Real-world impact of glucocorticoid replacement therapy on bone mineral density: retrospective experience of a large single-center CAH cohort spanning 24 years.

The congenital adrenal hyperplasia population seems to have an intrinsic tendency to a high frequency of low bone mass. However in this single-center and long-term evaluated cohort, the simplified corticoid regimen, with exclusive dexamethasone single dose reposition during adulthood, did not represent a risk factor for decrease in bone health. INTRODUCTION: The impact of long-term and supposedly physiological doses of gluco and mineralocorticoid (GC/MC) on bone mineral density (BMD) in congenital adrenal hyperplasia (CAH) remains discordant among studies, which contain different clinical forms and corticoid regimens. Our aim was to evaluate the BMD in CAH adults receiving similar GC regimen since childhood and to correlate it with GC/MC cumulative doses. METHODS: Only patients with good compliance, who used cortisone acetate (CA) during childhood and dexamethasone after the final height achievement. Cumulative GC/MC doses were calculated from diagnosis until last evaluation. BMD was analyzed by the first and last energy X-ray absorptiometry (DXA) scans performed. RESULTS: Twenty simple virilizing (SV) and 14 salt wasting (WS) whose mean age was 26 ± 6 years, mean CA, dexamethasone, and fludrocortisone cumulative doses were 63,813 ± 32,767, 812 ± 558, and 319 ± 325 mg/m2, respectively. Based on the last DXA, low BMD was observed in 11% of patients, total hip Z-score was lower in the SW than SV form (p = 0.04). Cumulative CA dose had an inverse correlation with femoral neck Z-score (p < 0.01). Total cumulative GC and MC doses had an inverse correlation with total hip Z-score (p < 0.01). In the analysis of sequential BMD during dexamethasone therapy, no association was observed among cumulative GC/MC doses, clinical forms, sex, and lumbar Z-score delta. CONCLUSIONS: Even though a low CA regimen during growth periods in addition to MC replacement appears to have an influence on BMD at femoral sites, interestingly a low dexamethasone one does not seem to be deleterious for bone health in adulthood.

Latent class analysis of attention and white matter correlation in children with attention-deficit/hyperactivity disorder.

This study aimed to explore attentional patterns among children with inattentive attention-deficit/hyperactivity disorder (ADHD-I) and children with typical development (TD), using a latent class analysis (LCA). Patterns of brain connectivity were also explored. The sample comprised 29 ADHD-I and 29 TD matched children. An LCA was conducted to reclassify subjects according to their attentional performance, considering cognitive measures of attention and behavioral symptoms, regardless of group of origin. The new clusters were then compared in respect to brain white matter measurements (extracted from diffusion tensor imaging). Participants were rearranged in 2 new latent classes, according to their performance in an attention task and the results of behavioral scales, resulting in groups with more homogeneous attentional profiles. A comparison of the 2 new classes using the white matter measurements revealed increased fractional anisotropy in the left inferior fronto-occipital fasciculus and left inferior longitudinal fasciculus for the class composed by participants with a higher risk of attentional problems. The findings indicated that it was possible to observe variability regarding neuropsychological profile, accompanied by underpinning neurobiological differences, even among individuals with the same disorder subtype - inattentive ADHD. This specific data-driven clustering analysis may help to enhance understanding of the pathophysiology of the disorder's phenotypes.

Evaluation of peptides release using a natural rubber latex biomembrane as a carrier.

The biomembrane natural (NRL-Natural Rubber Latex), manipulated from the latex obtained from the rubber tree Hevea brasiliensis, has shown great potential for application in biomedicine and biomaterials. Reflecting the biocompatibility and low bounce rate of this material, NRL has been used as a physical barrier to infectious agents and for the controlled release of drugs and extracts. The aim of the present study was to evaluate the incorporation and release of peptides using a latex biomembrane carrier. After incorporation, the release of material from the membrane was observed using spectrophotometry. Analyses using HPLC and mass spectroscopy did not confirm the release of the antimicrobial peptide [W6]Hylin a1 after 24 h. In addition, analysis of the release solution showed new compounds, indicating the degradation of the peptide by enzymes contained in the latex. Additionally, the release of a peptide with a shorter sequence (Ac-WAAAA) was evaluated, and degradation was not observed. These results showed that the use of NRL as solid matrices as delivery systems of peptide are sequence dependent and could to be evaluated for each sequence.

