Reversible acquired epileptic frontal syndrome and CSWS suppression in a child with congenital hemiparesis treated by hemispherotomy

A boy with a right congenital hemiparesis due to a left pre-natal middle cerebral artery infarct developed focal epilepsy at 33 months and then an insidious and subsequently more rapid, massive cognitive and behavioural regression with a frontal syndrome between the ages of 4 and 5 years with continuous spike-waves during sleep (CSWS) on the EEG. Both the epilepsy and the CSWS were immediately suppressed by hemispherotomy at the age of 5 years and 4 months. A behavioural-cognitive follow-up prior to hemispherotomy, an per-operative EEG and corticography and serial post-operative neuropsychological assessments were performed until the age of 11 years. The spread of the epileptic activity to the “healthy” frontal region was the cause of the reversible frontal syndrome. A later gradual long-term but incomplete cognitive recovery, with moderate mental disability was documented. This outcome is probably explained by another facet of the epilepsy, namely the structural effects of prolonged epileptic discharges in rapidly developing cerebral networks which are, at the same time undergoing the reorganization imposed by a unilateral early hemispheric lesion. Group studies on the outcome of children before and after hemispherectomy using only single IQ measures, pre- and post-operatively, may miss particular epileptic cognitive dysfunctions as they are likely to be different from case to case. Such detailed and rarely available complementary clinical and EEG data obtained in a single case at different time periods in relation to the epilepsy, including per-operative electrophysiological findings, may help to understand the different cognitive deficits and recovery profiles and the limits of full cognitive recovery.     

Health related quality of life among patients with chronic hepatitis C: a cross-sectional study of sociodemographic,psychopathological and psychiatric determinants

IntroductionChronic hepatitis C virus infection patients have higher rates of psychiatric disorders than the general population. Chronic hepatitis C virus infection is known to be associated with impaired health related quality of life. To our knowledge, there is no previous research of health related quality of life in chronic hepatitis C patients that combined structured psychiatric interview and careful psychopathological evaluation, including depression, anxiety and fatigue instruments. The aim of this study was to evaluate health related quality of life of chronic hepatitis C patients and to investigate the association with sociodemographic, psychopathological and psychiatric factors.Materials and methodsEighty-one individuals with chronic hepatitis C virus infection receiving care at a Brazilian public university-based outpatient service for infectious diseases were enrolled in the study. The World Health Organization Quality of Life Scale Brief Version was used to assess health related quality of life. Standard psychiatric interview (Mini International Neuropsychiatric Interview-Plus) was conducted to establish Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, Axis I psychiatric diagnosis. Further instruments completed psychopathological investigation: Beck Depression Inventory, Hospital Anxiety and Depression Scale, Brief Fatigue Inventory, Hamilton Depression Scale and Hamilton Anxiety Scale. Pearson Chi-Square and Kruskal–Wallis were performed for categorical and continuous univariate analysis, respectively. Correlation between psychopathological and health related quality of life scores was performed according to Spearman's correlation. Multivariate analysis was performed according to stepwise forward ordinal logistic regression. The significance threshold was fixed at α = 0.05.ResultsDepressive disorders were associated with worse scores in overall health related quality of life and in all domains. Fatigue was associated with lower scores in physical and psychological domains, and married status with higher scores in psychological health related quality of life. We found strong correlation among scores of depression, fatigue and health related quality of life.ConclusionDepression and fatigue must be properly investigated and managed in HCV patients in order to improve HRQL. WHOQOL-BREF proved to be a useful instrument to assess HRQL in HCV patients.     

