RESUMO
BACKGROUND: Marfan Syndrome is an autosomal dominant disease caused by pathogenetic variants in the FBN1 gene. The progressive dilatation of the aorta and the potential risk of acute aortic syndromes influence the prognosis of these patients. We aim to describe population characteristics, long-term survival, and re-intervention patterns in patients who underwent aortic surgery with a previously confirmed clinical diagnosis of Marfan Syndrome in a middle-income country. METHODS: A retrospective single-center case series study was conducted. All Marfan Syndrome patients who underwent aortic procedures from 2004 until 2021 were included. Qualitative variables were frequency-presented, while quantitative ones adopted mean ± standard deviation. A subgroup analysis between elective and emergent procedures was conducted. Kaplan-Meier plots depicted cumulative survival and re-intervention-free. Control appointments and government data tracked out-of-hospital mortality. RESULTS: Fifty patients were identified. The mean age was 38.79 ± 14.41 years, with a male-to-female ratio of 2:1. Common comorbidities included aortic valve regurgitation (66%) and hypertension (50%). Aortic aneurysms were observed in 64% without dissection and 36% with dissection. Surgical procedures comprised elective (52%) and emergent cases (48%). The most common surgery performed was the David procedure (64%), and the Bentall procedure (14%). The in-hospital mortality rate was 4%. Complications included stroke (10%), and acute kidney injury (6%). The average follow-up was 8.88 ± 5.78 years. Survival rates at 5, 10, and 15 years were 89%, 73%, and 68%, respectively. Reintervention rates at 1, 2.5, and 5 years were 10%, 14%, and 17%, respectively. The emergent subgroup was younger (37.58 ± 14.49 years), had the largest number of Stanford A aortic dissections, presented hemodynamic instability (41.67%), and had a higher requirement of reinterventions in the first 5 years of follow-up (p = 0.030). CONCLUSION: In our study, surveillance programs played a pivotal role in sustaining high survival rates and identifying re-intervention requirements. However, challenges persist, as 48% of the patients required emergent surgery. Despite not affecting survival rates, a greater requirement for reinterventions was observed, emphasizing the necessity of timely diagnosis. Enhanced educational initiatives for healthcare providers and increased patient involvement in follow-up programs are imperative to address these concerns.
Assuntos
Síndrome de Marfan , Humanos , Síndrome de Marfan/complicações , Síndrome de Marfan/cirurgia , Masculino , Feminino , Estudos Retrospectivos , Adulto , Pessoa de Meia-Idade , Dissecção Aórtica/cirurgia , Adulto Jovem , Aneurisma Aórtico/cirurgiaRESUMO
Resumen La cultura sobre seguridad se perfila como uno de los requisitos para evitar la aparición de efectos adversos, sin embargo, no se ha estudiado en el ámbito de la cardiología. Objetivo: es evaluar la cultura de seguridad en una unidad de cardiología que tiene implantado y certificado un sistema integrado de gestión de calidad y riesgos para la seguridad del paciente. Método: Se realizó un estudio observacional trasversal en 2 años consecutivos utilizando la encuesta Hospital Survey on Patient Safety Culture de la «Agency for Healthcare Research and Quality¼ en su versión española (42 ítems agrupados en 12 dimensiones) sobre todo el personal. Se comparó el porcentaje de respuestas positivas de cada dimensión en 2014 y 2015, así como con los datos a nivel nacional y en EE. UU., siguiendo las recomendaciones establecidas. Resultados: La valoración global, sobre un máximo de 5, fue de 4.5 en 2014 y de 4.7 en 2015. Identificamos 7 dimensiones como fortaleza. Las peor valoradas fueron: Dotación de personal, Apoyo de la gerencia y Trabajo en equipo entre unidades. La comparación mostró superioridad en todas las dimensiones a nivel nacional, y en 8 respecto a los datos del registro americano. Conclusiones: La cultura de seguridad en una unidad de cardiología con un sistema integrado de gestión de calidad y riesgos y seguridad del paciente es elevada, superior a la nacional en todas sus dimensiones y en la mayoría de ellas respecto al registro de EE. UU.