Latent class analysis of attention and white matter correlation in children with attention-deficit/hyperactivity disorder

This study aimed to explore attentional patterns among children with inattentive attention-deficit/hyperactivity disorder (ADHD-I) and children with typical development (TD), using a latent class analysis (LCA). Patterns of brain connectivity were also explored. The sample comprised 29 ADHD-I and 29 TD matched children. An LCA was conducted to reclassify subjects according to their attentional performance, considering cognitive measures of attention and behavioral symptoms, regardless of group of origin. The new clusters were then compared in respect to brain white matter measurements (extracted from diffusion tensor imaging). Participants were rearranged in 2 new latent classes, according to their performance in an attention task and the results of behavioral scales, resulting in groups with more homogeneous attentional profiles. A comparison of the 2 new classes using the white matter measurements revealed increased fractional anisotropy in the left inferior fronto-occipital fasciculus and left inferior longitudinal fasciculus for the class composed by participants with a higher risk of attentional problems. The findings indicated that it was possible to observe variability regarding neuropsychological profile, accompanied by underpinning neurobiological differences, even among individuals with the same disorder subtype - inattentive ADHD. This specific data-driven clustering analysis may help to enhance understanding of the pathophysiology of the disorder's phenotypes.

Porosity effects of natural latex (Hevea brasiliensis) on release of compounds for biomedical applications.

Natural rubber latex biomedical (NRLb) obtained from the rubber tree Hevea brasiliensis has shown great potential in biomedicine and biomaterial applications. NRLb has been utilized as a physical barrier against infectious agents and in the controlled release of drugs and extracts. In the present work, NRLb was polymerized in a lyophilizer using different volumes of water to control the resultant membrane porosity and characterized regarding the surface morphology, water vapour permeability (WVP), mechanical properties, haemolytic activity and cytotoxicity. The release of bovine serum albumin protein from the latex membranes was evaluated. Drug release rates increased with porosity and membranes were able to control protein release up to 12 h. In addition, WVP increased with the quantity of pores. The cell viability observed for the porous membrane was higher than that noted for conventional membranes. In summary, the porosity control of natural latex membranes can be used to modulate properties and make them suitable for biomedical applications, such as wound dressings, modulated gas-exchange membranes and controlled drug delivery systems.

Comparison of the lactate minimum speed and the maximal lactate steady state to determine aerobic capacity in purebred Arabian horses.

AIM: To compare five different protocols for estimating the lactate minimum speed (LMS) with that for estimating the maximal lactate steady state (MLSS) in Arabian horses, in order to obtain a more rapid method for monitoring aerobic capacity and prescribing training schedules. METHODS: Eight purebred Arabian horses were conditioned to exercise on a treadmill for 12 days then submitted to three to five exercise sessions to determine the MLSS. Blood samples were collected from a jugular catheter at specific intervals for measurement of lactate concentrations. The MLSS was the velocity maintained during the last 20 minutes of constant submaximal exercise, at which the concentration of lactate increased by no more than 1.0 mmol/L. The LMS test protocols (P1 - P5) included a warm-up period followed by a high-intensity gallop. The speed was then reduced to 4 m/s, and the incremental portion of the test was initiated. In P1, P2, and P3, the velocity increment was 0.5 m/s, and the duration of each incremental stage was three, five and seven minutes, respectively. In P4 and P5, the velocity increments were 1.0 and 1.5 m/s, respectively, and the duration of the stages was fixed at five minutes each. A second-degree polynomial function was fitted to the lactate-velocity curve, and the velocity corresponding to the lowest concentration of lactate was the LMS. RESULTS: Only the mean LMS determined by P1 and P2 did not differ from the velocity determined by the MLSS test (p > 0.1). There was a strong correlation (r >0.6) between P1 and the MLSS velocity. A limits of agreement plot revealed that the best agreement occurred between the MLSS test and P1 (mean bias = 0.14 m/s), followed by P2 (bias = -0.22 m/s). The lactate concentrations associated with the various LMS protocols did not differ. CONCLUSIONS: This study shows the variation between protocols of the LMS test for determining the onset of blood lactate accumulation but also reveals that, at least for Arabian horses, the P1 protocol of the LMS has good agreement with the MLSS.

