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1.
Am J Hematol ; 2024 Apr 23.
Artículo en Inglés | MEDLINE | ID: mdl-38651646

RESUMEN

Refractory chronic immune thrombocytopenia (r-cITP) is one of the most challenging situations in chronic immune thrombocytopenia (cITP). Pediatric r-cITP is inconsistently defined in literature, contributing to the scarcity of data. Moreover, no evidence is available to guide the choice of treatment. We compared seven definitions of r-cITP including five pediatric definitions in 886 patients with cITP (median [min-max] follow-up 5.3 [1.0-29.3] years). The pediatric definitions identified overlapping groups of various sizes (4%-20%) but with similar characteristics (higher proportion of immunopathological manifestations [IM] and systemic lupus erythematosus [SLE]), suggesting that they adequately captured the population of interest. Based on the 79 patients with r-cITP (median follow-up 3.1 [0-18.2] years) according to the CEREVANCE definition (≥3 second-line treatments), we showed that r-cITP occurred at a rate of 1.15% new patients per year and did not plateau over time. In multivariate analysis, older age was associated with r-cITP. One patient (1%) experienced two grade five bleeding events after meeting r-cITP criteria and while not receiving second-line treatment. The cumulative incidence of continuous complete remission (CCR) at 2 years after r-cITP diagnosis was 9%. In this analysis, splenectomy was associated with a higher cumulative incidence of CCR (hazard ratio: 5.43, 95% confidence interval: 1.48-19.84, p = 7.8 × 10-4). In sum, children with cITP may be diagnosed with r-cITP at any time point of the follow-up and are at increased risk of IM and SLE. Second-line treatments seem to be effective for preventing grade 5 bleeding. Splenectomy may be considered to achieve CCR.

2.
Br J Haematol ; 204(5): 1899-1907, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38432067

RESUMEN

Kabuki syndrome (KS) is now listed in the Human Inborn Errors of Immunity (IEI) Classification. It is a rare disease caused by KMT2D and KDM6A variants, dominated by intellectual disability and characteristic facial features. Recurrently, pathogenic variants are identified in those genes in patients examined for autoimmune cytopenia (AIC), but interpretation remains challenging. This study aims to describe the genetic diagnosis and the clinical management of patients with paediatric-onset AIC and KS. Among 11 patients with AIC and KS, all had chronic immune thrombocytopenic purpura, and seven had Evans syndrome. All had other associated immunopathological manifestations, mainly symptomatic hypogammaglobinaemia. They had a median of 8 (5-10) KS-associated manifestations. Pathogenic variants were detected in KMT2D gene without clustering, during the immunological work-up of AIC in three cases, and the clinical strategy to validate them is emphasized. Eight patients received second-line treatments, mainly rituximab and mycophenolate mofetil. With a median follow-up of 17 (2-31) years, 8/10 alive patients still needed treatment for AIC. First-line paediatricians should be able to recognize and confirm KS in children with ITP or multiple AIC, to provide early appropriate clinical management and specific long-term follow-up. The epigenetic immune dysregulation in KS opens exciting new perspectives.


Asunto(s)
Anomalías Múltiples , Proteínas de Unión al ADN , Cara , Enfermedades Hematológicas , Histona Demetilasas , Proteínas de Neoplasias , Enfermedades Vestibulares , Humanos , Enfermedades Vestibulares/genética , Enfermedades Vestibulares/diagnóstico , Niño , Cara/anomalías , Femenino , Masculino , Preescolar , Anomalías Múltiples/genética , Adolescente , Histona Demetilasas/genética , Proteínas de Neoplasias/genética , Enfermedades Hematológicas/genética , Proteínas de Unión al ADN/genética , Púrpura Trombocitopénica Idiopática/genética , Púrpura Trombocitopénica Idiopática/terapia , Púrpura Trombocitopénica Idiopática/diagnóstico , Lactante , Trombocitopenia/genética , Trombocitopenia/diagnóstico , Trombocitopenia/etiología , Trombocitopenia/terapia , Anemia Hemolítica Autoinmune/genética , Anemia Hemolítica Autoinmune/diagnóstico , Anemia Hemolítica Autoinmune/terapia , Enfermedades Autoinmunes/genética , Enfermedades Autoinmunes/diagnóstico , Rituximab/uso terapéutico , Mutación , Citopenia
3.
BMC Public Health ; 24(1): 582, 2024 Feb 23.
Artículo en Inglés | MEDLINE | ID: mdl-38395826

