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1.
Genet Med ; 2019 Jul 31.
Artigo em Inglês | MEDLINE | ID: mdl-31363182

RESUMO

PURPOSE: Kabuki syndrome (KS) (OMIM 147920 and 300867) is a rare genetic disorder characterized by specific facial features, intellectual disability, and various malformations. Immunopathological manifestations seem prevalent and increase the morbimortality. To assess the frequency and severity of the manifestations, we measured the prevalence of immunopathological manifestations as well as genotype-phenotype correlations in KS individuals from a registry. METHODS: Data were for 177 KS individuals with KDM6A or KMT2D pathogenic variants. Questionnaires to clinicians were used to assess the presence of immunodeficiency and autoimmune diseases both on a clinical and biological basis. RESULTS: Overall, 44.1% (78/177) and 58.2% (46/79) of KS individuals exhibited infection susceptibility and hypogammaglobulinemia, respectively; 13.6% (24/177) had autoimmune disease (AID; 25.6% [11/43] in adults), 5.6% (10/177) with ≥2 AID manifestations. The most frequent AID manifestations were immune thrombocytopenic purpura (7.3% [13/177]) and autoimmune hemolytic anemia (4.0% [7/177]). Among nonhematological manifestations, vitiligo was frequent. Immune thrombocytopenic purpura was frequent with missense versus other types of variants (p = 0.027). CONCLUSION: The high prevalence of immunopathological manifestations in KS demonstrates the importance of systematic screening and efficient preventive management of these treatable and sometimes life-threatening conditions.

2.
Blood ; 134(1): 9-21, 2019 Jul 04.
Artigo em Inglês | MEDLINE | ID: mdl-30940614

RESUMO

Evans syndrome (ES) is a rare severe autoimmune disorder characterized by the combination of autoimmune hemolytic anemia and immune thrombocytopenia. In most cases, the underlying cause is unknown. We sought to identify genetic defects in pediatric ES (pES), based on a hypothesis of strong genetic determinism. In a national, prospective cohort of 203 patients with early-onset ES (median [range] age at last follow-up: 16.3 years ([1.2-41.0 years]) initiated in 2004, 80 nonselected consecutive individuals underwent genetic testing. The clinical data were analyzed as a function of the genetic findings. Fifty-two patients (65%) received a genetic diagnosis (the M+ group): 49 carried germline mutations and 3 carried somatic variants. Thirty-two (40%) had pathogenic mutations in 1 of 9 genes known to be involved in primary immunodeficiencies (TNFRSF6, CTLA4, STAT3, PIK3CD, CBL, ADAR1, LRBA, RAG1, and KRAS), whereas 20 patients (25%) carried probable pathogenic variants in 16 genes that had not previously been reported in the context of autoimmune disease. Lastly, no genetic abnormalities were found in the remaining 28 patients (35%, the M- group). The M+ group displayed more severe disease than the M- group, with a greater frequency of additional immunopathologic manifestations and a greater median number of lines of treatment. Six patients (all from the M+ group) died during the study. In conclusion, pES was potentially genetically determined in at least 65% of cases. Systematic, wide-ranging genetic screening should be offered in pES; the genetic findings have prognostic significance and may guide the choice of a targeted treatment.

3.
J Clin Immunol ; 39(2): 171-181, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30671780

RESUMO

PURPOSE: Immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AIHA) are associated in the definition of Evans syndrome (ES). The occurrence of neurological involvement in this population is poorly described and suggests an underlying primary immunodeficiency (PID). We aimed to describe the clinical manifestations, evolution, and PID profiles of these patients. METHODS: OBS'CEREVANCE is a French, nationwide prospective cohort that includes children with chronic ITP, AIHA, and ES. Patients with a neurological involvement were described. Centralized radiological and pathological reviews and genetic analyses were performed. RESULTS: On October 2016, eight patients (7/181 ES, 1/371 AIHA, and 0/615 ITP) were identified, all male, with a median age (range) at cytopenia onset of 11.5 years (1.6-15.8). Neurological symptoms appeared with a median delay of 6 years (2.5-18) after cytopenia and were polymorphic: seizures (n = 4), cranial nerve palsy (n = 2), Brown-Sequard syndrome (n = 2), intracranial pressure (n = 2), vertigo (n = 1), and/or sensory neuropathy (n = 1). Magnetic resonance imaging (MRI) showed inflammatory lesions, confirmed by pathology for five patients with macrophagic or lymphoplasmocytic infiltrates. All patients had other relevant immunopathological manifestations: pulmonary nodules (n = 6), lymphoproliferation (n = 4), abnormal immunophenotype (n = 8), and hypogammaglobulinemia (n = 7). Treatment consisted of steroids that improved symptomatology and MRI. Five patients relapsed and three had an asymptomatic radiological progression. A PID was identified in 3/8 patients: 22q11.2 microdeletion (n = 1) and CTLA deficiency (n = 2). CONCLUSION: Neurological involvement is a rare and severe late event in the course of childhood ES, which can reveal an underlying PID. Imaging and pathology examination highlight a causative immune dysregulation that may guide targeted therapeutic strategies.

