[The natural history of cardiac involvement in Portuguese-type familial amyloid polyneuropathy].
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Biomedical subjects
Publications and source records attributed to C Fonseca.
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A study of heat-related deaths associated with the 1993 heat wave in Philadelphia, Pennsylvania, was conducted. Most of these deaths were in the susceptible elderly with preexisting natural diseases who lived alone without air conditioning in upstairs bedrooms with windows shut, thus creating an even hotter environment. These excessive deaths under such conditions did not meet the standard clinical criteria for hyperthermia because of varying postmortem intervals. Therefore, the authors stress the utility of a postmortem definition of heat-related death to better define the magnitude of health risk posed by hot weather and warn public health and other agencies to take preventative measures.
The purpose of this blind study was to determine the effect of a two-stage chlorhexidine varnish, after three months, on the gingival status of 11- to 15-year-old children attending a school in Rio de Janeiro, Brazil. Subjects participating in the study were randomly allocated to control (C) and treatment (T) groups, n = 53 and n = 57, respectively. All subjects were matched at baseline on age, salivary levels of mutans streptococci, and caries scores. After elimination of carious lesions, a prophylaxis was given to both groups. The chlorhexidine varnish was then painted on the entire dentition of Group T subjects only. Prior to caries elimination, and again after three months, the gingival index was used to assess the gingival status of study subjects. An average of 106.6 +/- 8.9 and 107.7 +/- 6.2 gingival sites per subject (four sites per tooth) in Groups C and T, respectively, were examined by the same calibrated examiner on two occasions. For statistical purposes, data were dichotomized [(0,1) (2,3)] for the gingival index. Independent t-tests and paired t-tests were used to analyze the data. The percentage of sites per subject with scores of two or three at the baseline were balanced between study groups (3.7 +/- 7.1 for T; 1.8 +/- 3.2 for C; p = 0.08). After three months, a statistically significant decrease in the average percentage of sites with scores of two or three was demonstrated in the T group (0.7 +/- 2.4, T, p < 0.0001; 1.3 +/- 3.0, C, p < 0.25). The authors concluded that the application of a chlorhexidine varnish significantly improved the gingival health of T subjects for up to three months. A significant improvement in the gingival health could not be demonstrated in the C group.
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The management of acute pulmonary edema remains an interesting challenge to the clinician. The early recognition of this frequent condition, based on clinical features, must lead to the institution of general measures of therapy that can save most patients; a delay or mistake in this early treatment is lethal. The subsequent treatment depends on the characterization of the etiology and the pathophysiological characteristics of each case. The authors present a review of the management of acute pulmonary edema for the general practitioner, which includes the general first line measures and also mention some therapeutic approaches, related to different etiologies, namely those requiring intensive care units.
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Congestive heart failure represents a clinical syndrome whose symptoms and signs result from different etiopathogenic and pathophysiological mechanisms. Diagnosing congestive heart failure remains mainly a matter for accurate clinical methodology. Therapeutical approaches to congestive heart failure require a profound knowledge of the behaviour of each system and mechanism involved in the process, namely neurohumoral activation, dysautonomia and endothelial responses, besides renal, skeletal muscle, cardiac and pulmonary participation. So, the treatment of congestive heart failure is always multifactorial. Preventing heart failure requires also an accurate knowledge of those mechanisms, in order to apply the most appropriate measures to stop vascular and cardiac remodeling.
Cefoxitin is a second generation cephalosporin commonly used to treat anaerobic and mixed infections. The authors reviewed the recently published data about the efficacy of cefoxitin; its utility in different clinical entities, patterns of resistance and resistance mechanisms, indications and reliability of in vitro susceptibility testing. These data indicate the need for determining susceptibility patterns of anaerobics at each hospital and point out to the essential close communication between the microbiologist and clinician to the rational treatment of anaerobic infections.
