Catatonia due to systemic lupus erythematosus

Objectives Discuss neuropsychiatric aspects and differential diagnosis of catatonic syndrome secondary to systemic lupus erythematosus (SLE) in a pediatric patient. Methods Single case report. Result A 13-year-old male, after two months diagnosed with SLE, started to present psychotic symptoms (behavioral changes, hallucinations and delusions) that evolved into intense catatonia. During hospitalization, neuroimaging, biochemical and serological tests for differential diagnosis with metabolic encephalopathy, neurological tumors and neuroinfections, among other tests, were performed. The possibility of neuroleptic malignant syndrome, steroid-induced psychosis and catatonia was also evaluated. A complete reversal of catatonia was achieved after using benzodiazepines in high doses, associated with immunosuppressive therapy for lupus, which speaks in favor of catatonia secondary to autoimmune encephalitis due to lupus. Conclusion Although catatonia rarely is the initial clinical presentation of SLE, the delay in recognizing the syndrome can be risky, having a negative impact on prognosis. Benzodiazepines have an important role in the catatonia resolution, especially when associated with parallel specific organic base cause treatment. The use of neuroleptics should be avoided for the duration of the catatonic syndrome as it may cause clinical deterioration.

Lewis Score - Prognostic value in patients with isolated small bowel Crohn's disease

Background and aims: Small bowel capsule endoscopy (SBCE) allows mapping of small bowel inflammation in Crohn’s disease (CD). We aimed to assess the prognostic value of the severity of inflammatory lesions, quantified by the Lewis score (LS), in patients with isolated small bowel CD. Methods: A retrospective study was performed in which 53 patients with isolated small bowel CD were submitted to SBCE at the time of diagnosis. The Lewis score was calculated and patients had at least 12 months of follow-up after diagnosis. As adverse events we defined disease flare requiring systemic corticosteroid therapy, hospitalization and/or surgery during follow-up. We compared the incidence of adverse events in 2 patient subgroups, i.e. those with moderate or severe inflammatory activity (LS =790) and those with mild inflammatory activity (135 = LS < 790). Results: The LS was =790 in 22 patients (41.5%), while 58.5% presented with LS between 135 and 790. Patients with a higher LS were more frequently smokers (p = 0.01), males (p = 0017) and under immunosuppressive therapy (p = 0.004). In multivariate analysis, moderate to severe disease at SBCE was independently associated with corticosteroid therapy during follow-up, with a relative risk (RR) of 5 (p = 0.011; 95% confidence interval [CI] 1.5–17.8), and for hospitalization, with an RR of 13.7 (p = 0 .028; 95% CI 1.3–141.9). Conclusion: In patients with moderate to severe inflammatory activity there were higher prevalences of corticosteroid therapy demand and hospitalization during follow-up. Thus, stratifying the degree of small bowel inflammatory activity with SBCE and LS calculation at the time of diagnosis provided relevant prognostic value in patients with isolated small bowel CD.

KIAA1549: BRAF gene fusion and FGFR1 hotspot mutations are prognostic factors in pilocytic astrocytomas

Up to 20% of patients with pilocytic astrocytoma (PA) experience a poor outcome. BRAF alterations and Fibroblast growth factor receptor 1 (FGFR1) point mutations are key molecular alterations in Pas, but their clinical implications are not established. We aimed to determine the frequency and prognostic role of these alterations in a cohort of 69 patients with PAs. We assessed KIAA1549:BRAF fusion by fluorescence in situ hybridization and BRAF (exon 15) mutations by capillary sequencing. In addition, FGFR1 expression was analyzed using immunohistochemistry, and this was compared with gene amplification and hotspot mutations (exons 12 and 14) assessed by fluorescence in situ hybridization and capillary sequencing. KIAA1549:BRAF fusion was identified in almost 60% of cases. Two tumors harbored mutated BRAF. Despite high FGFR1 expression overall, no cases had FGFR1 amplifications. Three cases harbored a FGFR1 p.K656E point mutation. No correlation was observed between BRAF and FGFR1 alterations. The cases were predominantly pediatric (87%), and no statistical differences were observed in molecular alterations-related patient ages. In summary, we confirmed the high frequency of KIAA1549:BRAF fusion in PAs and its association with a better outcome. Oncogenic mutations of FGFR1, although rare, occurred in a subset of patients with worse outcome. These molecular alterations may constitute alternative targets for novel clinical approaches, when radical surgical resection is unachievable.

