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- SomosHFF (30): Newsletter do Hospital Prof. Dr. Fernando Fonseca, E.P.E.Publication . Conselho Editorial SomosHFF
- Connective tissue disease-associated interstitial lung diseasePublication . Oliveira, RP; Ribeiro, R; Melo, L; Grima, B; Oliveira, S; Alves, JDBACKGROUND: Connective tissue diseases (CTD) are frequently associated with interstitial lung disease (ILD), significantly impacting their morbidity and mortality. AIM: Analyze the experience of an autoimmune specialized unit on treating CTD-ILD and characterize the population based on most frequent diseases, imaging patterns, lung function tests results, serology and treatment. Assess mortality and mortality predictors in these patients. METHODS: Retrospective, descriptive and statistical analysis of the CTD-ILD patients followed up at an autoimmune diseases unit during a 6-year period. RESULTS: Over the study period, 75 patients with CTD-ILD were treated with a mean follow-up of 49 ± 31 months. The most frequent CTD were systemic sclerosis and rheumatoid arthritis. ILD was diagnosed prior to CTD in 8% of patients and concomitantly in 35%. Nonspecific interstitial pneumonia was the CT pattern in 60% and 35% had an isolated diminished DLCO on lung function tests. Pulmonary hypertension was present in 12% and it was the single most important mortality predictor (OR 14.41, p = 0.006). Corticosteroids are the mainstay of treatment but biologics were prescribed in 39% of the patients (mostly tocilizumab and rituximab). Two scleroderma patients were recently treated with nintedanib. CONCLUSIONS: ILD is a potential complication of every CTD and can impose a dramatic burden on these patients. The clinical relevance of ILD together with their early expression in the course of the disease underlines the importance of the presence of chest physicians in these units.
- Utility of routine screening for alpha-1 antitrypsin deficiency in patients with bronchiectasisPublication . Carreto, L; Morrison, M; Donovan, J; Finch, S; Tan, GL; Fardon, T, et al.Alpha-1 antitrypsin deficiency (AATD) is a cause of bronchiectasis. Guidelines for bronchiectasis from the British Thoracic Society do not recommend to routinely test patients for AATD. In contrast, guidelines for AATD recommend routine screening. This contradiction, in part, results from the lack of data from large studies performing comprehensive screening. We screened 1600 patients with bronchiectasis at two centres in the UK from 2012 to 2016. In total, only eight individuals with AATD were identified representing 0.5% of the overall population. We conclude that routine screening for AATD in bronchiectasis in the UK has a low rate of detection. Further studies are required in different geographical regions, which may have a higher prevalence of AATD.
- SomosHFF (32): Newsletter do Hospital Prof. Dr. Fernando Fonseca, E.P.E.Publication . Conselho Editorial SomosHFF
- Gastrointestinal Tuberculosis Mimicking Crohn’s DiseasePublication . Rafael, MA; Figueiredo, L; Oliveira, AM; Costa, M; Manso, RT; Martins, AWe present the case of a 24-year-old woman with complaints of abdominal pain, bloody diarrhea, and weight loss for 3 months. An outpatient colonoscopy revealed scattered ulcers, suggestive of Crohn's disease (CD). Histopathology also favored the diagnosis of CD. However, after admission to our hospital for further investigation, a chest radiograph revealed pulmonary cavitations. A computed tomography scan suggested the diagnosis of active pulmonary tuberculosis (TB). Therefore, a bronchofibroscopy, a total colonoscopy with ileoscopy, and an upper endoscopy were performed. Not only were acid-fast bacilli present in both bronchoalveolar lavage fluid and gastric juice, but also in colonic biopsies. A complete resolution of gastrointestinal symptoms was achieved 2 weeks after starting anti-TB drugs.
- SomosHFF (35): Newsletter do Hospital Prof. Dr. Fernando Fonseca, E.P.E.Publication . Conselho Editorial SomosHFF
- Correction to: Clinical management of ageing people living with HIV in Europe: the view of the care providers.Publication . Boffito, M; Ryom, L; Spinner, C; Martinez, E; Behrens, G; Rockstroh, J; Hohenauer, J; Lacombe, K; Psichogyiou, M; Voith, N; Mallon, P; Branco, T, et al.
- 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
- Should We Use Papilla Morphology to Estimate the Size of the Terminal Common Bile Duct During Endoscopic Retrograde Cholangiopancreatography?Publication . Lopes, L; Canena, J; Fernandes, J; Moreira, M; Costa, I; Gomes-Fonseca, J, et al.Objective: A small common bile duct (CBD) diameter has been associated with complications and with a difficult biliary cannulation. Previous studies suggested that this diameter can be predicted during the endoscopic retrograde cholangiopancreatography (ERCP) simply by observing the papillary morphology. Despite this published suggestion there is no study addressing this topic. This study evaluated a possible association between the morphology of the major papilla and the diameter of the terminal CBD (t-CBD). Methods: Observational cross-sectional study including consecutive patients with naïve papillae was referred for ERCP in two affiliated university hospitals. The transverse (p-transv) and longitudinal measures (p-long) of the papilla were obtained using a visual method. Papillae were classified into nonprominent, prominent, bulging or other. The t-CBD was measured 1 cm from the papilla using fluoroscopic images in prone/supine. Measurements were performed by two senior endoscopists and outcomes were evaluated using correlation and linear regression model. Results: We included 245 patients with a median age of 76 years. The median p-transv for each type of papillae was as follows: nonprominent = 6 mm, prominent = 9 mm, bulging = 15 mm and other = 6 mm; P < 0.001. The median t-CBD for nonprominent = 7.62 mm, prominent = 8.34 mm, bulging = 8.60 mm and other = 8.52 mm; P = 0.40. The correlation between the transverse and longitudinal measures of papilla and the t-CBD were 0.0092 and 0.0614, respectively. In the regression model, the t-CBD diameter was not explained by papilla's size or morphology (R = 1.70%; P = 0.80). Conclusion: The morphology of the papilla must not be used as a predictor of the diameter of the CBD as there is no correlation between these two items.
- Wernicke-Korsakoff Syndrome: A Case Series in Liaison Psychiatry.Publication . Barata, PC; Serrano, R; Afonso, H; Luís, A; Maia, TWernicke-Korsakoff syndrome (WKS) is a life-threatening and underdiagnosed neuropsychiatric condition caused by thiamine deficiency that comprises Wernicke encephalopathy and Korsakoff syndrome. Although mainly associated with chronic alcoholism, WKS can arise from other circumstances. This report describes a series of cases of WKS that were clinically evaluated by liaison psychiatrists on a nonpsychiatric inpatient unit. The cases illustrate a deficit in the recognition and adequate treatment of WKS, demonstrating its clinical complexity and the need to improve physicians' knowledge