4.
Psychol Rep ; : 33294118805003, 2018 Nov 02.
Artigo em Inglês | MEDLINE | ID: mdl-30388392

RESUMO

The aims of this study were twofold: (i) to investigate the prevalence of social anxiety disorder and body dysmorphic disorder in a nonclinical, Brazilian population and (ii) to examine the effects of selected factors such as sociodemographic characteristics (sex, age, marital status, workload, education, and income), body mass index, current diet, physical activity, and use of aesthetic treatment. A total of 428 adults (279 women and 149 men) aged 18 to 60 years ( M = 31.51, SD = 10.73) participated in the study. Social anxiety disorder was measured using a Brazilian version of the Social Phobia Inventory ( Osório, Crippa, & Loureiro, 2009 ). Body dysmorphic disorder by using a body dysmorphic symptoms scale validated for the Brazilian population ( Ramos & Yoshida, 2012 ). The main results showed that 28.7% of the sample reported symptoms of social anxiety disorder. Body dysmorphic disorder was more prevalent among women, individuals who had sought aesthetic treatment and individuals who were physically inactive. Moreover, lower levels of social anxiety disorder were observed in physically active individuals who had sought aesthetic treatment compared with physically inactive individuals who had sought aesthetic treatment. Social anxiety disorder was negatively correlated with age, daily workload, and income, while body dysmorphic disorder was positively associated with body mass index and negatively with income. These results show that social anxiety disorder and body dysmorphic disorder are differentially influenced by the selected factors investigated in this study.

5.
Minerva Pediatr ; 2018 Jul 02.
Artigo em Inglês | MEDLINE | ID: mdl-29968447

RESUMO

BACKGROUND: The aim of the current study is to analyze the longitudinal effects of certain biopsychosocial variables (socioeconomic status, body mass index (BMI), body image and well-being (QL)), measured at three different menarche stages (S1: before menarche, S2: soon after menarche, and S3: one year after S2), on physical activity and inactivity levels in early adolescence. METHODS: The sample comprised 136 Brazilian teenagers (10-13 years old), showing BMI 18.98 kg/m2 at the initial assessment, who voluntarily participated in the study. Data concerning the following variables were collected in 2010 (S1), 2011 (S2) and 2012 (S3): family income (FI) (socioeconomic anamnesis), BMI (body mass and height), well-being (Autoquestionnaire Qualité de Vie Enfant Imagé) and body image (Body Shape Questionnaire). The physical activity and inactivity levels were measured in S3, only, through the International Physical Activity Questionnaire. The Pearson's correlation and the longitudinal path analysis technique were used to statistically analyze the data. RESULTS: The main results indicated that just family income and BMI, measured soon after menarche (S2), showed significant effect on physical activity and inactivity levels. More specifically, family income has positively influenced (ß = 0.25) physical inactivity levels and negatively influenced (ß = -0.14) moderate physical activity levels, whereas BMI has positively influenced (ß = 0.15) walking levels and negatively influenced (ß = -0.13) vigorous physical activity levels. CONCLUSIONS: These results suggest that the body image and well-being perceived by the teenagers at different menarche stages are not determining factors of their physical activity levels in early adolescence. On the other hand, the effects of socioeconomic status and body mass index on early adolescent girls' physical activity and inactivity levels depend on the assessment period.

6.
J Bodyw Mov Ther ; 22(2): 424-429, 2018 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-29861245

RESUMO

The aim of this study was to determine the effects of Pilates-based mat exercises on functional autonomy and life satisfaction among elderly women. A randomized controlled trial was conducted in Caxias do Sul, Brazil in 2015, in which 61 healthy older women were randomized into two groups (experimental group, EG = 31 participants with 64.25 mean years of age, SD = 0.14, and a control group, CG = 30 participants, 63.75 mean years of age; SD = 0.08). The EG participants performed the mat Pilates twice per week, 60-min per session. The same measurements were performed at baseline and after 16 weeks. No significant differences were found between groups at baseline (p > 0.05). In the EG, the following factors were increased after the 16-weeks training period: flexibility of lower limbs, flexibility of upper limbs, strength in lower and upper limbs, aerobic endurance and dynamic balance. This study revealed significant improvements on the life satisfaction scale's score for the EG when compared to the CG, after 16 weeks of intervention. No differences were found between baseline and after 16 weeks on the functional autonomy and life satisfaction scores for the CG. According to the results of this study, it was found that functional autonomy and life satisfaction for elderly women was improved after exposure to 16 weeks of the PM, suggesting this practice helps with healthy aging.

