Search

Nutritional deficiencies limit the growth of snap bean plants, therefore knowing the biological mechanisms involved in it is fundamental. This study is aimed to evaluate the damage caused by a deficiency of macronutrients in physiological variables that cause decreased growth and the appearance of visual symptoms in snap bean. Thus, we design a hydroponic system of snap bean cultivation in order to test the effect of macronutrient deficiencies in a controlled environment. The treatments consisted in evaluate the effects of lack of one macronutrient in time. To perform this, we used Hoagland and Arnon solution in its complete formulation (control) or without N, P; K; Mg, Ca or S in each treatment. Physiological, nutritional, and growth analyses were performed when visual deficiency symptoms of each omitted nutrient appeared. Thus, the omissions of N and P in the nutrient solution led to lower accumulations of all macronutrients in the shoot. And the K, Ca, Mg, and S omissions decreased the amounts of K, Ca, Mg, P, and S in the shoot of the snap bean plants when compared with the plants grown in the complete nutrient solution. With the lowest accumulation of macronutrients, the content of photosynthetic pigments and the photosynthetic rate were reduced, with harmful effects on plant growth. Thus, from the losses in dry matter production of the shoot, the order of limiting of macronutrients in bean plants was N < P < Ca < S < Mg < K, with a decrease of up to 86.2%, 80.1%, 51.2%, 46.5%, 25.6%, and 19.3%, respectively. The nitrogen deficiency is more evident, proven by symptoms such as chlorosis in the lower and upper third leaves and necrosis of the lower third leaves., Competing Interests: The authors have declared that no competing interests exist.

Objective: To describe the evolution of 15 patients who were treated for difficult-to-control episodic and chronic cluster headaches with clomiphene., Methods: Clomiphene treatment was used for seven chronic and eight episodic cluster headache patients. The chronic patients were refractory to the medication being used, and the episodic patients, in addition to being resistant to conventional medication, had longer cluster headache periods, exceeding the average time of previous cluster cycles. Our main analysis was of the time to pain-free, complete remission, and the length of pain-free time and complete remission., Results: Clomiphene was used for 45-180 days. The average time to being pain-free was 15 days and cluster remission was up to 60 days. The average time between being pain-free until cluster remission was 26 days., Conclusions: Clomiphene treatment was significantly efficient. It interrupted chronicity in all patients, suggesting the capability of changing the pattern of attacks. It proved to be safe and well tolerated.

We aimed to verify the changes in the microbial community during bioremediation of gasoline-contaminated soil. Microbial inoculants were produced from successive additions of gasoline to municipal solid waste compost (MSWC) previously fertilized with nitrogen-phosphorous. To obtain Inoculant A, fertilized MSWC was amended with gasoline every 3 days during 18 days. Inoculant B received the same application, but at every 6 days. Inoculant C included MSWC fertilized with N-P, but no gasoline. The inoculants were applied to gasoline-contaminated soil at 10, 30, or 50g/kg. Mineralization of gasoline hydrocarbons in soil was evaluated by respirometric analysis. The viability of the inoculants was evaluated after 103 days of storage under refrigeration or room temperature. The relative proportions of microbial groups in the inoculants and soil were evaluated by FAME. The dose of 50g/kg of inoculants A and B led to the largest CO 2 emission from soil. CO 2 emissions in treatments with inoculant C were inversely proportional to the dose of inoculant. Heterotrophic bacterial counts were greater in soil treated with inoculants A and B. The application of inoculants decreased the proportion of actinobacteria and increased of Gram-negative bacteria. Decline in the density of heterotrophic bacteria in inoculants occurred after storage. This reduction was bigger in inoculants stored at room temperature. The application of stored inoculants in gasoline-contaminated soil resulted in a CO 2 emission twice bigger than that observed in uninoculated soil. We concluded that MSWC is an effective material for the production of microbial inoculants for the bioremediation of gasoline-contaminated soil., (Copyright © 2016 Sociedade Brasileira de Microbiologia. Published by Elsevier Editora Ltda. All rights reserved.)

Introduction:: Ill-defined causes of death are common among the elderly owing to the high frequency of comorbidities and, consequently, to the difficulty in defining the underlying cause of death., Objective:: To analyze the validity and reliability of the "primary diagnosis" in hospitalization to recover the information on the underlying cause of death in natural deaths among the elderly whose deaths were originally assigned to "ill-defined cause" in their Death Certificate. The hospitalizations occurred in the state of Rio de Janeiro, in 2006., Methods:: The databases obtained in the Information Systems on Mortality and Hospitalization were probabilistically linked. The following data were calculated for hospitalizations of the elderly that evolved into deaths with a natural cause: concordance percentages, Kappa coefficient, sensitivity, specificity, and the positive predictive value of the primary diagnosis. Deaths related to "ill-defined causes" were assigned to a new cause, which was defined based on the primary diagnosis., Results:: The reliability of the primary diagnosis was good, according to the total percentage of consistency (50.2%), and fair, according to the Kappa coefficient (k = 0.4; p < 0.0001). Diseases related to the circulatory system and neoplasia occurred with the highest frequency among the deaths and the hospitalizations and presented a higher consistency of positive predictive values per chapter and grouping of the International Classification of Diseases. The recovery of the information on the primary cause occurred in 22.6% of the deaths with ill-defined causes (n = 14)., Conclusion:: The methodology developed and applied for the recovery of the information on the natural cause of death among the elderly in this study had the advantage of effectiveness and the reduction of costs compared to an investigation of the death that is recommended in situations of non-linked and low positive predictive values. Monitoring the mortality profile by the cause of death is necessary to periodically update the predictive values.

Objective: To describe the methodological features of a study on the association between restricted intrauterine growth and prevalence of overweight, obesity and hypertension in school aged children., Methods: The study was conducted in two stages in two public schools in Niterói (RJ), from June through December 2010. All students aged 6 to 14 years were eligible to participate. The first stage consisted of an interview to collect information on demographic variables, diet and other variables. A sample was selected for the second stage, in order to conduct an equivalent of a case-cohort study. There was an interval of about 15 days between the two stages. Cases were overweight students, defined as a Z-score for BMI/age/sex > +1.00 in the first stage. Controls were selected by using a random schedule in which the sample frame was the whole cohort. Bioelectrical impedance analysis, carotid ultrasound to measure intimal-medial thickness, blood measurements and interviews were obtained. Gestational age and weight at birth were used to define proxy variables of restricted intrauterine growth. Early health information was obtained from medical registers., Results: The study participation was 76.4% (n = 795) out of 1,040 eligible to participate). 85.1% of parent's questionnaires were returned. 62.5% of the eligible children participated in the case-control study (case: control ratio = 1:1.8). Early life health information was obtained from 292 children., Conclusion: The present study has the potential to provide important information about multiple outcomes and exposures related to restricted intrauterine growth and metabolic abnormalities.