Initial experience in Brazil with endoscopic submucosal dissection for early gastric cancer using insulation-tipped knife: a safety and feasibility study

Background  Endoscopic resection is an adequate treatment for subgroups of patients with early gastric cancer. Endoscopic submucosal dissection (ESD) represents a recent advance and leads to good results when adequately indicated. There is great experience with this technique in Japan and it is gaining acceptance among Western endoscopists. We present the first gastric ESD series performed in Brazil. Methods  Patients with well-differentiated adenocarcinomas macroscopically classified as early cancer, less than 30 mm with no ulcer or scar, were included. ESD was performed with an insulated-tip knife and electrosurgical unit with endocut mode. Clinicopathological aspects and morbidity were evaluated. The study was approved by the local ethics committee and informed consent was obtained from all participant subjects. Results  From October 2005 to July 2007, 160 patients received surgical treatment for gastric cancer; 44 patients (27.5%) had early gastric cancer. In this latter group, 15 procedures (ESD) were performed in 12 patients. The mean size of the lesions was 16.8 mm. Almost half of the lesions were located in the proximal third of the stomach and the mean elapsed time for the procedure was 140 min. In 80% of the cases resection was en bloc and 80% of the resections were considered curative. We had three perforations, which were managed clinically, and no bleeding. Conclusion  When adequately indicated, ESD is a safe and feasible technique.     

Azidothymidine (AZT) as a Potential Modifier of Radiation Response in Vitro

The potential effect of AZT as a thymidine analogue on radiation response in vitro was investigated. Two human cell lines (WiDr and HeLa) were used. The effect of 10 μM AZT on exponentially growing cells was studied after different exposure times (24, 48 and 72 h). The surviving fraction (clonogenic assay) or metabolic activity (MTT assay) after irradiation of AZT-exposed cells, was compared to unexposed irradiated controls. Flow cytometry was used to assess the cell-cycle effect of pre-exposure of exponentially growing cells to AZT. AZT had a radioprotective effect for all experimental time points as far as WiDr was concerned. For HeLa the effect was significant at 24 h. Cell-cycle analysis showed a significant accumulation in S-phase at 72 h for WiDr. For HeLa there was a significant accumulation in S-phase at 48 h. We conclude that under the reported experimental conditions, AZT as a thymidine analogue seems to reduce the cytotoxic effect of irradiation.     

Early benefits of pravastatin to experimentally induced atherosclerosis

There is little information regarding the time of hypolipidemic treatment of changes in atherosclerotic plaque, tissue cholesterol content, and also for the recovery of endothelial function. To assess the early effects of lipid-lowering treatment on these parameters, six groups of New Zealand male rabbits were studied. Animals in groups I and II were fed regular chow; groups III and IV received a 12-week 0.5% cholesterol diet followed by 12 weeks of 0.05% cholesterol diet. Finally, groups V and VI were fed a 12-week 0.5% cholesterol diet and were then shifted to a regular diet for 12 weeks. During the last four weeks, the rabbits in groups I, III, and V received low-dose pravastatin (2 mg/day), added to the diet. Group IV animals had the highest cholesterol plasma levels (vs. groups I, II, III, and V, p < 0.01) and presented atherosclerotic plaques in a more advanced stage. Nonatherogenic diet was insufficient to restore endothelial function in animals previously fed cholesterol-enriched diets (groups IV and VI). Conversely, pravastatin treatment promoted significant improvement in endothelial function and reduced the progression of atherosclerosis. Marked increase in cholesterol content was seen in aorta and liver in response to the atherogenic diet. However, neither treatment with pravastatin nor nonatherogenic diet was capable of modifying the tissue cholesterol content. Our study supports the hypothesis that the early use of statins can attenuate the progression of atherosclerosis and ameliorate endothelial function. In addition, significant changes in the tissue cholesterol pool probably need a longer period of treatment.     