RESUMEN

BACKGROUND: The vast majority of people have experienced the problem of back pain at least once in their lives for a variety of reasons. It is well known that regular physical activity at a moderate level has a number of health benefits regardless of age or gender with positive effects on the musculoskeletal, cardiovascular, respiratory or nervous systems improving fitness levels. During the pandemic, Poland experienced one of the longest periods of school lockdown in Europe. In Portugal, this period was considerably shorter. The aim of this study was to determine the level of physical activity and back pain as well as relationship between physical activity and back pain in a group of Polish and Portuguese students in the following year the COVID-19 pandemic. METHODS: The study was conducted via the Internet among students from Poland and Portugal (398 respondents - 229 from Poland and 169 from Portugal) in October 2022. In the pilot study, the International Physical Activity Questionnaire and The Oswestry Disability Index and Neck Disability Index questionnaires were used to assess the level of back pain. RESULTS: There are no statistically significant differences in the level of physical activity and pain complaints of respondents from the two countries. At least half of the students do not report any pain complaints (median in some groups being 0). In the Portuguese men, there is a correlation between the level of physical activity and the Neck Disability Index score (p = 0.0304). CONCLUSIONS: The following year, after the pandemic COVID-19 was declared, the prevalence of back pain among students in Poland and Portugal is still significant.


Asunto(s)
COVID-19 , Pandemias , Masculino , Humanos , Portugal/epidemiología , Proyectos Piloto , COVID-19/epidemiología , Control de Enfermedades Transmisibles , Dolor de Espalda/epidemiología , Ejercicio Físico , Encuestas y Cuestionarios , Polonia/epidemiología
4.
Blood ; 143(16): 1576-1585, 2024 Apr 18.
Artículo en Inglés | MEDLINE | ID: mdl-38227934

RESUMEN

ABSTRACT: Autoimmune cytopenia (AIC) in children may be associated with positive antinuclear antibodies (ANA) and may progress to systemic lupus erythematosus (SLE). We evaluated the risk of progression to SLE of childhood-onset ANA-associated AIC. In the French national prospective OBS'CEREVANCE cohort, the long-term outcome of children with ANA-associated AIC (ANA titer ≥1/160) and a subgroup of children who developed SLE were described. ANA were positive in 355 of 1803 (20%) children with AIC. With a median follow-up of 5.8 (range, 0.1-29.6) years, 79 of 355 (22%) patients developed SLE at a median age of 14.5 (1.1-21.4) years; 20% of chronic immune thrombocytopenic purpura, 19% of autoimmune hemolytic anemia, and 45% of Evans syndrome. None of the patients with ANA-negative test developed SLE. Severe manifestations of SLE were observed in 21 patients, and 2 patients died. In multivariate analysis including patients with positive ANA within the first 3 months after AIC diagnosis, age >10 years at AIC diagnosis (relative risk [RR], 3.67; 95% confidence interval [CI], 1.18-11.4; P = .024) and ANA titer >1/160 (RR, 5.28; 95% CI, 1.20-23.17; P = .027) were associated with the occurrence of SLE after AIC diagnosis. ANA-associated AIC is a risk factor for progression to SLE, especially in children with an initial ANA titer >1/160 and an age >10 years at AIC diagnosis. ANA screening should be recommended in children with AIC, and patients with ANA should be monitored long-term for SLE, with special attention to the transition period. This trial was registered at www.ClinicalTrials.gov as #NCT05937828.