Abstract Safety culture is one of the requirements for preventing the occurrence of adverse effects. However, this has not been studied in the field of cardiology. The aim of this study is to evaluate the safety culture in a cardiology unit that has implemented and certified an integrated quality and risk management system for patient safety. Methods: A cross-sectional observational study was conducted in 2 consecutive years, with all staff completing the Spanish version of the questionnaire, ''Hospital Survey on Patient Safety Culture'' of the ''Agency for Healthcare Research and Quality'', with 42 items grouped into 12 dimensions. The percentage of positive responses in each dimension in 2014 and 2015 were compared, as well as national data and United States data, following the established rules. Results: The overall assessment out of a possible 5, was 4.5 in 2014 and 4.7 in 2015. Seven dimensions were identified as strengths. The worst rated were: staffing, management support and teamwork between units. The comparison showed superiority in all dimensions compared to national data, and in 8 of them compared to American data. Conclusions: The safety culture in a Cardiology Unit with an integrated quality and risk management patient safety system is high, and higher than nationally in all its dimensions and in most of them compared to the United States.
Assuntos
Humanos , Serviço Hospitalar de Cardiologia/normas , Gestão da Segurança , Segurança do Paciente , Recursos Humanos em Hospital/estatística & dados numéricos , Gestão de Riscos/métodos , Espanha , Estados Unidos , Estudos Transversais , Inquéritos e Questionários , Serviço Hospitalar de Cardiologia/organização & administraçãoRESUMO
Safety culture is one of the requirements for preventing the occurrence of adverse effects. However, this has not been studied in the field of cardiology. The aim of this study is to evaluate the safety culture in a cardiology unit that has implemented and certified an integrated quality and risk management system for patient safety. METHODS: A cross-sectional observational study was conducted in 2 consecutive years, with all staff completing the Spanish version of the questionnaire, "Hospital Survey on Patient Safety Culture" of the "Agency for Healthcare Research and Quality", with 42 items grouped into 12 dimensions. The percentage of positive responses in each dimension in 2014 and 2015 were compared, as well as national data and United States data, following the established rules. RESULTS: The overall assessment out of a possible 5, was 4.5 in 2014 and 4.7 in 2015. Seven dimensions were identified as strengths. The worst rated were: staffing, management support and teamwork between units. The comparison showed superiority in all dimensions compared to national data, and in 8 of them compared to American data. CONCLUSIONS: The safety culture in a Cardiology Unit with an integrated quality and risk management patient safety system is high, and higher than nationally in all its dimensions and in most of them compared to the United States.
Assuntos
Serviço Hospitalar de Cardiologia/normas , Segurança do Paciente , Gestão da Segurança , Serviço Hospitalar de Cardiologia/organização & administração , Estudos Transversais , Humanos , Recursos Humanos em Hospital/estatística & dados numéricos , Gestão de Riscos/métodos , Espanha , Inquéritos e Questionários , Estados UnidosRESUMO
This study examined the latent structure and validity of inattention, hyperactivity-impulsivity, and sluggish cognitive tempo (SCT) symptomatology. We evaluated mother and teacher ratings of ADHD and SCT symptoms in 140 Puerto Rican children (55.7% males), ages 6 to 11 years, via factor and regression analyses. A three-factor model (inattention, hyperactivity-impulsivity, and SCT) provided the best fit for both sets of ratings. Inattention was the strongest correlate of lower scores on neuropsychological, achievement, and psychosocial measures. Externalizing problems were most strongly associated with hyperactivity-impulsivity, and internalizing problems were most strongly associated with parent-rated SCT and teacher-rated Inattention. SCT was not associated with executive function but was negatively associated with math. Inattention accounted for a disproportionate amount of ADHD-related impairment, which may explain the restricted discriminant validity of DSM-IV types. The distinct factors of hyperactivity-impulsivity and SCT had unique associations with impairing comorbidities and are roughly equivalent in predicting external correlates of ADHD-related impairment.
