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2.
Haematologica ; 2021 Jan 14.
Artigo em Inglês | MEDLINE | ID: mdl-33440924

RESUMO

Pediatric-onset Evans syndrome (pES) is defined by both immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AIHA) before the age of 18 years. There have been no comprehensive long-term studies of this rare disease, which can be associated to various immunopathological manifestations (IMs). We report outcomes of the 151 patients with pES and more than 5 years of follow-up from the nationwide French prospective OBS'CEREVANCE cohort. Median age at final follow-up was 18.5 (6.8-50.0) years and the median follow-up period was 11.3 (5.1-38.0) years. At 10 years, ITP and AIHA were in sustained complete remission in 54.5% and 78.4% of patients, respectively. The frequency and number of clinical and biological IMs increased with age: at 20 years old, 74% had at least one clinical cIM. A wide range of cIMs occurred, mainly lymphoproliferation, dermatological, gastrointestinal/hepatic and pneumological IMs. The number of cIMs was associated with a subsequent increase in the number of second-line treatments received (other than steroids and immunoglobulins; hazard ratio, 1.4; 95% confidence interval, 1.15-1.60; p = 0.0002, Cox proportional hazards method). Survival at 15 years after diagnosis was 84%. Death occurred at a median age of 18 (1.7-31.5) years, and the most frequent cause was infection. The number of second-line treatments and severe/recurrent infections were independently associated with mortality. In conclusion, longterm outcomes of pES showed remission of cytopenias but frequent IMs linked to high secondline treatment burden. Mortality was associated to drugs and/or underlying immunodeficiencies, and adolescents-young adults are a high-risk subgroup.

3.
J Pediatr ; 2020 Dec 17.
Artigo em Inglês | MEDLINE | ID: mdl-33340549

RESUMO

OBJECTIVE: To describe 4 subgroups of pediatric patients treated with splenectomy, hydroxychloroquine, azathioprine, or rituximab as the first-option, second-line treatment for chronic immune thrombocytopenia. STUDY DESIGN: Selection of patients with chronic immune thrombocytopenia from the French national prospective cohort of pediatric autoimmune cytopenia OBS'CEREVANCE and VIGICAIRE study, treated by splenectomy, hydroxychloroquine, azathioprine, or rituximab as a first second-line treatment. RESULTS: For 137 patients, treated between 1989 and 2016, the median follow-up after diagnosis and after treatment initiation was 8.5 (2.8-26.4) years and 4.7 (1.1-25.1) years, respectively. Median age at diagnosis and at initiation of treatment were 9 (0.7; 16) and 12 (2; 18.1) years, respectively without significant difference between subgroups. For the whole cohort, 24-month event-free survival was 62% (95% CI 55; 71). It was 85% (95% CI 77; 95) for the 56 patients treated with splenectomy, 60% (95% CI 44; 84) for the 23 patients treated with rituximab, 46% (95% CI 30; 71) for the 24 patients treated with azathioprine, and 37% (95% CI 24; 59) for the 34 patients treated with hydroxychloroquine (log-rank P < .0001). For the splenectomy subgroup, being older than 10 years at splenectomy tended to improve event-free survival (P = .05). Female teenagers with antinuclear antibody positivity benefited from hydroxychloroquine therapy. CONCLUSIONS: This national study, limiting pitfalls in the analysis of the effects of second-line therapies, showed that splenectomy remains the treatment associated with the better response at 24 months.

