Pediatric epilepsy surgery and sudden unexpected death epilepsy: the contribution of a Brazilian epilepsy surgery program

Individuals with epilepsy are at higher risk of death than those from the general population, and sudden unexpected death in epilepsy (SUDEP) is the most important direct epilepsy-related cause of death. Epilepsies in the pediatric group are more frequently associated with known potentially risk factors for SUDEP, and a treatment resulting in an improved seizure control may also decrease mortality. The aim of this study is to identify the incidence of SUDEP in a group of operated-on children and adolescents. We analyzed 267 patients up to 18 years old, with medically intractable epilepsy submitted to surgery. We considered the age at surgery, the seizure type, the pathological findings, and the seizure outcome. Data were prospectively collected, according to the protocols of our institution`s ethics committee. The percentage of boys was 58.05. A good outcome was achieved in 72.6% of the cases and a bad outcome in 27.4%. Nine patients died during follow-up, six from clinical complications, and one from SUDEP. All patients who died during the long-term follow-up had persisted with refractory postoperative seizures. The patient who died from SUDEP died during a generalized tonic-clonic seizure. Of the patients, 72.6% had excellent postoperative outcome, and one patient died of SUDEP. All patients who died had had disabling seizures` persistence. The surgical treatment of epilepsy in children and adolescents is an efficient therapy for the medically intractable symptomatic epilepsies and also for the reduction of mortality and SUDEP risks.

Characterization of juvenile and young adult mice following induction of hydrocephalus with kaolin

Hydrocephalus is a common neurological problem in humans, Usually caused by an impairment of cerebrospinal fluid (CSF) flow or absorption. A reliable induced model of chronic hydrocephalus in mice would be useful to test hypotheses using genetic mutants. Our goal was to characterize behavioral and histological changes in juvenile and Young adult mice with kaolin (aluminum silicate) -induced hydrocephalus. Seven-day old and 7-8 week old mice received injection of kaolin into the cisterna magna. Behavior was assessed repeatedly. Seven or 14 days following kaolin, magnetic resonance (MR) imaging was used to assess ventricle size. In hydrocephalic mice, body weight was significantly lower than in age-matched saline-injected sham controls and the gait and posture score were impaired. Juvenile mice developed severe ventriculomegaly and had reduced corpus callosum thickness with gross white matter destruction by 14 days. Reactive astroglial change in white matter and cortex and reduced cellular proliferation in the subependymal zone were also apparent. Young adult mice developed only moderate ventricular enlargement without overt white matter destruction, although there was corpus callosum atrophy and mild astroglial reaction in white matter. Glial fibrillary acidic protein content was significantly higher in juvenile and young adult hydrocephalic mice at 7 and 14 days, but myelin basic protein content was not significantly altered. In conclusion, hydrocephalus induced by percutaneous injection of kaolin in juvenile and young adult mice is feasible. The associated periventricular alterations are essentially the same as those reported in rats of comparable ages. (C) 2009 Elsevier Inc. All rights reserved.

Importance of psychological intervention for the recovery of children submitted to elective surgery

Purpose: The effects of anxiety on the patient and his/her family are known to be the main factors that influence health recovery in child surgery. When the whole family can be prepared and supported by psychologic intervention, the damage to child behavior and family anxiety is attenuated. Methods: This study was conducted in children between 2 and 6 years old, divided into 2 groups of 10 pairs each. The experimental group received psychologic intervention, whereas the control group did not. One month after the surgery, they were reevaluated and compared with the same instruments used in the beginning of the study. The instruments used were as follows: the State-Trait Anxiety Inventory with the mothers and the Rutter`s Child Behavior A2 Scale and the Posthospital Behavior Questionnaire with the children. Results: The results were compared by the Wilcoxon and Mann-Whitney nonparametric tests for independent samples, both at the P <.05 significance levels. Mothers stated that anxiety was different in the postsurgery period, showing a significant decrease when comparing the experimental and control groups. Both instruments to measure child behavior also showed that prepared children had less habit changes than the control group, which showed increased levels of inadequate behavior. Conclusions: These data confirm reports in literature regarding child preparation before medical intervention and reinforces the importance of specialized presurgery planning procedures by the proper professional interfaced with the surgical colleagues, all aiming toward the best recovery for the children. (C) 2009 Elsevier Inc. All rights reserved.

