Browsing by Issue Date, starting with "2015-12"
Now showing 1 - 8 of 8
Results Per Page
Sort Options
- Heart Transplantation in Patients Older than 65 Years: Worthwhile or Wastage of Organs?Publication . Prieto, D; Correia, P; Batista, M; Antunes, MJBACKGROUND: Patients older than 65 years have traditionally not been considered candidates for heart transplantation. However, recent studies have shown similar survival. We evaluated immediate and medium-term results in patients older than 65 years compared with younger patients. METHODS: From November 2003 to December 2013, 258 patients underwent transplantation. Children and patients with other organ transplantations were excluded from this study. Recipients were divided into two groups: 45 patients (18%) aged 65 years and older (Group A) and 203 patients (81%) younger than 65 years (Group B). RESULTS: Patients differed in age (67.0 ± 2.2 vs. 51.5 ± 9.7 years), but gender (male 77.8 vs. 77.3%; p = 0.949) was similar. Patients in Group A had more cardiovascular risk factors and ischemic cardiomyopathy (60 vs. 33.5%; p < 0.001). Donors to Group A were older (38.5 ± 11.3 vs. 34.0 ± 11.0 years; p = 0.014). Hospital mortality was 0 vs. 5.9% (p = 0.095) and 1- and 5-year survival were 88.8 ± 4.7 versus 86.8 ± 2.4% and 81.5 ± 5.9 versus 77.2 ± 3.2%, respectively. Mean follow-up was 3.8 ± 2.7 versus 4.5 ± 3.1 years. Incidence of cellular/humoral rejection was similar, but incidence of cardiac allograft vasculopathy was higher (15.6 vs. 7.4%; p = 0.081). Incidence of diabetes de novo was similar (p = 0.632), but older patients had more serious infections in the 1st year (p = 0.018). CONCLUSION: Heart transplantation in selected older patients can be performed with survival similar to younger patients, hence should not be restricted arbitrarily. Incidence of infections, graft vascular disease, and malignancies can be reduced with a more personalized approach to immunosuppression. Allocation of donors to these patients does not appear to reduce the possibility of transplanting younger patients.
- New combined CFH/MCP mutations and a rare clinical course in atypical haemolytic uraemic syndromePublication . Lopes, D; Gomes, AM; Cunha, C; Pinto, CS; Fidalgo, T; Fernandes, JCAtypical haemolytic uraemic syndrome (aHUS) is a rare, life-threatening, chronic, genetic disease due to uncontrolled alternative pathway complement activation. In this report, we discuss the case of a heterozygous carrier of a mutation on both factor H and membrane cofactor protein, who persistently presents haemolytic anaemia without need for blood transfusions, normal platelet count, normal renal function and no signs or symptoms of organ injury due to thrombotic microangiopathy 4 years after the diagnosis of aHUS.
- Outcome Measures in Polymyalgia Rheumatica. A Systematic ReviewPublication . Duarte, C; Ferreira, R; Mackie, SL; Kirwan, JR; Pereira da Silva, JAOBJECTIVE: To identify the instruments used to assess polymyalgia rheumatica (PMR) in published studies. METHODS: A systematic literature review of clinical trials and longitudinal observational studies related to PMR, published from 1970 to 2014, was carried out. All outcome and assessment instruments were extracted and categorized according to core areas and domains, as defined by the OMERACT (Outcome Measures in Rheumatology) Filter 2.0. RESULTS: Thirty-five articles (3221 patients) were included: 12 randomized controlled trials (RCT); 3 nonrandomized trials; and 20 observational studies. More than 20 domains were identified, measured by 29 different instruments. The most frequently used measures were pain, morning stiffness, patient global assessment and physician global assessment, erythrocyte sedimentation rate, and C-reactive protein. The definition of outcomes varied considerably between studies. CONCLUSION: The outcome measures and instruments used in PMR are numerous and diversely defined. The establishment of a core set of validated and standardized outcome measurements is needed.
- Prognosis following acute coronary syndromes according to prior coronary artery bypass grafting: Meta-analysisPublication . Teixeira, R; Vieira, MJ; Ribeiro, MA; Gonçalves, L; Gersh, BJPURPOSE: Conduct a meta-analysis to study the prognostic influence of a previous coronary artery bypass grafting (CABG) in patients admitted for an acute coronary syndrome (ACS). METHODS: A systematic review of the literature was performed using electronic reference databases through January 2013 (MEDLINE, Cochrane Library, Web of Knowledge, Google Scholar and references cited in other studies). Studies in which ACS outcomes with a previous history of CABG were compared with ACS outcomes with no history of previous CABG were considered for inclusion. The main endpoints of interest were mortality and non-fatal acute myocardial infarction. Data was aggregated at three follow-up times using random-effects meta-analysis models. RESULTS: Twenty-four studies were included which provided 387,181 patients for analysis. Previous CABG ACS patients were older, more diabetic and had a more frequent history of a previous myocardial infarction. Pooled in-hospital mortality was higher for the previous CABG ACS patients (OR 1.22 [1.04-1.44], p<0.01, I(2) 88%). The pooled adjusted OR showed no significant differences for the two groups (adjusted OR 1.13 [0.93-1.37], p=0.22, I(2) 92%). Previous CABG ACS patient had a higher pooled 30-day mortality (OR 1.28 [1.05-1.55], p=0.02, I(2) 74%); a higher non-adjusted (OR 1.61 [1.38-1.88], p<0.01, I(2) 70%) and adjusted (adjusted OR 1.37 [1.15-1.65], p<0.01, I(2) 0%) long-term mortality. Both the in-hospital and the long-term re-infarction rates were higher for the previous CABG ACS patients. CONCLUSIONS: According to our data, ACS patients with previous CABG history had a higher risk for short- and long-term adverse events.