Asunto(s)
Citopenia , Lupus Eritematoso Sistémico , Adolescente , Adulto , Niño , Humanos , Adulto Joven , Anticuerpos Antinucleares , Lupus Eritematoso Sistémico/diagnóstico , Estudios Prospectivos , Factores de Riesgo
5.
Nurs Rep ; 13(4): 1342-1353, 2023 Sep 28.
Artículo en Inglés | MEDLINE | ID: mdl-37873820

RESUMEN

Nursing education and formation is still heterogenous in spite of The Bologna Declaration in 1999. Apart from the existence of basic curriculum standards, universities have flexibility regarding its design. The objective of this study is to provide an overview of contemporary nursing education and contrast it with the legal frameworks in place in four Southern European countries: Portugal, Spain, Italy, and Greece. A scoping review was conducted in order to revise and obtain an up-to-date review of current nursing education and quality. Then, a content evaluation of the legal public framework was conducted. A total of nine articles were included in the review. Data analysis evoked three main themes from the results: nursing education heterogeneity owing to ECTS increased from 180 to 240 for the diversity of clinical practice hours; the nursing framework lacked a definition; and the evolution of nursing education, alongside quality improvement and more accurate guidelines were required. Regarding their legal framework, the main directives and legislation standards were assessed and compared with the current curriculum. To conclude, nursing heterogeneity education evokes competence inequality among students and future professionals as larger curricular programs (240 ECTS) offer more clinical practice. Nursing education uniformity could enhance international mobility and promote knowledge exchange and nursing competence framework definitions. These are facts that certainly bring nursing empowerment. This study was not registered.

6.
Healthcare (Basel) ; 11(18)2023 Sep 20.
Artículo en Inglés | MEDLINE | ID: mdl-37761787

RESUMEN

The purpose of this study was to examine and compare the psychometric properties (internal consistency and factorial validity) of different versions of the Body Shape Questionnaire (BSQ) in a sample of female aesthetic patients. The sample included 296 women attending aesthetic clinics, with ages ranging between 18 and 70 years (M = 32.23, SD = 11.35) and body mass index between 17.10 and 45.00 kg/m2 (M = 24.70, SD = 4.07). Nine different length versions of the BSQ (BSQ-34, BSQ-32, BSQ-16A, BSQ-16B, BSQ-14, BSQ-8A, BSQ-8B, BSQ-8C and BSQ-8D) were subjected to confirmatory factor analyses, using a robust maximum likelihood estimator. Robust fit indices indicated that the BSQ-8D version was the better-fitting and more parsimonious model (S-Bχ2/df = 1.81, CFI = 0.963, RMSEA = 0.052, SRMR = 0.043). This short version also showed appropriate reliability (McDonald's omega and composite reliability = 0.87) and a very high correlation with the original BSQ-34 version (r = 0.95). In sum, these findings suggest that the BSQ-8D is the most valid, reliable and suitable BSQ version for measuring body shape concerns in female aesthetic patients.

7.
J Clin Med ; 12(16)2023 Aug 10.
Artículo en Inglés | MEDLINE | ID: mdl-37629246

RESUMEN

AIM: The purpose of this study was to analyze the level of Internet addiction and physical activity, as well as the relationship between internet addiction and physical activity, among Polish and Portuguese students in the final year of the COVID-19 pandemic. METHODS: A web-based online survey was conducted among students in Poland and Portugal (398 respondents) aged between 17 and 26 in October 2022. The level of internet addiction was assessed by the Kimberly Young questionnaire in Polish and in Portuguese. The level of physical activity was measured by the international physical activity questionnaire in Polish and in Portuguese. RESULTS: In the study population, the majority of the respondents were mildly addicted to the internet. The average level of addiction was reported by 9.3-23.5% of the respondents. The average level of internet addiction was higher among Portuguese students and among men. Those who had suffered from COVID-19 (especially more than once) and were more physically active showed a higher tendency towards internet addiction. CONCLUSIONS: Internet addiction is a problem that has to be monitored and given more attention, particularly among male students. The results presented provide the first statistical insight into the problem of internet addiction among students in both countries and form the basis for further studies. They also highlight the severity of the problem and point to the need for early preventive and protective interventions against problematic internet use. It appears that effective strategies should include promoting the benefits of physical activity.