Cultural consonance and adult body composition in urban Brazil

In previous research in Brazil, we found socioeconomic and gender differences in body mass and percent body fat, consistent with a model in which individuals in higher socioeconomic strata, especially women, could achieve a cultural ideal of body size and shape. In this article, using new data, we examine these processes more precisely using measures of cultural consonance. Cultural consonance refers to the degree to which individuals approximate, in their own beliefs and behaviors, the shared prototypes for belief and behavior encoded in cultural models. We have found higher cultural consonance in several domains to be associated with health outcomes. Furthermore, there tends to be a general consistency in cultural consonance across domains. Here we suggest that measures of body composition can be considered indicators of individuals` success in achieving cultural ideals of the body, and that cultural consonance in several domains will be associated with body composition. Using waist circumference as an outcome, smaller waist size was associated with higher cultural consonance in lifestyle (beta = -0.311, P < 0.01) and higher cultural consonance in the consumption of high prestige foods (beta = -0.260, P < 0.01) for women (n = 161), but not for men (n = 106), controlling for age, family income, tobacco use, and dietary intake of protein and carbohydrates. Similar results were obtained using the body mass index and weight as outcomes, while there were no associations with height. These results help to illuminate the cultural mediation of body composition.

Outcome of acute myeloid leukemia patients with hyperleukocytosis in Brazil

Acute myeloid leukemia (AML) with a high white blood cell (WBC) count at presentation has been associated with an increased early mortality rate, usually secondary to leukostasis. However, the value of the WBC count at which there is a high risk of early death (ED) and the efficiency of supportive treatments remain unclear. In this report, a series of 187 consecutive adult patients with AML in our institution was reviewed. The outcome of 40 patients with WBC above 50 x 10(9) L(-1) (hyperleukocytosis) was compared to 147 patients with a leukocyte count lower than 50 x 10(9) L(-1). The group with hyperleukocytosis showed a significantly shorter OS (P < 0.0001) and a higher rate of ED (P = 0.0008). Even when the data from ED patients were removed from analysis, we still detected a shorter OS in patients with hyperleukocytosis (P = 0.0049), which suggests that high WBC number influences long-term survival, and not only ED. We also observed higher lactic dehydrogenase (LDH) and serum creatinine levels in the group of patients with hyperleukocytosis (P = 0.0003 and 0.0406, respectively). Besides considering all the patients with ED, we could observe higher levels of lactic dehydrogenase, a serum creatinine and nitrogen urea (P = 0.0056, P = 0.0008 and P < 0.0001, respectively). Pulmonary involvement was more frequent in patients with ED (P = 0.0277). In conclusion, hyperleukocytosis confers a poorer prognosis in patients with AML.

Effect of Oral Hygiene with 0.12% Chlorhexidine Gluconate on the Incidence of Nosocomial Pneumonia in Children Undergoing Cardiac Surgery

OBJECTIVE. To evaluate the effect of oral hygiene with 0.12% chlorhexidine gluconate on the incidence of nosocomial pneumonia and ventilator-associated pneumonia (VAP) in children undergoing cardiac surgery. DESIGN. Prospective, randomized, double-blind, placebo-controlled trial. SETTING. Pediatric intensive care unit (PICU) at a tertiary care hospital. patients. One hundred sixty children undergoing surgery for congenital heart disease, randomized into 2 groups: chlorhexidine (n = 87) and control (n = 73). INTERVENTIONS. Oral hygiene with 0.12% chlorhexidine gluconate or placebo preoperatively and twice a day postoperatively until PICU discharge or death. RESULTS. Patients in experimental and control groups had similar ages (median, 12.2 vs 10.8 months; P =. 72) and risk adjustment for congenital heart surgery 1 score distribution (66% in category 1 or 2 in both groups; P =. 17). The incidence of nosocomial pneumonia was 29.8% versus 24.6% (Pp. 46) and the incidence of VAP was 18.3% versus 15% (Pp. 57) in the chlorhexidine and the control group, respectively. There was no difference in intubation time (P =. 34), need for reintubation (P =. 37), time interval between hospitalization and nosocomial pneumonia diagnosis (P =. 63), time interval between surgery and nosocomial pneumonia diagnosis (P =. 10), and time on antibiotics (P =. 77) and vasoactive drugs (P =. 16) between groups. Median length of PICU stay (3 vs 4 days; P =. 53), median length of hospital stay (12 vs 11 days; P =. 67), and 28-day mortality (5.7% vs 6.8%; P =. 77) were also similar in the chlorhexidine and the control group. CONCLUSIONS. Oral hygiene with 0.12% chlorhexidine gluconate did not reduce the incidence of nosocomial pneumonia and VAP in children undergoing cardiac surgery.

