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2.
Rev Paul Pediatr ; 42: e2024111, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37194911

RESUMEN

OBJECTIVE: To revise the impact of telehealth on the quality of life, reduction in pulmonary exacerbations, number of days using antibiotics, adherence to treatment, pulmonary function, emergency visits, hospitalizations, and the nutritional status of individuals with asthma and cystic fibrosis. DATA SOURCE: Four databases were used, MEDLINE, LILACS, Web of Science and Cochrane, as well as manual searches in English, Portuguese and Spanish. Randomized clinical trials, published between January 2010 and December 2020, with participants aged 0 to 20 years, were included. DATA SYNTHESIS: Seventy-one records were identified after the removal of duplicates; however, twelve trials were eligible for synthesis. Included trials utilized: mobile phone applications (n=5), web platforms (n= 4), mobile telemedicine unit (n=1), software with an electronic record (n=1), remote spirometer (n=1), and active video games platform (n=1). Three trials used two tools, including telephone calls. Among the different types of interventions, improvement in adherence, quality of life, and physiologic variables were observed for mobile application interventions and game platforms compared to usual care. Visits to the emergency department, unscheduled medical appointments, and hospitalizations were not reduced. There was considerable heterogeneity among studies. CONCLUSIONS: The findings suggest that better control of symptoms, quality of life, and adherence to treatment can be attributed to the technological interventions used. Nevertheless, further research is needed to compare telehealth with face-to-face care and to indicate the most effective tools in the routine care of children with chronic lung diseases.


Asunto(s)
Asma , Aplicaciones Móviles , Telemedicina , Humanos , Niño , Adolescente , Calidad de Vida , Asma/tratamiento farmacológico , Teléfono , Enfermedad Crónica
3.
Rev Paul Pediatr ; 41: e2022092, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-36921178

RESUMEN

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.


Asunto(s)
Osteogénesis Imperfecta , Femenino , Humanos , Niño , Adolescente , Masculino , Estudios Transversales , Capacidad Vital/fisiología , Músculos Respiratorios , Fuerza Muscular/fisiología
4.
J. pediatr. (Rio J.) ; 99(1): 94-98, Jan.-Feb. 2023. tab, graf
Artículo en Inglés | LILACS-Express | LILACS | ID: biblio-1422013

RESUMEN

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.

5.
J Pediatr (Rio J) ; 99(1): 94-98, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-35940209

RESUMEN

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-CAT evaluation, 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.


Asunto(s)
Osteogénesis Imperfecta , Humanos , Estudios Transversales , Estado Funcional , Fuerza Muscular
7.
Rev. Paul. Pediatr. (Ed. Port., Online) ; 42: e2024111, 2023. tab, graf
Artículo en Inglés | LILACS-Express | LILACS | ID: biblio-1441062

RESUMEN

Abstract Objective: To revise the impact of telehealth on the quality of life, reduction in pulmonary exacerbations, number of days using antibiotics, adherence to treatment, pulmonary function, emergency visits, hospitalizations, and the nutritional status of individuals with asthma and cystic fibrosis. Data source: Four databases were used, MEDLINE, LILACS, Web of Science and Cochrane, as well as manual searches in English, Portuguese and Spanish. Randomized clinical trials, published between January 2010 and December 2020, with participants aged 0 to 20 years, were included. Data synthesis: Seventy-one records were identified after the removal of duplicates; however, twelve trials were eligible for synthesis. Included trials utilized: mobile phone applications (n=5), web platforms (n= 4), mobile telemedicine unit (n=1), software with an electronic record (n=1), remote spirometer (n=1), and active video games platform (n=1). Three trials used two tools, including telephone calls. Among the different types of interventions, improvement in adherence, quality of life, and physiologic variables were observed for mobile application interventions and game platforms compared to usual care. Visits to the emergency department, unscheduled medical appointments, and hospitalizations were not reduced. There was considerable heterogeneity among studies. Conclusions: The findings suggest that better control of symptoms, quality of life, and adherence to treatment can be attributed to the technological interventions used. Nevertheless, further research is needed to compare telehealth with face-to-face care and to indicate the most effective tools in the routine care of children with chronic lung diseases.


