ECE2017 Eposter Presentations: Adrenal and Neuroendocrine Tumours Adrenal cortex (to include Cushing's) (86 abstracts)
Primary adrenal insufficiency: a Portuguese multicentre study by the adrenal tumour study group
Lia Ferreira 1 , João Silva 2 , Ana Martins 3 , Carlos Belo 4 , Diana Oliveira 5 , Hélder Simões 6 , Isabel Paiva 5 , Joana Guimarães 7 , Marta Ferreira 8 , Rita Silva 9 , Susana Garrido 10 , Tiago Silva 11 , Vera Fernandes 12 & Maria Lopes Pereira 12
1Centro Hospitalar do Porto, Porto, Portugal; 2Hospital das Forças Armadas, Lisboa, Portugal; 3Centro Hospitalar Lisboa Norte, Lisboa, Portugal; 4Centro Hospitalar Lisboa Ocidental, Lisboa, Portugal; 5Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal; 6Instituto Português de Oncologia Lisboa, Lisboa, Portugal; 7Centro Hospitalar do Baixo Vouga, Aveiro, Portugal; 8Centro Hospitalar Leiria, Leiria, Portugal; 9Centro Hospitalar São João, Porto, Portugal; 10Hospital Tâmega e Sousa, Penafiel, Portugal; 11Hospital Garcia da Orta, Lisboa, Portugal; 12Hospital de Braga, Braga, Portugal.
Introduction: Primary adrenal insufficiency (PAI) is a rare but severe and potentially life-threatening condition. There are no studies characterizing portuguese patients with PAI.
Aims: To characterize the clinical presentation, diagnostic workup, treatment and follow-up of patients with confirmed PAI.
Methods: A multicentre retrospective study of PAI patients followed in 12 portuguese hospitals.
Results: We investigated 278 patients with PAI (55.8% were females) with mean age of diagnosis of 33.6±19.3 years, 7.3 years after the beginning of the symptom presentation. The most frequently reported symptoms and signs were asthenia (60.1%), hyperpigmentation (55.0%), weight loss (43.2%), hypotension (42.8%) and hypoglycaemia (8.6%). Hyponatremia was documented in 36.3% of cases and hyperkalaemia in 25.9%; 29.1% of patients were diagnosed in adrenal crisis. In 122 patients the diagnosis was established using morning cortisol <5 g/dl and plasma ACTH 2-fold the upper limit of the reference range and in 39 cases by the corticotropin stimulation test. The main causes were autoimmune adrenalitis (140 cases), idiopathic (48 cases), congenital adrenal hyperplasia (27 cases) and tuberculosis (17 cases). Concerning associated autoimmune diseases, the most common were autoimmune thyroiditis (81 patients) and type 1 diabetes mellitus (25 patients). Seventy-nine percent were treated with hydrocortisone (mean dose 26.3±8.3 mg/day) in three (57.5%), two (37.4%), one (3.7%) or four (0.5%) daily doses; the remainder with prednisolone (10.1%), dexamethasone (6.2%) and methylprednisolone (0.7%); 66.2% were medicated with fludrocortisone (median dose of 100 μg/day). Since diagnosis 33.5% have been hospitalized due to disease decompensation. At the last consultation, 17.2% of the patients had complaints (7.6% asthenia and 6.5% depression) and 9.7% presented changes in the ionogram.
Conclusion: This is the first multicentre Portuguese study about PAI. As described in international studies a significant number of PAI patients continue to have symptoms of over or under-substitution despite optimal steroid replacement.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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