8.
BMC Vet Res ; 14(1): 60, 2018 Feb 27.
Artigo em Inglês | MEDLINE | ID: mdl-29486759

RESUMO

BACKGROUND: In birds there are reports of intracranial lesions but not of the clinical, computed tomographic and histopathologic features of acute intraparenchymal cerebral haemorrhage in Iberian golden eagle. CASE PRESENTATION: The following report describes a case of a 30-year-old Iberian golden eagle (Aquila chrysaetos homeyeri) with no history of trauma, presented with acute opisthotonus, left head tilt and circling, anisocoria, positional nystagmus, and ataxia. The main differential diagnosis were hypovitaminosis B or E and intracranial disease due to trauma, infection, toxins or masses. A computed tomography (CT) of the head was performed with an 8-slices scanner and evidenced a hyperdense (63-65 HU) non-enhancing homogeneous well delineated round area in the midbrain, with 6 mm in its highest diameter. The attenuation values and the non-enhancing nature of the lesion strongly suggested the diagnosis of acute intraparenchymal haemorrhage, which was histologically confirmed after necropsy. CONCLUSIONS: In birds with a central neurological dysfunction, the diagnosis of acute brain haemorrhage should be considered when the CT evidences a non-enhancing, homogeneous, well circumscribed hyperattenuated round area.


Assuntos
Doenças das Aves/diagnóstico , Hemorragia Cerebral/veterinária , Águias , Animais , Animais de Zoológico , Doenças das Aves/diagnóstico por imagem , Doenças das Aves/patologia , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Hemorragia Cerebral/diagnóstico , Hemorragia Cerebral/diagnóstico por imagem , Hemorragia Cerebral/patologia , Neuroimagem/veterinária , Tomografia Computadorizada por Raios X/veterinária
9.
J Sci Med Sport ; 21(2): 185-189, 2018 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-28595866

RESUMO

OBJECTIVES: Previous studies have indicated that only a small proportion of Portuguese adolescents attain sufficient levels of physical activity for health. However, it is unknown whether the levels of moderate to vigorous physical activity (MVPA) have changed over a considerable time period in this country. Therefore, the aims of the present study were to examine the 10-year trend changes in self-reported MVPA among adolescents and also investigate differences by sex and age. DESIGN AND METHOD: This study compared two cross-sectional cohorts of youngsters aged 12-18 years, recruited in 2006 (N=1010; 461 boys and 549 girls) and in 2016 (N=1096; 461 boys and 635 girls), using identical procedures and instruments. The outcome variable, MVPA, was assessed by using the PACE+ screening measure. RESULTS: Main results showed a considerable decline in the overall levels of physical activity between 2006 and 2016 (-10.8%), as well as a small and declining proportion of adolescents achieving the international physical activity recommendations (2006: 10.7%; 2016: 8.1%). Age and sex-specific comparisons by time periods indicated that the 13- and 14-year-old male adolescents reported lower levels of MVPA in 2016 than their counterparts in 2006, and that the MVPA decline between the subgroups of 12- and 18-year-olds was higher in girls, in both 2006 and 2016. CONCLUSIONS: This study provides evidence of a secular decrease in self-reported MVPA levels among Portuguese adolescents. These results reinforce the need for interventions and policies aimed at promoting a more active lifestyle throughout adolescence.


Assuntos
Exercício , Fidelidade a Diretrizes/estatística & dados numéricos , Adolescente , Fatores Etários , Análise de Variância , Criança , Estudos Transversais , Feminino , Humanos , Estilo de Vida , Masculino , Portugal , Autorrelato , Fatores Sexuais , Fatores de Tempo
10.
Psicol. reflex. crit ; 31: 2, 2018. tab, graf
Artigo em Inglês | LILACS, Index Psicologia - Periódicos técnico-científicos | ID: biblio-895869

RESUMO

Abstract This study had the following aims: (i) to translate the Spiritual Health and Life-Orientation Measure (SHALOM) into Brazilian Portuguese and adapt it to ensure the semantic/conceptual equivalence and content validity of the Brazilian version and (ii) to analyse the psychometric properties—reliability, convergent validity, discriminant validity and factorial validity—of the lived experience component, also called the Spiritual Well-Being Questionnaire (SWBQ), in a calibration sample and in a validation sample of Brazilian adults. The calibration sample comprised 436 subjects, 159 men and 277 women, aged between 18 and 79 years (mean age = 32.20 years; SD = 11.46); the validation study sample comprised 388 subjects, 253 women and 135 men, aged between 18 and 59 years (mean age = 30.59 years; SD = 9.44). All subjects completed a sociodemographic questionnaire, the Brazilian SWBQ and the Psychological Well-being Scale (PWBS). The results provide evidence of the reliability and factorial validity of an oblique four-factor model of a reduced 17-item version but revealed some problems with the convergent validity of the communal and personal factors (average variance extracted < .50). Nonetheless, these results provide evidence that the Brazilian version of the lived experience component of SHALOM (or SWBQb) has good psychometric properties and is a valid method of evaluating the spiritual health of Brazilian adults. Further research is required to establish the convergent and discriminant validity of this reduced version.


Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Comparação Transcultural , Saúde , Inquéritos e Questionários , Reprodutibilidade dos Testes , Espiritualidade , Psicometria , Traduções , Brasil
11.
Rev. psicol. deport ; 27(1): 67-76, 2018.
Artigo em Espanhol | IBECS | ID: ibc-172509

RESUMO

Los posibles mecanismos psicológicos explicativos de la relación entre la actividad física y el bienestar todavía no han sido suficientemente estudiados y probados en la población adolescente. Como tal, este estudio tiene como objetivo investigar los efectos de la práctica de actividad física moderada a vigorosa en las diferentes dimensiones de la salud mental (bienestar psicológico y la ansiedad social), así como los efectos de las variables mediadoras autoestima y satisfacción corporal en esas relaciones. El total de la muestra consistió de 924 adolescentes (530 chicas y 394 chicos) de edades comprendidas entre los 12 y 18 años (M = 10.15, DT = 1.78), divididos aleatoriamente en dos submuestras (calibración y validación) con características similares. El modelo de relaciones directas y indirectas se examinó a través de la técnica de path analysis. Los resultados obtenidos en las submuestras de calibración y validación confirmaron la calidad del ajuste del modelo de relaciones propuesto (CFI y GFI > .95, RMSEA < .065). La autoestima presentó un efecto mediador en la relación de la actividad física con por la mayoría de las dimensiones de bienestar y de ansiedad social, con la excepción del efecto directo entre la actividad física y los propósitos en la vida. Estos resultados se discuten a la luz de investigaciones anteriores, y se incluyen algunas de sus implicaciones prácticas (AU)


Possible psychological mechanisms underlying the association between physical activity and mental health have not been sufficiently studied and tested in adolescents. As such, this study aims to investigate the effects of moderate-to-vigorous physical activity in different mental health dimensions (psychological well-being and social anxiety), as well, as the mediation effects of self-esteem and body satisfaction between these variables. Participants were 924 adolescents (530 girls and 394 boys) aged between 12 and 18 years (M = 15.10, SD = 1.78), randomly divided into two sub-samples (calibration and validation) with similar characteristics. The direct and indirect relationships model was tested by using the path analysis technique. The obtained results in both sub-samples confirmed the adequacy of the relationships model suggested (CFI e GFI > .95, RMSEA < .065) and supported the mediation effect of self-esteem on nearly all of the well-being and social anxiety dimensions, with the exception of the direct effect of physical activity on purpose in life. These results are discussed in the context of previous findings and practical implications are provided (AU)


Assuntos
Humanos , Masculino , Feminino , Criança , Adolescente , Exercício/psicologia , Saúde Mental , Autoimagem , Imagem Corporal/psicologia , Fobia Social/psicologia , Comportamento do Adolescente/psicologia , Ansiedade
12.
Pediatr Blood Cancer ; 64(12)2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28748541

RESUMO

BACKGROUND: Childhood autoimmune hemolytic anemia (AIHA) is a rare and severe disease characterized by hemolysis and positive direct antiglobulin test (DAT). Few epidemiologic indicators are available for the pediatric population. The objective of our study was to reliably estimate the number of AIHA cases in the French Aquitaine region and the incidence of AIHA in patients under 18 years old. PROCEDURE: In this retrospective study, the capture-recapture method and log-linear model were used for the period 2000-2008 in the Aquitaine region from the following three data sources for the diagnosis of AIHA: the OBS'CEREVANCE database cohort, positive DAT collected from the regional blood bank database, and the French medico-economic information system. RESULTS: A list of 281 different patients was obtained after cross-matching the three databases; 44 AIHA cases were identified in the period 2000-2008; and the total number of cases was estimated to be 48 (95% confidence interval [CI]: 45-55). The calculated incidence of the disease was 0.81/100,000 children under 18 years old per year (95% CI 0.76-0.92). CONCLUSION: Accurate methods are required for estimating the incidence of AIHA in children. Capture-recapture analysis corrects underreporting and provides optimal completeness. This study highlights a possible under diagnosis of this potentially severe disease in various pediatric settings. AIHA incidence may now be compared with the incidences of other hematological diseases and used for clinical or research purposes.


Assuntos
Anemia Hemolítica Autoimune/epidemiologia , Adolescente , Anemia Hemolítica Autoimune/mortalidade , Criança , Pré-Escolar , Teste de Coombs , Bases de Dados Factuais , Feminino , Humanos , Incidência , Lactente , Masculino , Estudos Retrospectivos
13.
Br J Haematol ; 177(5): 751-758, 2017 06.
Artigo em Inglês | MEDLINE | ID: mdl-28444729

RESUMO

Childhood autoimmune haemolytic anaemia (AIHA) requires second-line immunosuppressive therapy in 30-50% of cases. It appears that rituximab is indicated in such circumstances. This prospective national study reports the practice, efficacy and tolerance of rituximab in children with isolated AIHA and AIHA in the setting of Evans syndrome (ES). Sixty-one children were given rituximab between 2000 and 2014. The median interval from diagnosis to rituximab was 9·9 [interquartile range (IQR) 1·6-28·5] months. Forty-six patients responded (75%) and the 6-year relapse-free survival (RFS) was 48%. Twenty patients relapsed at a median interval of 10·8 (IQR 3·9-18·7) months, rituximab allowed steroid withdrawal in 44/61 (72%) of children. In isolated AIHA, complete response and 6-year RFS were significantly higher than in ES (P < 0·05). Ten out of 61 patients were infants, seven of who responded with a 6-year RFS of 71%. Among patients without immunoglobulin substitution before rituximab, 4 are still receiving substitutions. Five patients died, including one potentially attributable to rituximab. This large observational series of childhood AIHA established the rituximab benefit-risk ratio, allowing steroid withdrawal, with 37% of long-term responders, mainly in isolated AIHA. All subgroups of patients drew benefit. Our long-term results indicate the baseline to be challenged by new treatment approaches.