5.
Acta Neurol Scand ; 142(6): 563-573, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-32691850

RESUMO

OBJECTIVES: To assess the effectiveness and safety/tolerability of eslicarbazepine acetate (ESL) in patients included in the Euro-Esli study who had focal seizures associated with post-stroke epilepsy (PSE). MATERIALS AND METHODS: Euro-Esli was a pooled analysis of 14 European clinical practice studies. Effectiveness assessments (evaluated after 3, 6 and 12 months of ESL treatment and at final follow-up ["last visit"]) included rates of response (≥50% seizure frequency reduction), seizure freedom (no seizures since at least the prior visit) and retention. Safety/tolerability was assessed throughout ESL treatment by evaluating adverse events (AEs) and discontinuation due to AEs. A post hoc analysis was conducted of patients with PSE versus patients without PSE ("non-PSE"). RESULTS: Of 1656 patients included in the analysis, 76 (4.6%) had PSE and 1580 (95.4%) had non-PSE. Compared with non-PSE patients, PSE patients were significantly older, had significantly shorter epilepsy duration, significantly lower total baseline seizure frequency, and were treated with significantly fewer prior and concomitant antiepileptic drugs (P < .001 for all). At the last visit, the responder rate was significantly higher in PSE versus non-PSE patients (72.9% vs 60.6%; P = .040), as was the seizure freedom rate (48.6% vs 31.7%; P = .003). After 12 months, retention was significantly higher in PSE versus non-PSE patients (87.8% vs 77.4%; P = .035). The incidence of AEs was similar for PSE versus non-PSE patients (36.0% vs 35.8%; P = .966). CONCLUSIONS: These findings suggest that ESL may be an effective and well-tolerated treatment option for patients with focal seizures due to PSE.

6.
Br J Haematol ; 189(5): 931-942, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32130726

RESUMO

Childhood chronic immune thrombocytopenic purpura (cITP) is a rare disease. In severe cases, there is no evidence for the optimal therapeutic strategy. Our aim was to describe the real-life management of non-selected children with cITP at diagnosis. Since 2004, patients less than 18 years old with cITP have been enrolled in the national prospective cohort, OBS'CEREVANCE. From 1990 to 2014, in 29 centres, 392 children were diagnosed with cITP. With a median follow-up of six years (2·0-25), 45% did not need second-line therapy, and 55% (n = 217) received one or more second lines, mainly splenectomy (n = 108), hydroxychloroquine (n = 61), rituximab (n = 61) or azathioprine (n = 40). The overall five-year further second-line treatment-free survival was 56% [95% CI 49·5-64.1]. The use of splenectomy significantly decreased over time. Hydroxychloroquine was administered to children with positive antinuclear antibodies, more frequently older and girls, and reached 55% efficacy. None of the patients died. Ten years after the initial diagnosis, 55% of the 56 followed children had achieved complete remission. Children with cITP do not need second-line treatments in 45% of cases. Basing the treatment decision on the pathophysiological pathways is challenging, as illustrated by ITP patients with positive antinuclear antibodies treated with hydroxychloroquine.

7.
Genet Med ; 22(1): 181-188, 2020 01.
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.

8.
Referência ; serIV(23): 49-58, dez. 2019. tab
Artigo em Português | BDENF - Enfermagem | ID: biblio-1098631

RESUMO

Enquadramento: A cirurgia abdominal alta está associada a alta incidência de complicações pulmonares. A técnica breath stacking (BS) perspetiva-se como recurso com potencial terapêutico para a enfermagem de reabilitação. Objetivo: Avaliar a eficácia da técnica BS na melhoria da função respiratória. Metodologia: Estudo quasi-experimental com 36 mulheres submetidas a cirurgia bariátrica, distribuídas pelos grupos de controlo (GC) e intervenção (GI). Foram avaliadas no pré e pós-operatório as medidas capacidade vital forçada (CVF), volume expiratório forçado no primeiro segundo (VEF1), pressão inspiratória máxima (PIm), pressão expiratória máxima (PEm), saturação periférica de oxigénio (SpO2) e frequência respiratória (FR). Foi aplicada a técnica BS no GI no pré e pós-operatório. Recorreu-se à análise estatística descritiva e inferencial. Resultados: Observaram-se diferenças estatisticamente significativas pós-operatórias, entre o GC e o GI, na CVF ( -20,29 vs. -13,60), VEF1 (-23,05 vs. -13,38), PIm (-22,96 vs. -14,93), PEm (-14,10 vs -10,32) e FR (12,29 vs 6,45). Conclusão: A técnica de BS permitiu melhorar a função respiratória do GI e reverter as alterações previsíveis no pós-operatório de cirurgia bariátrica.


