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- Absolute Hyperglycemia versus Stress Hyperglycemia Ratio for the Prognosis of Hospitalized Patients with COVID-19 in the First Months of the Pandemic: A Retrospective StudyPublication . Matias, AA; Manique, I; Sabino, T; Rego, T; Mihon, C; Panarra, A; Rizzo, M; Silva-Nunes, JDiabetes is a risk factor for greater severity of coronavirus disease 2019 (COVID-19). The stress hyperglycemia ratio (SHR) is an independent predictor of critical illness, and it is reported to have a stronger association than absolute hyperglycemia. The aim of this study was to assess the relationship of absolute hyperglycemia and SHR with the severity of COVID-19, since there are no studies investigating SHR in patients with COVID-19. We conducted a retrospective observational study on hospitalized patients with COVID-19 in the first months of the pandemic, regarding absolute hyperglycemia, SHR, and severity outcomes. Of the 374 patients, 28.1% had a previous diagnosis of type 2 diabetes. Absolute hyperglycemia (64.8% versus 22.7%; p < 0.01) and SHR [1.1 (IQR 0.9-1.3) versus 1.0 (IQR 0.9-1.2); p < 0.001] showed a statistically significant association with previous diabetes. Absolute hyperglycemia showed a significant association with clinical severity of COVID-19 (79.0% versus 62.7%; p < 0.001), need for oxygen therapy (74.8% versus 54.4%; p < 0.001), invasive mechanical ventilation (28.6% versus 11.6%; p < 0.001), and intensive care unit (30.3% versus 14.9%; p = 0.002), but not with mortality; by contrast, there was no statistically significant association between SHR and all these parameters. Our results are in agreement with the literature regarding the impact of absolute hyperglycemia on COVID-19 severity outcomes, while SHR was not a significant marker. We therefore suggest that SHR should not be evaluated in all patients admitted in the hospital for COVID-19, and we encourage the standard measures at admission of blood glucose and HbA1c levels.
- Acute Sheehan's Syndrome Presenting as Central Diabetes InsipidusPublication . Robalo, R; Pedroso, C; Agapito, A; Borges, ASheehan's syndrome occurs as a result of ischaemic pituitary necrosis due to severe postpartum haemorrhage. Improvements in obstetrical care have significantly reduced its incidence in developed countries, but postpartum pituitary infarction remains a common cause of hypopituitarism in developing countries. We report a case of severe postpartum haemorrhage followed by headache, central diabetes insipidus and failure to lactate, which prompted us to investigate and identify both anterior and posterior pituitary deficiency compatible with Sheehan's syndrome. A timely diagnosis allowed us to implement an adequate treatment and follow-up plan, which are known to improve clinical status and patient outcome.
- Adrenal Vein Sampling in the Management of Primary Aldosteronism: The Added Value of Intraprocedural Cortisol AssessmentPublication . Manique, I; Amaral, S; Matias, A; Bouça, B; Serranito, S; Torres, J; Gutu, O; Bilhim, T; Coimbra, E; Rodrigues, I; Godinho, C; Cortez, L; Silva-Nunes, JIntroduction: Primary aldosteronism is the most common cause of secondary hypertension. Adrenal vein sampling is the gold standard for subtyping primary aldosteronism. However, this procedure is technically challenging and often has a low success rate. Our center is one of the very few performing this technique in our country with an increasing experience. Objective: The aim of this study was to evaluate the role of the cortisol intraprocedural assay in improving the performance of adrenal vein sampling. Design: We enrolled all of the patients with primary aldosteronism that underwent adrenal vein sampling from February 2016 to April 2023. The cortisol intraprocedural assay was introduced in October 2021. Methods: We enrolled a total of 50 adrenal vein samplings performed on 43 patients with the diagnosis of primary aldosteronism. In this sample, 19 patients and 24 patients underwent adrenal vein sampling before and after intraprocedural cortisol measurement, respectively. The procedure was repeated in seven patients (one before and six after intraprocedural cortisol measurement), given the unsuccess of the first exam. Selectivity of the adrenal vein sampling was assumed if the serum cortisol concentration from the adrenal vein was at least five times