Disfagia e Disartria. Forma Invulgar de Apresentação da Miastenia Gravis

Myasthenia Gravis is an autoimmune disorder that generally presents with ocular symptoms, specially diplopia and ptosis. Dysphagia may be a manifestation of the generalised type of the disease, but rarely is its presenting feature. The authors describe a case of Myasthenia Gravis in an old patient complaining of dysphagia and dysarthria. Because Myasthenia Gravis is a potentially serious but treatable disease, we emphasize the need to consider it in the differential diagnosis of dysphagia, namely in the elderly. In fact, the disorder is probably underdiagnosed in this population group, because clinicians tend to accept other more frequent diagnosis to explain these symptoms.

O Rim Revisitado

O autor faz a recapitulação do percurso histórico que conduziu ao conhecimento moderno da fisiologia renale refere-se ao fascínio que esse conhecimento exerceu sobre os internistas no início da década de 60. Depois de realçar a importância do aparecimento da diálise no tratamento e no prognóstico da insuficiência renal, faz algumas considerações acerca do tratamento das glomerulopatias e dos mistérios que o rim ainda persiste em nos ocultar.

The Arterial Switch Operation in Europe for Transposition of the Great Arteries: a Multi-Institutional Study from the European Congenital Heart Surgeons Association

OBJECTIVES: This study analyzes the results of the arterial switch operation for transposition of the great arteries in member institutions of the European Congenital Heart Surgeons Association. METHODS: The records of 613 patients who underwent primary arterial switch operations in each of 19 participating institutions in the period from January 1998 through December 2000 were reviewed retrospectively. RESULTS: A ventricular septal defect was present in 186 (30%) patients. Coronary anatomy was type A in 69% of the patients, and aortic arch pathology was present in 20% of patients with ventricular septal defect. Rashkind septostomy was performed in 75% of the patients, and 69% received prostaglandin. There were 37 hospital deaths (operative mortality, 6%), 13 (3%) for patients with an intact ventricular septum and 24 (13%) for those with a ventricular septal defect (P < .001). In 36% delayed sternal closure was performed, 8% required peritoneal dialysis, and 2% required mechanical circulatory support. Median ventilation time was 58 hours, and intensive care and hospital stay were 6 and 14 days, respectively. Although of various preoperative risk factors the presence of a ventricular septal defect, arch pathology, and coronary anomalies were univariate predictors of operative mortality, only the presence of a ventricular septal defect approached statistical significance (P = .06) on multivariable analysis. Of various operative parameters, aortic crossclamp time and delayed sternal closure were also univariate predictors; however, only the latter was an independent statistically significant predictor of death. CONCLUSIONS: Results of the procedure in European centers are compatible with those in the literature. The presence of a ventricular septal defect is the clinically most important preoperative risk factor for operative death, approaching statistical significance on multivariable analysis.

Results of Surgery for Ebstein Anomaly: a Multicenter Study from the European Congenital Heart Surgeons Association

OBJECTIVE: Since most centers' experience with Ebstein anomaly is limited, we sought to analyze the collective experience of participating institutions of the European Congenital Heart Surgeons Association with surgery for this rare malformation. METHODS: The records of all 150 patients (median age 6.4 years) who underwent surgery for Ebstein anomaly in the 13 participating Association centers between January 1992 and January 2005 were reviewed retrospectively. Patients with congenitally corrected transposition were excluded. RESULTS: Most patients (81%) had Ebstein disease type B or C and significant functional impairment (61% in New York Heart Association class III or IV) and 16% had prior operations. Surgical procedures (n = 179) included valve replacement (n = 60, 33.5%), valve repair (n = 49, 27.3%), 1(1/2) ventricle repair (n = 46, 25.6%), palliative shunt (n = 13, 7.26%), and other complex procedures (n = 11, 6.14%). There were 20 hospital deaths (operative mortality 13.3%) after valve replacement in 5 patients, valve repair in 3, 1(1/2) ventricle repair in 7, palliative procedures in 3, and miscellaneous procedures in 2. Younger age and palliative procedures were univariate risk factors for operative death, but only age was an independent predictor on multivariable analysis. CONCLUSIONS: Most patients coming to surgery presented in childhood and were significantly symptomatic. More than half underwent valve replacement or repair, but a considerable proportion had severe disease necessitating 1(1/2) ventricle repair or palliative procedures. Operative mortality did not differ significantly among repair, replacement, and 1(1/2) ventricle repair but was associated with palliative procedures for severe disease early in life, young age being the only independent predictor of operative death.