Assuntos
Transtorno do Deficit de Atenção com Hiperatividade/psicologia , Atenção , Transtornos Cognitivos/psicologia , Logro , Transtorno do Deficit de Atenção com Hiperatividade/diagnóstico , Transtorno do Deficit de Atenção com Hiperatividade/etnologia , Criança , Escolaridade , Feminino , Humanos , Hipercinese/psicologia , Comportamento Impulsivo/psicologia , Inteligência , Relações Interpessoais , Masculino , Memória de Curto Prazo , Mães , Testes Neuropsicológicos , Resolução de Problemas , Escalas de Graduação Psiquiátrica , Desempenho Psicomotor , Porto Rico , EnsinoRESUMO
OBJECTIVE: This study examined the impact of inattention, hyperactivity, and oppositional defiant disorder (ODD) behaviors and gender on family life. METHOD: We created scales for the Family Experiences Inventory (FEI) in a nonclinical sample of Spaniard families with children ages 6 to 12 years (N = 369) and analyzed the perceived impact of these three behavior dimensions on family experiences. RESULTS: Multiple regression analyses indicated that ODD behaviors were uniquely correlated with Total FEI and its dimensions. Inattention was also uniquely related to higher negative Impact on School Relations and lower Positive Impact on Parents scales. Finally, gender-hyperactivity interactions indicated that boys with higher hyperactivity scores were more likely to score higher on the FEI Total, School Relations, and Siblings scales, and more likely to score lower on the Positive Impact on Parents scale than girls. CONCLUSIONS: These findings suggested that parents perceive greater child-related impact and place greater burden from having a male child with hyperactivity. Inattention, hyperactivity, and oppositional defiant behaviors are associated with global parent-child interactive stress but the pattern of associations will vary depending upon the behavior, child gender, and context of family life examined.
Assuntos
Transtorno do Deficit de Atenção com Hiperatividade/psicologia , Transtornos de Deficit da Atenção e do Comportamento Disruptivo/psicologia , Família/psicologia , Hipercinese/psicologia , Criança , Comportamento Infantil/psicologia , Análise Fatorial , Feminino , Hispânico ou Latino/psicologia , Humanos , Modelos Lineares , Masculino , Percepção , Estresse Psicológico , Inquéritos e QuestionáriosRESUMO
BACKGROUND: The aim of this investigation was to examine the construct validity and distinctiveness of the inattentive type (IT) and combined type (CT) of Attention-Deficit/Hyperactivity Disorder (ADHD) in a Latino/Hispanic sample. METHOD: A comprehensive assessment was conducted with a clinically diagnosed school-based sample of 98 children aged 6 to 11 (CT=44; IT=25; control group=29). RESULTS: Both ADHD groups were impaired on academic achievement measures, presented more ADHD-type behaviors during math and vigilance tasks, and exhibited greater internalizing symptoms. The IT group had a later onset of inattention symptoms, presented more sluggish cognitive tempo symptoms, was less prone to initiate social interactions or to be assertive and more self-controlled in social interchanges, was less likely to have externalizing behaviors, had mothers who reported less child-related family stress, and was less impaired in their adaptive functioning. CONCLUSIONS: Findings supported the construct validity of ADHD in this culturally different sample and suggested that the CT and IT represent distinct disorders.
Assuntos
Transtorno do Deficit de Atenção com Hiperatividade/classificação , Transtorno do Deficit de Atenção com Hiperatividade/diagnóstico , Manual Diagnóstico e Estatístico de Transtornos Mentais , Transtorno do Deficit de Atenção com Hiperatividade/epidemiologia , Criança , Escolaridade , Feminino , Humanos , Modelos Logísticos , Masculino , Análise Multivariada , Testes Psicológicos , Porto Rico/epidemiologia , Reprodutibilidade dos Testes , Ajustamento SocialRESUMO
PURPOSE: Surgical results in patients with mesial temporal lobe epilepsy due to hippocampal sclerosis (MTLE/HS) are often reported in conjunction with other etiologies of TLE. METHODS: We prospectively collected surgical outcome data for 2 to 11 years for 134 consecutive patients who specifically had MTLE and unilateral HS, according to magnetic resonance imaging (MRI) and confirmed by histopathology. Sixty-five had postoperative neuropsychological testing. Outcome was analyzed by using Engel's classification (a) through Kaplan-Meier estimated survival curves (as a function of the time to seizure recurrence), (b) as percentage of patients in each outcome class on a yearly basis, and (c) at the last updated follow-up. RESULTS: Kaplan-Meier estimates of complete seizure freedom (Engel's class IA) for years 1, 2, 5, and 10 were 85%, 77%, 74%, and 66%, and of Engel's class I were 89%, 86%, 83%, and 81%. Only nine (6.7%) patients had outcome classes III or IV at any point during follow-up. Of the patients tested, 26% of those operated on the left side and 22% of those operated on the right had postoperative decline of >1 SD in verbal or visual memory, respectively. CONCLUSIONS: High rates of seizure freedom can be obtained and remain stable over the years in patients operated on for unilateral MTLE/HS, even in countries with limited resources.