Efeitos do extrato da parede de levedura na digestibilidade, no escore fecal e na palatabilidade de dietas para gatos / Effects of spray-dried yeast cell wall on digestibility, score of feces, and palatability of diets for cats

Para avaliar o efeito do extrato seco da parede de levedura (EPL) sobre a digestibilidade, o escore fecal e a palatabilidade de dietas para gatos, foram realizados três ensaios experimentais. No primeiro, 20 animais adultos foram distribuídos ao acaso em quatro tratamentos: dieta comercial úmida (controle) e dieta-controle + 0,2, ou dieta-controle + 0,4 ou dieta-controle + 0,6 por cento de EPL na matéria seca. No segundo, utilizaram-se alimento seco e as mesmas proporções com o mesmo delineamento do primeiro experimento. No ensaio 3, de palatabilidade, 20 gatos adultos receberam simultaneamente dieta comercial úmida sem e com a inclusão de 0,4 por cento de EPL. No experimento 1, não foram observadas diferenças quanto à digestibilidade da matéria seca, proteína bruta, extrato etéreo, matéria orgânica e energia bruta, assim como no escore fecal; no segundo, houve aumento linear (P<0,46) no coeficiente de digestibilidade da matéria seca, e, no terceiro, observou-se efeito negativo da inclusão de 0,4 por cento sobre a palatabilidade da dieta (P<0,004). Conclui-se que a inclusão de EPL em dietas úmidas não influi na digestibilidade, mas pode comprometer a palatabilidade, e que em dietas secas há melhora da digestibilidade da matéria seca.(AU)

The effects of spray-dried yeast cell wall (YCW) were evaluated on digestibility, score of feces, and palatability of diets for cats were evaluated. Three trials were carried out. In the first, 20 adult cats were randomly allotted in four treatments: wet commercial diet (control) and control plus 0.2, 0.4, or 0.6 percent of YCW in dry matter. In the second, a commercial dry diet was tested in an equal arrangement concerning concentration of YCW and number of animals of the first trial. In the third, 20 adult cats were fed at the same time a wet diet with or without 0.4 percent YCW. In the first trial, no differences among treatments for dry matter, crude protein, ether extract, organic matter, gross energy digestibility, and faecal score were observed. In second trial, positive linear effect on dry matter digestibility (P=0.046) was observed. In the third, negative effect of 0.4 percent YCW inclusion (P=0.004) on palatability of diet was observed. It was concluded that YCW inclusion in wet diet did not effectively alter the nutrients digestibilities but it decrease the palatability. However, the YCW inclusion in dry diets can be important to improve dry matter digestibility.(AU)

Efeitos do extrato da parede de levedura na digestibilidade, no escore fecal e na palatabilidade de dietas para gatos / Effects of spray-dried yeast cell wall on digestibility, score of feces, and palatability of diets for cats

Para avaliar o efeito do extrato seco da parede de levedura (EPL) sobre a digestibilidade, o escore fecal e a palatabilidade de dietas para gatos, foram realizados três ensaios experimentais. No primeiro, 20 animais adultos foram distribuídos ao acaso em quatro tratamentos: dieta comercial úmida (controle) e dieta-controle + 0,2, ou dieta-controle + 0,4 ou dieta-controle + 0,6 por cento de EPL na matéria seca. No segundo, utilizaram-se alimento seco e as mesmas proporções com o mesmo delineamento do primeiro experimento. No ensaio 3, de palatabilidade, 20 gatos adultos receberam simultaneamente dieta comercial úmida sem e com a inclusão de 0,4 por cento de EPL. No experimento 1, não foram observadas diferenças quanto à digestibilidade da matéria seca, proteína bruta, extrato etéreo, matéria orgânica e energia bruta, assim como no escore fecal; no segundo, houve aumento linear (P<0,46) no coeficiente de digestibilidade da matéria seca, e, no terceiro, observou-se efeito negativo da inclusão de 0,4 por cento sobre a palatabilidade da dieta (P<0,004). Conclui-se que a inclusão de EPL em dietas úmidas não influi na digestibilidade, mas pode comprometer a palatabilidade, e que em dietas secas há melhora da digestibilidade da matéria seca.