This study investigated the association between low birthweight and postnatal weight in full-term infants, taking social, economic, maternal and babies characteristics into account. A cross-sectional study was conducted with infants under six months old at 27 primary healthcare units in the Rio de Janeiro municipality, Brazil, in 2007. Only singleton full-term babies were included. The association between full-term low birthweight and postnatal weight was tested using the multiple regression model adjusted for the gender and age of the baby, as well as potential confounding factors. A total of 875 babies were evaluated, of whom 4.5% were small for gestational age. Small-for-gestational-age babies weighted, on average, 977.4 grams less than those born with adequate weight for gestational age, after adjustment by gender and age, as well as marital status and parity. Girls were 426.74 grams lighter than boys; children from mothers with live-in partners were 146.2 grams heavier than those of single mothers, and the babies of primiparae weighed 204.67 grams less than the children of multiparae. Low birthweight is an unfavorable factor for postnatal weight of full-term infants. These children, particularly daughters of primipara single mothers, must be followed more frequently in relation to their postnatal growth.

Background: Smoking epidemic in Brazilian women has later onset, smaller magnitude, and slower decreasing trend, compared to men. Among pregnant women, smoking has an additional deleterious effect. The purpose of this study was to analyze smoking prevalence during pregnancy and associated factors, and to describe the frequency of smoking reduction and cessation in public maternities of Rio de Janeiro State, southeastern Brazil, in 2011., Methods: A cross-sectional study was conducted in two maternities located at public hospitals in two cities of the Rio de Janeiro state, Niterói (maternity A) and of Rio de Janeiro (maternity B). Data were gathered by interviews 12 hours after the delivery, and analyses of prenatal cards and medical records. Smoking prevalence according to maternal characteristics, adequacy of prenatal care, and proportions of smoking reduction and cessation during pregnancy were calculated. Factors associated to smoking during pregnancy were estimated by logistic regression analysis., Results: Smoking prevalence at maternity A (24.8%, 95% CI: 21.1-29.0) and maternity B (17.9%, 95% CI: 15.8-20.1) were high. Prevalence rates were greater in women aged 20-34 years, mainly without partner, multiparous and brown or black skin color. Low education (OR = 2.14, 95% CI 1.21, 3.79) and multiparity (OR = 3.48, 95% CI 1.78, 6.81), at maternity A; adolescence (OR = 0.44, 95% CI 0.26, 0.75), black skin color (OR = 1.71, 95% CI 1.06, 2.74), low education (OR = 1.61, 95% CI 1.08, 2.40), and multiparity (OR = 1.58, 95% CI 1.03, 2.44), at maternity B, were associated with smoking in multivariable analysis. Adequacy of prenatal care and smoking prevalence showed an inverse association. More than half of the smokers kept the smoking habits during pregnancy. Reduction occurred mainly between the 1(st) and 2(nd) trimesters of pregnancy., Conclusion: Smoking prevalence during pregnancy was higher for multiparous and less educated women. Population and individual strategies for smoking prevention and control must include actions specific for women, especially during the reproductive period.

Background: The purpose of this study was to investigate the association between mean body mass index (BMI) and prevalence of obesity in adult populations living in Brazilian State capitals., Methods: An ecological study was conducted, using data from the National Household Budget Survey conducted in July 2002 through June 2003, including a representative sample of 48.470 households. Pearson's correlation and linear regression coefficients were estimated in order to define the relationships of mean BMI and sex-specific, age standardized obesity prevalence (BMI ≥ 30.0 kg/m(2)) in adults aged 20 to 59 years., Results: Stronger correlations between BMI and prevalence of obesity were observed in women (r = 0.9; p < 0.001) than in men (r = 0.6; p = 0.001) in all analyzes. A reduction of one unit in mean BMI predicted a decline in the prevalence of obesity of about 4.0% (95% CI: 1.7-6.3) in men, and 3.4% (95% CI: 2.6-4.3) in women., Conclusion: We found a correlation between BMI and prevalence of obesity, particularly among women, suggesting that population-based strategies would be effective to reduce the prevalence of obesity in adult populations living in Brazilian state capitals.

Objective: In most cases of pediatric liver transplantation, the clinical scenario of large-for-size transplants can lead to hepatic dysfunction and a decreased blood supply to the liver graft. The objective of the present experimental investigation was to evaluate the effects of ischemic preconditioning on this clinical entity., Methods: Eighteen pigs were divided into three groups and underwent liver transplantation: a control group, in which the weights of the donors were similar to those of the recipients, a large-for-size group, and a large-for-size + ischemic preconditioning group. Blood samples were collected from the recipients to evaluate the pH and the sodium, potassium, aspartate aminotransferase and alanine aminotransferase levels. In addition, hepatic tissue was sampled from the recipients for histological evaluation, immunohistochemical analyses to detect hepatocyte apoptosis and proliferation and molecular analyses to evaluate the gene expression of Bax (pro-apoptotic), Bcl-XL (anti-apoptotic), c-Fos and c-Jun (immediate-early genes), ischemia-reperfusion-related inflammatory cytokines (IL-1, TNF-alpha and IL-6, which is also a stimulator of hepatocyte regeneration), intracellular adhesion molecule, endothelial nitric oxide synthase (a mediator of the protective effect of ischemic preconditioning) and TGF-beta (a pro-fibrogenic cytokine)., Results: All animals developed acidosis. At 1 hour and 3 hours after reperfusion, the animals in the large-for-size and large-for-size + ischemic preconditioning groups had decreased serum levels of Na and increased serum levels of K and aspartate aminotransferase compared with the control group. The molecular analysis revealed higher expression of the Bax, TNF-alpha, I-CAM and TGF-beta genes in the large-for-size group compared with the control and large-for-size + ischemic preconditioning groups. Ischemic preconditioning was responsible for an increase in c-Fos, IL-1, IL-6 and e-NOS gene expression., Conclusion: Ischemia-reperfusion injury in this model of large-for-size liver transplantation could be partially attenuated by ischemic preconditioning.

Context: Severe obesity affects the body favoring the development of serious diseases, including hypertension, diabetes mellitus, atherosclerosis and non alcoholic fatty liver disease. Bariatric procedures increased in Brazil in the last decade., Objectives: The purpose of this study was to verify if gender and age in date of procedure resulted significant differences in metabolic syndrome parameters after surgery., Methods: The study involved 205 medical records of adult patients undergoing Roux-en-Y gastric bypass, stratified by gender and age groups and followed one year by a multidisciplinary team., Results: It was observed significant decrease in body mass index, fasting glucose and insulin at all ages and both genders. Lipid profile showed significant improvements except high density lipoprotein cholesterol. Ectopic fat in the liver has decreased after 6 months in patients classified with steatosis at baseline. Patients classified as hypertensive blood pressure levels decreased 6 months after surgical intervention., Conclusions: Roux-en-Y gastric bypass proved to be an important tool in remission of metabolic syndrome parameters. The reduction of body mass accompanied to decrease in insulin resistance resulted in lower prevalence of comorbidities associated with obesity. The benefits were similar and extended both genders and all age groups between 18 and 65 years old.