higher than that of the inferior vena cava. Lateralization was assumed if the aldosterone to cortisol ratio of one adrenal vein was at least four times the aldosterone to cortisol ratio of the contralateral side. Results: The mean age of the patients that underwent adrenal vein sampling (N = 43) was 55.2 ± 8.9 years, and 53.5% (n = 23) were female. The mean interval between the diagnosis of hypertension and the diagnosis of primary aldosteronism was 9.8 years (±9.9). At diagnosis, 62.8% of the patients (n = 27) had hypokalemia (mean value of 3 mmol/L (±0.34)), 88.4% (n = 38) had adrenal abnormalities on preprocedural CT scan, and 67.4% (n = 29) described as unilateral nodules. There were no statistically significant differences in the patients' baseline characteristics between the two groups (before and after intraprocedural cortisol measurement). Before intraprocedural cortisol measurement, adrenal vein sampling selectivity was achieved in 35% (n = 7) patients. Selectivity increased to 100% (30/30) after intraprocedural cortisol measurement (p < 0.001). With the exception of one patient who refused it, all patients with lateralized disease underwent unilateral adrenalectomy with normalization of the aldosterone to renin ratio postoperatively. Conclusions: The lack of effective alternatives in subtyping primary aldosteronism highlights the need to improve the success rate of adrenal vein sampling. In this study, intraprocedural cortisol measurement allowed a selectivity of 100%. Its addition to this procedure protocol should be considered, especially in centers with a low success rate.
- Agonistas dos Receptores do GLP-1 no Tratamento da Diabetes Tipo 2Publication . Carvalho, D; Silva Nunes, J; Raposo, J; Medina, J; Jácome de Castro, J; Carrilho, FA Diabetes Mellitus tipo 2 é um flagelo individual, social e económico, em particular nas sociedades ocidentais. A metformina surge como fármaco de primeira linha na terapêutica farmacológica desta patologia, mas na impossibilidade de controlo metabólico adequado sob esta em monoterapia, as recomendações colocam as restantes classes de fármacos ao mesmo nível, reflectindo uma mudança de paradigma para a individualização da terapêutica. A partir de 2007 foram disponibilizadas três novas classes de fármacos antidiabéticos: inibidores da dipeptidil peptidase 4, agonistas dos receptores do glucagon-like peptide-1, e inibidores do co-transportador 2 de sódio e glicose. Destas três, os agonistas dos receptores do glucagon-like peptide-1 são a classe considerada mais eficaz no controlo glicémico. Este artigo debruça-se sobre as mais recentes evidências científicas sobre os agonistas dos receptores do glucagon-like peptide-1, concluindo que, para além da eficácia no controlo glicémico, são bem tolerados, com a vantagem de reduzirem o peso e apresentarem benefícios cardiovasculares. No entanto, devido às actuais condições económicas e à impossibilidade de prescrever estes fármacos indiscriminadamente, propomos as características dos doentes que mais poderão beneficiar da terapêutica com esta classe, bem como um sistema de controlo de reembolso que permita que esta seja utilizada nos doentes que mais beneficiam.
- Anticorpos Anti-Receptor da TSH na Doença de GravesPublication . Sérgio, M; Godinho, C; Guerra, L; Agapito, A; Fonseca, F; Costa, CNeste trabalho os AA avaliam a sensibilidade, especificidade e valor predictivo do doseamen to dos anticorpos anti-receptor da TSH (TRAb) no diagnóstico da doença de Graves. A população estudada incluiu 80 doentes com doença de Graves recentemente diagnosticada e sem tratamento prévio (grupo 1), 63 doentes com outras patologias tiroideias (grupo II) e 60 indivíduos sem patologia tiroideia (grupo III). Utilizaram uma técnica de radioreceptor, o kit TRAK Henning, que considera positividade> 14 U TRAb L, negatividade 9 e zona cinzenta entre estes 2 valores. No grupo 1, 11 doentes tinham TRAb negativo e 7 situavam-se na zona cinzenta. No grupo II apenas 2 doentes tinham TRAb de 9 e todos os indivíduos do grupo controlo tinham TRAb negativo. Para efeito estatístico foram excluidos os doentes com valores na zona cinzenta. Os valores de sensibilidade e especificidade para o método ensaiado foram respectivamente de 84,5° o e 10000. O valor predictivo foi de 1000 o, o que permite afirmar com segurança que um doente com hipertiroidismo e TRAb positivo tem doença de Graves.
