ABSTRACT
Abstract Objective: To evaluate the functional status of individuals with Osteogenesis Imperfecta (OI) followed up at a reference center in the state of Bahia. Materials and methods: This is an observational, cross-sectional, descriptive study, which evaluated individuals with OI, based on a non-probabilistic sampling. To assess motor function, the Motor Function Measure (MFM) score was used, in addition to the measurement of muscle strength using the Medical Research Council (MRC) score. Functional performance was measured using the Pediatric Assessment of Disability Inventory, Computerized Adaptive Testing (PEDI-CAT). Results: Thirty-one individuals aged between two and 18 years old were evaluated. The overall score of MFM was 74.2%, and the lowest score was found in participants with type III OI (56.3%). The median of the MRC index was 80. The mobility domain was the most affected in the PEDI-CATevaluation, with a mean T score of 23.9, (14.2 in type III OI). Conclusions: Among the evaluated individuals, functional alterations were identified, reduced global gross motor functionality and muscle strength, impacting the mobility domain, with the most relevant findings in individuals with type III OI.
ABSTRACT
Abstract Objective: This study aims to evaluate the respiratory function of children and adolescents with osteogenesis imperfecta (OI) followed up at a referral center. Methods: A cross-sectional study was conducted with a non-probabilistic sample. Manovacuometry was performed with the measurement of maximal inspiratory pressure (MIP) and maximal expiratory pressure (MEP), and in addition, peak expiratory flow (PEF) and ventilometry were performed to measure forced vital capacity (FVC). Results: In total, 23 individuals were evaluated, with a mean age of 11.6±3.4 years, 56.5% of whom were females. Regarding the classification of OI, 56.5% of the sample belonged to type IV, 30.5% to type III, and 13% to type I. The mean MIP was 64.4% of the predicted, and the mean MEP was 56.2% of the predicted. Overall, the mean PEF was 213.9 L/min, but only 140.6 L/min in the OI type III group. Median FVC was 1.9 L, corresponding to 110% of the predicted. Conclusions: Respiratory function of the study subjects was altered, with respiratory muscle strength values lower than expected in the whole sample, and peak expiratory flow was significantly reduced in the OI type III group.
RESUMO Objetivo: Avaliar a função respiratória de crianças e adolescentes com osteogênese imperfeita (OI) acompanhados em um centro de referência. Métodos: Realizou-se um estudo de corte transversal, com amostragem não probabilística. Foi realizada manovacuometria com mensuração da pressão inspiratória máxima (PIM) e pressão expiratória máxima (PEM), além do pico de fluxo expiratório (PFE) e da ventilometria para a medida da capacidade vital forçada (CVF). Resultados: Foram avaliados 23 indivíduos, com média de idade de 11,6±3,4 anos, sendo 56,5% do sexo feminino. Com relação à classificação da OI, 56,5% da amostra pertencia ao tipo IV, 30,5% ao tipo III e 13% ao tipo I. A média de PIM foi de 64,4% do previsto, e a PEM foi de 56,2% do previsto. A média de PFE foi de 213,9 L/min, sendo 140,6 L/min no grupo de OI tipo III. A mediana da CVF foi de 1,9 L, correspondendo a 110% do previsto. Conclusões: A função respiratória dos indivíduos estudados encontrava-se alterada, com valores abaixo do esperado em toda a amostra para força muscular respiratória, além do PFE reduzido no grupo OI tipo III.
ABSTRACT
Objetivo: determinar a incidência, os fatores associados e o impacto das complicações pulmonares no pós-operatório de cirurgia cardíaca pediátrica. Metodologia: estudo de coorte, prospectivo, que incluiu lactentes e crianças submetidas à cirurgia cardíaca em um hospital pediátrico, no período de novembro de 2016 a julho de 2019. Foram coletados dados dos prontuários referentes ao sexo, idade, presença de outras malformações associadas, tipo de cardiopatia, ocorrência de complicações pulmonares, tempo de ventilação mecânica (VM) e de internamento na unidade de terapia intensiva (UTI) e óbito. Resultados: a amostra foi constituída por 111 lactentes e crianças, mediana da idade de 13 meses (7-32 meses), 54,1% do sexo feminino. Quanto ao tipo de cardiopatia, 80,2% foram cianogênicas. As complicações pulmonares ocorreram em 44,1% dos casos, sendo a mais frequente a atelectasia. A mediana do tempo de VM foi 8 horas (1-48h) e 45 (40,5%) permaneceram na VM por mais de 24h. A mediana do tempo de internamento na UTI foi de 7 dias (4-12dias). Evoluíram a óbito 7 (14,3%) pacientes. Conclusão: a amostra investigada apresentou incidência elevada de complicações pulmonares no pós-operatório de cirurgia cardíaca
Objective: to determine the incidence, associated factors and impact of pulmonary complications in the postoperative period of pediatric cardiac surgery. Methodology: prospective cohort study, which included infants and children undergoing cardiac surgery in a pediatric hospital, from November 2016 to July 2019. Data were collected from medical records regarding sex, age, presence of other associated malformations, type of heart disease, occurrence of pulmonary complications, duration of mechanical ventilation (MV) and admission to the intensive care unit (ICU) stay and death. Results: the sample consisted of 111 infants and children, median age 13 months (7-32 months), 54.1% female. As for the type of heart disease, 80.2% were acyanotic. Pulmonary complications occurred in 44.1% of cases, with atelectasis being the most frequent. The median time on mechanical ventilation (MV) was 8 hours (1-48h) and 45 (40.5%) remained on MV for more than 24h. The median length of stay in the ICU was 7 days (4-12 days). 7 (14.3%) patients died. Conclusion: the investigated sample had a high incidence of pulmonary complications in the postoperative period of cardiac surgery