Background: Upper abdominal surgery is associated with a high incidence of pulmonary complications. The breath-stacking (BS) technique is considered a resource with therapeutic potential for rehabilitation nursing. Objective: To assess the effectiveness of the BS technique in improving the respiratory function. Methodology: Quasi-experimental study with 36 women undergoing bariatric surgery, distributed into a control group (CG) and an intervention group (IG). The forced vital capacity (FVC), the forced expiratory volume in one second (FEV1), the maximal inspiratory pressure (MIP), the maximal expiratory pressure (MEP), the peripheral oxygen saturation (SpO2), and the respiratory rate (RR) were evaluated in the pre and postoperative periods. The BS technique was applied to the IG in the pre and postoperative periods. Both descriptive and inferential statistics were used. Results: Statistically significant postoperative differences were observed between the CG and the IG in FVC (-20.29 vs. -13.60), FEV1 (-23.05 vs. -13.38), MIP (-22.96 vs. -14.93), MEP (-14.10 vs. -10.32), and RF (12.29 vs. 6.45). Conclusion: The BS technique improved the respiratory function of the IG and reversed the predictable changes in the postoperative period of bariatric surgery.


Marco contextual: La cirugía abdominal alta se asocia con una elevada incidencia de complicaciones pulmonares. La técnica de breath stacking (BS) se considera un recurso con potencial terapéutico para la enfermería de rehabilitación. Objetivo: Evaluar la efectividad de la técnica de BS para mejorar la función respiratoria. Metodología: Estudio cuasi-experimental con 36 mujeres sometidas a cirugía bariátrica, distribuidas entre el grupo de control (GC) y el de intervención (GI). Las mediciones de la capacidad vital forzada (CVF), el volumen espiratorio forzado en el primer segundo (VEF1), la presión inspiratoria máxima (PIm), la presión espiratoria máxima (PEm), la saturación periférica de oxigeno (SpO2) y la frecuencia respiratoria (FR) se evaluaron GI que en el GC antes y después de la cirugía. La técnica BS se aplicó en el GI antes y después de la cirugía. Se utilizó un análisis estadístico descriptivo e inferencial. Resultados: Se observaron diferencias posoperatorias estadísticamente significativas entre el GC y el GI en la CVF (-20,29 frente a -13,60), VEF1 (-23,05 frente a -13,38), PIm (-22,96 frente a -14,93), PEm (-14,10 frente a -10,32) y FR (12,29 frente a 6,45). Conclusión: La técnica BS permitió mejorar la función respiratoria del GI y revertir los cambios predecibles en el posoperatorio de cirugía bariátrica.


Assuntos
Sistema Respiratório , Mulheres , Ventilação Pulmonar , Enfermagem em Reabilitação , Cirurgia Bariátrica , Obesidade
9.
Blood ; 134(1): 9-21, 2019 07 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.


Assuntos
Anemia Hemolítica Autoimune/genética , Anemia Hemolítica Autoimune/imunologia , Trombocitopenia/genética , Trombocitopenia/imunologia , Adolescente , Adulto , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Lactente , Masculino , Mutação , Adulto Jovem
10.
J Clin Immunol ; 39(2): 171-181, 2019 02.
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.

12.
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.

13.
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.


Assuntos
Técnicas de Exercício e de Movimento/métodos , Técnicas de Exercício e de Movimento/psicologia , Força Muscular/fisiologia , Músculo Esquelético/fisiologia , Satisfação Pessoal , Idoso , Brasil , Aptidão Cardiorrespiratória/fisiologia , Feminino , Humanos , Extremidade Inferior/fisiologia , Pessoa de Meia-Idade , Equilíbrio Postural/fisiologia , Amplitude de Movimento Articular/fisiologia , Extremidade Superior/fisiologia
15.
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
16.
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 Físico , 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
17.
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
18.
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 Físico/psicologia , Saúde Mental , Autoimagem , Imagem Corporal/psicologia , Fobia Social/psicologia , Comportamento do Adolescente/psicologia , Ansiedade
19.
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
20.
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
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