Primary adrenal insufficiency in adult population: a Portuguese Multicentre Study by the Adrenal Tumours Study Group
Introduction: Primary adrenal insufficiency (PAI) is a rare but severe and potentially life-threatening condition. No previous studies have characterized Portuguese patients with PAI. Aims: To characterize the clinical presentation, diagnostic workup, treatment and follow‐up of Portuguese patients...
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Bioscientifica
2017-11-01
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doaj-7600eda309ba4027891e66d2da2f9d1e2020-11-25T02:15:10ZengBioscientificaEndocrine Connections2049-36142049-36142017-11-016893594210.1530/EC-17-0295Primary adrenal insufficiency in adult population: a Portuguese Multicentre Study by the Adrenal Tumours Study GroupLia Ferreira0João Silva1Susana Garrido2Carlos Bello3Diana Oliveira4Hélder Simões5Isabel Paiva6Joana Guimarães7Marta Ferreira8Teresa Pereira9Rita Bettencourt-Silva10Ana Filipa Martins11Tiago Silva12Vera Fernandes13Maria LopesPereira14Adrenal Tumors Study Group of the Portuguese Society of EndocrinologyDepartment of Endocrinology, Centro Hospitalar do Porto, Porto, PortugalDepartment of Endocrinology, Hospital das Forças Armadas, Lisboa, PortugalDepartment of Endocrinology, Centro Hospitalar Tâmega e Sousa, Porto, PortugalDepartment of Endocrinology, Centro Hospitalar Lisboa Ocidental, Lisboa, PortugalDepartment of Endocrinology, Centro Hospitalar e Universitário de Coimbra, Coimbra, PortugalDepartment of Endocrinology, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisboa, PortugalDepartment of Endocrinology, Centro Hospitalar e Universitário de Coimbra, Coimbra, PortugalDepartment of Endocrinology, Centro Hospitalar do Baixo Vouga, Aveiro, PortugalDepartment of Endocrinology, Centro Hospitalar de Leiria, Leiria, PortugalDepartment of Endocrinology, Centro Hospitalar de Leiria, Leiria, PortugalDepartment of Endocrinology, Centro Hospitalar de São João, Porto, PortugalDepartment of Endocrinology, Centro Hospitalar Lisboa Norte, Lisboa, PortugalDepartment of Endocrinology, Hospital Garcia da Orta, Lisboa, PortugalDepartment of Endocrinology, Hospital de Braga, Braga, PortugalDepartment of Endocrinology, Hospital de Braga, Braga, PortugalIntroduction: Primary adrenal insufficiency (PAI) is a rare but severe and potentially life-threatening condition. No previous studies have characterized Portuguese patients with PAI. Aims: To characterize the clinical presentation, diagnostic workup, treatment and follow‐up of Portuguese patients with confirmed PAI. Methods: This multicentre retrospective study examined PAI patients in 12 Portuguese hospitals. Results: We investigated 278 patients with PAI (55.8% were females), with a mean age of 33.6 ± 19.3 years at diagnosis. The most frequent presenting clinical features were asthenia (60.1%), mucocutaneous hyperpigmentation (55.0%) and weight loss (43.2%); 29.1% of the patients presented with adrenal crisis. Diagnosis was established by high plasma ACTH and low serum cortisol in most patients (43.9%). The most common aetiology of PAI was autoimmune adrenalitis (61.0%). There were 38 idiopathic cases. Autoimmune comorbidities were found in 70% of the patients, the most frequent being autoimmune thyroiditis (60.7%) and type 1 diabetes mellitus (17.3%). Seventy-nine percent were treated with hydrocortisone (mean dose 26.3 ± 8.3 mg/day) mostly in three (57.5%) or two (37.4%) daily doses. The remaining patients were treated with prednisolone (10.1%), dexamethasone (6.2%) and methylprednisolone (0.7%); 66.2% were also on fludrocortisone (median dose of 100 μg/day). Since diagnosis, 33.5% of patients were hospitalized for disease decompensation. In the last appointment,17.2% of patients had complaints (7.6% asthenia and 6.5% depression) and 9.7% had electrolyte disturbances. Conclusion: This is the first multicentre Portuguese study regarding PAI. The results emphasize the need for standardization in diagnostic tests and etiological investigation and provide a framework for improving treatment.http://www.endocrineconnections.com/content/6/8/935.fullprimary adrenal insufficiencyAddison diseaseglucocorticoid replacementmineralocorticoid replacementPortugal |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Lia Ferreira João Silva Susana Garrido Carlos Bello Diana Oliveira Hélder Simões Isabel Paiva Joana Guimarães Marta Ferreira Teresa Pereira Rita Bettencourt-Silva Ana Filipa Martins Tiago Silva Vera Fernandes Maria LopesPereira Adrenal Tumors Study Group of the Portuguese Society of Endocrinology |