- ARMC5 Mutation in a Portuguese Family with Primary Bilateral Macronodular Adrenal Hyperplasia (PBMAH)Publication . Rego, T; Fonseca, F; Espiard, S; Perlemoine, K; Bertherat, J; Agapito, ASUMMARY: PBMAH is a rare etiology of Cushing syndrome (CS). Familial clustering suggested a genetic cause that was recently confirmed, after identification of inactivating germline mutations in armadillo repeat-containing 5 (ARMC5) gene. A 70-year-old female patient was admitted due to left femoral neck fracture in May 2014, in Orthopedics Department. During hospitalization, hypertension (HTA) and hypokalemia were diagnosed. She presented with clinical signs of hypercortisolism and was transferred to the Endocrinology ward for suspected CS. Laboratory workup revealed: ACTH <5 pg/mL; urinary free cortisol (UFC), 532 µg/24 h (normal range: 20-90); failure to suppress the low-dose dexamethasone test (0.5 mg every 6 h for 48 h): cortisol 21 µg/dL. Abdominal magnetic resonance imaging (MRI) showed enlarged nodular adrenals (right, 55 × 54 × 30 mm; left, 85 × 53 × 35 mm), and she was submitted to bilateral adrenalectomy. In 2006, this patient's 39-year-old daughter had been treated by one of the authors. She presented with severe clinical and biological hypercortisolism. Computed tomography (CT) scan showed massively enlarged nodular adrenals with maximal axis of 15 cm for both. Bilateral adrenalectomy was performed. In this familial context of PBMAH, genetic study was performed. Leucocyte DNA genotyping identified in both patients the same germline heterozygous ARMC5 mutation in exon 1 c.172_173insA p.I58Nfs*45. The clinical cases herein described have an identical phenotype with severe hypercortisolism and huge adrenal glands, but different ages at the time of diagnosis. Current knowledge of inheritance of this disease, its insidious nature and the well-known deleterious effect of hypercortisolism favor genetic study to timely identify and treat these patients. LEARNING POINTS: PBMAH is a rare etiology of CS, characterized by functioning adrenal macronodules and variable cortisol secretion.The asymmetric/asynchronous involvement of only one adrenal gland can also occur, making disease diagnosis a challenge.Familial clustering suggests a genetic cause that was recently confirmed, after identification of inactivating germline mutations in armadillo repeat-containing 5 (ARMC5) gene.The insidious nature of this disease and the well-known deleterious effect of hypercortisolism favor genetic study of other family members, to diagnose and treat these patients timely.As ARMC5 is expressed in many organs and recent findings suggest an association of PBMAH and meningioma, a watchful follow-up is required.
- Aspetos Práticos na Terapêutica com MetforminaPublication . Silva Nunes, JA diabetes tipo 2 apresenta uma evolução pandémica a nível global. Ao longo dos últimos 100 anos vários fármacos antidiabéticos foram sendo desenvolvidos com abandono, ulterior, de alguns destes. Tal não foi o caso da metformina. Na prática clínica há mais de 50 anos, a metformina continua a constituir o fármaco antidiabético de primeira linha na terapêutica da diabetes tipo 2. A metformina está indicada ao longo de toda a história natural da doença, exceto se presença de contraindicação para o seu uso. Apesar de não ser, ainda, totalmente conhecido o seu mecanismo de ação, a metformina constitui um fármaco antidiabético que apresenta benefícios que ultrapassam o estrito efeito anti-hiperglicémico. Caracterizada por um bom perfil de segurança, a terapêutica com metformina está associada à ocorrência de sintomas gastrointestinais que são, geralmente, transitórios e passíveis de serem minimizados. A acidose láctica constitui o efeito secundário mais temido desta terapêutica. Contudo, este risco pode, também ele, ser minimizado através da observação escrupulosa de todas as contraindicações para a terapêutica com metformina.