Temporal lobe epilepsies are associated with cognitive dysfunctions in memory which are important clues currently used clinically for the lateralization of the epileptic focus in evaluations for epilepsy surgery. Because these lobes also contain the primary auditory cortex, the study of auditory evoked potentials (AEPs) is a candidate, not yet established, complementary method to characterize epilepsy-induced dysfunction. We aimed to establish the clinical usefulness of auditory evoked potentials for the study of pediatric symptomatic temporal lobe epilepsies. A group of 17 patients (ages 4-16) with symptomatic epilepsies undergoing evaluation for epilepsy surgery epilepsy was submitted to auditory evoked potentials using 35-channel scalp EEG recordings. A control group of 10 healthy volunteers was studied with the same protocol. The P100 and mismatch negativity (MMN) potential latencies and normalized amplitudes were studied. We also performed a voxel-based lesion-symptom mapping (VLSM) to determine the anatomical areas associated with changes in the AEPs. Eleven patients had temporal lobe epilepsy, three had frontal lobe epilepsy, and three had occipital lobe epilepsy. Latencies for the P100 were normal in 15/17 and in 11/17 for the MMN, with no consistent correlation with the epilepsy type. The MMN amplitude was abnormal in 7/17 patients, all with temporal lobe epilepsies (sensitivity of 64%). Of these patients, four had a decreased MMN associated with a Heschl's gyrus lesion in the VLSM, and three had an increased MMN associated with hippocampal lesion. No extratemporal epilepsy showed MMN amplitude abnormalities (specificity of 100%). The P100 amplitude was abnormal in 3/17, two with temporal and one with frontal lobe epilepsies. The auditory MMN has a high specificity but a low sensitivity for temporal lobe epilepsy in symptomatic pediatric epilepsies. Amplitude decreases of the MMN are associated with homolateral Heschl's gyrus lesions, and MMN increases with hippocampal lesions., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Panayiotopoulos syndrome (PS) is a common epilepsy syndrome associated with rare clinical seizures and unknown localization of the epileptogenic area. Despite findings of normal development in patients with PS, recent neuropsychological studies point to subtle and diverse cognitive impairments. No well-outlined hypothesis about the localization of the brain dysfunction responsible for these impairments has been proposed. We further explored the cognitive dysfunctions in PS and made inferences on the most likely anatomical localization of brain impairment. A group of 19 patients (aged 6-12) with PS was rated according to spike activity and lateralization. The patients were submitted to a neuropsychological evaluation to assess general intelligence, memory, language, visual-perceptual abilities, attention, and executive functions. Using 35-channel scalp EEG recordings, the N170 face-evoked event-related potential (ERP) was obtained to assess the functional integrity of the ventral pathway. All patients with PS showed normal IQ but subtle and consistent neurocognitive impairments. Namely, we found abnormalities in the copy task of the Rey-Osterrieth Complex Figure and in the Narrative Memory Test. There was no correlation between neuropsychological impairments with spike activity and hemispheric spike lateralization. The N170 ERP was normal in all patients except for one. Our neuropsychological findings demonstrate impairments in visual-perceptual abilities and in semantic processing. These findings, paired with the absence of occipital lobe dysfunction in all neuropsychological studies of PS performed to this date, support the existence of parietal lobe dysfunction., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Esophagocoloplasty is a commonly performed procedure for esophageal replacement in children. Traditionally, mechanical bowel preparation (MBP) is performed before this operation. However, this practice has been questioned, initially in adults and now in children. The aim of this study was to evaluate the influence of MBP on esophagocoloplasty in a series of children. Data collected from 164 patients who underwent esophagocoloplasty in the Pediatric Surgery Division, University of São Paulo Medical School, from February 1978 to July 2011 were reviewed for postoperative complications. In 134 patients, at least one kind of MBP was performed before the surgery (PREP group). MBP was omitted in 30 patients (NO-PREP group). There was no statistical difference between the groups in the rates of evisceration, colocolic, or cologastric anastomotic dehiscence and death. However, in the NO-PREP group, the incidence of cervical leakage (6.6%) was significantly decreased in comparison with the classical PREP group (25.3%) (P= 0.03). The results of this study suggest that the omission of MBP has a positive impact on the incidence of postoperative complications in esophagocoloplasty., (© 2012 Copyright the Authors. Journal compilation © 2012, Wiley Periodicals, Inc. and the International Society for Diseases of the Esophagus.)

Objective: The ideal ratio between liver graft mass and recipient body weight for liver transplantation in small infants is unknown; however, if this ratio is over 4%, a condition called large-for-size may occur. Experimental models of large-for-size liver transplants have not been described in the literature. In addition, orthotopic liver transplantation is marked by high morbidity and mortality rates in animals due to the clamping of the venous splanchnic system. Therefore, the objective of this study was to create a porcine model of large-for-size liver transplantation with clamping of the supraceliac aorta during the anhepatic phase as an alternative to venovenous bypass., Method: Fourteen pigs underwent liver transplantation with whole-liver grafts without venovenous bypass and were divided into two experimental groups: the control group, in which the weights of the donors were similar to the weights of the recipients; and the large-for-size group, in which the weights of the donors were nearly 2 times the weights of the recipients. Hemodynamic data, the results of serum biochemical analyses and histological examination of the transplanted livers were collected., Results: The mortality rate in both groups was 16.5% (1/7). The animals in the large-for-size group had increased serum levels of potassium, sodium, aspartate aminotransferase and alanine aminotransferase after graft reperfusion. The histological analyses revealed that there were no significant differences between the groups., Conclusion: This transplant method is a feasible experimental model of large-for-size liver transplantation.

Objective: The aim of the study was to demonstrate the dynamics and structure of the epileptic network and provide a tentative correlation with the clinical manifestations, in a symptomatic case of Panayiotopoulos syndrome (PS)., Methods: JP, 5-year-old girl. Gestational period and developmental milestones were normal. At age 4 years, two episodes of recurrent vomiting, tonic eye deviation and consciousness impairment lasting for about 30 min occurred. Multifocal spikes were apparent over frontal areas in the EEG and MRI demonstrated an inferior parietal lobe (IPL) lesion., Results: A long-term 35-channel scalp EEG was obtained, which was processed with a Blind Source Separation algorithm. The most significant components with a dipolar field were submitted to source analysis and the recovered generators used to build the nodes of a brain network associated with each spike type. Analysis of information flow supported epileptic propagation from the left parietal lobe to both frontal and temporal lobes around spike peak. The good spatial overlap with physiological networks controlling eye movements, autonomic functions and consciousness, provides a tentative explanation to the diverse clinical manifestations of PS., Conclusions: Spreading patterns of epileptic activity form an extended network in PS., Significance: An epileptic focus in an IPL can reproduce both neurophysiological and clinical features of PS., (Copyright © 2012 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.)

Introduction: Different approaches have been developed in order to deal with maternal mortality. In 2008 the Brazilian Ministry of Health made mandatory the investigation of all deaths of women of reproductive age. So far, completeness of investigation has not been thoroughly accomplished in the State of Rio de Janeiro (RJ) due to the constrained capacity of public health services to investigate the large number of eligible deaths. Our aim was to develop a strategy to improve the selection of deaths of women of reproductive age for investigation under maternal death surveillance., Methods: The study population comprised all deaths of women of reproductive age that occurred in the counties of Belford Roxo and Niterói, RJ, between May and September of 2008. Deaths were classified according to priority for investigation, based on data regarding its underlying cause and relation to either pregnancy, delivery or puerperium, as registered in deaths certificate's fields 43 and 44 (related to maternal deaths)., Results: Data related to fields 43 and 44 was missing in around 47% of death certificates; following investigation it was recovered for approximately 74% of such deaths. Of the 132 deaths investigated, four occurred either during pregnancy, delivery or puerperium and were classified as priority for investigation, among which three were classified as maternal deaths. The strategy developed to improve the selection of deaths of women of reproductive age for investigation under maternal death surveillance was considered appropriate.

