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Primary adrenal insufficiency in adult population: a Portuguese multicentre study by the Adrenal Tumours Study Group

2017-10-31Journal article Open access
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dc.contributor.authorFerreira, L
dc.contributor.authorSilva, J
dc.contributor.authorGarrido, S
dc.contributor.authorBello, C
dc.contributor.authorOliveira, D
dc.contributor.authorSimões, H
dc.contributor.authorPaiva, I
dc.contributor.authorGuimarães, J
dc.contributor.authorFerreira, M
dc.contributor.authorPereira, MT
dc.contributor.authorBettencourt-Silva, R
dc.contributor.authorMartins, AF
dc.contributor.authorSilva, T
dc.contributor.authorFernandes, V
dc.contributor.authorFerreira, M
dc.date.accessioned2017-11-10T16:00:01Z
dc.date.available2017-11-10T16:00:01Z
dc.date.issued2017-10-31
dc.description.abstractINTRODUCTION: 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 etiology 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 g/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 aetiological investigation and provide a framework for improving treatment.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationEndocr Connect. 2017 Oct 31. pii: EC-17-0295.pt_PT
dc.identifier.doi10.1530/EC-17-0295pt_PT
dc.identifier.urihttp://hdl.handle.net/10400.23/1215
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.subjectInsuficiência Suprarrenalpt_PT
dc.subjectDoença de Addisonpt_PT
dc.subjectAdultopt_PT
dc.titlePrimary adrenal insufficiency in adult population: a Portuguese multicentre study by the Adrenal Tumours Study Grouppt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.startPageEC-17-0295pt_PT
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT

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