Browsing by Author "Silva, MJ"
Now showing 1 - 10 of 10
Results Per Page
Sort Options
- Burden of Liver Cirrhosis in Portugal Between 2010 and 2017Publication . Silva, JM; Silva, MJ; Calinas, F; Nogueira, PJIntroduction: Liver cirrhosis is a prevalent disease in Portugal. Recent changes in alcohol consumption, as well as the wide use of direct-acting antivirals for hepatitis C since 2015, may be contributing to changes in the national burden of liver cirrhosis in the last few years. Objectives: We aim to characterize the burden of cirrhosis in Portugal between 2010 and 2017. Patients and methods: We analyzed all hospital admission episodes due to cirrhosis in Portugal Mainland between 2010 and 2017, registered in the national Diagnosis-Related Group database, according to etiology of cirrhosis. We also analyzed data on mortality and potential years of life lost from liver cirrhosis and chronic liver disease, retrieved from Statistics Portugal (National Institute for Statistics). Results: Between 2010 and 2017, a total of 51,438 admissions for liver cirrhosis occurred in Portugal. The annual number of admissions decreased (p = 0.044) during the analyzed period. The most frequent cause of cirrhosis was alcoholic liver disease, present in 78.9% of all admissions (n = 40,595), followed by chronic hepatitis C virus infection, present in 11.3% (n = 5,823). A male predominance was identified in the admissions for every analyzed cause of cirrhosis. Annual admissions for alcoholic cirrhosis remained stable (p = 0.075) during the 8-year period. The same stable tendency was observed in the number of admissions for cirrhosis caused by hepatitis C virus (p = 0.861) and alcohol plus hepatitis C virus infection (p = 0.082), although these admissions for hepatitis C-related cirrhosis increased until 2014-2015 and steadily decreased thereafter. Annual deaths due to liver cirrhosis and chronic liver disease decreased from 1,357 in 2010 to 1,038 in 2017 (p = 0.002). The number of potential years of life lost decreased as well in the period (p = 0.001). Conclusion: The burden of cirrhosis, evaluated by hospital admissions, mortality, and potential years of life lost, decreased in Portugal between 2010 and 2017.
- Coordenação de Colheita de Órgãos e TecidosPublication . Silva, MJ
- Endoscopic Management of Sigmoid Volvulus in a Debilitated Population: What Relevance?Publication . Coelho da Rocha, M; Capela, T; Silva, MJ; Ramos, G; Coimbra, JBackground: Colonic volvulus, mainly from the sigmoid, is a relatively common cause of intestinal obstruction, particularly in the elderly and in patients with debilitating conditions. The high morbi-mortality of emergency surgery places the endoscopic approach as the first-line treatment for the resolution of this acute obstructive condition. Objectives: To assess the importance of endoscopic treatment for the resolution of colonic volvulus in a debilitated population. Method: This is a retrospective analysis of emergency lower gastrointestinal endoscopies in patients with colonic volvulus as diagnosis, performed over a 9-year period (2009-2018), as well as population characterization and follow-up after the first exam. Results: We performed 88 procedures in 52 patients (56.4% males, median age 83 years, range 33-94). Endoscopic resolution was effective in 86.4% (76/88) of the exams, without procedure complications. Eighty-one percent of patients presented with volvulus recurrence, 50% of which occurred during the 3 months after the initial procedure. Twenty-one were submitted to surgery, 12 of which were emergency procedures after endoscopic failure as the primary treatment (5 of which had mucosal necrosis at endoscopy). There was no mortality in elective surgery. In the emergency plus necrosis group, mortality was 60% (3/5) and 14.3% (1/7) in the emergency without necrosis group. Conclusions: Despite the high recurrence of volvulus after endoscopic treatment, it seems to be an adequate and low-risk first-line therapy for sigmoid volvulus in debilitated patients, allowing improvement of surgical conditions.
- Fungal GastroduodenitisPublication . Castelo Branco, S; Ferreira, AT; Saraiva, S; Silva, MJ; Garcia, TCandida infection of the gastrointestinal (GI) tract is rare in the immunocompetent individual. In immunocompromised patients, it frequently involves the oesophagus, but extra-oesophageal involvement is uncommon. We report a case of primary and isolated gastroduodenal candidiasis. Upper GI endoscopy with biopsy of gastric mucosa was crucial for making the diagnosis. The patient showed transient improvement after therapy with fluconazole. Learning points: Gastroduodenal candidiasis is rarely documented in the literature.Old age is an independent risk factor for fungal infection of the gastrointestinal tract.In this case, fungal gastroduodenitis was associated with gastroparesis.