Assuntos
Anemia Hemolítica Autoimune/tratamento farmacológico , Hematínicos/uso terapêutico , Rituximab/uso terapêutico , Adolescente , Transfusão de Sangue/estatística & dados numéricos , Criança , Pré-Escolar , Substituição de Medicamentos , Feminino , França , Humanos , Masculino , Estudos Prospectivos , Esteroides/uso terapêutico , Resultado do Tratamento
14.
Rev. bras. educ. fís. esp ; 30(2): 255-261, tab, graf
Artigo em Português | LILACS | ID: lil-787967

RESUMO

Resumo O estudo teve por objetivo analisar o efeito da adição do equivalente energético do lactato sanguíneo com a medida de VO2 durante a corrida em esteira horizontal (0%) e inclinada (10,5%), como forma de estimativa do custo energético da corrida. Treze corredores de meia e longa distância (idade 28,1 ± 4,2 anos; estatura 1,75 ± 0,07 m; massa corporal 65,2 ± 4,9 kg; VO2max 70,3 ± 4,9 ml·kg-1·min-1) cumpriram dois testes em esteira rolante (0% e 10,5%) que incluíram vários estágios em intensidade constante. Foram calculadas para cada atleta as regressões VO2-velocidade, bem como regressões alternativas com a adição de um equivalente energético de 3 ml O2 Eq·kg-1·mM [La-] às medições de VO2. Não se verificou interação significativa entre a adição do equivalente do lactato e a inclinação da esteira. A ANOVA indicou um efeito significativo da adição do equivalente do lactato na inclinação da reta de regressão e na estimativa do custo energético. Os tamanhos do efeito obtidos indicam que este efeito é mais forte na corrida horizontal. Estes resultados sugerem que em testes laboratoriais com corredores treinados se deverá considerar a adição dos valores de VO2 com os equivalentes energéticos do lactato.(AU)


Abstract This study aimed to analyze the influence of adding blood lactate ([La-]) energy equivalent (Eq) to the VO2 measurements during running exercise at different grades (0% and 10.5%) in order to estimate energy cost of running. Thirteen male highly-trained middle- and long-distance runners (age 28.1 ± 4.2 years; stature 1.75 ± 0.07 m; body mass 65.2 ± 4.9 kg; and maximal oxygen uptake 70.3 ± 4.9 ml·kg-1·min-1) volunteered after medical approval and performed two treadmill tests (0% and 10.5% grade) which included several bouts at a constant speed. Individual VO2-speed regressions were determined for each subject and alternative regressions were established by adding an energy equivalent of 3 ml O2 Eq·kg-1·mM [La-] to the mean VO2 values. No significant interaction between [La-] O2 equivalent inclusion and grade running was found. Results of within-subjects ANOVAs indicated a significant effect of [La-] inclusion in the regression slope and in the estimated energy cost of running at both level and grade running. However, the obtained effect sizes suggest that this effect is considerably higher at level compared with grade running. These findings indicate that the inclusion of [La-] measurements in VO2-intensity regression estimates at sub maximal running should be considered when testing highly trained runners on the treadmill.(AU)


Assuntos
Humanos , Masculino , Adulto , Metabolismo Energético , Consumo de Oxigênio , Corrida
15.
Front Pediatr ; 3: 79, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26484337

RESUMO

Evans syndrome (ES) is a rare autoimmune disorder whose long-term outcome is not well known. In France, a collaborative pediatric network set up via the National Rare Disease Plan now provides comprehensive clinical data in children with this disease. Patients aged less than 18 years at the initial presentation of autoimmune cytopenia have been prospectively included into a national observational cohort since 2004. The definition of ES was restricted to the simultaneous or sequential association of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP). Cases were deemed secondary if associated with a primitive immunodeficiency or systemic lupus erythematosus. In December 2014, we analyzed the data pertaining to 156 children from 26 centers with ES whose diagnosis was made between 1981 and 2014. Median age (range) at the onset of cytopenia was 5.4 years (0.2-17.2). In 85 sequential cases, the time lapse between the first episodes of AIHA and ITP was 2.4 years (0.1-16.3). The follow-up period as from ES diagnosis was 6.5 years (0.1-28.8). ES was secondary, revealing another underlying disease, in 10% of cases; various associated immune manifestations (mainly lymphoproliferation, other autoimmune diseases, and hypogammaglobulinemia) were observed in 60% of cases; and ES remained primary in 30% of cases. Five-year ITP and AIHA relapse-free survival were 25 and 61%, respectively. Overall, 69% of children required one or more second-line immune treatments, and 15 patients (10%) died at the age of 14.3 years (1.7-28.1). To our knowledge, this is the first consistent long-term clinical description of this rare syndrome. It underscores the high rate of associated immune manifestations and the burden of long-term complications and treatment toxicity. Future challenges include (1) the identification of the underlying genetic defects inducing immune dysregulation and (2) the need to better characterize patient subgroups and second-line treatment strategies.