The effects of spray-dried yeast cell wall (YCW) were evaluated on digestibility, score of feces, and palatability of diets for cats were evaluated. Three trials were carried out. In the first, 20 adult cats were randomly allotted in four treatments: wet commercial diet (control) and control plus 0.2, 0.4, or 0.6 percent of YCW in dry matter. In the second, a commercial dry diet was tested in an equal arrangement concerning concentration of YCW and number of animals of the first trial. In the third, 20 adult cats were fed at the same time a wet diet with or without 0.4 percent YCW. In the first trial, no differences among treatments for dry matter, crude protein, ether extract, organic matter, gross energy digestibility, and faecal score were observed. In second trial, positive linear effect on dry matter digestibility (P=0.046) was observed. In the third, negative effect of 0.4 percent YCW inclusion (P=0.004) on palatability of diet was observed. It was concluded that YCW inclusion in wet diet did not effectively alter the nutrients digestibilities but it decrease the palatability. However, the YCW inclusion in dry diets can be important to improve dry matter digestibility.

Diagnosis and molecular characterization of non-classic forms of Tay-Sachs disease in Brazil.

Molecular analysis of five Brazilian families, including eight patients presenting with nonclassic Tay-Sachs disease, was performed to identify frequent causative mutations and their correlation with clinical course. Three patients were affected by the B1 subacute variant and were shown to carry the R178H mutation (the DN allele), which is also common among Portuguese patients. Two of them were compound heterozygotes, whereas the third presented with the mutation in both alleles. Since Brazil was a Portuguese colony for over two centuries, common ancestry might be the probable explanation. The fourth patient presented with a juvenile phenotype and carries the R499H mutation, which has been reported only once worldwide and is associated with residual enzyme activity, responsible for a slower clinical course. The fifth family, of an Ashkenazi Jewish background, showed an extensive intrafamilial clinical variability among three affected sibs presenting with muscle atrophy, ataxia, and psychiatric symptoms. They were first diagnosed as having atypical spinal muscular atrophy and, subsequently, spinocerebellar ataxia, but, recently, the diagnosis of late-onset Tay-Sachs disease was confirmed based on reduced plasma hexosaminidase A activity and the G269S/InsTATC1278 genotype. It is therefore highly recommended to test patients with a similar clinical history for Tay-Sachs disease. In the same family, one first cousin committed suicide at the age of 24 years, presenting with a clinical phenotype that suggested an undiagnosed case and highlighting the effect of the intrafamilial clinical variability in delaying a prompt diagnosis. It is now recognized that his parents are, in fact, a carrier couple. Additionally, another relative had been previously identified as a heterozygote in a Tay-Sachs disease screening program, but the information was not shared among the family. Since this information might anticipate diagnosis and genetic counseling, it is advisable that heterozygote screening programs encourage families to share genetic information.

Dithiocarbamates residues in Brazilian food and the potential risk for consumers.

Dithiocarbamates are a non-systemic group of pesticides widely used to protect crops from fungal diseases. The current methodology used by monitoring laboratories to determine dithiocarbamates in food involves the analysis of CS(2) generated after hydrolysis of the compound present in the sample. This method does not identify the origin of the CS(2) detected, which may or may not be related to the presence of pesticides leading to a potential overestimation of the dietary dithiocarbamate intake. In this paper, 520 food samples (papaya, banana, apple, strawberry, orange, potato, tomato, rice and dry beans) collected in the local market of the Federal District, Brazil, were analyzed for dithiocarbamate content. Detectable levels (> or =10.10 mg/kg CS(2)) were found in 60.8% of the samples, with the highest levels (up to 3.8 mg/kg) found in strawberry, papaya and banana. No residues were found in rice (polished) and only one dry bean sample had detectable levels of the fungicides. Detectable residues were found in the pulp of banana, papaya (including the seeds) and orange (50-62% of the analyzed samples). An exposure assessment, based on dithiocarbamate levels detected in the food crops analyzed in this study, confirms that the intake of dithiocarbamates through food consumption in the country does not represent a health risk to consumers, i.e., the estimated daily intake is less than the acceptable daily intake. Furthermore, the implementation of more selective methodologies to individually analyze these compounds in food monitoring programs in Brazil is not necessary.

