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- Abordagem Diagnóstica e Terapêutica da Diabetes Mellitus no Doente com Infeção pelo Vírus da Imunodeficiência HumanaPublication . Gomes, F; Vasconcelos, P; Pacheco, PA terapêutica antirretroviral levou a uma redução significativa da morbi e mortalidade nos doentes infetados pelo vírus da imunodeficiência humana. No entanto, as complicações cardiometabólicas do tratamento e o envelhecimento desta população têm alargado a complexidade de cuidados para a gestão de comorbilidades cardiovasculares. O aumento da insulinorresistência e da prevalência da diabetes mellitus tipo 2 nestes doentes é já conhecida há alguns anos, sendo, deste modo, importante conhecer as particularidades do rastreio e da abordagem terapêutica desta doença metabólica. O presente trabalho tem como objetivo rever a abordagem diagnóstica e terapêutica da diabetes mellitus no doente com infeção pelo vírus da imunodeficiência humana, assim como as interações farmacológicas entre a terapêutica antirretroviral e os antidiabéticos, visto que a literatura é escassa neste tema, que consideramos ser útil na prática clínica.
- Secular Trends in the Mortality by Cerebrovascular Diseases in Portugal: 1902-2012Publication . Ramalheira, C; Gonçalves, A; Gomes, F; Dutschmann, R; Gusmão, RIntroduction: Cerebrovascular diseases (CVD) are one of the main causes of death in Portugal. Research on the epidemiologic history might help to understand the phenomenon and guide intervention strategies. Objectives: (1) Describe historic trends in mortality. (2) Estimate the impact of demographic variations on the registered number of cases. Methods: (1) We calculated rates, specific and standardized, for deaths registered as CVD (ICD-10: I60-I69, G45; ICD8/9: 430-438; ICD-6/7: 330-334) by sex (1902-2012) and by sex and age groups (1913-2012). We used Join point analysis to identify statistically significant changes in standardized death rates, and multivariate regression models, Poisson and negative binomial, controlling for demographic dynamics and time trend, from 1913 to 2012. (2) We calculated the contribution of demographic variations using the application RiskDiff. (3) We evaluated if changes in coding rules might have been a source of bias. Data source: National Institute of Statistics. Results: (1) We gathered the longest and most discriminated mortality series from CVD in Portugal with data since the beginning of nationwide collection. Mortality increases exponentially with age and is higher in men. (2) We observed significant variations in age-standardized time trends (1913-1933: APC 2.0%; 1933-1955: APC -0.9%; 1955-1974: 2.9%; 1974- 1996: -2.4%; 1996-2012: -6.5%). (3) Population ageing exerted a significant pressure to increase the number of deaths, particularly in the second half of the twentieth century. However, this effect was counterweighted by protective factors. Conclusion: CVD mortality in Portugal is particularly expressive when compared to other European countries, although significant gains have been observed in the last decades. Population ageing combined with a transition between the morbid and mortality dimensions changed the public health paradigm.
- Etiology, Treatment and Prognosis of Acute PericarditisPublication . Serodio, J; Trindade, M; Achega, M; Faria, D; Maurício, J; Batista, F; Favas, C; Alves, JDIntroduction: Acute pericarditis is a common inflammatory condition of the pericardium usually assumed to be viral or idiopathic. Despite recent treatment improvements, information is scarce regarding etiology and prognosis. Our aim was to determine the incidence of pericarditis with a known etiology and assess clinical prognostic predictors. Material and Methods: A clinical retrospective analysis of hospitalized patients with acute pericarditis was conducted from 2012 to 2016. Population was characterized according to etiology, clinical presentation, treatment and prognosis. Outcomes of interest, evaluated at one year after hospital discharge, were pericarditis recurrence, hospitalization, constriction and overall mortality. Results: A total of 94 patients were enrolled, median age 46 years (inter-quartile range 32-61), 65% were male. Idiopathic etiology was responsible for 68% of cases. A specific etiology was found in the remaining 32% of patients, being the most frequent autoimmune disease (12%) and malignancy (5%). Idiopathic pericarditis was associated with myopericarditis (p = 0.049) and a known etiology with pericardial effusion (p = 0.001) and cardiac tamponade (p = 0.027). Recurrence of pericarditis was found in 13% of patients. Corticosteroid treatment in patients with an identified etiology was not associated with an increase in recurrence (p = 0.220). Overall 1-year mortality was 9%. A defined etiology was the only independent predictor of mortality at multivariate analysis (OR 40.3; 95% CI 1.9 – 137.2; p = 0.016). Conclusion: Up to one third of hospitalized patients with acute pericarditis have an identified cause of pericarditis and these patients are at increased risk of mortality.