Precise Temporal Regulation of roughest is Required for Correct Salivary Gland Autophagic Cell Death in Drosophila

The Drosophila roughest (rst) locus encodes an immunoglobulin superfamily transmembrane glycoprotein implicated in a variety of embryonic and postembryonic developmental processes. Here we demonstrate a previously unnoticed role for this gene in the autophagic elimination of larval salivary glands during early pupal stages by showing that overexpression of the Rst protein ectodomain in early pupa leads to persistence of salivary glands up to at least 12 hours after head eversion, although with variable penetrance. The same phenotype is observed in individuals carrying the dominant regulatory allele rst(D), but not in loss of function alleles. Analysis of persistent glands at the ultrastructural level showed that programmed cell death starts at the right time but is arrested at an early stage of the process. Finally we describe the expression pattern and intracellular distribution of Rst in wild type and rstD mutants, showing that its downregulation in salivary glands at the beginning of pupal stage is an important factor in the correct implementation of the autophagic program of this tissue in space and time. genesis 47:492-504, 2009. (C) 2009 Wiley-Liss, Inc.

In vitro schistosomicidal activity of curcumin against Schistosoma mansoni adult worms

The in vitro schistosomicidal activity of curcumin (doses ranging from 5 to 100 mu M) was carried out against Schistosoma mansoni adult worms. Curcumin (at 50 and 100 mu M) caused death of all worms. When tested at the doses of 5 and 20 mu M, it decreased the worm viability in comparison with negative (Roswell Memorial Park Institute (RPMI) 1640 medium alone or RPMI 1640 medium with 10% dimethyl sulfoxide) and positive (heat-killed worms at 56A degrees C or praziquantel 10 mu M) control groups. All pairs of coupled adult worms were separated into individual male and female by the action of curcumin at the doses of 20 to 100 mu M. When tested at 5 and 10 mu M, curcumin reduced egg production by 50% in comparison with the positive control group. It is the first time that the schistosomicidal activity has been reported for curcumin.

Total gastric transposition is better than partial gastric tube esophagoplasty for esophageal replacement in children

Whenever the surgeon uses the stomach as an esophageal substitute, either one of two techniques is generally performed: total gastric transposition or gastric tube esophagoplasty. No existing reports compare the complications associated with these two surgical procedures. The purpose of this study is to review the authors` experience with total gastric transposition and verify whether this technique is superior to gastric tube esophagoplasty in children by comparing the main complications with those reported in the publications of gastric tubes esophagoplasties in the English language literature published in the last 38 years. A total of 35 children underwent total gastric transposition according to the classical technique. Most of these patients (27, or 77.1%) had long gap esophageal atresia. The most frequently observed complications were compared to those reported in nine studies of gastric tube esophagoplasty comprising 184 patients. Mortality and graft failure rates were also compared. Seven patients (20.0%) presented with leaks, all of which closed spontaneously. Six children were reoperated, three experienced gastric outlet obstruction secondary to axial torsion of the stomach placed in the retrosternal space and the other three experienced delayed gastric emptying that required revision of the piloroplasty. There were two deaths (5.7%) and no graft failure. Strictures were observed in five patients (14.2%) and all of these were resolved with endoscopic dilatations. Six patients had diarrhea that spontaneously resolved. In the late follow-up period, all patients were on full feed and thriving well. The comparisons with gastric tube patients demonstrated that the total gastric transposition group presented with significantly less leaks and strictures (P = 0.0001 and 0.001, respectively). The incidence of death and graft failure was not statistically different. In conclusion, gastric transposition is as a simple technical procedure for esophageal replacement in children with satisfactory results, and is superior to gastric tube esophagoplasty.