8.
J Clin Med ; 12(7)2023 Mar 28.
Artículo en Inglés | MEDLINE | ID: mdl-37048625

RESUMEN

Objectives: The aim of the study was to make a preliminary assessment of depression rates and levels of physical activity and the association between physical activity and depression among Polish and Portuguese students in the second year of the COVID-19 pandemic. Methods: A web-based online survey was conducted among students in Poland and Portugal (398 respondents-229 from Poland and 169 from Portugal) aged between 17 and 26 in October 2022. The level of depression was assessed by the Beck Depression Inventory (BDI) in Polish and in Portuguese. The level of physical activity was measured by the International Physical Activity Questionnaire (IPAQ) in Polish and in Portuguese. Results: Overall, 20-35% of respondents suffered from moderate depression, with a lower proportion among men. There were no differences in the percentage distribution of depression classification between Polish and Portuguese students. A decrease in the severity of depression was observed with increasing physical activity. In both countries, walking more often had the strongest effect on reducing depression scores. Conclusions: The continued and alarming prevalence of depressive disorders among university students in the year after the pandemic began had a significant impact on this population-further research on the impact of physical activity on mental health in different populations should be conducted to adjust the optimal level of physical activity for alleviating negative psychiatric symptoms.

9.
Am J Hematol ; 98(6): 857-868, 2023 06.
Artículo en Inglés | MEDLINE | ID: mdl-36882195

RESUMEN

Pediatric chronic immune thrombocytopenia (cITP) is a heterogeneous condition in terms of bleeding severity, second-line treatment use, association with clinical and/or biological immunopathological manifestations (IMs), and progression to systemic lupus erythematosus (SLE). No risk factors for these outcomes are known. Specifically, whether age at ITP diagnosis, sex, or IMs impact cITP outcomes is unknown. We report the outcomes of patients with pediatric cITP from the French nationwide prospective cohort OBS'CEREVANCE. We used multivariate analyses to investigate the effect of age at ITP diagnosis, sex, and IMs on cITP outcomes. We included 886 patients with a median (min-max) follow-up duration of 5.3 (1.0-29.3) years. We identified an age cutoff that dichotomized the risk of the outcomes and defined two risk groups: patients with ITP diagnosed <10 years (children) and ≥ 10 years (adolescents). Adolescents had a two to four-fold higher risk of grade ≥3 bleeding, second-line treatment use, clinical and biological IMs, and SLE diagnosis. Moreover, female sex and biological IMs were independently associated with higher risks of biological IMs and SLE diagnosis, second-line treatment use, and SLE diagnosis, respectively. The combination of these three risk factors defined outcome-specific risk groups. Finally, we showed that patients clustered in mild and severe phenotypes, more frequent in children and adolescents, respectively. In conclusion, we identified that age at ITP diagnosis, sex, and biological IMs impacted the long-term outcomes of pediatric cITP. We defined risk groups for each outcome, which will help clinical management and further studies.


Asunto(s)
Lupus Eritematoso Sistémico , Púrpura Trombocitopénica Idiopática , Femenino , Humanos , Púrpura Trombocitopénica Idiopática/tratamiento farmacológico , Estudios Prospectivos , Factores de Riesgo , Hemorragia , Lupus Eritematoso Sistémico/complicaciones , Lupus Eritematoso Sistémico/diagnóstico , Estudios Retrospectivos
10.
Midwifery ; 119: 103609, 2023 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-36804674

RESUMEN

Maternity health care professionals' attitudes on the option of home birth can influence the choices and decisions women and their partners make about place of birth. Midwives are particularly influential in this space. The study outlined in this paper aimed to translate and validate the Provider Attitudes towards Planned Home Birth (PAPHB) scale questionnaire for use in the Portuguese maternity context. METHODS: A total of 118 Portuguese midwives were selected through intentional sampling. The procedure was divided into two phases. In the first phase, a triple translation from the original language into Portuguese and a cross-cultural adaptation of the Provider Attitudes towards Planned Home Birth (PAPHB) scale were carried out, obtaining three versions of the same questionnaire. The second phase consisted of the validation of the questionnaire, for which the Provider Attitudes towards Planned Home Birth (PAPHB) scale was submitted to a panel of 20 experts and to a pilot test. Subsequently, the reliability and statistical validity of the scale were evaluated. RESULTS: After content analysis, the results confirmed a four-dimensional structure with a Cronbach's α value of 0.933 for the Provider Attitudes towards Planned Home Birth (PAPHB) scale as a whole, showing good internal consistency. Finally, a bivariate analysis was carried out identifying associations between variables and midwives' attitudes towards home birth. Positive attitudes towards homebirth were strongly influenced by previous clinical experience and exposure to home birth during midwives' academic education. CONCLUSION: The 18-item scale is a reliable and valid tool to quantify attitudes towards planned home births in Portugal as the results obtained in the study showed very good internal consistency.