Background/purpose: Gallstones and cholelithiasis are being increasingly diagnosed in children owing to the widespread use of ultrasonography. The treatment of choice is cholecystectomy, and routine intraoperative cholangiography is recommended to explore the common bile duct. The objectives of this study were to describe our experience with the management of gallstone disease in childhood over the last 18 years and to propose an algorithm to guide the approach to cholelithiasis in children based on clinical and ultrasonographic findings., Methods: The data for this study were obtained by reviewing the records of all patients with gallstone disease treated between January 1994 and October 2011. The patients were divided into the following 5 groups based on their symptoms: group 1, asymptomatic; group 2, nonbiliary obstructive symptoms; group 3, acute cholecystitis symptoms; group 4, a history of biliary obstructive symptoms that were completely resolved by the time of surgery; and group 5, ongoing biliary obstructive symptoms. Patients were treated according to an algorithm based on their clinical, ultrasonographic, and endoscopic retrograde cholangiopancreatography (ERCP) findings., Results: A total of 223 patients were diagnosed with cholelithiasis, and comorbidities were present in 177 patients (79.3%). The most common comorbidities were hemolytic disorders in 139 patients (62.3%) and previous bariatric surgery in 16 (7.1%). Although symptoms were present in 134 patients (60.0%), cholecystectomy was performed for all patients with cholelithiasis, even if they were asymptomatic; the surgery was laparoscopic in 204 patients and open in 19. Fifty-six patients (25.1%) presented with complications as the first sign of cholelithiasis (eg, pancreatitis, choledocolithiasis, or acute calculous cholecystitis). Intraoperative cholangiography was indicated in 15 children, and it was positive in only 1 (0.4%) for whom ERCP was necessary to extract the stone after a laparoscopic cholecystectomy (LC). Preoperative ERCP was performed in 11 patients to extract the stones, and a hepaticojejunostomy was indicated in 2 patients. There were no injuries to the hepatic artery or common bile duct in our series., Conclusions: Based on our experience, we can propose an algorithm to guide the approach to cholelithiasis in the pediatric population. The final conclusion is that LC results in limited postoperative complications in children with gallstones. When a diagnosis of choledocolithiasis or dilation of the choledocus is made, ERCP is necessary if obstructive symptoms persist either before or after an LC. Intraoperative cholangiography and laparoscopic common bile duct exploration are not mandatory., (Published by Elsevier Inc.)

Objective: The aim of this study was to demonstrate the quality of the National Mortality Information System (SIM) in a special cohort of inpatients submitted to lower limb amputation (LLA) as a result of diabetes mellitus (DM), and compare the mortality pattern by causes for the cohort and for the population base of the State of Rio de Janeiro, from 2000 to 2003., Methods: Data were generated from the linkage of the National Hospital Admittance Authorization-SIH-SUS (2000) and SIM (2000-2003) databases. Individuals under 30 years were excluded from the study, and deaths due to violent causes were also excluded in order to analyze the mention of DM as cause of death. Analyses of causes of death were conducted both in the cohort and the population base. Quality markers of SIM in the cohort were the frequency of mention of diabetes mellitus in the death certificate and the proportion of deaths classified as non-specific causes of death, and non-specific causes of death in the population base., Results: 38.0% in the special cohort (n = 977) died during the four years following LLA as a result of diabetes mellitus (DM), and 49.1% of these deaths occurred during the first year. Endocrine, nutritional metabolic (41.5%), and cardiovascular diseases (28.5%), and non-specific causes of death (8.1%) were the main underlying causes of death (UC). DM was mentioned as UC for 41.0%, as a sequential cause for 1.6%, and as contributive cause for 10.0%, among the other natural underlying causes of death besides DM on death certificates. Non-specific underlying causes of death accounted for 11.8% of death certificates in the population base., Conclusions: The high risk of death, the causes of death and the underreporting of DM on death certificates of adults with diabetes were similar to other population-based mortality studies. In the special cohort, the quality of the causes of death on certificates was considered bad. The improvement in the reliability of cause-of-death depends on the ability of physicians to register, and of healthcare facilities to codify correctly the causes of death on death certificates. The method (linkage of data and multiple causes of death) applied in this study to qualify death information was efficient and effective.

Background: Neuroblastoma is one of the most common solid tumors in the pediatric population and the adrenal gland is the main abdominal site of this tumor. The laparoscopic approach has become the standard of care for most benign adrenal tumors in adults, but the role of laparoscopic adrenalectomy in children for malignant tumor is still a point of controversy. However, there is a growing experience with laparoscopic neuroblastoma resection of small lesions and the use of minimally invasive techniques for the initial management of infiltrative neuroblastoma in the last years. The aim of this study is to describe our initial experience with laparoscopic adrenalectomy for neuroblastoma in children, based on surgical outcomes., Methods: A retrospective review of 7 laparoscopic adrenalectomies performed in a single institution between October 2008 and October 2009. We focused our analysis on early surgical outcomes., Results: The mean tumoral size was 2.8±0.9 cm, the average surgical time was 138.6±65.5 minutes, and the mean hospital stay was 2.9±1.6 days. One stage IV patient was submitted to conversion due to bleeding and needed blood transfusion. There were no late complications or deaths and the mean follow-up time was 18.8±6.1 months., Conclusions: The laparoscopic approach for adrenal neuroblastoma resection is feasible in children with good outcomes, but should be reserved to patients with small, well-circumscribed adrenal lesions, without invasive or infiltrative disease.

Objectives: Newborns who undergo surgery for gastroschisis correction may present with oliguria, anasarca, prolonged postoperative ileus, and infection. New postoperative therapeutic procedures were tested with the objective of improving postoperative outcome., Patients and Methods: One hundred thirty-six newborns participated in one of two phases. Newborns in the first phase received infusions of large volumes of crystalloid solution and integral enteral formula, and newborns in the second phase received crystalloid solutions in smaller volumes, with albumin solution infusion when necessary and the late introduction of a semi-elemental diet. The studied variables were serum sodium and albumin levels, the need for albumin solution expansion, the occurrence of anasarca, the length of time on parenteral nutrition, the length of time before initiating an enteral diet and reaching a full enteral diet, orotracheal intubation time, length of hospitalization, and survival rates., Results: Serum sodium levels were higher in newborns in the second phase. There was a correlation between low serum sodium levels and orotracheal intubation time; additionally, low serum albumin levels correlated with the length of time before the initiation of an oral diet and the time until a full enteral diet was reached. However, the discharge weights of newborns in the second phase were higher than in the first phase. The other studied variables, including survival rates (83.4% and 92.0%, respectively), were similar for both phases., Conclusions: The administration of an albumin solution to newborns in the early postoperative period following gastroschisis repair increased their low serum sodium levels but did not improve the final outcome. The introduction of a semi-elemental diet promoted an increase in body weight at the time of discharge.