spellingShingle |
Lia Ferreira João Silva Susana Garrido Carlos Bello Diana Oliveira Hélder Simões Isabel Paiva Joana Guimarães Marta Ferreira Teresa Pereira Rita Bettencourt-Silva Ana Filipa Martins Tiago Silva Vera Fernandes Maria LopesPereira Adrenal Tumors Study Group of the Portuguese Society of Endocrinology Primary adrenal insufficiency in adult population: a Portuguese Multicentre Study by the Adrenal Tumours Study Group Endocrine Connections primary adrenal insufficiency Addison disease glucocorticoid replacement mineralocorticoid replacement Portugal |
author_facet |
Lia Ferreira João Silva Susana Garrido Carlos Bello Diana Oliveira Hélder Simões Isabel Paiva Joana Guimarães Marta Ferreira Teresa Pereira Rita Bettencourt-Silva Ana Filipa Martins Tiago Silva Vera Fernandes Maria LopesPereira Adrenal Tumors Study Group of the Portuguese Society of Endocrinology |
author_sort |
Lia Ferreira |
title |
Primary adrenal insufficiency in adult population: a Portuguese Multicentre Study by the Adrenal Tumours Study Group |
title_short |
Primary adrenal insufficiency in adult population: a Portuguese Multicentre Study by the Adrenal Tumours Study Group |
title_full |
Primary adrenal insufficiency in adult population: a Portuguese Multicentre Study by the Adrenal Tumours Study Group |
title_fullStr |
Primary adrenal insufficiency in adult population: a Portuguese Multicentre Study by the Adrenal Tumours Study Group |
title_full_unstemmed |
Primary adrenal insufficiency in adult population: a Portuguese Multicentre Study by the Adrenal Tumours Study Group |
title_sort |
primary adrenal insufficiency in adult population: a portuguese multicentre study by the adrenal tumours study group |
publisher |
Bioscientifica |
series |
Endocrine Connections |
issn |
2049-3614 2049-3614 |
publishDate |
2017-11-01 |
description |
Introduction: Primary adrenal insufficiency (PAI) is a rare but severe and potentially
life-threatening condition. No previous studies have characterized Portuguese patients with PAI.
Aims: To characterize the clinical presentation, diagnostic workup, treatment and follow‐up of Portuguese patients with confirmed PAI.
Methods: This multicentre retrospective study examined PAI patients in 12 Portuguese hospitals.
Results: We investigated 278 patients with PAI (55.8% were females), with a mean age of 33.6 ± 19.3 years at diagnosis. The most frequent presenting clinical features were asthenia (60.1%), mucocutaneous hyperpigmentation (55.0%) and weight loss (43.2%); 29.1% of the patients presented with adrenal crisis. Diagnosis was established by high plasma ACTH and low serum cortisol in most patients (43.9%). The most common aetiology of PAI was autoimmune adrenalitis (61.0%). There were 38 idiopathic cases. Autoimmune comorbidities were found in 70% of the patients, the most frequent being autoimmune thyroiditis (60.7%) and type 1 diabetes mellitus (17.3%). Seventy-nine percent were treated with hydrocortisone (mean dose 26.3 ± 8.3 mg/day) mostly in three (57.5%) or two (37.4%) daily doses. The remaining patients were treated with prednisolone (10.1%), dexamethasone (6.2%) and methylprednisolone (0.7%); 66.2% were also on fludrocortisone (median dose of 100 μg/day). Since diagnosis, 33.5% of patients were hospitalized for disease decompensation. In the last appointment,17.2% of patients had complaints (7.6% asthenia and 6.5% depression) and 9.7% had electrolyte disturbances.
Conclusion: This is the first multicentre Portuguese study regarding PAI. The results emphasize the need for standardization in diagnostic tests and etiological investigation and provide a framework for improving treatment. |
topic |
primary adrenal insufficiency Addison disease glucocorticoid replacement mineralocorticoid replacement Portugal |
url |
http://www.endocrineconnections.com/content/6/8/935.full |
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