Browsing by Author "Silva, T"
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- Hiperaldosteronismo primário: resultados do primeiro estudo multicêntrico português realizado pelo Grupo de Estudos de Tumores da Supra-RenalPublication . Fernandes, V; Silva, T; Martins, D; Gonçalves, D; Almeida, R; Monteiro, AM; Neves, C; Simões, H; Marques, P; Serra, F; Pereira, ML; Grupo de Estudos de Tumores da Supra-RenalIntroduction: Primary Aldosteronism (PA) is the most prevalent cause of secondary hypertension. Nevertheless, there is no portuguese multicenter study characterizing PA patients. Aims: To characterize the clinical presentation, diagnostic workup, treatment and follow-up of patients with confirmed PA. Methods: Retrospective multicenter study of PA patients followed in 9 portuguese hospitals. Results: Sixty-three cases were selected with a mean age of diagnosis of 52.1 ± 13.1 years, 9.9 years after the diagnosis of hypertension. At presentation, 37 cases (60.7%) had hypertension, 11 (22.9%) had resistant hypertension and 20 (32.8%) of patients had hipokalemia (mean 3.2 mmol/L). Baseline laboratory showed a mean serum aldosterone of 33.4ng/dL, plasmatic renin activity (PRA) of 0.2ng/mL/h with an aldosterone/PRA ratio of 97.1. Confirmatory testing was performed with saline infusion in 55 patients (positive in 84.4%) and captopril test in 14 (positive in 85.7%). Imaging showed adenomas in 55 cases, hyperplasia in 2 and bilateral cases in 8. Arterial venous sampling (AVS) was performed in 9 patients (14,5%) and was conclusive in 1. Iodocholesterol scintigraphy was done in 14 cases (22%) with unilateral fixation in 9 and no fixation in 4. Patients were treated with laparoscopic adrenalectomy in 28 cases (58.3%) and mineralocorticoid receptor antagonists in 20 cases (41.7%). The surgical treated group had less duration of hypertension (8 versus 14 years, p = 0.002), higher prevalence of anti-hypertensive drugs at presentation (100 versus 75% p = 0.009) and bigger tumour size (1.8 versus 1.5 cm, p = 0.022). During follow-up there was a trend towards a greater proportion of patients with no hypertension improvement in the medical treatment group (29.4% versus 7.4%, p = 0.089). Conclusion: This is the first Portuguese PA multicenter study. It suggests that PA remains an under- -diagnosed condition with a significant delay in diagnosis. Surgical treated patients had a more aggressive disease and showed a trend towards better hypertension control.
- Primary adrenal insufficiency in adult population: a Portuguese multicentre study by the Adrenal Tumours Study GroupPublication . Ferreira, L; Silva, J; Garrido, S; Bello, C; Oliveira, D; Simões, H; Paiva, I; Guimarães, J; Ferreira, M; Pereira, MT; Bettencourt-Silva, R; Martins, AF; Silva, T; Fernandes, V; Ferreira, MINTRODUCTION: 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.