Asunto(s)
Parto Domiciliario , Femenino , Humanos , Embarazo , Portugal , Reproducibilidad de los Resultados , Actitud del Personal de Salud , Encuestas y Cuestionarios , Lenguaje , Psicometría
11.
Psychol Health ; 38(1): 73-93, 2023 01.
Artículo en Inglés | MEDLINE | ID: mdl-34355628

RESUMEN

ObjectiveTo examine short- and long-term effects of a group singing program on older adults' perceived physical and mental health levels, and also investigate subjective well-being (life satisfaction, positive/negative affect and hedonic balance), body balance and serum biomarkers (C-reactive protein and erythrocyte sedimentation rate) as putative mediating mechanisms, controlling for the cognitive status of the participants. Design: The randomized controlled trial included 149 participants (60 to 95 years), allocated to an immediate intervention group (IG) or a wait-list active control group (WLG). The intervention comprised 34 sessions of group singing during 4-months. Main outcome measures: Self-report measures of physical health, anxiety, stress, and depression. Blinded assessments were conducted at baseline, post-intervention (4 months) and follow-up (6 months). Results: Participants in the IG reported a lower decline in perceived physical health after the intervention, as compared to the WLG. These benefits were maintained at follow-up. Singing-related changes in physical and mental health outcomes were mediated via an increase in positive affect. Moderation results showed that participants with very low cognitive functioning reported more anxiety and depression symptoms after the intervention. Conclusions: These findings provide further understanding on the psychological and physical mechanisms and effects of group singing in older adults.


Asunto(s)
Salud Mental , Canto , Anciano , Humanos , Ansiedad , Trastornos de Ansiedad , Cognición , Persona de Mediana Edad , Anciano de 80 o más Años
12.
Mundo saúde (Impr.) ; 47: e14442022, 2023.
Artículo en Inglés, Portugués | LILACS-Express | LILACS | ID: biblio-1517664

RESUMEN

Os sentidos atribuídos ao corpo estão comumente relacionados a sua limitação de nascer, viver e morrer. À vista disso, essas concepções atravessam todos os ciclos geracionais do indivíduo, desde antes do nascimento, na simbiose do binômio mãe-filho, até a morte. Sendo assim, há uma limitação da compreensão corporal na sua totalidade e a perpetuação da desinformação. Nesse sentido, o estudo tem como objetivo assimilar as percepções acerca da imagem corporal no decorrer dos ciclos de vida. Sendo adotada a abordagem qualitativa de um estudo comparativo e narrativo de casos múltiplos, constitui-se como cenário da pesquisa, uma unidade básica de saúde do município de Aracati/ CE, situado no litoral leste. A população foi estratificada em quatro grupos: infância, adolescência, adultos e velhice. Seguidamente, elencou-se a amostra pela escolha de dois indivíduos, masculino e feminino, de cada estrato etário. A aplicação das quatro oficinas ocorreu entre setembro e novembro de 2021, baseadas nas dinâmicas de sensibilidade e criatividade do método criativo sensível, alicerçado nas ideias de educação problematizadora de Paulo Freire. Já a análise foi realizada através da técnica de Análise do Discurso de Pêcheux. No decorrer desse estudo, percebeu-se que o corpo sofre representações mutáveis de signos e símbolos durante as gerações. Durante toda a pesquisa, a identificação da imagem corporal transitou nas experiências recentes e armazenadas de cada indivíduo. Sendo assim, a contribuição desta investigação para a atuação do profissional de saúde refere-se ao fato de que a comunicação é fundamental na relação entre profissional e cliente.