16.
Cuad. psicol. deporte ; 15(3): 135-144, oct. 2015. tab
Artigo em Inglês | IBECS | ID: ibc-147215

RESUMO

This study aims to provide the translation and the cultural adaptation of the Physical Education State Anxiety Scale (PESAS) in Portuguese, assess its psychometric properties, and investigate the effects of gender, age and extracurricular physical activity, on the different dimensions of PESAS (somatic anxiety, cognitive processes and worry). The sample comprised 496 students (221 male, 275 female) aged between 13 and 21 years (M =15.71, SD = 1.34). Confirmatory factor analysis confirmed the original factor structure of the scale. There was appropriate factorial validity of the 3-factor structure (somatic anxiety, cognitive processes and worry). Female students reported higher levels in the dimensions of anxiety, in comparison to boys. Students who practiced extracurricular physical activity revealed lower levels of cognitive processes and worries/fears. Age negatively correlated with cognitive processes and somatic anxiety. These findings support the use of the Portuguese version of PESAS and provide additional information about state anxiety in school physical education (AU)


El objetivo de este estudio fue la traducción y adaptación cultural de la escala PESAS en portugués, evaluar sus propiedades psicométricas, e investigar los efectos del género, la edad y la actividad física extracurricular, en las diferentes dimensiones de PESAS (ansiedad somática, procesos cognitivos y preocupación). La muestra fue de 496 estudiantes (221 varones, 275 mujeres) con edades comprendidas entre 13 y 21 años (M = 15,71, SD = 1,34). El análisis factorial confirmatorio confirmó una adecuada validez factorial de la estructura multidimensional original de tres factores (procesos cognitivos, ansiedad somática y preocupaciones/miedos). Las estudiantes reportaron niveles más altos en las dimensiones de la ansiedad, en comparación con los varones. Los practicantes de actividad física extracurricular revelaron niveles más bajos de los procesos cognitivos y preocupaciones/ miedos. La edad correlacionó negativamente con los procesos cognitivos y la ansiedad somática. Estos resultados apoyan el uso de la versión portuguesa de la escala PESAS y proporcionan información adicional sobre el estado de ansiedad en la educación física escolar (AU)


O presente estudo tem como objetivos a tradução e adaptação transcultural do PESAS para Português, assim como, verificar as suas propriedades psicométricas e investigar o efeito do sexo, idade e atividade física extraescolar nas dimensões do PESAS (ansiedade somática, processos cognitivos e preocupações/receios). A amostra foi constituída por 496 alunos (221 rapazes e 275 raparigas) com idades compreendidas entre os 13 e os 21 anos (M =15,71, DP = 1,34). A análise fatorial confirmatória confirmou uma apropriada validade fatorial para a estrutura multidimensional original de 3 fatores (processos cognitivos, ansiedade somática e preocupações/ receios). As alunas reportaram níveis significativamente superiores nas dimensões de ansiedade em comparação aos rapazes. Os alunos praticantes de atividade física extraescolar revelaram menores níveis de processos cognitivos e preocupações/receios. A idade associou-se negativamente com os processos cognitivos e a ansiedade somática. Estes resultados suportam a utilização da versão portuguesa do PESAS e revelam novas evidências sobre a ansiedade-estado no contexto da educação física escolar (AU)


Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto Jovem , Escala de Ansiedade Frente a Teste/estatística & dados numéricos , Psicometria/instrumentação , Educação Física e Treinamento , Distribuição por Idade e Sexo , Atividade Motora , Estudantes/psicologia
17.
Psicol. reflex. crit ; 28(1): 136-146, Jan-Mar/2015. tab
Artigo em Português | LILACS | ID: lil-736139

RESUMO

Este estudo tem como objetivos proceder à tradução e adaptação transcultural (equivalência semântica/conceitual e validade de conteúdo) do Test of Performance Strategies (TOPS), assim como, verificar as suas propriedades psicométricas (confiabilidade e validade fatorial) em atletas brasileiros. A amostra foi constituída por 413 atletas (295 homens e 118 mulheres) com idades compreendidas entre os 16 e 69 anos (M = 25,87, DP = 7,82). As propriedades psicométricas foram avaliadas através do alpha de Cronbach e da análise factorial confirmatória (AFC). Na análise da confiabilidade dos fatores, foram obtidos valores entre 0,41 e 0,82. Os resultados das AFC dos modelos multidimensionais revelaram índices de adequação insatisfatórios tanto para a situação de treino (GFI = 0,832; CFI = 0,761 e RMSEA = 0,058), como de competição (GFI = 0,855; CFI = 0,843 e RMSEA = 0,054). Quando analisadas as dimensões individualmente, verificaram-se problemas de validade fatorial (GFI e CFI < 0,90; RMSEA > 0,06) em quatro dos dezesseis fatores (ativação em situação de treino e ativação, controle emocional e pensamentos negativos em situação de competição). Em conclusão, os resultados providenciam evidências relativamente às propriedades psicométricas do TOPS em atletas brasileiros, sugerindo-se, contudo, precaução na interpretação dos escores de certas dimensões.