Clinical pharmacokinetics of intranasal sumatriptan

A substantial proportion of migraine patients have gastric stasis and suffer severe nausea and/or vomiting during their migraine attack. This may lead to erratic absorption from the gastrointestinal tract and make oral treatment unsatisfactory. For such patients, an intranasal formulation may be advantageous. Sumatriptan is a potent serotonin 5HT(1B/1D) agonist widely used in the treatment of migraine; the effectiveness of the intranasal formulation (20mg) has been well established in several clinical studies. This article reviews the pharmacokinetics of intranasal sumatriptan and includes comparisons with oral and subcutaneous administration. After intranasal administration, sumatriptan is directly and rapidly absorbed, with 60% of the maximum plasma concentration (C(max)) occurring at 30 minutes after administration of a single 20mg dose. Following intranasal administration, approximately 10% more sumatriptan is absorbed probably via the nasal mucosa when compared with oral administration. Mean C(max) after a 20mg intranasal dose is approximately 13.1 to 14.4 ng/mL, with median time to C(max) approximately 1 to 1.75 hours. When given as a single dose, intranasal sumatriptan displays dose proportionality in its extent of absorption and C(max) over the dose range 5 to 10mg, but not between 5 and 20mg for C(max). The elimination phase half-life is approximately 2 hours, consistent with administration by other routes. Sumatriptan is metabolised by monoamine oxidase (MAO; predominantly the A isozyme, MAO-A) to an inactive metabolite. Coadministration with a MAO-A inhibitor, moclobemide, leads to a significant increase in sumatriptan plasma concentrations and is contraindicated. Single-dose pharmacokinetics in paediatric and adolescent patients following intranasal sumatriptan were studied to determine the effect of changes in nasal morphology during growth, and of body size, on pharmacokinetic parameters. The pharmacokinetic profile observed in adults was maintained in the adolescent population; generally, factors such as age, bodyweight or height did not significantly affect the pharmacokinetics. In children below 12 years, C(max) is comparable to that seen in adolescents and adults, but total exposure (area under the concentration-time curve from zero to infinity) was lower in children compared with older patients, especially in younger children treated with 5mg. Clinical experience suggests that intranasal sumatriptan has some advantages over the tablet (more rapid onset of effect and use in patients with gastrointestinal complaints) or subcutaneous (noninvasive and fewer adverse events) formulations.     

View of the different social agents on the social participation in the health system in northeastern Brazil. A qualitative approximation

BACKGROUND: Social participation in the health system constitutes one of the basic principles of Brazilian health sector reform. This paper analyses the concepts held by different groups of stakeholders with respect to social participation in health and identifies their opinions on hindering and enabling factors for this participation. METHODS: A qualitative study was conducted using individual interviews (II) and focus groups (FG) with different groups of stakeholders: 135 users (II, GF), 14 community leaders (II, GF), 12 Municipal Health Council (MHC) members (II), 8 policymakers and (II) 37 health personnel (II, GF) were interviewed. A narrative content analysis of the results was conducted. Two municipalities in North-eastern Brazil, Cabo de Santo Agostinho and Camaragibe, made up the area of study. RESULTS: In all groups, a distinction between individual and group participation emerged and reached different levels of elaboration. Many informants' ideas described also their own ways of action. Individual participation concepts and the utilization of health services were predominant among users. Population and institutional factors were identified as obstacles to participation: users and community leaders referred both type of factors, while the health personnel and MHC members put more emphasis on the former. Some informants among the health personnel did not identify any barriers. Policymakers emphasised the lack of political will. The enabling factors mentioned reflected the same logic. CONCLUSIONS: Concepts and perceived hindering factors to participation which were described appear to show different ways of interaction among the diverse stakeholders and the health services. The results point towards a limited effect of the health policy on social participation. Elements that could contribute to an improvement are identified.     

Long-term complete haematological and molecular remission after allogeneic bone marrow transplantation in a patient with a stem cell myeloproliferative disorder associated with t(8;13)(p12;q12)

A rare atypical myeloproliferative disorder (aMPD) associated with chromosomal translocations involving the short arm of chromosome 8, region p11-p12 has been described. In most patients, the cytogenetic abnormality is a t(8;13)(p12;q12) that fuses fibroblast growth factor receptor 1, the 8p12 key gene, to FIM/ZNF198 gene. Prognosis is poor with frequent evolution to acute myeloid leukaemia within 1 year of diagnosis. We report a new patient with aMPD with a t(8;13) translocation. Complete haematological, cytogenetic and molecular remission was demonstrated 39 months after allogeneic bone marrow transplantation. This is the first report to demonstrate a molecular remission in this disorder.