The meanings attributed to the body are commonly related to its limitation of being born, living and dying. Given this, these conceptions cross all the individual's generational cycles, from before birth, in the symbiosis of the mother-child binomial, until death. Therefore, there is a limitation of bodily understanding in its entirety and the perpetuation of misinformation. In this sense, the study aims to assimilate perceptions about body image throughout life cycles. The qualitative approach of a comparative and narrative study of multiple cases was adopted, the research setting was a basic health unit in the municipality of Aracati/CE, located on the east coast. The population was stratified into four groups: childhood, adolescence, adulthood and old age. Next, the sample was selected by choosing two individuals, a male and a female, from each age group. The implementation of the four workshops took place between September and November 2021, based on the dynamics of sensitivity and creativity of the sensitive creative method, based on the ideas of problematizing education by Paulo Freire. The analysis was carried out using the Pêcheux Discourse Analysis technique. During this study, it was noticed that the body undergoes changing representations of signs and symbols over the generations. Throughout the research, the identification of body image was reflected in the recent and stored experiences of each individual. Therefore, the contribution of this investigation to the performance of health professionals refers to the fact that communication is fundamental in the relationship between professional and client.

13.
Pharmacol Rep ; 74(5): 956-968, 2022 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-36097257

RESUMEN

BACKGROUND: Anxiolytic benzodiazepines, due to their clinical effectiveness, are one of the most prescribed drugs worldwide, despite being associated with sedative effects and impaired psychomotor and cognitive performance. Not every GABAA receptor functions in the same manner. Those containing α1 subunits are associated with sleep regulation and have a greater effect on the sedative-hypnotic benzodiazepines, whereas those containing α2 and/or α3 subunits are associated with anxiety phenomena and have a greater effect on the anxiolytic benzodiazepines. Therefore, characterization of the selectivity profile of anxiolytic drugs could translate into a significant clinical impact. METHODS: The present study pharmacodynamically evaluated chlornordiazepam, the main active metabolite of mexazolam, upon GABAA receptors containing α2 and/or α3, anxiety-related, and those containing an α1 subunit, associated with sleep modulation. RESULTS: As shown by whole-cell patch-clamp data, chlornordiazepam potentiated GABA-evoked current amplitude in α2 and α3 containing receptors without changing the current amplitude in α1 containing receptors. However, current decay time increased, particularly in GABAA receptors containing α1 subunits. In contrast, other anxiolytic benzodiazepines such as alprazolam, bromazepam, and zolpidem, all increased currents associated with GABAA receptors containing the α1 subunit. CONCLUSIONS: This novel evidence demonstrates that mexazolam (through its main metabolite chlornordiazepam) has a "pharmacodynamic fingerprint" that correlates better with an anxiolytic profile and fewer sedative effects, when compared to alprazolam, bromazepam and zolpidem, explaining clinical trial outcomes with these drugs. This also highlights the relevance of the pharmacological selectivity over GABAA receptor subtypes in the selection of benzodiazepines, in addition to their clinical performance and pharmacokinetic characteristics.


Asunto(s)
Ansiolíticos , Bromazepam , Receptores de GABA-A/metabolismo , Zolpidem , Alprazolam/farmacología , Ansiolíticos/farmacología , Bromazepam/farmacología , Benzodiazepinas/farmacología , Hipnóticos y Sedantes/farmacología , Ácido gamma-Aminobutírico
14.
Blood ; 140(3): 253-261, 2022 07 21.
Artículo en Inglés | MEDLINE | ID: mdl-35443028

RESUMEN

Splenectomy is effective in ∼70% to 80% of pediatric chronic immune thrombocytopenia (cITP) cases, and few data exist about it in autoimmune hemolytic anemia (AIHA) and Evans syndrome (ES). Because of the irreversibility of the procedure and the lack of predictions regarding long-term outcomes, the decision to undertake splenectomy is difficult in children. We report here factors associated with splenectomy outcomes from the OBS'CEREVANCE cohort, which prospectively includes French children with autoimmune cytopenia (AIC) since 2004. The primary outcome was failure-free survival (FFS), defined as the time from splenectomy to the initiation of a second-line treatment (other than steroids and intravenous immunoglobulins) or death. We included 161 patients (cITP, n = 120; AIHA, n = 19; ES, n = 22) with a median (minimum-maximum) follow-up of 6.8 years (1.0-33.3) after splenectomy. AIC subtype was not associated with FFS. We found that immunopathological manifestations (IMs) were strongly associated with unfavorable outcomes. Diagnosis of an IM before splenectomy was associated with a lower FFS (hazard ratio [HR], 0.39; 95% confidence interval [CI], 0.21-0.72, P = .003, adjusted for AIC subtype). Diagnosis of an IM at any timepoint during follow-up was associated with an even lower FFS (HR, 0.22; 95% CI, 0.12-0.39; P = 2.8 × 10-7, adjusted for AIC subtype) as well as with higher risk of recurrent or severe bacterial infections and thrombosis. In conclusion, our results support the search for associated IMs when considering a splenectomy to refine the risk-benefit ratio. After the procedure, monitoring IMs helps to identify patients with higher risk of unfavorable outcomes.


