Heart involvement in hepatolenticular degeneration

The case of a 10-year-old boy with a mild abdominal form of hepatolenticular degeneration, showing clinical, laboratory and histological manifestations of heart involvement is reported. The microscopical examination of myocardial fragments collected by intracavitary puncture biopsy, using hematoxylin-eosine and the histochemical method with rubeanic acid, showed a medium degree of myocardial damage and the presence of copper deposition in the tissue. Copper concentration on a myocardial biopsy fragment was almost 10 times the normal heart content. Comments are made on the relationship of the clinical, laboratory and pathological findings with the degree of copper storage on the myocardium.     

The Abortion and Sterilization Act in 1975--experience of the Johannesburg Hospital Pregnancy Advisory Clinic

The experience of a Pregnancy Advisory Service operating in terms of the Abortion and Sterilization Act of 1975 is reviewed. A total of 454 applications for abortion were considered, of which 198 (44%) were accepted and the abortions performed. The urgent need for sex education at schools, colleges and universities is stressed, in view of the large number of scholars and students under the age of 17 who seek an abortion. There are also patients who discontinue contraception for a variety of reasons, on medical advice or otherwise, without an alternative being suggested or sought. Motivation of patient, nurse and doctor needs urgent improvement.     

Phase-II trial with vindesine for regression induction in patients with leukemias and hematosarcomas

Summary Vindesine (VDS) has been submitted to a phase-II trial, the results of which were assessed in terms of regression induction. VDS was given weekly IV in doses of 2 mg/m² on two consecutive days to 59 patients, 55 of whom were evaluable. A high proportion of complete (36%) and over 50% partial regressions were obtained in acute lymphoid leukemias (ALL) (overall response 63%) whatever the perceptible phase, in blastic crisis of chronic myeloid leukemia (55%), and some responses were recorded in lymphosarcoma (40%). No effect has so far been seen in acute myeloid leukemia or in Hodgkin's disease. Malignant neoplasms of the immunoblastic type seem to be particularly sensitive to VDS. Continuous 48 h IV infusion can induce a remission where an IV push administration of the same dose has failed. One remarkable characteristic of VDS is the apparent absence of cross-resistance with VCR: in acute leukemic forms, 55% of patients who failed to obtain remission induction after three weekly injections of VCR (used in combination chemotherapy) achieved a complete or partial remission with VDS. The toxicity was mainly neurologic (paralytic ileus, constipation, paresthesias, loss of reflexes) and hematologic (leukopenia and thrombopenia), and was not more significant than with the other agents: four patients died of infection or hemorrhage.     

A monitoring system for work-related accidents in Piracicaba, São Paulo, Brazil

The authors report on the development of a work accident monitoring system in Piracicaba, S?o Paulo State, Brazil, with the following characteristics: information feeding the system is obtained in real time directly from accident treatment centers; the system has universal monitoring, covering all work-related accidents in Piracicaba, regardless of the nature of the worker's employment conditions, place of work, or place of residence; health surveillance and promotion of health initiatives are triggered by identification of sentinel events; spatial distribution analysis of work-related accidents is a basic tool in designing accident awareness strategies and accident prevention policies. The system was implemented in November 2003 and by October 2004 had identified 5,320 work-related accidents, or a 3.8% annual proportional incidence of work-related accidents in the municipal area. We illustrate spatial analysis of registered work-related accidents and present a detailed investigation of one example of a serious accident.     

Robotics and other devices in the treatment of patients recovering from stroke

Stroke is the leading cause of permanent disability in the United States despite advances in prevention and novel interventional treatments. Randomized controlled studies have demonstrated the effectiveness of specialized post-stroke rehabilitation units, but administrative orders have severely limited the length of stay, so novel approaches to the treatment of recovery need to be tested in outpatients. Although the mechanisms of stroke recovery depend on multiple factors, a number of techniques that concentrate on enhanced exercise of the paralyzed limb have demonstrated effectiveness in reducing the motor impairment. For example, interactive robotic devices are new tools for therapists to deliver enhanced sensorimotor training for the paralyzed upper limb, which can potentially improve patient outcome and increase patient productivity. New data support the idea that for some post-stroke patients and for some aspects of training-induced recovery, timing of the training may be less important than the quality and intensity of the training. The positive outcome that resulted in the interactive robotic trials contrasts with the failure to find a beneficial result in trials that used a noninteractive device that delivered continuous passive motion only. New pilot data from novel devices to move the wrist demonstrate benefit and suggest that successive improvement of the function of the arm progressing to the distal muscles may eventually lead to significant disability reduction. These data from robotic trials continue to contribute to the emerging scientific basis of neuro-rehabilitation.     