Purpose: We aimed to examine the relationship of birthweight to cognitive performance in middle aged participants of the Atherosclerosis Risk in Communities Study (ARIC)., Methods: Cognitive function, assessed by means of three neuropsychological tests-the Delayed Word Recall Test (DWR), the Digit Symbol Subtest of the Wechsler Adult Intelligence Scale-Revised (DSS/WAIS-R), and the Word Fluency (WF) Test, was evaluated in relation to birthweight, as recalled through standardized interviews, by the use of data from the second and fourth follow-up visits of the Atherosclerosis Risk in Communities study cohort (1990-1992 and 1996-1998, respectively). Overall, 6785 participants satisfied the inclusion criteria and were included in the analysis., Results: After adjusting for adult sociodemographic factors, childhood socioeconomic environment and parental risk factors, and adult anthropometric, health status-related. and behavioral variables, we observed linear trends for the relationship of birthweight to WF scores, although the trend was statistically significant only for those reporting exact birthweights (p for trend = .004). For the other cognitive test results, results were either null or inconsistent with the a priori hypotheses., Conclusions: Except for WF in those reporting exact birthweights, our study does not support the notion that birthweight influences cognitive function in adults., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background: International ecological studies have shown a positive association between infant mortality as a proxy for low birth weight and cardiovascular disease mortality in adult life., Methods: Mortality rates due to Cardiovascular Diseases (CVDMR) standardised by age in adults between 45 and 69 years of age and by place of birth (pob) and residence (res) were related to Infant Mortality Rates (IMR) in the Brazilian 1935 birth cohort., Results: Two relationship patterns were noted between IMR and CVDMR: for the Southeast, South and Centre-West group of regions (r(pob)=0.46; r(res)=0.29) and for the North and Northeast group of regions (r(pob)=0.21; r(res)=0.33). For the latter pattern, two states were identified (Rio Grande do Norte and Paraíba) as atypical areas, whose exclusion strengthened the association (r(pob)=0.73; r(res)=0.91)., Conclusions: The direction of the associations changed after the analysis by group of Brazilian regions (indirect control of socio-economic levels, coverage and quality of the information). There is a positive, although weak association between IMR and CVDMR. Attempts to control or minimise the interference of migratory movements, cohort effects and socio-economic levels represented methodological progress in ecological analyses of foetal programming in Brazil., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Unlabelled: OBJECTIVES AND INTRODUCTION: Gastroschisis is a congenital abdominal wall defect with increasing occurrence worldwide over the past 20-30 years. Our aim was to analyze the morbidity of newborns after gastroschisis closure, with emphasis on metabolic and hydroelectrolyte disturbances in patients at three tertiary university centers., Methods: From January 2003 to June 2009, the following patient data were collected retrospectively: (A) Background maternal and neonatal data: maternal age, prenatal diagnosis, type of delivery, Apgar scores, birth weight, gestational age and sex; (B) Surgical modalities: primary or staged closure; and (C) Hospital course: levels of serum sodium and levels of serum albumin in the two first postoperative days, number of ventilation days, other postoperative variables and survival. Statistical analyses were used to examine the associations between some variables., Results: 163 newborns were included in the study. Primary closure of the abdominal defect was performed in 111 cases (68.1%). The mean serum sodium level was 127.4 ± 6.7 mEq/L, and the mean serum albumin level was 2.35 ± 0.5 g/dL. Among the correlations between variables, it was verified that hyponatremia and hypoalbuminemia correlated with the number of days on the ventilator but not with the number of days on total parenteral nutrition (TPN); mortality rate correlated with infection. The final survival rate was 85.9%., Conclusion: In newborns with gastroschisis, more aggressive attention to hyponatremia and hypoalbuminemia would improve the outcome.

Objective: The objective of this study was to report our experience with pediatric orthotopic liver transplantation (OLT) with living related donors., Methods: We performed a retrospective chart analysis of 121 living related donor liver transplantations (LRDLT) from June 1998 to June 2010., Results: Indications were biliary atresia (BA; n = 81), primary sclerosing cholangitis (n = 5), α-1 antitrypsin deficiency (n = 4); cholestasis (n = 9), fulminant hepatic failure (n = 8), autoimmune hepatitis (n = 2), Alagille syndrome (n = 4), hepatoblastoma (n = 3), tyrosinemia (n = 2), and congenital hepatic fibrosis (n = 3). The age of the recipients ranged from 7-174 months (median, 22) and the weights ranged from 6-58 kg (median, 10). Forty-nine children (40.5%) weighed ≤10 kg. The grafts included the left lateral segment (n = 108), the left lobe (n = 12), and the right lobe (n = 1). The donors included 71 mothers, 45 fathers, 2 uncles, 1 grandmother, 1 grandfather, and 1 sister with a median age of 29 years (range, 16-53 ys) and a median weight of 68 kg (range, 47-106). Sixteen patients (12.9%) required retransplantation, most commonly due to hepatic artery thrombosis (HAT; n = 13; 10.7%). The other complications were biliary stenosis (n = 25; 20.6%), portal vein thrombosis (PVT; n = 11; 9.1%), portal vein stenosis (n = 5; 4.1%), hepatic vein stenosis (n = 6; 4.9%), and lymphoproliferative disorders (n = 8; 6.6%). The ultimate survival rate of recipients was 90.3% after 1 year and 75.8% after 3 years. Causes of early death within 1 month were HAT (n = 6), PVT (n = 2), severe graft dysfunction (n = 1), sepsis (n = 1), and intraoperative death in children with acute liver failure (n = 2). Causes of late deaths included lymphoproliferative disease (n = 3), chronic rejection (n = 2), biliary complications (n = 3), and recurrent disease (n = 3; hepatoblastoma and primary sclerosing cholangitis)., Conclusions: Despite the heightened possibility of complications (mainly vascular), LRDLT represented a good alternative to transplantation from cadaveric donors in pediatric populations. It was associated with a high survival ratio., (Copyright © 2011 Elsevier Inc. All rights reserved.)

The study describes the characteristics of maternal deaths in the city of Rio de Janeiro, Brazil, during 2000-2003. After investigation by public-health services, 217 maternal deaths were identified among predominantly non-white (48.9%), single (57.1%) women aged 29.6 +/- 7.3 years on average. Direct obstetric causes corresponded to 77.4% of the maternal deaths, mainly due to hypertensive disorders. HIV-related diseases accounted for 4% of the maternal deaths. Almost three-fourths of the mothers who died were aged 20-39 years, although the highest risk of maternal death corresponded to the age-group of 40-49 years (248.9 per 100,000 livebirths). The socioeconomic and demographic profiles of maternal deaths in the city of Rio de Janeiro reflected a vulnerable social situation. Appropriate interventions aimed at reducing maternal mortality need to encompass all women of childbearing age, irrespective of the magnitude of the risk of maternal death.

Objective: To investigate the correlation between the consumption of refined carbohydrates and fats and the prevalence of obesity in the state capitals of Brazil., Design: An ecological evaluation of obesity and dietary risk factors was carried out in twenty-six state capitals of Brazil., Setting: Analysis was based on the age-standardized prevalence of obesity (BMI >or= 30.0 kg/m2) among adults aged 20-59 years. Both intake and obesity prevalence were obtained from the last National Family Household Budget Survey (HBS). The survey was conducted from July 2002 to June 2003, based on a probabilistic national sample of 48 470 households. In each household, during seven consecutive days, all monetary and non-monetary expenses for food and beverages for family consumption were transformed into energy. The relative contribution of foods and food groups was expressed as the proportion (%) of total energy. Fruits and vegetables were also measured by the quantity bought in grams., Results: Prevalence of obesity varied from 5.1 % to 13.6 % among women and from 5.2 % to 17.6 % among men. For women, there were statistically significant correlations between obesity and intake of sugar and soft drinks (rS = 0.60; P = 0.001), ready-to-eat meals (rS = 0.39; P = 0.05) and potatoes (rS = 0.40; P = 0.04). For men there were no such associations., Conclusions: Increasing intake of refined carbohydrates, mainly soft drinks, may play a role in the prevalence of obesity among women in Brazil. Effecting changes in family purchase patterns may be a strategy to reduce obesity.