UNLABELLED: Heart failure (HF) is a dynamic clinical syndrome depending on multiple hemodynamic and neurohormonal factors. This syndrome concerns not only left ventricular systolic dysfunction but also left ventricular diastolic dysfunction and right ventricular dysfunction. Clinical features and therapeutic approaches are different for each of them. NYHA class IV is just one of the various prognostic factors of heart failure; consequently, severe heart failure is not synonymous of NYHA class IV. OBJECTIVE: To study hospitalised patients with heart failure in NYHA class IV, in order to characterise the predominant dysfunction, and analyze evolution and mortality. DESIGN: A retrospective analysis of a prospective study. SETTING: Hundred and eight hospitalised patients (1985-89). Patients with chronic obstructive pulmonary disease and acute myocardial infarction were excluded. PATIENTS: Sixty nine patients: 29 female and 40 male, aged 18 to 81 years old (m = 59 +/- 15.5). METHODS: Patients were clinically examined and had chest radiographs, electrocardiogram and M + 2D mode echocardiogram. Three groups were identified: Group I-patients with predominant left ventricular systolic dysfunction; Group II-patients with predominant left ventricular diastolic dysfunction; Group III-patients with predominant right ventricular dysfunction. RESULTS: 41% of the patients had coronary artery disease; 44%, valvular heart disease; 11.8% dilated cardiomyopathy; 8.7% hypertrophic cardiomyopathy; 8.7% hypertensive cardiomyopathy. Fifty five percent of the patients were in group I and the major aetiology were coronary artery disease and valvular heart disease; 25% of the patients were in group II and the major aetiology were coronary artery disease and hypertrophic cardiomyopathy; 20% of the patients were in group III, all had valvular heart disease. The global mortality during the hospitalisation period was 15.9%, mostly group III (29%) and II (17%). CONCLUSIONS: Heart failure patients in NYHA class IV formed an heterogeneous group, requiring individualised therapeutic approaches. Left ventricular systolic dysfunction was the major pathophysiological mechanism, however, diastolic dysfunction and right ventricular dysfunction were frequent. Coronary artery disease presented as a frequent aetiology of heart failure, resulting in diastolic and/or systolic dysfunction. Valvular heart disease can be present as left ventricular systolic or diastolic dysfunction or as a right ventricular dysfunction, depending on the valvulopathy and the time of evolution. Echocardiography, in association with clinical features, has been very useful for each patient approach, allowing HF aetiology and pathophysiological mechanisms characterisation. The low mortality observed in this study was related certainly to the correction of precipitating factors, together with early etiologic diagnosis and pathophysiological characterisation, and adequate individualised treatment.
OBJECTIVE: To assess safety and efficacy of ibopamine, 200 mg TID, added to conventional treatment of congestive heart failure. DESIGN: A prospective, longterm, open study over two years (1986-88). A multicenter trial. SETTING: Outpatients of Departments of Internal Medicine of S. Francisco Xavier Hospital and Sta. Maria Hospital, and Departments of Cardiology of Sta. Cruz Hospital and Hospital Militar Principal, Lisbon. PATIENTS AND METHODOLOGY: 63 patients, 49 males from 34 to 80 years (m = 55.6 +/- 11.36) and 14 females from 41 to 80 years (m = 63 +/- 10.2), with congestive heart failure, NYHA class II in 52 patients (82.5%) and NYHA class III in 11 patients (17.5%) with a mean disease duration of 47.9 months entered into the study. Digoxin, diuretics, nitrates and antiarrhythmic drugs were allowed as concomitant therapy. Patients carried out clinical examination, ECG and laboratory tests monthly and X-Ray at the beginning and at the end of each year of the study. RESULTS: 42 patients completed one year of treatment and 20/42 continued for an additional year, 17 patients completed this second year of follow-up. From the 42 patients who completed the first year period, the NYHA class changed from II to I in 17/38 from II to III in 2/38 patients from III to II in 3/4 patients, and from III to IV in one patient. Twenty patients dropped during the first year of treatment. Six for non-compliance (less than 80% of the treatment). Two were submitted to cardiac valve surgery. Seven had cardiovascular clinical events: one ventricular tachycardia, one atrial fibrillation, one pulmonary edema, one patient had no therapeutic effect, two patients had anxiety and fatigue and one patient died suddenly. One diabetic patient had uncontrolled hyperglycemia. One patient had gastric ulcer. Two had nausea and vomiting. Dysrhythmia and nausea and vomiting were the only clinical events, considered, respectively, possibly related and related, to ibopamine. During the second year of treatment 9/11 patients were stabilized in NYHA class I and 6/9 in NYHA class II, one patient changed from class II to class I, and one patient changed from class I to class II of the NYHA. Three patients did not complete the second year of treatment; one due to abnormal creatininemia; one for probable pulmonary embolism with CHF worsening; the third died suddenly. None of these events was considered related to ibopamine. Heart rate, arterial pressure, laboratory values and cardiothoracic index did not vary over the two years of the study. CONCLUSIONS: This has been the first study with data from patients treated with 200 mg TID of ibopamine during two years. Ibopamine has been shown to be a safe and useful drug added to conventional treatment of cardiac heart failure. Clinical events were few and well controlled after ibopamine interruption.