HOXA9 as a key regulator in glioma initiation, aggressiveness and response to therapy

Tese de Doutoramento em Ciências da Saúde

Mitral valve repair. Quadrangular resection of the posterior leaflet in patients with myxomatous degeneration

OBJECTIVE - To analyze the immediate and late results of mitral valve repair with quadrangular resection of the posterior leaflet without the use of a prosthetic ring annuloplasty. METHODS - Using this technique, 118 patients with mitral valve prolapse who underwent mitral repair from January '84 through December '96 were studied. Age ranged from 30 to 86 (mean = 59.1±11.8) years and 62.7% were males. An associated surgery was performed in 22% of the patients, and coronary artery bypass graft was the most frequently performed surgery (15 patients - 12.7%). In 20 (16.9%) patients other associated techniques of mitral valve repair were used and shortening of elongated chordae tendineae was the most frequent one (6 patients). RESULTS - Immediate mortality was 0.9% (one patient). Long-term rates for thromboembolism, endocarditis, re-operation and death in the late postoperative period were 0.4%, 0.4%, 1.7% and 2.2% patients/year, respectively. The actuarial curve of survival was 83.8±8.6% over 12 years; survival free from re-operation was 91.8±4.3%, free from endocarditis was 99.2±0.8% and free from thromboembolism was 99.2±0.8%. In the late postoperative period, 93.8% of the patients were in functional class 1 (NYHA), with a complete follow-up in 89.7% of the patients. CONCLUSION - Patients with mitral valve prolapse who undergo mitral valve repair using this technique have a satisfactory prognosis over 12 years.

Permanent and temporary pacemaker implantation after orthotopic heart transplantation

PURPOSE:To determine the indication for and incidence and evolution of temporary and permanent pacemaker implantation in cardiac transplant recipients. METHODS: A retrospective review of 114 patients who underwent orthotopic heart transplantation InCor (Heart Institute USP BR) between March 1985 and May 1993. We studied the incidence of and indication for temporary pacing, the relationship between pacing and rejection, the need for pemanent pacing and the clinical follow-up. RESULTS: Fourteen of 114 (12%)heart transplant recipients required temporary pacing and 4 of 114 (3.5%) patients required permanent pacing. The indication for temporary pacing was sinus node dysfunction in 11 patients (78.5%) and atrioventricular (AV) block in 3 patients (21.4%). The indication for permanent pacemaker implantation was sinus node dysfunction in 3 patients (75%) and atrioventricular (AV) block in 1 patient (25%). We observed rejection in 3 patients (21.4%) who required temporary pacing and in 2 patients (50%) who required permanent pacing. The previous use of amiodarone was observed in 10 patients (71.4%) with temporary pacing. Seven of the 14 patients (50%) died during follow-up. CONCLUSION: Sinus node dysfunction was the principal indication for temporary and permanent pacemaker implantation in cardiac transplant recipients. The need for pacing was related to worse prognosis after cardiac transplantation.

Beta-adrenergic blocking agents in heart failure

Cardiac dysfunction in heart failure is widely recognized as a progressive process, regardless of the clinical signs and symptoms. An increase in cardiac sympathetic drive is one of the earliest neurohormonal responses occurring in patients with heart failure and may be one of the major causes of the progressive remodeling leading to the decline in myocardial function, and responsible for the poor prognosis of patients with heart failure. Therefore, recent data provided by several appropriately designed clinical trials clearly indicate the benefits of beta-adrenoceptor blocking agents, combined with diuretics, ACE inhibitors, and digoxin in chronic heart failure class II to IV due to systolic ventricular dysfunction. The benefits are related to symptoms, functional capacity, remodeling, and improvement in left ventricular function, reduction in cardiovascular hospitalization, a decrease in the overall and sudden cardiac death rate, and are similar in patients with ischemic or nonischemic cardiomyopathy, independent of age, gender, or functional class. In this review we describe the cardiovascular effects of the increase in sympathetic drive, the pharmacological properties of the beta-blockers most evaluated in heart failure therapy (metoprolol, bisoprolol, and carvedilol), the major clinical trials related to these agents in heart failure, the recommendations for their appropriate use in clinical practice, the precautions to be adopted, and how to handle the more common adverse reactions.

