Cardiorespiratory fitness and mortality risk in patients receiving hemodialysis: a prospective cohort / Aptidão cardiorrespiratória e risco de mortalidade em pacientes em hemodiálise: uma coorte prospectiva

ABSTRACT Background: Kidney failure reduces life expectancy by one-third compared with the general population, and cardiovascular complications and poor cardiorespiratory fitness (CRF) are the main causes. We aimed to evaluate the association between severely low CRF and all-cause mortality risk in HD patients. Methods: This observational prospective cohort study followed-up patients receiving HD from August 2015 until March 2022. Cardiorespiratory fitness was evaluated through the cardiopulmonary exercise test, and the peak oxygen uptake (VO2peak) value was used to determine severely low CRF (< 15 mL∙kg−1∙min−1). Cox regression and univariate Kaplan-Meier analysis were used to evaluate the association of severely low CRF with mortality risk and survival rate. Results: Forty-eight patients were followed-up for a median of 33.0 [14.3 - 49.3] months. A total of 26 patients had severely low CRF. During the follow-up period, 11 patients (22.92%) died from all causes. From these, eight (30.8%) had severely low CRF. Even so, severely low CRF was not associated with crude death rates for patients stratified by CRF levels (p = 0.189), neither in unadjusted (HR 2.18; CI 95% 0.58−8.23) nor in adjusted (HR 1.32; CI 95% 0.31−5.59) Cox proportional hazard models. As a continuous variable, VO2peak was not associated with mortality risk (HR 1.01; CI 95% 0.84−1.21). Univariate Kaplan-Meier analysis showed that patients with severely low CRF did not have significantly worse survival rates than those with mild-moderate CRF (p = 0.186). Conclusion: Our findings indicated that severely low CRF was not associated with all-cause mortality in patients on HD. Despite severely low CRF being prevalent, larger cohort studies are needed to establish strong conclusions on its association with all-cause mortality.

RESUMO Introdução A insuficiência renal reduz a expectativa de vida em um terço comparada à população em geral. Complicações cardiovasculares e baixa aptidão cardiorrespiratória (ACR) são as principais causas. Avaliamos a associação entre ACR muito baixa e risco de mortalidade por todas as causas em pacientes em HD. Métodos Este estudo de coorte prospectivo observacional acompanhou pacientes em HD de agosto/2015 a março/2022. Avaliou-se a aptidão cardiorrespiratória pelo teste de exercício cardiopulmonar, e o valor do pico do consumo de oxigênio (VO2pico) foi usado para determinar ACR muito baixa (< 15 mL∙kg−1∙min−1). Utilizamos regressão de Cox e análise univariada de Kaplan-Meier para avaliar associação da ACR muito baixa com o risco de mortalidade e taxa de sobrevida. Resultados Acompanhamos 48 pacientes por uma média de 33,0 [14,3 - 49,3] meses. Um total de 26 pacientes apresentaram ACR muito baixa. No período de acompanhamento, 11 pacientes (22,92%) foram a óbito por todas as causas. Destes, oito (30,8%) apresentavam ACR muito baixa. Mesmo assim, ACR muito baixa não foi associada a taxas brutas de mortalidade para pacientes estratificados por níveis de ACR (p = 0,189), nem em modelos de risco proporcional de Cox não ajustados (HR 2,18; IC 95% 0,58-8,23) ou ajustados (HR 1,32; IC 95% 0,31-5,59). Como variável contínua, VO2pico não foi associado ao risco de mortalidade (HR 1,01; IC 95% 0,84-1,21). A análise univariada de Kaplan-Meier mostrou que pacientes com ACR muito baixa não apresentaram taxas de sobrevida significativamente piores do que aqueles com ACR leve-moderada (p = 0,186). Conclusão Nossos achados indicaram que a ACR muito baixa não foi associada à mortalidade por todas as causas em pacientes em HD. Apesar de ACR muito baixa ser prevalente, são necessários estudos de coorte maiores para estabelecer conclusões sólidas sobre sua associação com mortalidade por todas as causas.