Asunto(s)
Anemia Hemolítica Autoinmune , Trombocitopenia , Anemia Hemolítica Autoinmune/diagnóstico , Niño , Estudios de Cohortes , Humanos , Esplenectomía/efectos adversos , Trombocitopenia/complicaciones
15.
Haematologica ; 107(2): 457-466, 2022 02 01.
Artículo en Inglés | MEDLINE | ID: mdl-33440924

RESUMEN

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 (IM). 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 years (range, 6.8-50.0 years) and the median follow-up period was 11.3 years (range, 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 IM increased with age: at the age of 20 years, 74% had at least one clinical IM (cIM). A wide range of cIM occurred, mainly lymphoproliferation, dermatological, gastrointestinal/hepatic and pneumological IM. The number of cIM 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 years (range, 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, long-term outcomes of pES showed remission of cytopenias but frequent IM linked to high second-line treatment burden. Mortality was associated to drugs and/or underlying immunodeficiencies, and adolescents-young adults are a high-risk subgroup.


Asunto(s)
Anemia Hemolítica Autoinmune , Adolescente , Adulto , Anemia Hemolítica Autoinmune/diagnóstico , Anemia Hemolítica Autoinmune/terapia , Niño , Preescolar , Estudios de Seguimiento , Humanos , Lactante , Estudios Prospectivos , Estudios Retrospectivos , Trombocitopenia , Adulto Joven
16.
Healthcare (Basel) ; 9(11)2021 Oct 23.
Artículo en Inglés | MEDLINE | ID: mdl-34828476

RESUMEN

BACKGROUND: Schizophrenia is a chronic psychiatric disorder with symptoms that can severely affect patients' quality of life. While drug administration inhibits most of the positive symptoms, it fails to effectively treat the negative symptoms and in turn relies on rehabilitation therapies. With technological progress, virtual reality (VR) has been the target of recent studies in terms of mental health rehabilitation and has been shown to be a tool with ecological validity in order to introduce the individual to situations similar to their daily life. METHODS: From July to August 2021, we conducted a systematic review with the purpose of understanding the impact of virtual reality rehabilitation on negative symptoms and psychosocial rehabilitation in the schizophrenia spectrum. The searches were performed in the databases Web of Science, Scopus, and PubMed. RESULTS: In our search, we identified 201 results, where 108 duplicates were excluded, resulting in a final balance of 93. After reading and assessing the titles and abstracts, 66 studies were excluded. Of the remaining 27 reports, 23 were excluded for not meeting the previously defined eligibility criteria, resulting in the inclusion of four studies in this systematic review. CONCLUSIONS: The available data on the specific topic was limited and could have been more complete. However, in our review, we were able to identify statistically relevant results demonstrating the effectiveness of intervention. We could find medium to large effects, allowing VR to be an ally for rehabilitation of symptoms related to schizophrenia.