Association between ambient air pollution and birth weight in São Paulo, Brazil

Objectives: Previous studies have implicated air pollution in increased mortality and morbidity, especially in the elderly population and children. More recently, associations with mortality in infants and with some reproductive outcomes have also been reported. The aim of this study is to explore the association between exposure to outdoor air pollution during pregnancy and birth weight. Design: Cross sectional study using data on all singleton full term live births during a one year period. For each individual birth, information on gestational age, type of delivery, birth weight, sex, maternal education, maternal age, place of residence, and parity was available. Daily mean levels of PM₁₀, sulphur dioxide, nitrogen dioxide, carbon monoxide, and ozone were also gathered. The association between birth weight and air pollution was assessed in regression models with exposure averaged over each trimester of pregnancy. Setting: São Paulo city, Brazil. Results: Birth weight was shown to be associated with length of gestation, maternal age and instruction, infant gender, number of antenatal care visits, parity, and type of delivery. On adjusting for these variables negative effects of exposure to PM₁₀ and carbon monoxide during the first trimester were observed. This effect seemed to be more robust for carbon monoxide. For a 1 ppm increase in mean exposure to carbon monoxide during the first trimester a reduction of 23 g in birth weight was estimated. Conclusions: The results are consistent in revealing that exposure to air pollution during pregnancy may interfere with weight gain in the fetus. Given the poorer outlook for low birthweight babies on a number of health outcomes, this finding is important from the public health perspective.     

Robotic technology and stroke rehabilitation: translating research into practice

Research on the effectiveness of robotic therapy for the paretic upper limb after stroke has shown statistically significant reductions in motor impairment during both acute and chronic phases of recovery. Despite growing empirical support for this technology and a stronger focus on optimizing rehabilitation outcomes and productivity, there continues to be a disconnect between research and clinical practice. We review studies on the use of robot-aided neurorehabilitation for the paretic arm after stroke and discuss ways in which this technology may provide opportunities for intensive training that complement more conventional therapy methods.     

Drinking practices and other health-related behaviors among adolescents of São Paulo City,Brazil

PURPOSE: (a) detect possible demographic and behavioral differences among young episodic heavy drinkers and other young drinkers; (b) investigate the association of youth drinking patterns with other health-compromising behaviors. METHODS: The data presented are part of a health behavior survey, which used a modified version of the Youth Health Risk Behavior Survey (YHRBS) questionnaire. Students from public (n = 993) and private schools (n = 815), from 7th to 11th grades were investigated in S?o Paulo city. Multivariate logistic regression analyses were employed to investigate the association among different drinking practices and the various health-compromising behaviors, controlling for age group, gender, and school system. Odds Ratios (OR) and their 95% Confidence Intervals (CI) were calculated. Episodic Heavy Drinking was defined as having five or more drinks in a 2-hour interval, at least once in the last 30 days. RESULTS: Episodic Heavy Drinkers (EHDs) tend to be older and males and prefer to drink with friends at commercial facilities instead of drinking with their relatives at home. EHDs also reported higher percentages of adverse consequences, such as physical fights, accidents, and school absenteism after drinking. EHDs are more likely to engage in other high-risk behaviors. In the public schools, they were more likely to carry guns (OR = 17.0; CI = 3.9-74.8), get involved in physical fights (OR = 8.9; CI = 4.4-18.0), attempt suicide (OR = 4.2; CI = 2.0-8.7), and use inhalants (OR = 2.7; CI = 1.3-5.7) than abstainers. They are also more likely to use marijuana (OR = 4.7; CI = 2.2-9.9) and smoke cigarettes (OR = 5.3; CI = 2.7-10.4) than moderate drinkers. The results were similar for private schools, with even higher ratios of inhalant use (OR = 15.2; CI = 6.2-37.1), when EHDs were compared with abstainers and cigarette (OR = 64.5; CI = 8.6-481.0) and marijuana use (OR = 6.5; CI = 4.3-9.7), when EHDs were compared with moderate drinkers. CONCLUSIONS: EHDs display different drinking habits than other adolescents who drink. Also, they are at increased risk for a range of health-compromising behaviors, when compared with both abstainers and moderate drinkers.     

