Browsing by Author "Costa, F"
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- Alpha-1 Antitrypsin Deficiency: Principles of CarePublication . Rodrigues, J; Mineiro, A; Reis, A; Ventura, D; Fernandez-Llimos, F; Costa, F; Gomes, J; Silva, JM; Lopes, P; Robalo Cordeiro, CAlpha-1 antitrypsin deficiency is an autosomal co-dominant inherited disorder that results in decreased circulating levels of alpha-1 antitrypsin (also known as alpha-1 proteinase inhibitor) and predisposes affected individuals to early onset lung and liver disease. There is currently no cure for alpha-1 antitrypsin deficiency. However, appropriate treatment and a high standard of clinical care can prevent patients from being seriously affected and having to undergo major medical interventions, such as organ transplantation. Beyond managing the symptoms associated with alpha-1 antitrypsin deficiency, alpha-1 proteinase inhibitor therapy is the only treatment for the condition's underlying cause. Early diagnosis is important to ensure efficient therapeutic strategies and to minimize further deterioration of lung function. alpha-1 antitrypsin deficiency is under diagnosed globally, partly because the disease has no unique presenting symptoms. This document was prepared by a Portuguese multidisciplinary group and it aims to set out comprehensive principles of care for Alpha-1 antitrypsin deficiency. These include the importance of registries, the need for clinical research, the need for consistent recommendations (regarding diagnosis, treatment and monitoring), the role of reference centres, the requirement for sustained access to treatment, diagnostic and support services, and the role of patient organizations.
- Assessment of Patient Dose Reduction by Bismuth Shielding in CT Using Measurements, GEANT4 and MCNPX SimulationsPublication . Mendes, M; Costa, F; Figueira, C; Madeira, P; Teles, P; Vaz, PThis work reports on the use of two different Monte Carlo codes (GEANT4 and MCNPX) for assessing the dose reduction using bismuth shields in computer tomography (CT) procedures in order to protect radiosensitive organs such as eye lens, thyroid and breast. Measurements were performed using head and body PMMA phantoms and an ionisation chamber placed in five different positions of the phantom. Simulations were performed to estimate Computed Tomography Dose Index values using GEANT4 and MCNPX. The relative differences between measurements and simulations were <10 %. The dose reduction arising from the use of bismuth shielding ranges from 2 to 45 %, depending on the position of the bismuth shield. The percentage of dose reduction was more significant for the area covered by the bismuth shielding (36 % for eye lens, 39 % for thyroid and 45 % for breast shields).
- Criopreservação a - 80ºC de Progenitores Hematopoiéticos para Transplantação Autóloga: Resultados a Longo PrazoPublication . Santos, A; Costa, F; Ferreira, G; Botelho de Sousa, A
- Demographic and Clinical Characteristics of Human Immunodeficiency Virus-Infected Patients Receiving Dialysis in Portugal: a Nationwide Multicentre SurveyPublication . Lopes, JA; Abreu, F; Almeida, E; Carvalho, B; Carmo, C; Carvalho, D; Barber, E; Costa, F; Silva, G; Boquinhas, H; Graça Silva, J; Inchaustegui, L; Dias, L; Batista, M; Neves, P; Mendes, TBackground: Data on human immunodeficiency virus (HIV) infected patients receiving dialysis in Portugal is scarce. Methods: This nationwide epidemiological survey retrospectively evaluates HIV-infected patients on chronic dialysis in Portugal between 1997 and 2002. Results: Sixty-six patients were evaluated (mean age: 39.1±1.6 years, 47 men, 35 black African). Sixty-two patients started dialysis and 4 patients who were receiving dialysis had HIV seroconversion. Eighty-five percent of patients were treated in Lisbon. The annual incidence of HIV-infected patients on chronic dialysis was 0.5% in 1997 and 0.9% in 2002. Seventy-eight percent of patients were HIV-1 infected , 13% had hepatitis B and 31% hepatitis C. Sexual contact was the mode of transmission of HIV in 53% of cases. Four patients had biopsy-proved HIV-associated nephropathy. Ninety-five percent of patients were on chronic hemodialysis. Fifty percent of patients had acquired immunodeficiency syndrome. At follow-up, 12 patients died. HIV-infected CKD patient survival after starting dialysis was 80% at 3 years. Conclusion: The incidence of HIV-infected patients on chronic dialysis in Portugal has almost doubled. Widespread use of highly active antiretroviral therapy and the increasing number of black Africans from former overseas Portuguese colonies now living in Portugal are possible reasons for this large increase.