17.
Work ; 69(1): 275-282, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-33998587

RESUMEN

BACKGROUND: Several studies have suggested that trait and ability-based measures of emotional intelligence (EI) contribute to the promotion of well-being in adults. However, this relationship has not been sufficiently explored among adolescents. OBJECTIVE: The present study aims to: i) investigate the associations between EI dimensions and well-being indicators (self-esteem, life satisfaction and social anxiety) in adolescents; and ii) analyze the effect of sex and age on dimensions of EI. METHODS: The sample consisted of 1066 adolescents (57.9%girls and 42.1%boys), aged between 11 and 18 years (M = 14.36; SD = 1.58). Participants completed sociodemographic and validated psychosocial measures. RESULTS: Correlation analysis indicated that EI dimensions (self-emotion appraisal, use of emotions and regulation of emotions) were positively and moderately correlated with self-esteem and life satisfaction, whereas associations between EI and social anxiety dimensions were small and negative. Boys reported higher levels of self-emotion appraisal, use of emotions and regulation of emotions, whereas girls showed higher levels of other-emotions appraisal. Age was negatively associated with the use of the emotions dimension and positively correlated with the emotional appraisal of others, although both correlation coefficients were small. CONCLUSIONS: Our study findings suggest the need and importance of implementing emotional education practices during adolescence.


Asunto(s)
Inteligencia Emocional , Emociones , Adolescente , Adulto , Niño , Femenino , Humanos , Masculino , Autoimagen
18.
Epilepsy Res ; 174: 106653, 2021 08.
Artículo en Inglés | MEDLINE | ID: mdl-34049227

RESUMEN

Eslicarbazepine acetate (ESL) is a once-daily antiseizure medication (ASM) that is approved in Europe and the USA for the treatment of focal-onset seizures. The Euro-Esli study, which included over 2000 patients, investigated the real-world effectiveness, safety and tolerability of ESL when used in everyday clinical practice in Europe. This post-hoc analysis of Euro-Esli employed univariate and multivariate binary logistic regression analyses to investigate the relationship between demographic and baseline characteristics (including epilepsy- and treatment-related factors) and the likelihood of seizure freedom, response and retention in adult patients with focal seizures after 12 months of ESL treatment in the real-world setting. Multivariate analysis revealed that the factors associated with seizure freedom and response at 12 months (N = 1054) were generally those characterising patients who were relatively early in their disease course and/or less refractory to treatment, such as older age at onset of epilepsy, absence of seizures at baseline and lower number of concomitant ASMs at baseline. Although it was not possible to construct a multivariate model to predict retention on ESL treatment at 12 months, when the univariate regression model was adjusted for age and epilepsy duration, the factors found to be significantly associated with retention at 12 months (N = 1559) comprised shorter duration of epilepsy, absence of any seizures at baseline, lower baseline seizure frequency (<5 vs. ≥ 5 seizures/month), lower number of previous ASMs, lower number of concomitant ASMs, and the absence of concomitant use of lamotrigine at baseline. These findings therefore identify baseline characteristics that are predictive of the effectiveness of ESL treatment in clinical practice, which may help clinicians choose appropriate ASM therapy for patients.


Asunto(s)
Dibenzazepinas , Adulto , Anticonvulsivantes/uso terapéutico , Dibenzazepinas/uso terapéutico , Libertad , Humanos , Convulsiones/inducido químicamente , Convulsiones/tratamiento farmacológico , Resultado del Tratamiento
19.
Artículo en Inglés | MEDLINE | ID: mdl-33562438

RESUMEN

The purpose of this study was to compare measures of anthropometry characteristics and physical fitness performance between rugby union players (17.9 ± 0.5 years old) recruited (n = 39) and non-recruited (n = 145) to the Portuguese under-19 (U19) national team, controlling for their playing position (forwards or backs). Standardized anthropometric, physical, and performance assessment tests included players' body mass and height, push up and pull-up test, squat test, sit-and-reach test, 20 m shuttle run test, flexed arm hang test, Sargent test, handgrip strength test, Illinois agility test, and 20-m and 50-m sprint test. Results showed that recruited forwards players had better agility scores (p = 0.02, ES = -0.55) than the non-recruited forwards, whereas recruited backs players had higher right (p < 0.01, ES = 0.84) and left (p = 0.01, ES = 0.74) handgrip strength scores than their counterparts. Logistic regression showed that better agility (for the forwards) and right handgrip strength scores (for the backs) were the only variables significantly associated with an increased likelihood of being recruited to the national team. In sum, these findings suggest that certain well-developed physical qualities, namely, agility for the forwards players and upper-body strength for the back players, partially explain the selection of U19 rugby players to their national team.


Asunto(s)
Fútbol Americano , Antropometría , Fuerza de la Mano , Illinois , Fuerza Muscular , Aptitud Física
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