Intrahepatic arterioportal fistula (IAPF) is a rare cause of portal hypertension in young children. We report the case of a 2-year-old girl with severe undernutrition, chronic watery diarrhea, and gastrointestinal bleeding because of a congenital intrahepatic arterioportal fistula. Radiographic embolization and surgical ligation of the left hepatic artery were attempted, with no resolution of the symptoms. So, a left lobectomy was performed, with excellent results and prompt disappearance of the diarrhea. Hepatectomy should be considered as a definitive and reliable therapy for congenital IAPF.

Hypothalamic hamartomas (HHs) have been demonstrated as the cause of gelastic epilepsy, both by intracranial electrodes and functional imaging. The neocortex becomes secondarily involved, through poorly characterized propagation pathways. The detailed dynamics of seizure spread have not yet been demonstrated, owing to the limited spatial-temporal resolution of available functional mapping. We studied a patient with epilepsy associated with HH and gelastic epilepsy. Simultaneous electroencephalography (EEG) and functional magnetic resonance imaging (fMRI) of several seizure events were obtained, with blood oxygen level dependent (BOLD) activation of the hamartoma, and left hemisphere hypothalamus, hippocampus, parietal-occipital area, cingulate gyrus, and dorsal-lateral frontal area. Integration of regional BOLD kinetics and EEG power dynamics strongly suggests propagation of the epileptic activity from the HH through the left fornix to the temporal lobe, and later through the cingulate fasciculus to the left frontal lobe. The EEG/fMRI method has the spatial-temporal resolution to study the dynamics of seizure activity, with detailed demonstration of origin and propagation pathways.

Objective: Early onset benign occipital lobe epilepsy (Panayiotopoulos syndrome [PS]) is a common and easily recognizable epilepsy. Interictal EEG spike activity is often multifocal but most frequently localized in the occipital lobes. The origin and clinical significance of the extra-occipital spikes remain poorly understood., Methods: Three patients with the PS and interictal EEG spikes with frontal lobe topography were studied using high-resolution EEG. Independent component analysis (ICA) was used to decompose the spikes in components with distinct temporal dynamics. The components were mapped in the scalp with a spline-laplacian algorithm., Results: The change in scalp potential topography from spike onset to peak, suggests the contribution of several intracranial generators, with different kinetics of activation and significant overlap. ICA was able to separate the major contributors to frontal spikes and consistently revealed an early activating group of components over the occipital areas in all the patients. The local origin of these early potentials was established by the spline-laplacian montage., Conclusions: Frontal spikes in PS are consistently associated with early and unilateral occipital lobe activation, suggesting a postero-anterior spike propagation., Significance: Frontal spikes in the PS represent a secondary activation triggered by occipital interictal discharges and do not represent an independent focus.

Objective: The epilepsies associated with the tuberous sclerosis complex (TSC) are very often refractory to medical therapy. Surgery for epilepsy is an effective alternative when the critical link between the localization of seizure onset in the scalp and a particular cortical tuber can be established. In this study we perform analysis of ictal and interictal EEG to improve such link., Methods: The ictal and interictal recordings of four patients with TSC undergoing surgery for epilepsy were submitted to independent component analysis (ICA), followed by source analysis, using the sLORETA algorithm. The localizations obtained for the ictal EEG and for the average interictal spikes were compared., Results: The ICA of ictal EEG produced consistent results in different events, and there was good agreement with the tubers that were successfully removed in three of the four patients (one patient refused surgery). In some patients there was a large discrepancy between the localization of ictal and interictal sources. The interictal activity produced more widespread source localizations., Conclusions: The use of ICA of ictal EEG followed by the use of source analysis methods in four cases of epilepsy and TSC was able to localize the epileptic generators very near the lesions successfully removed in surgery for epilepsy., Significance: The ICA of ictal EEG events may be a useful add-on to the tools used to establish the connection between epileptic scalp activity and the cortical tubers originating it, in patients with TSC considered for surgery of epilepsy.

The aim of this study was to evaluate the lipid profile of patients with different degrees of hypothyroidism and the effect of levothyroxine replacement in subclinical hypothyroidism (SH). Initially, a cross-sectional study was performed with 226 participants [SH = 133 participants, manifest hypothyroidism (MH) = 23 participants, and euthyroidism (EU) = 70 participants]. The mean levels of atherogenic lipid variables were greater in MH than in SH and were greater in SH than in EU, although the differences between SH and EU did not reach statistical significance. The SH subgroup with greater serum thyrotropin (TSH) levels and that with positive antithyroperoxidase antibodies (TPO-Ab) had greater levels of triglycerides and of the atherogenic index Apo B/Apo A. A positive correlation exists between serum TSH and total cholesterol (rs = 0.167; P = 0.006), triglycerides (rs = 0.219; P < 0.001), and ApoB levels (rs = 0.205; P < 0.001). Eleven patients who received levothyroxine (L-T4) treatment and 15 patients who received placebo were reevaluated 1 year after TSH adjusted intervention. A fall in atherogenic variables was observed in the L-T4-treated group, with significance for total cholesterol (-20.0 vs +16.1 mg/dL in the placebo group) and LDL-c (-21.7 vs +17.2 mg/dL). We concluded that SH leads to an intermediary lipid profile between euthyroid individuals and that found in manifest hypothyroidism and that a significant lipid profile improvement occurred 1 year after L-T4 replacement therapy.

This study focused on access to and utilization of dental services in the State of Rio de Janeiro, Brazil, based on data from the 1998 National Household Sample Survey. The study population included 7,756 individuals, stratified by age (15 to 19, 35 to 44, and 65 to 74 years). Measurement of access to dental services was based on answers to the question regarding the last visit to a dentist. The answer "never visited a dentist" was interpreted as lack of access and was analyzed according to demographic, socioeconomic, and epidemiological variables. The proportions of individuals who had never visited a dentist were 7.6%, 1.8%, e 2.6%, respectively for young people, young adults, and the elderly. The results suggest that low socioeconomic status was associated with lack of access to dental services, characterized by low schooling (youth and young adults), low per capita income (youth and the elderly), and family wealth as measured by number of home appliances. Health insurance coverage was inversely associated with lack of access among youth and young adults.