RESUMO Objetivo: Revisar o impacto da telessaúde na qualidade de vida, redução das exacerbações pulmonares, número de dias em uso de antibióticos, adesão ao tratamento, função pulmonar, visitas à emergência, hospitalizações e estado nutricional de indivíduos com asma e fibrose cística. Fontes de dados: Foram utilizadas quatro base de dados, sendo, MEDLINE, LILACS, Web of Science e Cochrane, além de pesquisas manuais nos idiomas inglês, português e espanhol. Foram incluídos ensaios clínicos randomizados, publicados no período de janeiro de 2010 a dezembro de 2020, com participantes de 0 a 20 anos. Síntese dos dados: Setenta e um registros foram identificados após a remoção das duplicatas e doze estudos foram elegíveis para síntese. Os ensaios utilizaram aplicativos para celular (n=5), plataformas da web (n= 4), unidade de telemedicina móvel (n=1), software com registro eletrônico (n=1), espirômetro remoto (n=1) e plataforma ativa de videogames (n=1). Três ensaios utilizaram duas ferramentas, incluindo chamadas telefônicas. Entre os diferentes tipos de intervenções, observou-se melhora na adesão, qualidade de vida e de variáveis fisiológicas para intervenções de aplicativos móveis e plataformas de jogos em comparação com os cuidados habituais. Visitas ao pronto-socorro, consultas médicas não agendadas e internações não foram reduzidas. Houve considerável heterogeneidade entre os estudos. Conclusões: Os achados sugerem que a melhora do controle dos sintomas, da qualidade de vida e da adesão ao tratamento podem ser atribuídos às intervenções tecnológicas utilizadas. No entanto, mais pesquisas são necessárias para comparar a telessaúde com o atendimento presencial e indicar as ferramentas mais efetivas na rotina de cuidados à população infantil com doenças crônicas pulmonares.

8.
Artículo en Inglés | LILACS-Express | LILACS | ID: biblio-1422824

RESUMEN

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.

9.
Arq. Asma, Alerg. Imunol ; 4(3): 277-288, jul.set.2020. ilus
Artículo en Portugués | LILACS | ID: biblio-1381995

RESUMEN

A via inalatória é a mais adequada para o tratamento das doenças respiratórias. Muitos fatores influenciam na deposição pulmonar do fármaco inalado, e, consequentemente, no sucesso terapêutico, desde fatores relacionados ao indivíduo, como questões anatômicas das vias aéreas, dinâmica respiratória, doença de base e técnica correta, até situações relacionadas às questões aerodinâmicas das partículas que compõem o aerossol, como o tamanho (diâmetro aerodinâmico mediano de massa) e a homogeneidade das partículas (desvio padrão geométrico). Nos últimos anos os dispositivos inalatórios se aperfeiçoaram, buscando atender às características necessárias que garantam uma deposição pulmonar satisfatória dos fármacos. A escolha do dispositivo inalatório deve ser individualizada, e o conhecimento das particularidades de cada dispositivo e das vantagens e desvantagens instrumentaliza o profissional na decisão, e impacta diretamente no sucesso terapêutico da medicação utilizada.


The inhalation route is the most adequate for the treatment of respiratory diseases. Many factors influence pulmonary deposition of the inhaled drug and, consequently, therapeutic success. They include individual factors such as airway anatomy, respiratory dynamics, underlying disease, and correct technique, as well as factors related to the aerodynamics of aerosol particles such as size (mass median aerodynamic diameter) and homogeneity of the particles (geometric standard deviation). In recent years, inhalation devices have improved to comply with the necessary characteristics that guarantee a satisfactory pulmonary deposition of drugs. The choice of the inhalation device must be individualized, and the knowledge about the features of each device and their advantages and disadvantages instrumentalizes health professionals in the decision and impacts directly the therapeutic success of the medication used.


Asunto(s)
Humanos , Masculino , Femenino , Niño , Adolescente , Sociedades Médicas , Nebulizadores y Vaporizadores , Aerosoles , Pediatría , Enfermedades Respiratorias , Terapéutica , Preparaciones Farmacéuticas , Inhalación , Alergia e Inmunología , Equipos y Suministros , Material Particulado
10.
Int J Pediatr Otorhinolaryngol ; 116: 186-191, 2019 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-30554696