Análise de timol por CLAE na tintura de Lippia sidoides Cham. (alecrim-pimenta) produzida em diferentes estágios de desenvolvimento da planta

O teor de timol, princípio ativo usado como marcador nas tinturas das folhas de Lippia sidoides Cham. (Verbenaceae) preparadas com material coletado antes, durante e após a floração e designadas como T1, T2 e T3, foi determinado por CLAE, utilizando-se cromatógrafo Shimadzu CLASS-VP, coluna RP-18 (Supelco), com fase móvel isocrática acetonitrila:água (78:22) e fluxo 0,8 ml/min com detecção em 254 nm. As amostras foram injetadas num volume de 20 μl e analisadas em triplicata. Os teores de timol encontrados foram 1,97 ± 0,07 em T1; 2,00 ± 0,03 em T2 e 2,34 ± 0,06 mg/ml em T3. Os resultados mostram discretas diferenças nas concentrações de timol nas tinturas das folhas de alecrim-pimenta preparadas em diferentes momentos de seu desenvolvimento. Contudo, observou-se que o melhor momento para a coleta da planta parece ser após a sua floração (T3), que mostrou o maior teor de timol.

Lippia sidoides Cham. (Verbenaceae) is popularly known in Northeast Brazil as "alecrim-pimenta". The goal of the present work is to perform a quantitative analysis of timol, used as a marker in phytomedicine prepared from the leaves of L. sidoides, collected before, during and after the flower. At least three samples of tincture of L. sidoides were analyzed by HPLC. Among the samples analyzed, the tincture produced from the leaves after the flower showed slightly high concentration of timol (2,34 ± 0,06 mg/ml) when compared with before (1,97 ± 0,07 mg/ml) or during the flower (2,00 ± 0,03 mg/ml). The best period to collect leaves of L. sidoides seems to be after the flower.

[Insulin-glucose ratio and body fat composition in patients with chronic anovulation and sterility]. / Relación insulina-glucosa y composición corporal grasa en pacientes con anovulación crónica y esterilidad.

A clinical, descriptive, and transversal study was conducted in a group of patients with chronic anovulation and sterility, to correlate insulin resistance, determined by the fasting glucose/insulin ratio, with body fat composition using anthropometrics parameters and the interaction of light near infrared region method, we studied 41 young patients with chronic anovulation and sterility. Based on their body mass index, all patients had obesity or overweight. Similarly, most of them presented with a percentage of body fat over the recommended limits. Forty percent of all studied patients had a fasting glucose/insulin ratio below 4.5, which corresponds to insulin resistance. The correlation between the percentage of body fat and fasting glucose/insulin ratio was significant, as was the correlation between body mass index and the percentage of body fat. We found overweight or obesity in the majority of our patients, and insulin resistance in almost half of them. Such disturbances were positively associated with the percentage of body fat and android distribution. Therefore, we recommend a routinely anthropometrics evaluation in these patients as well as fasting glucose/insulin ratio determination in order to act in an early stage over the natural history of metabolic syndrome, whose common denominator is insulin resistance.

Determination of dithiocarbamate fungicide residues in food by a spectrophotometric method using a vertical disulfide reaction system.

Dithiocarbamates are a class of fungicides extensively used in many crops worldwide. The current residue definition of dithiocarbamates in food for compliance with maximum residue limits, at national and international levels, is total residues arising from the use of any or each dithiocarbamate fungicide, determined as CS(2). The analytical method most frequently used to analyze dithiocarbamate residues in food for monitoring purposes was proposed more than 30 years ago. In this method, total dithiocarbamates are decomposed to CS(2), which is purified and reacted with a cupric reagent. The yellow complex formed is quantified by spectrophotometry. In this paper, a new reaction system for the purification and complexation of CS(2) is proposed. The new system is less fragile than the traditional design, is easier to assemble, and allows for a higher sample throughput, in addition to being of low cost. Recovery of added mancozeb, thiram, or ziram (0.15-8.0 mg/kg) in rice, beans, apple, banana, orange, papaya, tomato, cucumber, and potato ranged from 82 to 120%, with relative standard deviations from 0 to 10% (n = 3 or 5). Analysis of apple, tomato, and papaya samples with field-incurred dithiocarbamate residues showed comparable results using both the traditional and the new reaction systems.