Objective: To evaluate the prevalence of psychiatric disorders and symptoms in patients with subclinical hypothyroidism., Method: Ninety-four outpatients with at least two elevated serum thyrotrophin levels (> 4 microU/ml) and normal FT4, and 43 euthyroid outpatients, both groups from HUCFF-UFRJ, were evaluated. Psychiatric diagnosis was based on the Structured Clinical Interview Diagnostic for the DSM-IV axis I (SCID-I/DSM-IV), the psychopathological symptoms on Hamilton anxiety and depression scales, and the Beck Inventory., Results: Our data showed an increased prevalence of psychiatric disorders in the subclinical hypothyroidism patients when compared to the euthyroid group (45.7% vs 25.6%; p = 0.025), mood disorders being the most frequent. The prevalence of depressive symptoms based on Beck's Scale among subclinical hypothyroidism patients was about 2.3 times higher than among euthyroid ones (45.6% vs 20.9%, p = 0.006). Anxiety symptoms were also more frequent among subclinical hypothyroidism patients (87.0% vs 60.5%, p < 0.001), mainly clinical anxiety (44.6% vs 23.3%; p = 0.001)., Conclusion: Our results showed a significant association of subclinical hypothyroidism with psychiatric disorders and an increased frequency of subsyndromic depression and anxiety symptoms in subclinical hypothyroidism in relation to the euthyroid group.

The Panayiotopoulos type of occipital lobe epilepsy has generated great interest, but the particular brain areas involved in the peculiar seizure manifestations have not been established. We studied a patient with the syndrome, using high-resolution EEG and simultaneous EEG and functional magnetic resonance imaging (fMRI). Resolution of the scalp EEG was improved using a realistic spline Laplacian algorithm, and produced a complex distribution of current sinks and sources over the occipital lobe. The spike-related blood oxygen level dependent (BOLD) effect was multifocal, with clusters in lateral and inferior occipital lobe and lateral and anterior temporal lobe. We also performed regional dipole seeding in BOLD clusters to determine their relative contribution to generation of scalp spikes. The integrated model of the neurophysiologic and vascular data strongly suggests that the epileptic activity originates in the lateral occipital area, spreading to the occipital pole and lateral temporal lobe.

Introduction: Neuropsychological changes are still controversial in patients with subclinical hypothyroidism (SH). The objective of this study is to assess these changes., Method: Cross-sectional study comparing the results of the neurocognitive evaluation of 65 SH patients and 31 individuals without thyroid disease. Subclinical hypothyroidism was defined as at least two elevated serum TSH levels (> 4 microUI/ml) with normal serum free T4 levels (0.9-1.8 ng/dl). The participants underwent the following neuropsychological assessment: Buschkes Selective Reminding Procedure, Rey-Osterrieth Complex Figure Test, Warrington's Recognition Memory Test for Words and Faces, and the Vocabulary subtest of the WAIS-R., Results: The groups were similar in regard to mean age, sex and educational level. No neuropsychological change was found in patients with SH when compared with euthyroid individuals., Conclusion: No difference was observed in the performance of the neuropsychological tests between both groups in regard to the functions studied.

Objective: The Panayiotopoulos type of idiopathic occipital epilepsy has peculiar and easily recognizable ictal symptoms, which are associated with complex and variable spike activity over the posterior scalp areas. These characteristics of spikes have prevented localization of the particular brain regions originating clinical manifestations. We studied spike activity in this epilepsy to determine their brain generators., Methods: The EEG of 5 patients (ages 7-9) was recorded, spikes were submitted to blind decomposition in independent components (ICs) and those to source analysis (sLORETA), revealing the spike generators. Coherence analysis evaluated the dynamics of the components., Results: Several ICs were recovered for posterior spikes in contrast to central spikes which originated a single one. Coherence analysis supports a model with epileptic activity originating near lateral occipital area and spreading to cortical temporal or parietal areas., Conclusions: Posterior spikes demonstrate rapid spread of epileptic activity to nearby lobes, starting in the lateral occipital area. In contrast, central spikes remain localized in the rolandic fissure., Significance: Rapid spread of posterior epileptic activity in the Panayitopoulos type of occipital lobe epilepsy is responsible for the variable and poorly localized spike EEG. The lateral occipital cortex is the primary generator of the epileptic activity.

Background: This investigation evaluated and correlated clinical, laboratorial aspects and psychiatric symptoms in sub clinical hypothyroidism (SH)., Methods: Cross sectional study involving 103 patients with SH and 60 subjects without thyroid disease. Clinical and psychiatric evaluation was respectively based on the Zulewski score and Hamilton A, Hamilton D and Beck questionnaires. Serum thyreotropin (TSH), (thyroxine) FT4 and (antithyroperoxidase) ATPO were measured in all participants. Analysis of continuums data was assessed by the Student t- test, for normally distributed data, and by the Mann- Whitney and Kruskal Wallis tests for non-parametric data. The chi2, Fisher's and Kruskal Wallis tests were used to study qualitative variables. Multivariate analyses were used to study confounding variables., Results: Mean serum TSH level was 7.76 +/- 2.9 micro UI/mL in SH and 1.66 +/- 0.6 micro UI/mL in the group without thyroid disease (p=0.001). Mean serum T4L was slightly lower among patients with HS, and showed a negative linear correlation with TSH. Higher frequencies of abnormal clinical score (48.3 vs. 67.0%; p=0.02), depression self reported symptoms (20.5 vs. 44.2%; p=0.011) and anxiety symptoms (86.0 vs. 63.4%; p=0.004) occurred more frequently in SH. Frequency of psychiatric symptoms had a positive correlation with the clinical score and serum TSH. There was no association between the clinical and psychiatric findings and the etiology of SH, presence of ATPO, age or menopause., Conclusions: The study showed that SH was associated with clinical findings and with psychiatric symptoms. Clinical trials are required to evaluate possible improvement with levotiroxine.

Objective: Gelastic seizures are a frequent and well established manifestation of the epilepsy associated with hypothalamic hamartomas. The scalp EEG recordings very seldom demonstrate clear spike activity and the information about the ictal epilepsy dynamics is limited. In this work, we try to isolate epileptic rhythms in gelastic seizures and study their generators., Methods: We extracted rhythmic activity from EEG scalp recordings of gelastic seizures using decomposition in independent components (ICA) in three patients, two with hypothalamic hamartomas and one with no hypothalamic lesion. Time analysis of these rhythms and inverse source analysis was done to recover their foci of origin and temporal dynamics., Results: In the two patients with hypothalamic hamartomas consistent ictal delta (2-3 Hz) rhythms were present, with subcortical generators in both and a superficial one in a single patient. The latter pattern was observed in the patient with no hypothalamic hamartoma visible in MRI. The deep generators activated earlier than the superficial ones, suggesting a consistent sub-cortical origin of the rhythmical activity., Conclusions: Our data is compatible with early and brief epileptic generators in deep sub-cortical regions and more superficial ones activating later., Significance: Gelastic seizures express rhythms on scalp EEG compatible with epileptic activity originating in sub-cortical generators and secondarily involving cortical ones.

Some symptoms and signs of hypothyroidism, as well as some laboratory abnormalities, may be present in subclinical hypothyroidism (SH). This study evaluates the prevalence of signs and symptoms of hypothyroidism and skeletal muscle dysfunction in 57 patients with SH compared to 37 euthyroid controls. The participants received a clinical score based on signs and symptoms of hypothyroidism. The muscle strength was estimated by manual testing and chair dynamometer and inspiratory force by manuvacuometer. Thyroid hormones and muscle enzymes were measured. The SH group presented with higher score (p< 0.01), complained about myalgia and weakness more frequently (p< 0.05), and showed strength disability in scapular and pelvic girdles (p< 0.05). The median free T4 serum levels were lower in SH (p< 0.001). These findings suggest that signs and symptoms of thyroid dysfunction may be related to lower levels of FT4 in SH and should be taken into account in the decision of beginning LT4 therapy.