This study aimed to provide the translation and cultural adaptation (semantic/conceptual equivalence and content validity) of the Test of Performance Strategies (TOPS) as well as to investigate its psychometric properties (reliability and factorial validity) in Brazilian athletes. The sample consisted of 413 athletes (295 men and 118 women) aged between 16 and 69 years (M = 25.87, SD = 7.82). The psychometric properties were assessed by means of reliability (Cronbach's alpha) and confirmatory factor analysis (CFA). The internal consistency of the factors ranged between .41 and .82. The results of the CFA of the multidimensional models showed unsatisfactory levels of adequacy for both training (GFI = .832, CFI = .761 and RMSEA = .058) and competition situations (GFI = .855, CFI = .843 and RMSEA = .054). At the individual level subscale, four of the sixteen subscales showed unacceptable fit (GFI and CFI < .90; RMSEA > .06) (training activation and activation, emotional control and negative thinking in competition situations). In conclusion, the results provide evidence of support for the psychometric properties of the TOPS in Brazilian athletes, although some caution is warranted particularly regarding the interpretation of some scales' scores.


Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto Jovem , Pessoa de Meia-Idade , Atletas/psicologia , Desempenho Atlético/psicologia , Psicometria , Reprodutibilidade dos Testes , Traduções , Brasil
18.
Psicol. reflex. crit ; 28(1): 136-146, Jan-Mar/2015. tab
Artigo em Português | Index Psicologia - Periódicos técnico-científicos | ID: psi-68145

RESUMO

Este estudo tem como objetivos proceder à tradução e adaptação transcultural (equivalência semântica/conceitual e validade de conteúdo) do Test of Performance Strategies (TOPS), assim como, verificar as suas propriedades psicométricas (confiabilidade e validade fatorial) em atletas brasileiros. A amostra foi constituída por 413 atletas (295 homens e 118 mulheres) com idades compreendidas entre os 16 e 69 anos (M = 25,87, DP = 7,82). As propriedades psicométricas foram avaliadas através do alpha de Cronbach e da análise factorial confirmatória (AFC). Na análise da confiabilidade dos fatores, foram obtidos valores entre 0,41 e 0,82. Os resultados das AFC dos modelos multidimensionais revelaram índices de adequação insatisfatórios tanto para a situação de treino (GFI = 0,832; CFI = 0,761 e RMSEA = 0,058), como de competição (GFI = 0,855; CFI = 0,843 e RMSEA = 0,054). Quando analisadas as dimensões individualmente, verificaram-se problemas de validade fatorial (GFI e CFI < 0,90; RMSEA > 0,06) em quatro dos dezesseis fatores (ativação em situação de treino e ativação, controle emocional e pensamentos negativos em situação de competição). Em conclusão, os resultados providenciam evidências relativamente às propriedades psicométricas do TOPS em atletas brasileiros, sugerindo-se, contudo, precaução na interpretação dos escores de certas dimensões.(AU)


This study aimed to provide the translation and cultural adaptation (semantic/conceptual equivalence and content validity) of the Test of Performance Strategies (TOPS) as well as to investigate its psychometric properties (reliability and factorial validity) in Brazilian athletes. The sample consisted of 413 athletes (295 men and 118 women) aged between 16 and 69 years (M = 25.87, SD = 7.82). The psychometric properties were assessed by means of reliability (Cronbach's alpha) and confirmatory factor analysis (CFA). The internal consistency of the factors ranged between .41 and .82. The results of the CFA of the multidimensional models showed unsatisfactory levels of adequacy for both training (GFI = .832, CFI = .761 and RMSEA = .058) and competition situations (GFI = .855, CFI = .843 and RMSEA = .054). At the individual level subscale, four of the sixteen subscales showed unacceptable fit (GFI and CFI < .90; RMSEA > .06) (training activation and activation, emotional control and negative thinking in competition situations). In conclusion, the results provide evidence of support for the psychometric properties of the TOPS in Brazilian athletes, although some caution is warranted particularly regarding the interpretation of some scales' scores.(AU)


Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Atletas/psicologia , Traduções , Psicometria , Reprodutibilidade dos Testes , Desempenho Atlético/psicologia , Brasil , Reprodutibilidade dos Testes
19.
J Strength Cond Res ; 29(1): 74-9, 2015 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-25028991