- Gynecological History Up to Diagnosis and Pregnancy Outcomes in Diagnosed Wilson's Disease Under Therapy - a Bicentric Matched Control Cohort StudyPublication . Roseira, J; Lopes, R; Silva, MJ; Vieira, AM; Sampaio, M; Calinas, FIntroduction: most studies narrowly focus on pregnancy outcome comparisons between Wilson’s disease (WD) patients on and off treatment. We aimed to identify menses irregularities in untreated WD, and to evaluate pregnancy outcomes in treated WD patients as compared to matched controls (with and without liver disease). Methods: females with WD, hepatitis C (liver disease controls), and other gastrointestinal conditions (controls without liver disease) were identified at two tertiary hospital gastroenterology departments. Gynecological and obstetric data were retrospectively collected. A comparison of gynecological and obstetric outcomes was performed between the groups, and regression models were used to further assess obstetric outcomes. Results: a total of 18 females with WD were identified, comprising 19 pregnancies under treatment in 11 patients, and 20 females were included in each control group. Age and liver disease stage were adjusted between groups. The incidence of menses irregularities was higher for WD (late menarche, 83 % vs. 10 % vs. 10 %, p < 0.01; irregular cycles, 100 % vs. 20 % vs. 20 %, p < 0.01; amenorrhea, 67 % vs. 10 % vs. 5 %, p < 0.01). Logistic regression models identified WD as a predictor of miscarriage and low birth weight (OR: 6.0; CI: 1.1-33.3; p < 0.05) but not of birth defects. Neither therapies (D-penicillamine 300 mg or zinc acetate 150 mg) nor disease presentation (hepatic and/or neurological) were associated with obstetric complications in WD subjects. Conclusion: there was a higher incidence of menses irregularities in untreated females with WD. In addition, our data suggest that treated WD still carries a higher risk of spontaneous abortion and low birth weight when compared to matched control groups with and without liver disease.
- Interventional Algorithm in Gastrointestinal Bleeding-An Expert Consensus Multimodal Approach Based on a Multidisciplinary TeamPublication . Rodrigues, A; Carrilho, A; Almeida, N; Baldaia, C; Alves, A; Gomes, M; Gonçalves, L; Robalo Nunes, A; Leal Pereira, C; Silva, MJ; Aguiar, J; Órfão, R; Duarte, P; Tato Marinho, RThe approach to the patient with gastrointestinal bleeding (GIB) can be very complex. A multidisciplinary panel of physicians with expertise in Gastroenterology, Anesthesiology, and Transfusion Medicine worked together to provide the best knowledge and guide clinical practitioners in the real setting of health institutions, characterized by disparate availability of human and technical resources. The authors propose a global and personalized approach according to different clinical scenarios to improve the outcomes of patients with GIB, for whom the reduction of inappropriate transfusions is crucial. The goal of this document is to provide clear and objective guidance through interventional algorithms toward a goal-directed approach according to the clinical situation and supported by the latest available scientific data on GIB management in different settings.
- Intramural Hematoma of the Esophagus After Thrombolysis for Ischemic StrokePublication . Silva, MJ; Saiote, J; Salvado, V; Paiva Nunes, A; Duarte, PIntramural dissecting hematoma is an unusual esophageal condition with a threatening presentation but excellent prognosis when managed conservatively.We report the case of an 88-year-old woman who developed an intramural hematoma of the esophagus after intravenous thrombolysis for an acute ischemic stroke. Before thrombolysis, nasogastric intubation was attempted unsuccessfully. She was kept on nil by mouth, intravenous hydration, proton pump inhibitor, antiemetics,and an antibiotic initiated 2 days before for periodontal disease. The esophageal hematoma regressed, and she resumed oral diet asymptomatically.To our knowledge, this is the first report of this type of lesion after thrombolysis for an ischemic stroke. A brief discussion and literature review are presented.