- Unusual Manifestations of a Rare Clinical Entity: Erdheim-Chester DiseasePublication . Martins, N; Achega, M; Rodrigues, A; Aldomiro, FA 69-year-old woman presented with sudden left hemiparesis. Computed tomography (CT) scan excluded acute brain injuries. Patient was admitted with acute ischemic stroke of right hemisphere. There was full recovery within 24 hours and etiologic studies were normal. There was a concomitant history of ingestion of 6 litres of water per day over the previous 2 years. Water deprivation test followed by the administration of desmopressin confirmed central diabetes insipidus diagnosis. Brain magnetic resonance imaging (MRI) was normal. Full body CT scan showed arterial wall thickening suggestive of large and medium vessels vasculitis, retroperitoneal fibrosis, perinephric infiltration, pleural and pericardial thickening and diffuse densification of greater omentum. Angio-MRI showed occlusive micro-arterial injuries. Laparoscopic biopsy of greater omentum revealed multinucleated giant Touton CD68+, CD1A- cells. This finding is pathognomonic of Erdheim-Chester Disease, a rare non-Langerhans histiocytosis.
- Drug-Induced Aseptic Meningitis Following Spinal Anesthesia.Publication . Oliveira, R; Teixeira, M; Cochito, S; Furtado, A; Grima, B; Alves, JDMeningitis is a very uncommon complication of spinal anesthesia, and drug-induced aseptic meningitis (DIAM) is even rarer. We present two cases of DIAM following spinal anesthesia with bupivacaine and ropivacaine, respectively. The patients presented shortly after the procedure with typical meningitis symptoms. Since CSF (cerebrospinal fluid) analysis could not initially exclude bacterial meningitis, they were started on empirical antibiotics. CSF was subsequently found to be negative for viruses and bacteria in both cases, and antibiotics were promptly stopped. Both patients improved rapidly and without neurological sequelae. While it remains a diagnosis of exclusion, it is important to be aware of DIAM as recognition of the condition can lead to shorter admission times and avoid unnecessary use of antibiotics. LEARNING POINTS: A diagnosis of DIAM should be considered when a patient who recently underwent spinal anesthesia is admitted with symptoms and CSF compatible with meningitis.Clinical and laboratory findings (including CSF analysis) cannot distinguish between bacterial meningitis and DIAM.A negative CSF culture and rapid recovery confirm the diagnosis and stopping antibiotics at this point is effective.
- Scrofula Caused by Multidrug-Resistant TuberculosisPublication . Pedro, B; Alves, L; Magano, R; Nunes, T; Marques, NTuberculosis (TB) is one of the top 10 causes of death worldwide. Multidrug-resistant tuberculosis (MDR-TB) occurs when at the minimum there is resistance to isoniazid and rifampin. Prevention of new infections of Mycobacterium tuberculosis and progression to TB disease is critical to reduce the burden and mortality of this disease. We present the case of a 73-year-old human immunodeficiency virus (HIV)-negative female who presented with cervical lymphadenopathy and who was diagnosed with MDR-TB. LEARNING POINTS: Tuberculosis is one of the most important causes of morbidity and mortality worldwide and approximately a quarter of the world's population is estimated to be latently infected by Mycobacterium tuberculosis.Multidrug-resistant tuberculosis lymphadenitis is a rare manifestation of the disease in Portugal.With the advent of multidrug-resistant tuberculosis, disease surveillance by the government should be intensified, with the aim of national and global TB control, focusing on prevention.
