary hypothyroidism the increased TRH levels, due to a long-standing hypothyroidism state and loss of negative thyroxin feedback, causes thyrotroph and lactotroph cells...

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Autores principales: Braz, Délia, Bastos, Dyrlanne, Mota, Gabriella, Matos Rezek Rodrigues, Stela, Nobrega, Naiara, Oliveira, Lais, Florencio, Augusto, Lofrano-Porto, Adriana, Oliveira, Renata, Lopes, Fernanda, Castro, Luiz Claudio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Endocrine Society 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6553287/
http://dx.doi.org/10.1210/js.2019-SUN-580
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author Braz, Délia
Bastos, Dyrlanne
Mota, Gabriella
Matos Rezek Rodrigues, Stela
Nobrega, Naiara
Oliveira, Lais
Florencio, Augusto
Lofrano-Porto, Adriana
Oliveira, Renata
Lopes, Fernanda
Castro, Luiz Claudio
author_facet Braz, Délia
Bastos, Dyrlanne
Mota, Gabriella
Matos Rezek Rodrigues, Stela
Nobrega, Naiara
Oliveira, Lais
Florencio, Augusto
Lofrano-Porto, Adriana
Oliveira, Renata
Lopes, Fernanda
Castro, Luiz Claudio
author_sort Braz, Délia
collection PubMed
description Background: Pathologic pituitary hyperplasia (PH) in Pediatrics may be observed in several clinical settings. In PH secondary to primary hypothyroidism the increased TRH levels, due to a long-standing hypothyroidism state and loss of negative thyroxin feedback, causes thyrotroph and lactotroph cells hypertrophy, leading to an enlarged pituitary gland. Herein we report on two pediatric patients presenting this condition. Patient 1: A 9 year-old female was referred due to severe short stature (height Z-score: -5 SDS), face myxedema and a suprasellar mass on MRI. She had a past of headache, fatigue and learning disabilities.  Laboratory evaluation showed anaemia and dyslipidemia (total cholesterol 282 mg/ dL, LDL 222 mg/dL); severe hypothyroidism: TSH 1,300 IU/L (NR 0.27-4.2), fT4 0.14 ng/dL (NR 0.93-1.7), negative thyroid antibodies TgAb 0.9 mcg/L (NR <4); and hyperprolactinemia (PRL: 110 ng/mL; NR 4.79-23.3). Her bone age was delayed (2,6 yr).  Pituitary MRI revealed a 19×16×12-mm mass pushing the optic chiasm. Thyroid ultrasound revealed a diminished gland. After fourteen months on LT4 therapy, she presented catch up growth (height Z-score: -3,8 SDS); and improvement in school duties. Lab evaluation showed improvement on thyroid function (fT4 0.89 ng/dL, TSH 1.57 IU/L), PRL (18.25 ng/mL) and lipids (total cholesterol 151 mg/ dL, LDL 83 mg/dL). A follow up pituitary MRI evidenced a normal-sized pituitary gland inside a still enlarged sella. Patient 2: A 19 year-old female was referred due to severe short stature (height Z-score: -4.61 SDS), myxedema, madarosis, and delayed puberty (Tanner B2PH1). Neurological complaints were absent, except for a mild slowing of the speech. Bone age was delayed (10 yrs). Her labs showed a severe primary autoimune hypothyroidism: TSH 606.60 IU/L, fT4 < 0.023 ng/dL, anti-TPO 85.8 IU/mL (NR < 4); and a mildly elevated PRL 67.59 ng/mL. Thyroid ultrasound revealed a small gland volume with heterogeneous texture. MRI evidenced a pituitary macroadenoma measuring 11x9x8 mm. After eight months on LT4 therapy, her thyroid function (fT4 1.04ng/dL, TSH 1.48 IU/L) and PRL (11.73 ng/mL) were in the normal range. A follow up MRI revealed a normal-sized pituitary gland. Conclusion: Evaluation of the hypothalamic-pituitary axis must always be taken into account when investigating pituitary masses, since MRI alone is unable to reliably differentiate the underlying condition. In both reported cases, hormonal profile was unequivocal, unmasking the diagnosis of pituitary hyperplasia secondary to primary hypothyroidism. A specialized and integrated approach is essential to adequately define treatment and prevent unnecessary intervention.