RESUMEN

OBJECTIVE: To evaluate the auditory system for hidden hearing loss (HHL) and its association with clinical variables and endothelial dysfunction (ED) in children and adolescents with sickle cell anemia (SCA). METHODS: Participants included 37 patients with stable SCA and 44 healthy controls (HC group) (aged 6-18 years) with hearing thresholds ≤ 20 dB (dB) were evaluated for pure tone audiometry, tympanometry, acoustic reflex, otoacoustic emission, and auditory evoked potentials. Laboratory analysis of the lipid profile, and C-reactive protein levels and endothelial function using ultrasonographic imaging of the brachial artery to assess flow-mediated dilation were performed. RESULTS: The SCA group presented with a higher rate of increased contralateral acoustic reflex thresholds, compared to those in the HC group at all frequencies and in both ears (p < 0.05). There were significant differences in the brainstem auditory evoked potentials between the SCA and HC groups. In the SCA group, the waves III and V latencies were increased (p = 0.006 and 0.004 respectively), and the I-III and I-V interpeak intervals were longer (p = 0.015 and 0.018 respectively) than those in the HC group. There was no association between the audiological measures and clinical and metabolic variables and sickle cell anemia complications including endothelial function and therapy. CONCLUSION: In conclusion, our findings suggest that damage in the auditory system in SCA patients can be present involving retrocochlear structures, causing functional deficits without deterioration of auditory sensitivity.


Asunto(s)
Anemia de Células Falciformes/complicaciones , Pérdida Auditiva/etiología , Adolescente , Arteria Braquial/diagnóstico por imagen , Niño , Endotelio/fisiopatología , Potenciales Evocados Auditivos/fisiología , Femenino , Audición/fisiología , Pérdida Auditiva/diagnóstico , Pruebas Auditivas/métodos , Humanos , Lípidos/sangre , Masculino , Emisiones Otoacústicas Espontáneas/fisiología , Ultrasonografía
11.
Sleep Med ; 53: 9-15, 2019 01.
Artículo en Inglés | MEDLINE | ID: mdl-30384138

RESUMEN

OBJECTIVE: We sought to assess sleep characteristics in children with sickle cell anemia (SCA) to investigate the possible association between sleep-related disorders and parameters of endothelial function. METHODS: Sleep Disturbance Scale for Children (SDSC) and endothelial function variables (flow-mediated dilatation using brachial artery ultrasound, cytokines) were compared in children with SCA and healthy children aged 6-18 years. RESULTS: Flow-mediated dilation (FMD) median (IQR) values were 10.1 (6.4; 14) vs 16.9 (12; 26.4), in the SCA and comparison groups (p = 0.001), respectively. Associations were found between difficulty initiating and maintaining sleep as well as IL-12p70 (rs = 0.3, p = 0.03) and IL-1b (rs = 0.4, p = 0.01); disorders of excessive sleepiness and TNF-a (rs = 0.3, p = 0.02), IL-6 (rs = 0.3, p = 0.03), e IL-17A (rs = 0.3, p = 0.04), and disorders of arousal with IL-6 (rs = 0.3, p = 0.04). Regarding SDSC subscales, the score for sleep breathing disorders was higher in the SCA group than in the comparison group [6.7 (3.4) vs. 5.2 (2.7), p = 0.04]. Sleep breathing disorders, disorders of excessive sleepiness and sleep hyperhidrosis scores were also higher among those SCA patients who were using hydroxyurea than those with SCA who were not using hydroxyurea [7.4 (3.9) vs. 5.9 (2.9); p = 0.026; 7.5 (3.5) vs. 6.9 (3); p = 0.028; 4.3 (3.1) vs. 3.9 (2.6); p = 0.044, respectively]. CONCLUSION: The SCA group presented higher sleep-disordered breathing scores and lower FMD values. Patients with SCA using hydroxyurea exhibited a higher frequency of scores on SDSC subscales. A positive correlation was found between SDSC subscales and cytokines.


Asunto(s)
Anemia de Células Falciformes/sangre , Anemia de Células Falciformes/complicaciones , Arteria Braquial/diagnóstico por imagen , Endotelio Vascular/fisiopatología , Trastornos del Sueño-Vigilia/complicaciones , Adolescente , Niño , Citocinas , Células Endoteliales/patología , Femenino , Humanos , Masculino , Ultrasonografía , Ultrasonografía Doppler
12.
Mol Biol Rep ; 45(6): 2045-2051, 2018 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-30232781