- Expert Perspectives on the Management of Alpha 1-Antitrypsin DeficiencyPublication . Conde, B; Costa, F; Gomes, J; Lopes, AP; Mineiro, A; Rodrigues, O; Santos, C; Semedo, L; Sucena, M; Guimarães, CAlpha 1-antitrypsin deficiency is an inherited autosomal codominant disorder, which predisposes patients to lung and/or liver disease. Even though it is considered rare, it is one of the most frequent genetic disorders worldwide, albeit remaining underdiagnosed. Several organizations and societies, including the Portuguese Society of Pulmonology have been elaborating guidelines and recommendations for the diagnosis and management of alpha 1-antitrypsin deficiency. Nevertheless, some important matters are yet to be included in those, mainly due to lack of robust scientific evidence, and continue to represent a point of discussion. This article reviews some important scientific publications and expresses the perspectives of a group of Portuguese experts regarding the management of alpha 1-antitrypsin deficiency, namely in terms of the pre and neonatal diagnosis, the impact of the COVID-19 pandemic, the validity of replacement therapy in lung transplant-receiving, and finally, alternative strategies of alpha 1-antitrypsin deficiency treatment to improve the patients' quality of life.
- Polymerase Chain Reaction Screening for Fungemia and/or Invasive Fungal Infections in Patients with Hematologic MalignanciesPublication . Ribeiro, P; Costa, F; Monteiro, A; Caldas, J; Silva, M; Ferreira, G; Veiga, J; Sousa, MO; Viegas, MP; Santos, E; Gonçalves, AJ; Botelho de Sousa, AINTRODUCTION: Invasive fungal infections (IFIs) are a life-threatening complication in patients with hematologic malignancies, mainly in acute leukemia patients, following chemotherapy. IFI incidence is increasing, and associated mortality remains high due to unreliable diagnosis. Antifungal drugs are often limited by inadequate antimicrobial spectrum and side effects. Thus, the detection of circulating fungal DNA has been advocated as a rapid, more sensitive diagnostic tool. PATIENTS AND METHODS: Between June 01 and January 03, weekly blood samples (1,311) were screened from 193 patients undergoing intensive myelosuppressive or immunosuppressive therapy. IFI cases were classified according to European Organization for Research and Treatment of Cancer/Mycoses Study Group criteria. Fungal DNA was extracted from whole blood and amplified using polymerase chain reaction (PCR) published primers that bind to the conserved regions of the fungal 18S rRNA gene sequence. In our study, two or more consecutive positive samples were always associated with fungal disease. RESULTS: PCR screening predicted the development of IFI to be 17 days (median). This test had a specificity of 91.1% and a sensitivity of 75%. IFI incidence was 7.8%. DISCUSSION: Therefore, our results confirm the potential usefulness of PCR serial screening and the clinical applicability in everyday routine. PCR screening offers a noninvasive repeatable aid to the diagnosis of IFI.
- Tomografia Computorizada Cardíaca Prévia a Ablação de Fibrilhação Auricular - Efeitos da Evolução Tecnológica e Otimização de ProtocolosPublication . Marques, H; Araújo Gonçalves, P; Ferreira, AM; Cruz, R; Lopes, J; Santos, R; Radu, L; Costa, F; Mesquita, J; Carmo, P; Cavaco, D; Parreira, L; Pisco, J; Goyri O'Neill, J; Adragão, PIntrodução: A capacidade de a TC cardíaca fornecer um mapa anatómico preciso e excluir a presença de trombo intracardíaco é conhecida. O objetivo deste estudo foi avaliar o impacto da otimização de protocolos e evolução tecnológica nas doses de radiação e contraste e na qualidade de imagem dos exames de TC cardíaca prévia a ablação de fibrilhação auricular (FA). Métodos: Registo prospetivo de doentes consecutivos de centro único, foram incluídos os que fizeram TC cardíaca num contexto de avaliação prévia a ablação de FA (n = 270), distribuídos em três grupos: Grupo 1, constituído pelos primeiros 150 doentes; Grupo 2, os últimos 60 doentes feitos no mesmo aparelho; Grupo 3, os primeiros 60 doentes do novo aparelho. Avaliámos a otimização do protocolo com base na dose de radiação, no volume de contraste, na necessidade de aquisição complementar e na avaliação objectiva da qualidade de imagem (rácios sinal/ruído, contraste/ruído e homogeneizac¸ão de densidade AE/AAE). Resultados: Houve uma redução significativa da radiação entre cada um dos grupos e da dose de contraste entre o primeiro e o último grupo (G1: 5,6 mSv e 100 ml; G2: 1,3 mSv e 90 ml; G3: 0,6 mSv e 65 ml). Apesar das menores doses de radiação e contraste, o Grupo 3 apresentou resultados significativamente melhores de qualidade de imagem (rácios sinal/ruído 13,5; contraste/ruído 14,8; homogeneização de densidade 0,92).Conclusão: A otimização de protocolos e a evolução tecnológica permitiram reduções significativas nas doses de radiação e de contraste usadas na TC cardíaca pré-ablação de FA, sem prejudicar a qualidade de imagem.