Clinical significance of in-hospital reocclusion after mechanical reperfusion and percutaneous transluminal coronary angioplasty for acute myocardial infarction

OBJECTIVE: To analyze the effects of in-hospital reocclusion of reperfused AMI culprit coronary arteries in mortality and to identify the predictors. METHODS: The present study comprises a sample of 155 patients with AMI who underwent successful mechanical reperfusion by direct coronary angioplasty and angiographic control during hospitalization or before discharge. Patients were classified into group A: reoccluded patients (n=30) and group B: non-reoccluded patients (n=125). RESULTS: We identified in-hospital reocclusion predictors and found a greater significance in mortality among reoccluded patients (23,3% x 1.6%; p=0.00004). Silent reocclusion or typical angina at reocclusion had a good prognosis. The independent predictors of in-hospital mortality were hypertension, multiarterial lesions, totally occluded AMI culprit lesions, failed redilatation, failed redilatation in comparison with no intention to redilate, no redilatation in comparison with no atempt to redilate, and reocclusion within the first 48 to 72 hours. The decision to redilate, independently of the result, led to a 50.0% reduction in hospital mortality (p=0.0366). CONCLUSION: In-hospital AMI culprit coronary artery reocclusion had an adverse effect similar to that reported in clinical studies with high mortality rates (23.3% x 1.6%; p=0.00004). The major contribution of this study is to recommend the reopening of reoccluded AMI culprit coronary arteries as a means for the management of coronary artery reocclusion.

Estimation of statistical cure from cancer using population-based data

Dissertação de mestrado em Estatística

Chagas' heart disease: evolutive evaluation of electrocardiographic and echocardiographic parameters in patients with the indeterminate form

OBJECTIVE: To identify and associate potential electrocardiographic and echocardiographic changes in patients with the indeterminate form of Chagas' disease during long-term follow-up. METHODS: One hundred sixty patients underwent standard electrocardiography and two-dimensional guided M-mode echocardiography for left ventricular ejection fraction determination. Patients were followed up for 98.6±30.4 months, undergoing repeat electrocardiographic studies at 6-month intervals and echocardiographic studies at 12-month intervals. RESULTS: Based on the electrocardiographic findings, the patients were divided into group I, 125 patients (78.6%) with normal electrocardiograms throughout follow-up, and group II, 34 patients (21.3%) who developed electrocardiographic changes. Group II was further divided into group IIA (9 patients, 5.6%) with permanent electrocardiographic changes, group IIB (14 patients, 8.8%) with transitory electrocardiographic changes, and group IIC (11 patients, 6.9%) with changes appearing only on the final electrocardiogram. Left ventricular ejection fractions remained normal in the entire population studied and did not differ among groups. CONCLUSION: The indeterminate form of Chagas' disease clearly represents a benign condition with a favorable long-term prognosis. Although some patients develop electrocardiographic changes, left ventricular systolic function is well preserved.

Prognostic factors of the results of cardiopulmonary resuscitation in a cardiology hospital

OBJECTIVE: To analyze the early and late results of cardiopulmonary resuscitation in a cardiology hospital and to try to detect prognostic determinants of both short- and long-term survival. METHODS: A series of 557 patients who suffered cardiorespiratory arrest (CRA) at the Dante Pazzanese Cardiology Institute over a period of 5 years was analyzed to examine factors predicting successful resuscitation and long-term survival. RESULTS: Ressuscitation maneuvers were tried in 536 patients; 281 patients (52.4%) died immediately, and 164 patients (30.6%) survived for than 24 hours. The 87 patients who survived for more than 1 month after CRA were compared with nonsurvivors. Coronary disease, cardiomyopathy, and valvular disease had a better prognosis. Primary arrhythmia occurred in 73.5% of the >1-month survivor group and heart failure occurred in 12.6% of this group. In those patients in whom the initial mechanism of CRA was ventricular fibrillation, 33.3% survived for more than 1 month, but of those with ventricular asystole only 4.3% survived. None of the 10 patients with electromechanical dissociation survived. There was worse prognosis in patients included in the extreme age groups (zero to 10 years and 70 years or more). The best results occurred when the cardiac arrest took place in the catheterization laboratories. The worst results occurred in the intensive care unit and the hemodialysis room. CONCLUSION: The results in our series may serve as a helpful guide to physicians with the difficult task of deciding when not to resuscitate or when to stop resuscitation efforts.