RESUMEN

Knowledge of the genetic profile of Cystic Fibrosis (CF) contributes to a better understanding of the genotype/phenotype relationship, particularly in mixed populations such as in Brazil. To describe clinical data of CF patients with rare or not yet observed CFTR gene mutations in Brazil. It was a case series of CF patients followed-up at a referral center. Clinical and laboratory data were obtained through medical records. Molecular analysis of the mutations was performed by conventional methods and/or by next-generation sequencing. Ten patients were studied, seven had five pathogenic mutations without previous description in Brazil (Q1100P, Y109C, A107P, E1409K and K162E), one of which has not yet been reported in patients with CF (A107P). Among the seven patients, three (two siblings) had the second mutant allele of rare occurrence among Brazilians patients (G1069R and 2307insA). Three other patients also had at least one rare variant (V201M, S466X and G1069R). The age of the CF diagnosis ranged from 1 to 190 months in the ten cases and the main clinical manifestations were respiratory symptoms and difficulty in gaining weight. All but one patient presented clinical and/or laboratory data compatible with pancreatic insufficiency. The identification of rare or not yet described CFTR mutations in patients with CF in Brazil highlights the high genetic heterogeneity in this population. Knowledge of the genotypic profile of Brazilian CF patients can contribute to the development of specific mutation panels for the genetic investigation targeting each region of the country, as well as helping to understand the complex genotype/phenotype relationship, especially in mixed populations.


Asunto(s)
Regulador de Conductancia de Transmembrana de Fibrosis Quística/genética , Fibrosis Quística/genética , Adolescente , Adulto , Alelos , Brasil , Niño , Preescolar , Femenino , Frecuencia de los Genes/genética , Heterogeneidad Genética , Predisposición Genética a la Enfermedad/genética , Pruebas Genéticas , Genotipo , Secuenciación de Nucleótidos de Alto Rendimiento/métodos , Humanos , Masculino , Mutación
13.
PLoS One ; 12(9): e0184076, 2017.
Artículo en Inglés | MEDLINE | ID: mdl-28863145

RESUMEN

BACKGROUND: Hematological changes can drive damage of endothelial cells, which potentially lead to an early endothelial dysfunction in patients with sickle cell anemia (SCA). An association may exist between endothelial dysfunction and several clinical manifestations of SCA. The present study aims to evaluate the links between changes in endothelial function and clinical and laboratory parameters in children and adolescents with SCA. METHODS: This study included 40 children and adolescents with stable SCA as well as 25 healthy children; aged 6-18 years. All study subjects were evaluated for endothelial function using Doppler ultrasonography. In addition, a number of laboratory assays were performed, including reticulocyte and leukocyte counts as well as measurement of circulating levels of total bilirubin, C-reactive protein (CRP), glucose, lipoproteins and peripheral oxyhemoglobin saturation. These parameters were also compared between SCA patients who were undertaking hydroxyurea (HU) and those who were not. RESULTS: Flow-mediated vasodilation (FMD) values were found to be reduced in SCA patients compared with those detected in healthy controls. SCA individuals with lower FMD values exhibited higher number of hospital admissions due to vaso-occlusive events. Additional analyses revealed that patients who had decreased FMD values exhibited higher odds of acute chest syndrome (ACS) episodes. A preliminary analysis with limited number of individuals failed to demonstrate significant differences in FMD values between SCA individuals who were treated with HU and those who were not. CONCLUSIONS: Children and adolescents with SCA exhibit impaired endothelial function. Reductions in FMD values are associated with ACS. These findings underline the potential use of FMD as screening strategy of SCA patients with severe prognosis at early stages.


Asunto(s)
Anemia de Células Falciformes/sangre , Anemia de Células Falciformes/complicaciones , Endotelio Vascular/fisiopatología , Enfermedades Vasculares/sangre , Enfermedades Vasculares/complicaciones , Adolescente , Antidrepanocíticos/uso terapéutico , Bilirrubina/sangre , Proteína C-Reactiva/análisis , Estudios de Casos y Controles , Niño , Células Endoteliales/patología , Femenino , Glucosa/análisis , Humanos , Hidroxiurea/uso terapéutico , Recuento de Leucocitos , Lipoproteínas/sangre , Masculino , Oxihemoglobinas/análisis , Reticulocitos/citología , Ultrasonografía Doppler , Enfermedades Vasculares/diagnóstico por imagen
14.
Hematology ; 22(5): 299-303, 2017 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-27934541