- Atrasos Pubertários. Orientação Clínica, Diagnóstica e TerapêuticaPublication . Agapito, A; Estriga, A; Cortes, E; Malheiro, F; Charneco da Costa, JA variabilidade individual da maturação do ser humano faz que a definição e diagnóstico de atraso pubertário sejam apoiadas, por um lado, em dados estatísticos, por outro lado em critérios bem estabelecidos (tabelas de Tanner). A classificação etiológica, com as consequentes implicações terapêuticas, poderá apresentar dificuldades mormente no que respeita à distinção entre atraso pubertário simples e hipogonadismo hipoganodotrófico – as provas dinâmicas requerem uma interpretação atenta, conjugada com a clínica e, necessariamente, o estabelecimento dum protocolo que permita respostas com valor diferencial. Salientam-se os atrasos pubertários simples pela sua frequência (90% dos casos) e apontam-se linhas gerais da terapêutica desta e das outras etiologias de puberdade atrasada.
- Case Report: Artifactual Hypoglycemia: A Condition That Should Not Be ForgottenPublication . Amaral, A; Palha, A; Bernardino, V; Silva-Nunes, JBackground: Hypoglycemia is uncommon in people who are not being treated for diabetes mellitus and, when present, the differential diagnosis is broad. Artifactual hypoglycemia describes discrepancy between low capillary and normal plasma glucose levels regardless of symptoms and should be considered in patients with Raynaud's phenomenon. Case presentation: A 46-year-old female patient with a history of a sleeve gastrectomy started complaining about episodes of lipothymias preceded by sweating, nausea, and dizziness. During one of these episodes, a capillary blood glucose was obtained with a value of 24 mg/dl. She had multiple emergency admissions with low-capillary glycemia. An exhaustive investigation for possible causes of hypoglycemia was made for 18 months. The 72h fasting test was negative for hypoglycemia. A Raynaud's phenomenon was identified during one appointment. Conclusion: Artifactual hypoglycemia has been described in various conditions including Raynaud's phenomenon, peripheral arterial disease, Eisenmenger syndrome, acrocyanosis, or hypothermia. With this case report, we want to reinforce the importance of being aware of this diagnosis to prevent anxiety, unnecessary treatment, and diagnostic tests.
- Casuística da Consulta de Diabetes da Maternidade Dr. Alfredo da Costa no Ano de 1997Publication . Baleiras, C; Nabais, H; Gonçalves, G; Neves, V; Rocha, T; Aleixo, F; Delgado, EA Diabetes Mellitus é uma doença crónica que frequentemente complica a gravidez. Por outro lado, as perturbações do metabolismo glucídico podem ser exageradas pela gravidez, com as suas alterações endócrino-metabólicas. A vigilância da grávida diabética é complexa e deve envolver uma equipa multidisciplinar. Com o objectivo de avaliar as repercussões da diabetes no prognóstico da gravidez e vice-versa, os autores efectuaram a casuística da Consulta de Diabetes da Maternidade Dr. Alfredo da Costa durante o ano de 1997. Das 138 doentes que recorreram à consulta, 108 apresentavam Diabetes Gestacional/Intolerância à glicose oral (DG/IGO) e 30 Diabetes prévia à gravidez. Apenas 2 destas últimas tiveram uma consulta pré-concepcional. Cerca de 44% das mulheres com DG/IGO necessitaram de insulina em algum momento da gestação. A doença hipertensiva constituiu a complicação obstétrica mais frequente. A taxa de cesarianas (49%) situou-se acima da mencionada por outros estudos. Não se registaram mortes fetais in útero e a morbi/mortalidade neonatal foi semelhante à referida por outros autores. O dado mais importante que interessa salientar deste estudo reside no trabalho desta equipa multidisciplinar, sem o qual não se teria conseguido uma diminuição da morbilidade materna e peri-natal.