- Generation of a High Number of Healthy Erythroid Cells from Gene-Edited Pyruvate Kinase Deficiency Patient-Specific Induced Pluripotent Stem CellsPublication . Garate, Z; Quintana-Bustamante, O; Crane, AM; Olivier, E; Poirot, L; Galetto, R; Kosinski, P; Hill, C; Kung, C; Agirre, X; Orman, I; Cerrato, L; Alberquilla, O; Rodriguez-Fornes, F; Fusaki, N; Garcia-Sanchez, F; Maia, TM; Ribeiro, ML; Sevilla, J; Prosper, F; Jin, S; Mountford, J; Guenechea, G; Gouble, A; Bueren, JA; Davis, BR; Segovia, JCPyruvate kinase deficiency (PKD) is a rare erythroid metabolic disease caused by mutations in the PKLR gene. Erythrocytes from PKD patients show an energetic imbalance causing chronic non-spherocytic hemolytic anemia, as pyruvate kinase defects impair ATP production in erythrocytes. We generated PKD induced pluripotent stem cells (PKDiPSCs) from peripheral blood mononuclear cells (PB-MNCs) of PKD patients by non-integrative Sendai viral vectors. PKDiPSCs were gene edited to integrate a partial codon-optimized R-type pyruvate kinase cDNA in the second intron of the PKLR gene by TALEN-mediated homologous recombination (HR). Notably, we found allele specificity of HR led by the presence of a single-nucleotide polymorphism. High numbers of erythroid cells derived from gene-edited PKDiPSCs showed correction of the energetic imbalance, providing an approach to correct metabolic erythroid diseases and demonstrating the practicality of this approach to generate the large cell numbers required for comprehensive biochemical and metabolic erythroid analyses.
- "Gunslinger's gait": a new cause of unilaterally reduced arm swingPublication . Araújo, R; Ferreira, JJ; Antonini, A; Bloem, BROBJECTIVE: To postulate a new possible cause of a unilaterally reduced arm swing in addition to the known medical conditions such as shoulder pathology, Erb's palsy, stroke, and Parkinson's disease. METHODS: Analysis of YouTube videos depicting the gait of highly ranked Russian officials. RESULTS: We found a similar walking pattern in President Vladimir Putin, Prime Minister Dmitry Medvedev and three other highly ranked Russian officials, all presenting with a consistently reduced right arm swing in the absence of other overt neurological abnormalities. CONCLUSIONS: We propose that this new gait pattern, which we term "gunslinger's gait," may result from a behavioural adaptation, possibly triggered by KGB or other forms of weapons training where trainees are taught to keep their right hand close to the chest while walking, allowing them to quickly draw a gun when faced with a foe. This should be included in the differential diagnosis of a unilaterally reduced arm swing.
- Phenotypic profile of expanded NK cells in chronic lymphoproliferative disorders: a surrogate marker for NK-cell clonalityPublication . Bárcena, P; Jara-Acevedo, M; Tabernero, MD; López, A; Sánchez, ML; García-Montero, AC; Muñoz-García, N; Vidriales, MB; Paiva, A; Lecrevisse, Q; Lima, M; Langerak, AW; Böttcher, S; van Dongen, JM; Orfão, A; Almeida, JCurrently, the lack of a universal and specific marker of clonality hampers the diagnosis and classification of chronic expansions of natural killer (NK) cells. Here we investigated the utility of flow cytometric detection of aberrant/altered NK-cell phenotypes as a surrogate marker for clonality, in the diagnostic work-up of chronic lymphoproliferative disorders of NK cells (CLPD-NK). For this purpose, a large panel of markers was evaluated by multiparametric flow cytometry on peripheral blood (PB) CD56(low) NK cells from 60 patients, including 23 subjects with predefined clonal (n = 9) and polyclonal (n = 14) CD56(low) NK-cell expansions, and 37 with CLPD-NK of undetermined clonality; also, PB samples from 10 healthy adults were included. Clonality was established using the human androgen receptor (HUMARA) assay. Clonal NK cells were found to show decreased expression of CD7, CD11b and CD38, and higher CD2, CD94 and HLADR levels vs. normal NK cells, together with a restricted repertoire of expression of the CD158a, CD158b and CD161 killer-associated receptors. In turn, NK cells from both clonal and polyclonal CLPD-NK showed similar/overlapping phenotypic profiles, except for high and more homogeneous expression of CD94 and HLADR, which was restricted to clonal CLPD-NK. We conclude that the CD94(hi)/HLADR+ phenotypic profile proved to be a useful surrogate marker for NK-cell clonality.
- Locally advanced adenocarcinoma of the rectum presenting with necrotising fasciitis of the perineum: successful management with early aggressive surgery and multimodal therapyPublication . Ferreira, L; Alexandrino, H; Soares Leite, J; Castro e Sousa, FColorectal cancer is a common malignant neoplasm and its treatment usually involves surgery associated, in some cases, depending on the staging, with chemoradiotherapy. Necrotising fasciitis of the perineum is a highly lethal infection of the perineum, perirectal tissues and genitals, requiring emergency surgical debridement, broad-spectrum antibiotics and control of sepsis. We present the case of a 59-year-old man with necrotising fasciitis of the perineum as the first clinical manifestation of locally advanced adenocarcinoma of the rectum, in which successful management consisted of early and aggressive surgical debridement, followed by multimodal therapy with curative intent. 2 years and 6 months after surgery the patient is well, with no evidence of local or systemic relapse.