Regulation of Trypanosoma cruzi-Induced Myocarditis by Programmed Death Cell Receptor

Trypanosoma cruzi infection causes intense myocarditis, leading to cardiomyopathy and severe cardiac dysfunction. Protective adaptive immunity depends on balanced signaling through a T cell receptor and coreceptors expressed on the T cell surface. Such coreceptors can trigger stimulatory or inhibitory signals after binding to their ligands in antigen-presenting cells (APC). T. cruzi modulates the expression of coreceptors in lymphocytes after infection. Deregulated inflammation may be due to unbalanced expression of these molecules. Programmed death cell receptor 1 (PD-1) is a negative T cell coreceptor that has been associated with T cell anergy or exhaustion and persistent intracellular infections. We aimed to study the role of PD-1 during T. cruzi-induced acute myocarditis in mice. Cytometry assays showed that PD-1 and its ligands are strongly upregulated in lymphocytes and APC in response to T. cruzi infection in vivo and in vitro. Lymphocytes infiltrating the myocardium exhibited high levels of expression of these molecules. An increased cardiac inflammatory response was found in mice treated with blocking antibodies against PD-1, PD-L1, and to a lesser extent, PD-L2, compared to that found in mice treated with rat IgG. Similar results in PD-1(-/-) mice were obtained. Moreover, the PD-1 blockade/deficiency led to reduced parasitemia and tissue parasitism but increased mortality. These results suggest the participation of a PD-1 signaling pathway in the control of acute myocarditis induced by T. cruzi and provide additional insight into the regulatory mechanisms in the pathogenesis of Chagas` disease.

Saethre-Chotzen Syndrome, Pro136His TWIST Mutation, Hearing Loss, and External and Middle Ear Structural Anomalies: Report on a Brazilian Family

Objective: To describe the clinical, speech, hearing, and imaging findings in three members of a Brazilian family with Saethre-Chotzen syndrome (SCS) who presented some unusual characteristics within the spectrum of the syndrome. Design: Clinical evaluation was performed by a multidisciplinary team. Direct sequencing of the polymerase chain reaction amplified coding region of the TWIST1 gene, routine and electrophysiological hearing evaluation, speech evaluation, and imaging studies through computed tomography (CT) scan and magnetic resonance imaging (MRI) were performed. Results: TWIST1 gene analysis revealed a Pro136His mutation in all patients. Hearing evaluation showed peripherial and mixed hearing loss in two of the patients, one of them with severe unilateral microtia. Computed tomography scan showed structural middle ear anomalies, and MRI showed distortion of the skull contour as well as some of the brain structures. Conclusions: We report a previously undescribed TWIST1 gene mutation in patients with SCS. There is evidence that indicates hearing loss (conductive and mixed) can be related both with middle ear (microtia, high jugular bulb, and enlarged vestibules) as well as with brain stem anomalies. Here we discuss the relationship between the gene mutation and the clinical, imaging, speech, and hearing findings.

Eag1 potassium channel immunohistochemistry in the CNS of adult rat and selected regions of human brain

Eag1 (K(v)10.1) is the founding member of an evolutionarily conserved superfamily of voltage-gated K+ channels. In rats and humans Eag1 is preferentially expressed in adult brain but its regional distribution has only been studied at mRNA level and only in the rat at high resolution. The main aim of the present study is to describe the distribution of Eag1 protein in adult rat brain in comparison to selected regions of the human adult brain. The distribution of Eag1 protein was assessed using alkaline-phosphatase based immunohistochemistry. Eag1 immunoreactivity was widespread, although selective, throughout rat brain, especially noticeable in the perinuclear space of cells and proximal regions of the extensions, both in rat and human brain. To relate the results to the relative abundance of Eag1 transcripts in different regions of rat brain a reverse-transcription coupled to quantitative polymerase chain reaction (real time PCR) was performed. This real time PCR analysis showed high Eag1 expression in the olfactory bulb, cerebral cortex, hippocampus, hypothalamus, and cerebellum. The results indicate that Eag1 protein expression greatly overlaps with mRNA distribution in rats and humans. The physiological relevance of potassium channels in the different regions expressing Eag1 protein is discussed. (C) 2008 IBRO. Published by Elsevier Ltd. All rights reserved.