Clinical Meaning of Ascites in Patients with Endomyocardial Fibrosis

OBJECTIVE: To evaluate the clinical meaning of ascites and the main features of patients with ascites and endomyocardial fibrosis. METHODS: We studied 166 patients with endomyocardial fibrosis (mean age 37 years, 114 women) treated over the last 20 years. Ventriculography findings, surgery or necropsy confirmed the diagnosis in all patients. Most patients belonged to New York Heart Association Functional Class III/IV (134, 83.7%). Eighty-one (50.6%) had biventricular, 28 (17.5%) had right ventricular, and 51 (31.8%) had left ventricular involvement. During follow-up, 56 patients died. RESULTS: Ascites was present in 67 (41.8%) patients, and right ventricular involvement was present in 59 (88%). In the comparison between patients with or without ascites, those with ascites had higher mortality (49.2% and 24.7%, respectively). Patients with ascites had a higher incidence of edema (95% vs. 43%), hepatomegaly (5.8cm vs. 4.1cm), mean right atrium pressure (19.3 vs. 12mmHg), and final right ventricle diastolic pressure (18.7 vs. 12.9mmHg). Also, patients with ascites had a longer history of illness (5.1 and 3.9 years, respectively) and had atrial fibrillation more frequently (44.7% vs. 30.1%). CONCLUSION: Ascites was observed in less than 50% of cases of endomyocardial fibrosis and was associated with greater involvement of the right ventricle and with a longer duration of the disease, thus being a characteristic of a worse prognosis.

A Real-Time intelligent system for tracking patient condition

Hospitals have multiple data sources, such as embedded systems, monitors and sensors. The number of data available is increasing and the information are used not only to care the patient but also to assist the decision processes. The introduction of intelligent environments in health care institutions has been adopted due their ability to provide useful information for health professionals, either in helping to identify prognosis or also to understand patient condition. Behind of this concept arises this Intelligent System to track patient condition (e.g. critic events) in health care. This system has the great advantage of being adaptable to the environment and user needs. The system is focused in identifying critic events from data streaming (e.g. vital signs and ventilation) which is particularly valuable for understanding the patient’s condition. This work aims to demonstrate the process of creating an intelligent system capable of operating in a real environment using streaming data provided by ventilators and vital signs monitors. Its development is important to the physician because becomes possible crossing multiple variables in real-time by analyzing if a value is critic or not and if their variation has or not clinical importance.

Electrocardiographic Alterations in Patients Hospitalized with Leptospirosis in the Brazilian City of Salvador

OBJECTIVE: To report the frequency and types of electrocardiographic alterations in patients with leptospirosis in the first 24 hours of hospitalization. METHODS: We analyzed the electrocardiograms of 157 patients admitted to the Hospital Couto Maia in the city of Salvador, in the State of Bahia, Brazil, from March 1998 to June 1999. The electrocardiograms were performed in the first 24 hours after hospital admission, independent of the clinical manifestations of the patients. RESULTS: The mean ± SD for patients' age was 35.5± 13.7 (median = 32) years, and jaundice was present in 95.5% of them. Alterations in the electrocardiogram were detected in 68.2% (107/157) of the patients (95% confidence interval = 60.6% - 75.1%). Atrial fibrillation was the most frequent arrhythmia, occurring in 10.8% (17/157) of the patients. Other frequent findings were alterations in ventricular repolarization detected in 38.9% (61/157) of patients and first-degree atrioventricular block in 10.2% (16/157). The patients with atrial fibrillation were older and had higher levels of creatinine and aminotransferases. CONCLUSION: In this sample, approximately 2/3 of the patients had electrocardiographic alterations after hospital admission. Of the major arrhythmias, atrial fibrillation was the most frequent, and the patients with this arrhythmia had evidence of more severe disease. The relation between the presence and type of electrocardiographic alteration and the prognosis of leptospirosis is yet to be assessed.

Improvement in left ventricular dysfunction after surgical correction of mitral regurgitation

OBJECTIVE: To evaluate whether left ventricular end-systolic (ESD) diameters £ 51mm in patients (pt) with severe chronic mitral regurgitation (MR) are predictors of a poor prognosis after mitral valve surgery (MVS). METHODS: Eleven pt (aged 36±13 years) were studied in the preoperative period (pre), median of 36 days; in the early postoperative period (post1), median of 9 days; and in the late postoperative period (post2), mean of 38.5±37.6 months. Clinical and echocardiographic data were gathered from each pt with MR and systolic diameter ³51mm (mean = 57±4mm) to evaluate the result of MVS. Ten patients were in NYHA Class III/IV. RESULTS: All but 2 pt improved in functional class. Two pt died from heart failure and infectious endocarditis 14 and 11 months, respectively, after valve replacement. According to ejection fraction (EF) in post2, we identified 2 groups: group 1 (n=6), whose EF decreased in post1, but increased in post2 (p=0.01) and group 2 (n=5), whose EF decreased progressively from post1 to post2 (p=0.10). All pt with symptoms lasting £ 48 months had improvement in EF in post2 (p=0.01). CONCLUSION: ESD ³51mm are not always associated with a poor prognosis after MVS in patients with MR. Symptoms lasting up to 48 months are associated with improvement in left ventricular function.