A set of 400 pictures standardised for Portuguese: norms for name agreement, familiarity and visual complexity for children and adults.

The present article provides normative measures for 400 pictured objects (Cycowicz et al., 1997) viewed by Portuguese speaking Brazilian University students and 5-7 year-old children. Name agreement, familiarity and visual complexity ratings were obtained. These variables have been shown to be important for the selection of adequate stimuli for cognitive studies. Children's name agreement was lower than that of adults. The children also failed to provide adequate modal names for 103 concepts, rated drawings as less familiar and less complex, and chose shorter names for pictures. The differences in ratings between adults and children were higher than those observed in the literature employing smaller picture sets. The pattern of correlations among measures observed in the present study was consistent with previous reports, supporting the usefulness of the 400 picture set as a tool for cognitive research in different cultures and ages.

Cognitive profile of patients with relapsing remitting multiple sclerosis.

Multiple sclerosis (MS) is a common disease in Western countries of temperate/cold climate, but in tropical countries an increasing number of cases have been diagnosticated. Moved by the lack of information about cognitive dysfunction of Brazilian MS patients, the present study attempted to describe features of neuropsychological alterations in patients with relapsing remitting MS living in the city of São Paulo. They were compared to healthy volunteers, matched for age and education. In the absence of global intellectual deterioration, the patients had a deficit: a) in learning and verbal long-term memory tasks and in visual long-term memory of complex figure; b) in timed tasks, accounted for by a slowness of mental processes; c) in tasks with a motor component. Tendency to depression was observed; anxiety levels were normal.

[Progressive hereditary dystonia with diurnal fluctuations: report of 2 cases]. / Distonía hereditaria progresiva con fluctuaciones diurnas: descripción de dos casos.

We report two patients with diurnally fluctuating hereditary progressive dystonia, that presented with dystonic movements and parkinsonian symptoms with marked diurnal fluctuations. There was a significant and maintained improvement of symptoms with small doses of L-Dopa. The pathogenesis of this disease could be a functional alteration of basal ganglia in the dopaminergic striatonigral region. The diurnal fluctuation of symptoms would be related to the circadian variation of tyrosine hydroxylase function.

Estudio clinico y electroencefalografico de crisis febriles en el nino. / Clinical and electroencephalographic study of febrile crisis in children

Se realizo un estudio clinico y electroencefalografico de 87 casos de ninos menores de cinco anos con crisis convulsivas febriles. Se considero caracteristicas clinicas de las crisis febriles, antecedentes personales y familiares alteraciones electroencefalograficas y factores predictivos de recurrencia. Destaca la elevada incidencia de indicadores de dano neurologico (41,7%) y de antecedentes familiares de crisis convulsivas (39,3%). Hubo correlacion entre la edad de comienzo de los fenomenos ictiales en el momento de la primera crisis y la presencia de crisis complejas (p < 0.05) lo que hace a los menores de un ano mas expuestos a recurrencia del cuadro. Otros factores descritos en la literatura como predictores de recurrencia no fueron demostrados en este trabajo

El tratamiento de la hidrocefalia infantil. Experiencia en 80 casos. / Treatment of hydrocephalic children.Experience on 80 cases

Se analizan 80 ninos con hidrocefalia, a los que se les instalo valvula de derivacion de L.C.R. ventriculo-cardiaca y ventriculo-peritoneal entre 1972 y 1978. Se realizo seguimiento satisfactorio en el 88% de los casos por un periodo de 3-10 anos, promedio cinco y medio anos. Hubo un 25% de mortalidad atribuible a la valvula, en su mayoria secundarias a infeccion valvular. La malfuncion y obstruccion valvular contribuyo en menor proporcion a la mortalidad, pero fue causa de reoperaciones. El grupo de mayor mortalidad lo formo el de los ninos con grave hidrocefalia congenita. Las infecciones rara vez postoperatorias (4%), fueron preferentemente alejadas de la cirugia y presumiblemente hematogenas. La frecuencia y gravedad de las infecciones valvulares fueron mayores en derivaciones ventriculo-cardiacas. El mejor resultado de valvulas ventriculo-peritoneales se deberia a estar en compartimiento menos expuesto a contaminacion microbiana