Routine Transthoracic Echocardiography in a General Intensive Care Unit: an 18 Month Survey in 704 Patients

The authors analyzed 704 transthoracic echocardiographic (TTE) examinations, performed routinely to all admitted patients to a general 16-bed Intensive Care Unit (ICU) during an 18-month period. Data acquisition and prevalence of abnormalities of cardiac structures and function were assessed, as well as the new, previously unknown severe diagnoses. A TTE was performed within the first 24 h of admission on 704 consecutive patients, with a mean age of 61.5+/-17.5 years, ICU stay of 10.6+/-17.1 days, APACHE II 22.6+/-8.9, and SAPS II 52.7+/-20.4. In four patients, TTE could not be performed. Left ventricular (LV) dimensions were quantified in 689 (97.8%) patients, and LV function in 670 (95.2%) patients. Cardiac output (CO) was determined in 610 (86.7%), and mitral E/A in 399 (85.9% of patients in sinus rhythm). Echocardiographic abnormalities were detected in 234 (33%) patients, the most common being left atrial (LA) enlargement (n=163), and LV dysfunction (n=132). Patients with these alterations were older (66+/-16.5 vs 58.1+/-17.4, p<0.001), presented a higher APACHE II score (24.4+/-8.7 vs 21.1+/-8.9, p<0.001), and had a higher mortality rate (40.1% vs 25.4%, p<0.001). Severe, previously unknown echocardiographic diagnoses were detected in 53 (7.5%) patients; the most frequent condition was severe LV dysfunction. Through a multivariate logistic regression analysis, it was determined that mortality was affected by tricuspid regurgitation (p=0.016, CI 1.007-1.016) and ICU stay (p<0.001, CI 1-1.019). We conclude that TTE can detect most cardiac structures in a general ICU. One-third of the patients studied presented cardiac structural or functional alterations and 7.5% severe previously unknown diagnoses.

Síndrome de DiGeorge e Anel Vascular. Associação Invulgar e Abordagem Multidisciplinar

A Síndrome velocardiofacial/DiGeorge/CATCH 22 consiste num espectro de associação caracterizado por fácies invulgar, insuficiência velo-palatina, anomalias cardíacas conotruncais, ausência do timo e das glândulas paratiróides, frequentemente associada também a perturbações do desenvolvimento. A alteração genética associada a esta anomalia é a microdelecção no cromossoma 22 (22q11.2). É descrito o caso de um lactente de 4 meses, com antecedentes de crises de dificuldade respiratória obstrutiva baixa e má progressão ponderal desde os 2 meses de idade. Após diagnóstico tardio de truncus arteriosus foi submetido a correcção cirúrgica com pós-operatório complicado, mantendo a dependência de ventilação mecânica, com atelectasia persistente no pulmão esquerdo. A broncofibroscopia mostrou obstrução completa do brônquio principal esquerdo por compressão extrínseca. O cateterismo cardíaco revelou origem anómala da artéria subclávia direita, comum com a artéria subclávia esquerda, formando o anel vascular com o ligamento arterioso, comprimindo o brônquio esquerdo. Foi submetido a segunda cirurgia que consistiu na divisão do anel vascular por toractomia lateral esquerda. No pós-operatório a manutenção de colapso do brônquio principal esquerdo levou à colocação de stent endobrônquico. O desenvolvimento de um uqdro de dificuldade respiratória por migração do stent do brônquico esquerdo para a traqueia obrigou a terapêutica endoscópica emergente com melhoria da sintomatologia respiratória e boa evolução posterior. A associação a este síndorme DiGeorge e anel vascular é incomum. O relato deste caso petende salientar a necessidadae de abordagem técnica multidisciplinar, por vezes de forma emergente, em situações de evolução atípica condicionada pela variabilidade anatómica.