- Case Report: Pure Red Cell Aplasia due to Angioimmunoblastic T-Cell Lymphoma.Publication . Vitorino, M; Nunes, F; Costa, M; Porteiro, B; Borges, A; Machado, JPure red cell aplasia (PRCA) is a rare bone marrow failure characterized by a progressive normocytic anemia and reticulocytopenia without leukopenia and thrombocytopenia. It can be associated with various hematological disorders but exceedingly rarely with angioimmunoblastic T-cell lymphoma (AITL). We report the case of a 72-year-old woman with PRCA associated with AITL. The patient presented with severe anemia (hemoglobin 2.6 g/dL) and a low reticulocyte count 0.7%. Direct and indirect Coombs tests were positive. A CT scan of the chest, abdomen, and pelvis revealed multiple lymphadenopathies. A cervical lymph node biopsy was compatible with AITL. A bone marrow biopsy showed medullary involvement by AITL and a severe erythroid hypoplasia with a myeloid:erythroid ratio of 19.70. The patient was started on CHOP and after 6 cycles the PET scan confirmed complete remission
- Pregnancy with anti-PP1Pk antibody managed with prednisolone and low-molecular-weight heparin – A case report and literature reviewPublication . Nascimento, S; Rodrigues, M; Henriques, M; Duarte, FP; Barra, A; Matos, TThe anti-PP1Pk is a rare antibody associated with recurrent miscarriages, mainly in the first half of pregnancy. There seems to be a direct correlation between the antibody titer and risk of miscarriage. As this is a rare entity, few case reports have been published. The most frequently proposed therapeutic approaches are doublefiltration plasmapheresis and plasma exchange therapy. The rationale behind them is to remove the cytotoxic antibodies from maternal circulation. Here, we present the case of a 30-year-old woman with a history of two spontaneous miscarriages and a pre-conception anti-PP1Pk antibody titer of 1:4. As soon as she became pregnant, she was placed on prednisolone and low-molecular-weight heparin (LMWH). Biweekly antibody titers were performed throughout the entire gestation and remained below 1:16. As the titers were considered to be low, plasmapheresis was not performed. The pregnancy was uneventful and she delivered a healthy newborn child at 37 weeks of gestation, with no signs of anaemia.
- Not All ENT Granulomas Are Wegener's - Keep Tuberculosis in Mind.Publication . Pedro, B; Meleiro, M; Marinho, AMycobacterium tuberculosis affects the middle ear in rare cases and is a challenging diagnosis. In this case, we present a 57-year-old patient diagnosed with anti-neutrophil cytoplasmic antibody (ANCA)-negative granulomatosis with polyangiitis (GPA) following a biopsy result of nasal granulomas, who was immediately started on immunosuppressive treatment. Years later, she developed progressive hypoacusis. Magnetic resonance imaging (MRI) revealed an extensive mass in the tympanic cavity extending to the mastoid. A biopsy of the mass was positive for Mycobacterium tuberculosis. Immunosuppressants were weaned and the patient was started on anti-tuberculous therapy with resolution of the complaints and findings. Tuberculous infections are difficult to diagnose and frequently mimic other illnesses, but in our case, we believe that an indolent tuberculous process was present from the beginning and evolved under immunosuppressive therapy. LEARNING POINTS: The differential diagnosis of rhinosinus granulomatous findings includes inflammatory and infectious diseases (for example, tuberculous infections), in addition to neoplasms, cocaine abuse and trauma.A comprehensive differential diagnosis list is essential to mitigate diagnostic errors, especially in patients where auto-immune studies are negative or there is any doubt in the diagnosis.Latent tuberculosis screening should be a concern for physicians treating patients with immunosuppressive therapy, especially in endemic countries.
- Bone Marrow Langerhans Cell Histiocytosis in Association with Kasabach-Merritt Syndrome: The Difficulty of a Differential DiagnosisPublication . Serra, JT; Silva, R; Aldomiro, F; Duarte, FPLangerhans cell histiocytosis is a rare haematological disorder with variable clinical findings and a high mortality rate. On the other hand, Kasabach-Merritt syndrome is of rare onset at adult age, requiring the simultaneous presentation of vascular lesion, thrombocytopenia, and consumptive coagulopathy. We present the first reported case of both diseases in a single patient and highlight the difficulties of diagnostic. A 69-year-old woman with immune thrombocytopenic purpura underwent surgery for the removal of giant skin haemangiomas. During post-operative care, intravascular disseminated coagulopathy developed. After weeks of corticosteroids and immunosuppressive therapy with no clinical improvement, pulmonary tuberculosis was diagnosed and appropriate treatment initiated. Despite all the efforts, the patient's clinical condition kept worsening and she eventually died. An autopsy revealed bone marrow Langerhans cell histiocytosis. In this case, the patient's autoimmune background together with tuberculosis and intravascular disseminated coagulopathy masked the presentation and made the diagnosis of a rapidly progressive fatal disease very difficult.