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spelling pubmed-65532872019-06-13 SUN-580 Pituitary Hyperplasia Secondary to Primary Hypothyroidism in Pediatrics: A Diagnosis Not to Be Forgotten Braz, Délia Bastos, Dyrlanne Mota, Gabriella Matos Rezek Rodrigues, Stela Nobrega, Naiara Oliveira, Lais Florencio, Augusto Lofrano-Porto, Adriana Oliveira, Renata Lopes, Fernanda Castro, Luiz Claudio J Endocr Soc Thyroid Background: Pathologic pituitary hyperplasia (PH) in Pediatrics may be observed in several clinical settings. In PH secondary to primary hypothyroidism the increased TRH levels, due to a long-standing hypothyroidism state and loss of negative thyroxin feedback, causes thyrotroph and lactotroph cells hypertrophy, leading to an enlarged pituitary gland. Herein we report on two pediatric patients presenting this condition. Patient 1: A 9 year-old female was referred due to severe short stature (height Z-score: -5 SDS), face myxedema and a suprasellar mass on MRI. She had a past of headache, fatigue and learning disabilities.  Laboratory evaluation showed anaemia and dyslipidemia (total cholesterol 282 mg/ dL, LDL 222 mg/dL); severe hypothyroidism: TSH 1,300 IU/L (NR 0.27-4.2), fT4 0.14 ng/dL (NR 0.93-1.7), negative thyroid antibodies TgAb 0.9 mcg/L (NR <4); and hyperprolactinemia (PRL: 110 ng/mL; NR 4.79-23.3). Her bone age was delayed (2,6 yr).  Pituitary MRI revealed a 19×16×12-mm mass pushing the optic chiasm. Thyroid ultrasound revealed a diminished gland. After fourteen months on LT4 therapy, she presented catch up growth (height Z-score: -3,8 SDS); and improvement in school duties. Lab evaluation showed improvement on thyroid function (fT4 0.89 ng/dL, TSH 1.57 IU/L), PRL (18.25 ng/mL) and lipids (total cholesterol 151 mg/ dL, LDL 83 mg/dL). A follow up pituitary MRI evidenced a normal-sized pituitary gland inside a still enlarged sella. Patient 2: A 19 year-old female was referred due to severe short stature (height Z-score: -4.61 SDS), myxedema, madarosis, and delayed puberty (Tanner B2PH1). Neurological complaints were absent, except for a mild slowing of the speech. Bone age was delayed (10 yrs). Her labs showed a severe primary autoimune hypothyroidism: TSH 606.60 IU/L, fT4 < 0.023 ng/dL, anti-TPO 85.8 IU/mL (NR < 4); and a mildly elevated PRL 67.59 ng/mL. Thyroid ultrasound revealed a small gland volume with heterogeneous texture. MRI evidenced a pituitary macroadenoma measuring 11x9x8 mm. After eight months on LT4 therapy, her thyroid function (fT4 1.04ng/dL, TSH 1.48 IU/L) and PRL (11.73 ng/mL) were in the normal range. A follow up MRI revealed a normal-sized pituitary gland. Conclusion: Evaluation of the hypothalamic-pituitary axis must always be taken into account when investigating pituitary masses, since MRI alone is unable to reliably differentiate the underlying condition. In both reported cases, hormonal profile was unequivocal, unmasking the diagnosis of pituitary hyperplasia secondary to primary hypothyroidism. A specialized and integrated approach is essential to adequately define treatment and prevent unnecessary intervention. Endocrine Society 2019-04-30 /pmc/articles/PMC6553287/ http://dx.doi.org/10.1210/js.2019-SUN-580 Text en Copyright © 2019 Endocrine Society https://creativecommons.org/licenses/by-nc-nd/4.0/ This article has been published under the terms of the Creative Commons Attribution Non-Commercial, No-Derivatives License (CC BY-NC-ND; https://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Thyroid
Braz, Délia
Bastos, Dyrlanne
Mota, Gabriella
Matos Rezek Rodrigues, Stela
Nobrega, Naiara
Oliveira, Lais
Florencio, Augusto
Lofrano-Porto, Adriana
Oliveira, Renata
Lopes, Fernanda
Castro, Luiz Claudio
SUN-580 Pituitary Hyperplasia Secondary to Primary Hypothyroidism in Pediatrics: A Diagnosis Not to Be Forgotten
title SUN-580 Pituitary Hyperplasia Secondary to Primary Hypothyroidism in Pediatrics: A Diagnosis Not to Be Forgotten
title_full SUN-580 Pituitary Hyperplasia Secondary to Primary Hypothyroidism in Pediatrics: A Diagnosis Not to Be Forgotten
title_fullStr SUN-580 Pituitary Hyperplasia Secondary to Primary Hypothyroidism in Pediatrics: A Diagnosis Not to Be Forgotten
title_full_unstemmed SUN-580 Pituitary Hyperplasia Secondary to Primary Hypothyroidism in Pediatrics: A Diagnosis Not to Be Forgotten
title_short SUN-580 Pituitary Hyperplasia Secondary to Primary Hypothyroidism in Pediatrics: A Diagnosis Not to Be Forgotten
title_sort sun-580 pituitary hyperplasia secondary to primary hypothyroidism in pediatrics: a diagnosis not to be forgotten
topic Thyroid
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6553287/
http://dx.doi.org/10.1210/js.2019-SUN-580
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