Specificity and Sensitivity of Screening for Anti-HLA Antibodies in Kidney Allograft Dysfunction

BACKGROUND: Prospective testing for posttransplant circulating anti-HLA antibodies seems to be a critical noninvasive tool, but confirmatory data are lacking. MATERIALS AND METHODS: Over the last 3 years, peritubular capillary (PTC) C4d deposition was prospectively sought by an immunofluorescence technique applied to frozen tissue in biopsies obtained for allograft dysfunction. Screening for circulating anti-HLA class I/II alloantibodies (AlloAb) by the flow cytometric test was performed simultaneously. RESULTS: We evaluated 132 sets of biopsies and simultaneous serum samples. PTC C4d deposition was demonstrated in 15.9% (21/132) of biopsies. Circulating anti-HLA I/II AlloAb were detected in 25% (33/132) of serum samples. Employing receiver-operator characteristic (ROC) curves for all C4d-positive biopsies, screening for AlloAb showed a global specificity of 82% and sensitivity of 61.9%. When this analysis was restricted to biopsies obtained in the first month posttransplantation, the sensitivity increased to 81.8%, but the specificity decreased to 76.9%. After the first month posttransplantation, we observed sensitivity of 40.0% and a specificity of 86.4%. In the first month posttransplantation, all patients with a diagnosis of acute antibody-mediated rejection displayed circulating anti-HLA class I/II, but not always at the same time as the C4d-positive biopsy. CONCLUSIONS: In the first month posttransplantation, prospective monitoring of anti-HLA antibodies may be useful. The high sensitivity allows the identification of patients at risk, affording an earlier diagnosis of antibody-mediated rejection. After the first month, the test can be used to evaluate allograft dysfunction episodes, since positivity is highly suggestive of an antibody-mediated process.

Oral Contraceptive Use and Clinical Outcomes in Patients with Multiple Sclerosis

Experimental and clinical data suggest a role of sex steroids in the pathogenesis of multiple sclerosis (MS). Scant information is available about the potential effect of oral contraceptive (OC) use on the prognosis of the disease. We aimed to evaluate this. The study population consisted of 132 women with relapsing-remitting MS before receiving disease modifying treatment and a mean disease duration 6.2 (SD 5.1) years. Three groups of patients were distinguished according to their OC behavior: [1] never-users, patients who never used OC [2] past-users, patients who stopped OC use before disease onset, and [3] after-users, those who used these drugs after disease onset. Multiple linear and logistic regression models were used to analyze the association between oral contraceptive use and annualized relapse rates, disability accumulation and severity of the disease. After-user patients had lower Expanded Disability Status Scale (EDSS) and Multiple Sclerosis Severity Score (MSSS) values than never users (p<0.001 and p=0.002, respectively) and past users (p=0.010 and p=0.002, respectively). These patients were also more likely to have a benign disease course (MSSS<2.5) than never and past users together (OR: 4.52, 95%CI: 2.13-9.56, p<0.001). This effect remained significant after adjustment for confounders, including smoking and childbirths (OR: 2.97, 95%CI: 1.24, 6.54, p=0.011 and for MSSS β: -1.04; 95% C.I. -1.78, -0.30, p=0.006). These results suggest that OC use in women with relapsing-remitting MS is possible associated with a milder disabling disease course.