RESUMEN

OBJECTIVE: To evaluate the association between endothelial dysfunction and otoneurological symptoms and vaso-occlusive phenomena in children with sickle cell disease (SCD). METHODS: Cross-sectional study with 54 children, aged between 6 and19 years of age, of whom 28 had genotype SS and 26 apparently healthy (AA genotype) whose parents or guardians, or the children themselves, filled out a questionnaire designed to assess their otoneurological symptoms. All the individuals were submitted assessment of endothelial function by flow-mediated dilation (FMD) percentage with reactive hyperemia of brachial artery Doppler. RESULTS: Otoneurological symptoms (tinnitus and/or vertigo) predominated in the SCD group (46.4 vs. 15.4%; p = 0.006). A negative correlation was observed between FMD percentage and time of evolution of vertigo SCD (r = -0.432; p = 0.022) and the linear regression analysis demonstrated that for every reduction in FMD percentage there was an increase in time of evolution of vertigo of 1.79 months (ß = -1.79; p = 0.022). The positive correlation between episodes of painful crisis and time of evolution of vertigo (r = 0.3; p = 0.04). DISCUSSION: The presence of vascular endothelial damage in the labyrinthine artery in patients with SCD is capable of compromising the semicircular canals, shown by clinical expression of otoneurological symptoms, such as vertigo. In the present study, an association was observed between endothelial dysfunction with otoneurological symptoms and otoneurological symptoms and vaso-occlusive phenomena in SCD.


Asunto(s)
Anemia de Células Falciformes/fisiopatología , Arteria Braquial/fisiopatología , Endotelio Vascular/fisiopatología , Acúfeno/fisiopatología , Vértigo/fisiopatología , Adolescente , Anemia de Células Falciformes/complicaciones , Anemia de Células Falciformes/diagnóstico por imagen , Arteria Braquial/diagnóstico por imagen , Niño , Estudios Transversales , Ecocardiografía Doppler , Femenino , Humanos , Hiperemia , Masculino , Acúfeno/diagnóstico por imagen , Acúfeno/etiología , Vértigo/diagnóstico por imagen , Vértigo/etiología
15.
Fisioter. Bras ; 18(1): f: 69-I: 79, 2017.
Artículo en Portugués | LILACS | ID: biblio-883889

RESUMEN

Objetivos: Caracterizar o perfil ventilatório e a capacidade funcional de pacientes com mucopolissacaridoses (MPS). Métodos: O perfil ventilatório caracterizou-se pela espirometria e pela forma da caixa torácica. A capacidade funcional foi obtida pela distância percorrida no teste de caminhada de seis minutos (DTC6M) e por questionários funcionais, Childhood Health Assessment Questionaire (CHAQ) e Health Assessment Questionaire (HAQ). Resultados: Dezenove pacientes com MPS, maioria do sexo masculino 16 (84,2%) e com mediana e intervalo interquartilíco (IIQ) de idade de 13,4 (6,3) anos. O MPS VI foi o mais frequente, 57,9%. Alteração na espirometria em 17/18 pacientes, com predomínio para o distúrbio ventilatório restritivo (DVR), 72,2%. Apresentaram alteração da caixa torácica 73,7%. Houve piora da capacidade vital forçada (CVF) (% previsto) com o aumento da idade para todos os tipos (r = - 0,37) e, ao retirar da análise o tipo II, a correlação aumentou consideravelmente (r = -0,726). A DTC6M em mediana e IIQ foi de 349 (106,5) metros, com variação mediana percentual, 64,7%, da distância percorrida prevista obtida pela equação de referência para indivíduos saudáveis, 575,2 (128,5) metros. Nos questionários funcionais, 13/19 pacientes tiveram leve comprometimento da capacidade funcional. Conclusão: Houve alterações da função pulmonar na espirometria, da caixa torácica e comprometimento da capacidade funcional. (AU)


Aims: To characterize the ventilatory profile and functional capacity of patients with mucopolysaccharidosis. Methods: The ventilatory profile was characterized by spirometry and evaluating the shape of the rib cage. Functional capacity was obtained by the distance covered on the six-minute walk test (6MWD) and, through functional questionnaires, the Childhood Health Assessment Questionnaire (CHAQ) and the Health Assessment Questionnaire (HAQ). Results: Nineteen patients were evaluated with MPS, most males, 16 (84.2%) with median and interquartile range (IQR) age of 13.4 (6.3) years. The most common types were VI, 57.9%. Change in spirometry in 17/18 patients who were examined, with predominance of restrictive ventilatory disorder, 72.2%. 73.7% patients had abnormal rib cage. There was worsening on forced vital capacity (FVC) (% predicted) with increasing age for all types (r = -0.37) and when withdrawing from Type II analysis, the correlation increased significantly (r = -0.726). The 6MWD median was 349 (IQR = 106.5) meters, with median percentage change, 64.7%, of distance traveled expected and obtained by reference equation for healthy individuals, 575.2(128.5) meters. Thirteen among 19 patients were assessed by functional questionnaires with mild impairment of functional capacity. Conclusion: Alterations were found in lung function through spirometry, abnormal rib cage and impaired functional capacity. (AU)


