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SUN-358 Dual Release Hydrocortisone as a New Treatment for Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency

In patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency, long-acting glucocorticoids (GCs) and/or multiple daily dose short-acting GCs are historically suggested to disease management. However, these treatment strategies are generally associated with GC overexposure, w...

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Autores principales: Simeoli, Chiara, Ferrigno, Rosario, Angellotti, Domenico, Iacuaniello, Davide, Pivonello, Claudia, Negri, Mariarosaria, Di Gennaro, Gilda, De Martino, Maria Cristina, Colao, Annamaria, Pivonello, Rosario
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Endocrine Society 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6552856/
http://dx.doi.org/10.1210/js.2019-SUN-358
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author Simeoli, Chiara
Ferrigno, Rosario
Angellotti, Domenico
Iacuaniello, Davide
Pivonello, Claudia
Negri, Mariarosaria
Di Gennaro, Gilda
De Martino, Maria Cristina
Colao, Annamaria
Pivonello, Rosario
author_facet Simeoli, Chiara
Ferrigno, Rosario
Angellotti, Domenico
Iacuaniello, Davide
Pivonello, Claudia
Negri, Mariarosaria
Di Gennaro, Gilda
De Martino, Maria Cristina
Colao, Annamaria
Pivonello, Rosario
author_sort Simeoli, Chiara
collection PubMed
description In patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency, long-acting glucocorticoids (GCs) and/or multiple daily dose short-acting GCs are historically suggested to disease management. However, these treatment strategies are generally associated with GC overexposure, which may induce metabolic syndrome (MS) and impairment of quality of life (QoL), and poor treatment compliance (TC). Dual-release hydrocortisone (DR-HC) with a generally once-daily administration has never been evaluated in cohorts of patients with CAH. The current study aimed at investigating the impact of the switch from conventional GCs to DR-HC on 12-month metabolic and androgens profile, QoL and TC in 39 CAH patients (26F, 13M, 18-50 years, 25 treated with immediate release hydrocortisone (IR-HC), 7 with prednisone, 3 with cortisone acetate, 3 with dexamethasone (DMX), one with IR-HC and DMX). The same cohort, stably treated with conventional GCs, was evaluated during the 12 months before the switch. In the switched cohort, at 12-month-follow-up total (p=0.028) and LDL-cholesterol (p=0.001) were significantly improved, and the diagnosis of MS, performed in two patients at baseline, was not confirmed in one patient. Interestingly, no significant increase in adrenal androgens and no clinical worsening of symptoms and signs related to hyperandrogenism in females were observed. QoL did not significantly changed, whereas TC significantly improved (p=0.001) during the year of DR-HC treatment. Conversely, no significant changes were observed during the control period before the treatment switch. In conclusion, in CAH patients the switch from conventional GCs to DR-HC appears able to induce a beneficial effect on lipid profile, MS and TC, surprisingly maintaining an optimal control of androgen levels.
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spelling pubmed-65528562019-06-13 SUN-358 Dual Release Hydrocortisone as a New Treatment for Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency Simeoli, Chiara Ferrigno, Rosario Angellotti, Domenico Iacuaniello, Davide Pivonello, Claudia Negri, Mariarosaria Di Gennaro, Gilda De Martino, Maria Cristina Colao, Annamaria Pivonello, Rosario J Endocr Soc Adrenal In patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency, long-acting glucocorticoids (GCs) and/or multiple daily dose short-acting GCs are historically suggested to disease management. However, these treatment strategies are generally associated with GC overexposure, which may induce metabolic syndrome (MS) and impairment of quality of life (QoL), and poor treatment compliance (TC). Dual-release hydrocortisone (DR-HC) with a generally once-daily administration has never been evaluated in cohorts of patients with CAH. The current study aimed at investigating the impact of the switch from conventional GCs to DR-HC on 12-month metabolic and androgens profile, QoL and TC in 39 CAH patients (26F, 13M, 18-50 years, 25 treated with immediate release hydrocortisone (IR-HC), 7 with prednisone, 3 with cortisone acetate, 3 with dexamethasone (DMX), one with IR-HC and DMX). The same cohort, stably treated with conventional GCs, was evaluated during the 12 months before the switch. In the switched cohort, at 12-month-follow-up total (p=0.028) and LDL-cholesterol (p=0.001) were significantly improved, and the diagnosis of MS, performed in two patients at baseline, was not confirmed in one patient. Interestingly, no significant increase in adrenal androgens and no clinical worsening of symptoms and signs related to hyperandrogenism in females were observed. QoL did not significantly changed, whereas TC significantly improved (p=0.001) during the year of DR-HC treatment. Conversely, no significant changes were observed during the control period before the treatment switch. In conclusion, in CAH patients the switch from conventional GCs to DR-HC appears able to induce a beneficial effect on lipid profile, MS and TC, surprisingly maintaining an optimal control of androgen levels. Endocrine Society 2019-04-30 /pmc/articles/PMC6552856/ http://dx.doi.org/10.1210/js.2019-SUN-358 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 Adrenal
Simeoli, Chiara
Ferrigno, Rosario
Angellotti, Domenico
Iacuaniello, Davide
Pivonello, Claudia
Negri, Mariarosaria
Di Gennaro, Gilda
De Martino, Maria Cristina
Colao, Annamaria
Pivonello, Rosario
SUN-358 Dual Release Hydrocortisone as a New Treatment for Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency
title SUN-358 Dual Release Hydrocortisone as a New Treatment for Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency
title_full SUN-358 Dual Release Hydrocortisone as a New Treatment for Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency
title_fullStr SUN-358 Dual Release Hydrocortisone as a New Treatment for Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency
title_full_unstemmed SUN-358 Dual Release Hydrocortisone as a New Treatment for Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency
title_short SUN-358 Dual Release Hydrocortisone as a New Treatment for Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency
title_sort sun-358 dual release hydrocortisone as a new treatment for congenital adrenal hyperplasia due to 21-hydroxylase deficiency
topic Adrenal
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6552856/
http://dx.doi.org/10.1210/js.2019-SUN-358
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