Measures of functional levels, commonly used to assess the safety and quality of life of individuals and populations, have not yet been derived from a fuzzy framework. The aim of this study is to estimate the degree of disability associated with varying functional levels, through a model based on fuzzy sets theory. A fuzzy linguistic model was developed to measure varying levels of functional disability, in accordance with the definitions of an individual's social and physical activities and mobility. One year of an adult's life whose mobility, social and physical activities were somewhat limited, was judged to be equivalent to 0.575 years free of functional disability. Results obtained from the fuzzy model approach those obtained with the quality of well-being scale (QWB), used as a conceptual framework. Such findings are encouraging, since the QWB is considered a consistent and valid approach for disability assessment and quality-of-life evaluation.

In clinical or epidemiologic research, the measurement of variables always implies some degree of error. Because it is impossible to control the various sources of variation, the assessment of the reliability of a measurement is essential. Otherwise, concordance analysis must take into account the "clinical" interpretation of the measurement under study, because its practical usefulness is of central importance. In this article, we propose a new approach to assess the reliability of a quantitative measurement. We use a graphical approach familiar to statisticians and data analysts of the biomedical area, associating to it the useful feature of interpretation based on the proportion of concordant cases. We believe that the proposed graphical approach can serve as a complement, or as a alternative, to the Altman-Bland method for agreement analysis. It allows a simple interpretation of agreement that takes into account the "clinical" importance of the differences between observers or methods. In addition, it allows the analysis of reliability or agreement, by means of survival analysis techniques.

Purpose: The epilepsy associated with hypothalamic hamartomas (HHs) has typical clinical, electrophysiologic, and behavioral manifestations refractory to drug therapy and with unfavorable evolution. It is well known that only sessile lesions produce epilepsy, but no correlation has been established between the different types of sessile hamartomas and the diverse manifestations of the epilepsy. We correlate anatomic details of the hamartoma and the clinical and neurophysiologic manifestations of the associated epilepsy., Methods: HHs of seven patients with epilepsy (ages 2- 25 years) were classified as to lateralization and connection to the anteroposterior axis of the hypothalamus by using high-resolution brain magnetic resonance imaging. We correlated the anatomic classification with the clinical and neurophysiologic manifestations of the epilepsy as evaluated in long-term (24 h) video-EEG recordings., Results: HHs ranged in size from 0.4 to 2.6 cc, with complete lateralization in six of seven patients. Ictal manifestations showed good correlation with the lobar involvement of ictal/interictal EEGs. These manifestations suggest the existence of two types of cortical involvement, one associated with the temporal lobe, produced by hamartomas connected to the posterior hypothalamus (mamillary bodies), and the other associated with the frontal lobe, seen in lesions connecting to the middle hypothalamus., Conclusions: A consistent clinical and neurophysiologic pattern of either temporal or frontal lobe cortical secondary involvement was found in the patients of our series. It depends on whether the hamartoma connects to the mamillary bodies (temporal lobe cases) or whether it connects to the medial hypothalamus (frontal lobe cases).

Background: cardiovascular system, strongly inhibits growth factor-stimulated phenotypic modulation of vascular smooth muscle cells in vitro and in vivo. The function of Gax in vascular endothelium is unknown, but we hypothesized that it may play a similar role there. We therefore studied Gax expression in vascular endothelial cells and its effects on proliferation and tube formation., Materials and Methods: Gax expression in normal endothelial cells was examined in vitro by Northern blot and reverse transcriptase polymerase chain reaction and in vivo by immunohistochemistry. A replication-deficient adenovirus was then used to express Gax in human umbilical vein endothelial cells (HUVECs). HUVEC proliferation, 3H-thymidine uptake, p21 expression, and tube formation on reconstituted basement membrane were measured at different viral multiplicities of infection., Results: Gax mRNA was detected in HUVECs by reverse transcriptase polymerase chain reaction and Northern blot analysis and in normal vascular endothelium by immunohistochemistry. Compared with controls transduced with a virus expressing beta-galactosidase, Gax strongly inhibited HUVEC proliferation and mitogen-stimulated 3H-thymidine uptake. p21 expression in HUVECs transduced with Gax was increased up to 5-fold as measured by Northern blot, and p21 promoter activity was activated by 4- to 5-fold. Tube formation on Matrigel was strongly inhibited by Gax expression., Conclusions: Gax is expressed in vascular endothelium and strongly inhibits endothelial cell activation in response to growth factors and tube formation in vitro. These observations suggest that Gax inhibits endothelial cell transition to the angiogenic phenotype in response to proangiogenic growth factors and, as a negative regulator of angiogenesis, may represent a target for the antiangiogenic therapy of cancer.

Objective: The epilepsy associated with the hypothalamic hamartomas constitutes a syndrome with peculiar seizures, usually refractory to medical therapy, mild cognitive delay, behavioural problems and multifocal spike activity in the scalp electroencephalogram (EEG). The cortical origin of spikes has been widely assumed but not specifically demonstrated., Methods: We present results of a source analysis of interictal spikes from 4 patients (age 2-25 years) with epilepsy and hypothalamic hamartoma, using EEG scalp recordings (32 electrodes) and realistic boundary element models constructed from volumetric magnetic resonance imaging (MRIs). Multifocal spike activity was the most common finding, distributed mainly over the frontal and temporal lobes. A spike classification based on scalp topography was done and averaging within each class performed to improve the signal to noise ratio. Single moving dipole models were used, as well as the Rap-MUSIC algorithm., Results: All spikes with good signal to noise ratio were best explained by initial deep sources in the neighbourhood of the hamartoma, with late sources located in the cortex. Not a single patient could have his spike activity explained by a combination of cortical sources., Conclusions: Overall, the results demonstrate a consistent origin of spike activity in the subcortical region in the neighbourhood of the hamartoma, with late spread to cortical areas.

Involuntary rhythmic leg movements in childhood is an uncommon condition, the generators of which remain unknown. We report on a male 3 years of age with distinct features providing important clues concerning the location of one of these generators. At the age of 7 months, the previously healthy young male started with low frequency, rhythmic, and continuous (both during wakefulness and sleep) flexion/extension movements of the lower limbs. Movements interfered significantly with gait acquisition, and, despite normal cognitive development, he was able to walk only at age 2 years, 4 months. The neurologic examination revealed the absence of automatic stepping in the neonatal period, but was otherwise normal. A polygraphic electroencephalogram/electromyogram (EEG/EMG) recording, at the age of 2 years, 9 months, revealed rhythmic and synchronous legs with EMG activity at 0.5 Hz. A more complete polygraphic recording at the age of 3 years, 10 months, showed a lower frequency (0.35 Hz) for the movements, which were time-locked with the respiratory cycle. Magnetic resonance imaging (MRI) of the brain revealed an increased T(2) signal in the upper medulla-lower pons regions. The generator of the rhythmic legs movements is postulated to be the respiratory center, connecting with the reticulospinal projecting neurons through an aberrant pathway.