Asunto(s)
Humanos , Masculino , Femenino , Niño , Adolescente , Adulto , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años , Actividades Cotidianas , Prueba de Esfuerzo , Mucopolisacaridosis , Enfermedad Pulmonar Obstructiva Crónica , Espirometría
16.
J Bras Pneumol ; 39(5): 604-12, 2013.
Artículo en Inglés, Portugués | MEDLINE | ID: mdl-24310634

RESUMEN

Symptoms of sleep-disordered breathing, especially obstructive sleep apnea syndrome (OSAS), are common in asthma patients and have been associated with asthma severity. It is known that asthma symptoms tend to be more severe at night and that asthma-related deaths are most likely to occur during the night or early morning. Nocturnal symptoms occur in 60-74% of asthma patients and are markers of inadequate control of the disease. Various pathophysiological mechanisms are related to the worsening of asthma symptoms, OSAS being one of the most important factors. In patients with asthma, OSAS should be investigated whenever there is inadequate control of symptoms of nocturnal asthma despite the treatment recommended by guidelines having been administered. There is evidence in the literature that the use of continuous positive airway pressure contributes to asthma control in asthma patients with obstructive sleep apnea and uncontrolled asthma.


Asunto(s)
Asma/epidemiología , Apnea Obstructiva del Sueño/epidemiología , Asma/terapia , Biomarcadores/sangre , Humanos , Inflamación/sangre , Leptina/sangre , Obesidad/epidemiología , Prevalencia , Rinitis/epidemiología , Factores de Riesgo , Apnea Obstructiva del Sueño/clasificación , Apnea Obstructiva del Sueño/terapia
17.
J. bras. pneumol ; 39(5): 604-612, Sep-Oct/2013. graf
Artículo en Inglés | LILACS | ID: lil-695177

RESUMEN

Symptoms of sleep-disordered breathing, especially obstructive sleep apnea syndrome (OSAS), are common in asthma patients and have been associated with asthma severity. It is known that asthma symptoms tend to be more severe at night and that asthma-related deaths are most likely to occur during the night or early morning. Nocturnal symptoms occur in 60-74% of asthma patients and are markers of inadequate control of the disease. Various pathophysiological mechanisms are related to the worsening of asthma symptoms, OSAS being one of the most important factors. In patients with asthma, OSAS should be investigated whenever there is inadequate control of symptoms of nocturnal asthma despite the treatment recommended by guidelines having been administered. There is evidence in the literature that the use of continuous positive airway pressure contributes to asthma control in asthma patients with obstructive sleep apnea and uncontrolled asthma.


Tem-se observado que sintomas dos distúrbios respiratórios do sono, especialmente a síndrome da apneia obstrutiva do sono (SAOS), são comuns em asmáticos; além disso, associam-se com a gravidade da asma. Sabe-se que durante a noite tende a haver maior gravidade dos sintomas da asma, assim como uma maior proporção de mortalidade durante a noite e as primeiras horas da manhã. Sintomas noturnos ocorrem entre 60-74% dos pacientes com asma e são marcadores de controle inadequado da doença. Vários mecanismos fisiopatológicos são relacionados a esse agravamento. A SAOS está incluída entre os fatores mais importantes. A investigação da SAOS em pacientes com asma deve ser realizada sempre que não houver um controle adequado dos sintomas noturnos da asma com o tratamento recomendado por diretrizes. Há evidências da literatura que sugerem que o uso de pressão positiva contínua nas vias aéreas pode contribuir para o controle da asma, quando o paciente asmático tem apneia obstrutiva do sono e sua asma não está controlada.


Asunto(s)
Humanos , Asma/epidemiología , Apnea Obstructiva del Sueño/epidemiología , Asma/terapia , Biomarcadores/sangre , Inflamación/sangre , Leptina/sangre , Obesidad/epidemiología , Prevalencia , Factores de Riesgo , Rinitis/epidemiología , Apnea Obstructiva del Sueño/clasificación , Apnea Obstructiva del Sueño/terapia
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