Impedimentos à Prescrição de Anticoagulação na Fibrilhação Auricular e Atitude Face aos Novos Anticoagulantes Orais

Introdução e Objetivos: Avaliar a taxa de prescrição de anticoagulantes orais na fibrilhação auricular, os fatores associados à não prescrição, os motivos referidos pelos clínicos para não prescrição de anticoagulantes incluindo os de nova geração e realizar estudo evolutivo a médio prazo. Material e Métodos: Estudo prospetivo sobre casos consecutivos de doentes com fibrilhação auricular com alta hospitalar. Registaram- se os scores CHA2DS2VASc e HASBLED, comorbilidades associadas e a medicação prévia e à data de alta. Na alta hospitalar, o médico assistente indicou em questionário o motivo de não prescrição de anticoagulantes orais e dos novos anticoagulantes orais. Exclusão: contra-indicação absoluta para anticoagulação, CHA2DS2VASc ≤ 1 e doença valvular. Os doentes foram reavaliados um ano após o recrutamento do primeiro doente. Resultados: Identificaram-se 103 candidatos a anticoagulação oral (79,6 ± 8,0 anos; CHA2DS2VASc 5,8 ± 1,4; HASBLED 2,6 ± 1,0; HASBLED ≥ 3 em 55,3%); os anticoagulantes foram prescritos em 34,0%. Fatores associados à não prescrição por ordem decrescente de relevância: uso prévio de antiagregantes, doente acamado e/ou demente, ausência de insuficiência cardíaca e número de fatores de risco hemorrágico. Razões invocadas para não prescrição por ordem decrescente de frequência: risco hemorrágico elevado, pequeno benefício, incapacidade de seguir o esquema terapêutico e dificuldade na monitorização da razão normalizada internacional(INR). Os novos anticoagulantes não foram prescritos e as razões invocadas foram, por ordem decrescente de frequência: informação insuficiente sobre estes fármacos, risco hemorrágico elevado, custo elevado e pequeno benefício. Aos 8,2 ± 2,5 meses de estudo evolutivo 33,3% dos doentes encontravam-se sob anticoagulação sem que os novos anticoagulantes tivessem sido prescritos. Conclusões: Nesta amostra, a taxa de prescrição de anticoagulação oral foi baixa e o fator mais associado à não prescrição foi o uso prévio de antiagregantes. O impedimento à prescrição mais referido foi o risco hemorrágico, seguido do pequeno benefício reconhecido. Os principais impedimentos referidos à prescrição dos novos anticoagulantes foram a informação insuficiente e o alto risco hemorrágico. A médio prazo, a proporção de doentes sob anticoagulação mantinha-se baixa e os novos anticoagulantes não tinham sido prescritos.

One Anastomosis Gastric Bypass versus Roux en Y Gastric Bypass as Salvage Technique after Failed Gastric Band: a Retrospective Analysis of 80 Cases

The classical “Roux en Y Gastric Bypass” (RYGBP) is still the standard technique between all the ones being used nowadays. The “One anastomosis gastric bypass” (OAGBP), is an evolution of the “Minigastic bypass” described by Robert Rutledge in 2001, is a well known and progressively frequent but still controversial technique. In our group, after an experience of 10 years using the RYGBP as a salvage surgery after failed gastric banding, in 132 cases, we decided to adopt the OAGBP as our preferential bariatric technique also in this situation. The theoretical main reasons for that shift are related to the increased safety, maximized weight loss, long term weight loss maintenance and reversibility of the operation. Method: Retrospectively we evaluated data of the surgical management of revisional cases for conversion, after failed or complicated gastric bands to gastric bypass. We selected the last 40 cases of each technique since May 2010. Results: All cases were performed by laparoscopy without any conversion. In both groups the conversion has been performed in one single step (17 cases, 42,5%). Data showed lower morbidity with OAGBP (2,5% against 7,5%) and better weight loss in theOAGBP cohort after a median follow up of 16months (67%against 55%) in patients revised after gastric band failure or complications. None had statistic significance (p>0,1) by the chi-square contingency table analysis.Conclusion: It seems to there is a difference in favour of OAGBP for conversion of complicated gastric bands. In this study we didn’t found statistic significance probably because of the short numbers. Prospective and more powerful studies are necessary to evaluate the benefit of the studied procedure.