RESUMO

Concurrent training (CT) has been widely used in fitness centers to simultaneously optimize cardiovascular and neuromuscular fitness, and induce a high-energy expenditure. Therefore, the aim of this study was to compare the acute effects of 2 different orders of CT on hormonal responses in concurrently trained men. Fourteen men (mean ± SD: 24.7 ± 5.1 years) were randomly divided into 2 groups: endurance training followed by strength (ES, n = 7) and strength training followed by endurance (SE, n = 7). Serum concentrations of testosterone, cortisol, growth hormone, and IGF-1 binding protein 3 (IGFBP-3) were measured before and after both training orders. A significant interaction between exercise order and time was only found in the IGFBP-3 levels (p = 0.022). The testosterone and IGFBP-3 concentrations significantly increased in the ES group after the exercise trainings (57.7 ± 35.1%, p = 0.013 and 17.0 ± 15.5%, p = 0.032, respectively) but did not change significantly in the SE group (15.5 ± 36.6%, p = 0.527 and -4.2 ± 13.9%, p = 0.421, respectively). Conversely, cortisol and growth hormone concentrations significantly increased in both ES (169.2 ± 191.0%, p = 0.021 and 13,296.8 ± 13,009.5%, p = 0.013, respectively) and SE (92.2 ± 81.5%, p = 0.017 and 12,346.2 ± 9714.1%, p = 0.001, respectively) groups compared with baseline values. No significant correlations were found between the changes in the hormonal concentrations. In conclusion, these results suggest that immediately postexercise testosterone and IGFPB-3 responses are significantly increased only after the ES order. Therefore, an ES training order should be prescribed if the main focus of the training intervention is to induce an acute postexercise anabolic environment.


Assuntos
Hormônio do Crescimento Humano/sangue , Hidrocortisona/sangue , Proteína 3 de Ligação a Fator de Crescimento Semelhante à Insulina/sangue , Resistência Física/fisiologia , Treinamento de Resistência/métodos , Testosterona/sangue , Adulto , Biomarcadores/metabolismo , Metabolismo Energético , Humanos , Masculino
20.
Rev. psicol. deport ; 23(2): 445-449, jul.-dic. 2014.
Artigo em Inglês | IBECS | ID: ibc-125645

RESUMO

Los estudios epidemiológicos han demostrado que, cada año, un número considerable de atletas y de practicantes de actividad física sufre una lesión causante de discapacidad y de otras repercusiones negativas para el bienestar físico, psicológico y social. Además, las investigaciones actuales revelan que la prevalencia de las lesiones asociadas a los deportes varía según el género, la edad, el deporte, el nivel competitivo, la posición en el juego, entre otros. Aunque los factores físicos constituyen las principales causas de las lesiones más comunes, varios estudios han sugerido que los factores psicológicos y sociales también ejercen un efecto significativo en la prevención y rehabilitación de las lesiones deportivas. Entre los factores psicosociales estudiados, el apoyo o soporte social percibido por el propio deportista se ha destacado como un importante mecanismo para controlar el distrés emocional y afrontar mejor las dificultades inherentes al proceso de recuperación de las lesiones deportivas. Sin embargo, la investigación también indica que las fuentes de apoyo social tienden a no satisfacer las expectativas y necesidades de soporte social de los atletas, siendo insuficientes en determinadas fases de la rehabilitación. Igualmente, algunos estudios han sugerido que el apoyo social puede inducir efectos perjudiciales en determinadas circunstancias. Por lo tanto, los entrenadores y profesionales de la salud (médicos, fisioterapeutas, psicólogos, etc.) deben tener conocimiento de estos factores influyentes y implementar intervenciones desde un punto de vista más integral con el fin de promover la recuperación de los atletas y mejorar su bienestar (AU)


Epidemiologically-based evidence has demonstrated that a substantial number of athletes and exercisers are injured each year, which can result in physical disability as well as other negative physical, social and psychological consequences. Accumulated research has indicated that the prevalence of sport and physical activity-related injuries varies based on gender, age group, type of sport, level of participation, and role on the team, among other considerations. Although physical causes are the primary contributors to injury, a considerable number of studies have suggested that psychological and social factors also have importance in injury prevention and rehabilitation. Among the psychosocial factors investigated, social support has emerged as a significant buffering and coping resource in the recovery process from athletic injuries. However, research has also indicated that sources of social support tend to be less frequently available to athletes during some stages of rehabilitation and do not necessarily meet the athletes’ expectations and needs. Moreover, some studies have suggested that social support may have detrimental effects under certain circumstances. As such, coaches, athletic trainers and health care professionals should be aware of these complex forms of influence and develop and implement injury rehabilitation processes that are based on a holistic approach in order to promote the athletes’ recovery and well-being (AU)


Assuntos
Humanos , Traumatismos em Atletas/psicologia , Apoio Social , Esportes/psicologia , Resultado do Tratamento , Traumatismos em Atletas/reabilitação , Estatísticas de Sequelas e Incapacidade
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