Itraconazole oral solution for primary prophylaxis of fungal infections in patients with hematological malignancy and profound neutropenia: a randomized, double-blind, double-placebo, multicenter trial comparing itraconazole and amphotericin B

Systemic and superficial fungal infections are a major problem among immunocompromised patients with hematological malignancy. A double-blind, double-placebo, randomized, multicenter trial was performed to compare the efficacy and safety of itraconazole oral solution (2.5 mg/kg of body weight twice a day) with amphotericin B capsules (500 mg orally four times a day) for prophylaxis of systemic and superficial fungal infection. Prophylactic treatment was initiated on the first day of chemotherapy and was continued until the end of the neutropenic period (>0.5 x 10(9) neutrophils/liter) or up to a maximum of 3 days following the end of neutropenia, unless a systemic fungal infection was documented or suspected. The maximum treatment duration was 56 days. In the intent-to-treat population, invasive aspergillosis was noted in 5 (1.8%) of the 281 patients assigned to itraconazole oral solution and in 9 (3.3%) of the 276 patients assigned to oral amphotericin B; of these, 1 and 4 patients died, respectively. Proven systemic fungal infection (including invasive aspergillosis) occurred in 8 patients (2.8%) who received itraconazole, compared with 13 (4.7%) who received oral amphotericin B. Itraconazole significantly reduced the incidence of superficial fungal infections as compared to oral amphotericin B (2 [1%] versus 13 [5%]; P = 0.004). Although the incidences of suspected fungal infection (including fever of unknown origin) were not different between the groups, fewer patients were administered intravenous systemic antifungals (mainly intravenous amphotericin B) in the group receiving itraconazole than in the group receiving oral amphotericin B (114 [41%] versus 132 [48%]; P = 0.066). Adequate plasma itraconazole levels were achieved in about 80% of the patients from 1 week after the start of treatment. In both groups, the trial medication was safe and well tolerated. Prophylactic administration of itraconazole oral solution significantly reduces superficial fungal infection in patients with hematological malignancies and neutropenia. The incidence of proven systemic fungal infections, the number of deaths due to deep fungal infections, and the use of systemic antifungals tended to be lower in the itraconazole-treated group than in the amphotericin B-treated group, without statistical significance. Itraconazole oral solution is a broad-spectrum systemic antifungal agent with prophylactic activity in neutropenic patients, especially for those at high risk of prolonged neutropenia.     

Kaposi sarcoma herpesvirus K5 removes CD31/PECAM from endothelial cells

The transmembrane ubiquitin ligase K5/MIR2 of Kaposi sarcoma herpesvirus (KSHV) mediates internalization and lysosomal degradation of glycoproteins involved in antigen presentation and co-stimulation. In endothelial cells (ECs), K5 additionally reduced expression of CD31/platelet-endothelial cell adhesion molecule (PECAM), an adhesion molecule regulating cell-cell interactions of ECs, platelets, monocytes, and T cells. K5 also reduced EC migration, a CD31-dependent process. Unlike other K5 substrates, both newly synthesized and pre-existing CD31 molecules were targeted by K5. K5 was transported to the cell surface and ubiquitinated pre-existing CD31, resulting in endocytosis and lysosomal degradation. In the endoplasmic reticulum, newly synthesized CD31 was degraded by proteasomes, which required binding of phosphofurin acidic cluster sorting protein-2 (PACS-2) to acidic residues in the carboxyterminal tail of K5. Thus, CD31, a novel target of K5, is efficiently removed from ECs by a dual degradation mechanism that is regulated by the subcellular sorting of the ubiquitin ligase. K5-mediated degradation of CD31 is likely to affect EC function in KS tumors.