Recent evidence suggests that a key feature in the pathogenesis of hypertrophic osteoarthropathy (HOA) is an enhanced local platelet vessel wall interaction in the affected extremities. In our investigation we measured plasma levels of von Willebrand Factor Antigen (vWF:Ag; ELISA assay), a marker of platelet and/or endothelial activation, in 5 patients with the primary form of HOA and in 6 patients with the secondary form (due to cyanotic heart disease). Seven subjects matched for sex and age were used as controls. Patients showed statistically significantly higher levels of vWF:Ag in the primary (163.8 +/- 13.9%) and secondary form (152.6 +/- 9.6%, p less than 0.001 for both) when compared to controls (100.4 +/- 6.3%). Our results support the notion that platelet endothelial cell interaction may play a key role in the development of HOA.
AIM OF THE STUDY: To assess the cardiac involvement of familial amyloidotic polyneuropathy--Portuguese type (FAP) in a prospective study. INTRODUCTION: FAP is a sensitive, motor and autonomic familial polyneuropathy, due to amyloid deposits on nerve and vascular structures, related to abnormal transthyretin synthesis. FAP is a progressive systemic disease. The associated cardiomyopathy is well characterized in the Japanese form, but studies on FAP--Portuguese type cardiomyopathy remains controversial, regarding its occurrence and clinical and morphofunctional aspects. POPULATION: The Authors studied 60 Portuguese caucasoid patients, belonging to 55 different families: 23 women from 27 to 67 years old (m = 40.78 +/- 18.4) and 37 men, from 31 to 60 years old (m = 40.8 +/- 12.8). The diagnosis was established by characteristic clinical aspects, electromyograms and nerve or gut biopsies, positive for amyloid. Patients were classified in phases from I to VI according to the severity of neurological involvement and disability. METHODS: All the patients carried out clinical evaluation, thorax X-Ray, conventional EKG, Holter EKG (20-24 hours), M + 2D echocardiography and phonocardiography. Morphological and functional data were obtained from 85 echocardiographic recordings; special attention was paid to the evidence of structural abnormalities, evaluation of internal chamber dimension, left ventricular mass and systolic function indexes (left ventricular fractional shortening, left ventricular ejection fraction, left ventricular end-systolic stress index and Vcf). CONCLUSIONS: Suggestive aspects of pericardial and myocardial amyloid infiltration were found even in the earliest phases of the disease; all the records of patients in advanced phases (III to VI) were abnormal. The most frequent abnormalities founded were: a bright sparkling pericardial and myocardial echo pattern and increased ventricular wall thickness, with increased left ventricular mass index in 85% of patients in phases III to VI. Vcf was abnormally increased when plotted together with left ventricular end-systolic stress index, which was normal or decreased, probably reflecting "excessive" inotropism, in 1/3 of patients in phases III to VI. Systolic function was never depressed; no cardiac chamber enlarged. So, this study points out the presence of an infiltrative and/or hypertrophic cardiomyopathy in FAP--Portuguese type; suggestive aspects of diastolic dysfunction and decrease in systolic function were recorded. However, in the clinical setting of FAP, symptoms and signs like dyspnea, fatigue, oedema, may not be diagnosis of congestive heart disease.
We review our radiographic experience with hypertrophic osteoarthropathy. Soft tissue abnormalities such as finger clubbing, "elephant feet" and cutis verticis gyrata are well appreciated by plain radiography. Hypertrophic osteoarthropathy is characterized by a bone remodeling process at the tip of the digits. In older patients it is manifested as tuftal hypertrophy whereas in younger individuals acroosteolysis takes place. Radiographic signs of inflammatory arthropathy are conspicuously absent. Periosteal proliferation is an orderly evolving process in 3 dimensions: in the number of affected bones, in the area of involvement of a given bone and in the shape of periostitis.
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The utility of 99mTc-IDA imaging in the differential diagnosis of jaundice was assessed. Although overall accuracy was 84%, this method is still inferior to the reported efficiency of ultrasonography and CT in making the correct diagnosis. Radionuclide imaging is a good alternative when these other modalities are not available and it functions well in an adjuvant capacity. The diagnostic limitations of 99mTc-dimethyl IDA imaging in cases of moderate to severe jaundice are largely overcome by the use of 99mTc-p-butyl IDA.
99mTc-IDA (99mTc-dimethyl-acetanilide-iminodiacetic acid) hepato-biliary imaging was evaluated for its efficacy in distinguishing acute cholecystitis from acute pancreatitis. In a retrospective review, gallbladders were demonstrated by 99mTc-IDA in 13 of 15 patients (87%) with acute pancreatitis. This is significantly higher than reports on the frequency of gallbladder filling with oral and intravenous cholangiography in the presence of acute cholecystitis.
Bile flow patterns were studied with serial 99mTc-IDA images in 19 patients with cholecysto- and choledochointestinal anastomoses, gastroenteric bypasses, and combinations of the two. Complications such as anastomotic, afferent, and efferent loop obstruction and bile leakage were readily detected even in the presence of jaundice. This noninvasive technique warrants further investigation to determine its indications and weaknesses.