- Pyruvate Dehydrogenase Complex Deficiency: Updating the Clinical, Metabolic and Mutational Landscapes in a Cohort of Portuguese PatientsPublication . Pavlu-Pereira, H; Silva, MJ; Florindo, C; Sequeira, S; Ferreira, AC; Duarte, S; Rodrigues, AL; Janeiro, P; Oliveira, A; Gomes, D; Bandeira, A; Martins, E; Gomes, R; Soares, S; Tavares de Almeida, I; Vicente, JB; Rivera, IBackground: The pyruvate dehydrogenase complex (PDC) catalyzes the irreversible decarboxylation of pyruvate into acetyl-CoA. PDC deficiency can be caused by alterations in any of the genes encoding its several subunits. The resulting phenotype, though very heterogeneous, mainly affects the central nervous system. The aim of this study is to describe and discuss the clinical, biochemical and genotypic information from thirteen PDC deficient patients, thus seeking to establish possible genotype-phenotype correlations. Results: The mutational spectrum showed that seven patients carry mutations in the PDHA1 gene encoding the E1α subunit, five patients carry mutations in the PDHX gene encoding the E3 binding protein, and the remaining patient carries mutations in the DLD gene encoding the E3 subunit. These data corroborate earlier reports describing PDHA1 mutations as the predominant cause of PDC deficiency but also reveal a notable prevalence of PDHX mutations among Portuguese patients, most of them carrying what seems to be a private mutation (p.R284X). The biochemical analyses revealed high lactate and pyruvate plasma levels whereas the lactate/pyruvate ratio was below 16; enzymatic activities, when compared to control values, indicated to be independent from the genotype and ranged from 8.5% to 30%, the latter being considered a cut-off value for primary PDC deficiency. Concerning the clinical features, all patients displayed psychomotor retardation/developmental delay, the severity of which seems to correlate with the type and localization of the mutation carried by the patient. The therapeutic options essentially include the administration of a ketogenic diet and supplementation with thiamine, although arginine aspartate intake revealed to be beneficial in some patients. Moreover, in silico analysis of the missense mutations present in this PDC deficient population allowed to envisage the molecular mechanism underlying these pathogenic variants. Conclusion: The identification of the disease-causing mutations, together with the functional and structural characterization of the mutant protein variants, allow to obtain an insight on the severity of the clinical phenotype and the selection of the most appropriate therapy.
- Relevância da Infecção VHC em Coagulopatias CongénitasPublication . Caiado, A; Antunes, M; Santos, AL; Esteves, J; Diniz, MJ; Costa, MN; Silva, MJIntrodução: A infecção pelo vírus da hepatite C (VHC) em doentes com coagulopatias congénitas (CC), como consequência da terapêutica empregue entre os anos 70 e 80 com transfusão de derivados de plasma humano, constitui um problema de saúde substancial e relevante. Objectivos: Análise e avaliação da relevância representada pela infecção VHC e suas complicações no tratamento duma população de doentes com CC. Métodos: Análise retrospectiva duma série de 161 doentes com CC tratados no Serviço de Imunohemoterapia do Centro Hospitalar de Lisboa Central (Lisboa, Portugal). Revisão sistemática de processos clínicos. Elaboração duma base de dados compreendendo a informação reunida e estudo estatístico das suas variáveis: idade, género, tipo e gravidade da coagulopatia e modalidade de tratamento. Relativamente à infecção por VHC: genotipo, tipo e duração do tratamento, frequência de resposta mantida ao tratamento e recidiva, co-infecções e complicações major e minor. Resultados: Dos 161 doentes 65 (40%) estão infectados pelo VHC. Dos doentes com hemofilia A: 36% são grave e 62% dos quais estão infectados pelo VHC; 9% moderada com 57%; 25% ligeira com 20%. No grupo da hemofilia B: 8% são grave com 23% infectados e 6% moderada ou ligeira com 10%. Relativamente ao grupo com doença de von Willebrand: 12% são tipo 2 com 16% infectados e 4% tipo 3 com 86%. Uma coorte de 26 doentes foi submetida a terapêutica para a infecção pelo VHC, com o primeiro doente a receber tratamento em 1993. Destes, 5 eram seropositivos para o VIH. O tratamento variou de monoterapia com interferão a terapêutica combinada de interferão ou interferão-peguilado com ribavirina. Conclusões: A infecção pelo VHC representa uma complicação significativa do tratamento empregue no passado na população em estudo. Considerando que a maioria destes doentes foi infectada nos finais dos anos 70 e início dos anos 80 assim como a evolução natural da infecção pelo VHC em doentes sem CC, prevê-se que a prevalência de complicações major deverá aumentar significativamente nos próximos anos. É de suma importância a implementação de medidas profilácticas na revisão e adaptação dos protocolos de seguimento de forma a prevenir a progressão da patologia hepática nestes doentes.
- Response Regarding: Endoscopic Management of Sigmoid Volvulus in a Debilitated Population: What Relevance?Publication . Simões, G; Coelho da Rocha, M; Capela, T; Silva, MJ; Ramos, G; Coimbra, J