Estimation of pulmonary artery systolic pressure in hemodialysis patients and its association with cardiorespiratory fitness and pulmonary function.

PURPOSE: To evaluate the estimated pulmonary arterial systolic pressure (PASP) through transthoracic echocardiography in hemodialysis (HD) patients and associate it with cardiorespiratory fitness and pulmonary function. METHODS: This study was a cross-sectional analysis of HD patients that performed evaluations of cardiac function, cardiorespiratory fitness, and pulmonary function, through transthoracic echocardiography, cardiopulmonary exercise test, spirometry, and manovacuometry, respectively. All patients underwent the evaluations on a non-dialysis day. RESULTS: Thirty-five HD patients were evaluated and separated according to the presence of probable pulmonary hypertension (PH) (estimated PASP ≥ 35 mmHg) or not (estimated PASP < 35 mmHg). Those HD patients with probable PH had the worst cardiorespiratory fitness, evaluated by the peak oxygen consumption (VO2peak) (17.11 ± 4.40 versus 12.90 ± 2.73 mL/kg/min; p = 0.011), and pulmonary function, evaluated by absolute and predicted of forced vital capacity (FVC) (85.52 ± 12.29 versus 71.38 ± 11.63%; p = 0.005) and absolute and predicted of forced expiratory volume in the first second (FEV1) (83.37 ± 14.98 versus 69.21 ± 13.48%; p = 0.017). The secondary analysis showed that estimated PASP was correlated with VO2peak (r = - 0.508; p = 0.002), FVC (r = - 0.450; p = 0.007), and FEV1 (r = - 0.361; p = 0.033). Moreover, the adjusted odds ratio by HD vintage, dry weight and gender showed that increments in VO2peak (OR 1.62; CI 95% 1.04-2.54; p = 0.034), FVC (OR 39.67; CI 95% 1.74-902.80; p = 0.021), and FEV1 (OR 39.54; CI 95% 1.89-826.99; p = 0.018) were associated with 1-fold and 39-fold higher chance, respectively, for not having PH. However, all these associations were lost when age was included in the analysis. CONCLUSIONS: The HD patients with probable PH had the worst cardiorespiratory fitness and pulmonary function. Exploratory analyses showed that greater cardiopulmonary fitness was associated with better cardiac function. Moreover, increments in cardiorespiratory fitness and pulmonary function may increase the chance of not having PH.

Efeitos da doença crítica no status funcional de crianças com histórico de prematuridade / Effects of critical illness on the functional status of children with a history of prematurity

RESUMO Objetivo: Avaliar os efeitos da doença crítica no status funcional de crianças de zero a 4 anos com e sem histórico de prematuridade após a alta da unidade de terapia intensiva pediátrica. Métodos: Estudo transversal secundário aninhado a uma coorte de caráter observacional de sobreviventes de uma unidade de terapia intensiva pediátrica. A avaliação funcional aconteceu por meio da Functional Status Scale no período de até 48 horas após a alta da unidade de terapia intensiva pediátrica. Resultados: Participaram do estudo 126 pacientes, sendo 75 prematuros e 51 nascidos a termo. Na comparação entre o status funcional basal e o status funcional da alta da unidade de terapia intensiva pediátrica, ambos os grupos apresentaram diferenças significativas (p < 0,001). Na alta da unidade de terapia intensiva pediátrica, as alterações funcionais aumentaram significativamente em ambos os grupos (p < 0,001). Os pacientes prematuros apresentaram maior declínio funcional na alta da unidade de terapia intensiva pediátrica (61%). Nos pacientes nascidos a termo, houve correlação significativa entre Pediatric Index of Mortality, tempo de sedação, tempo de ventilação mecânica e tempo de internação com os desfechos funcionais (p = 0,05). Conclusão: A maior parte dos pacientes estudados apresentou declínio funcional na alta da unidade de terapia intensiva pediátrica. Apesar de os pacientes prematuros apresentarem maior declínio funcional na alta, os pacientes nascidos a termo apresentaram influência do tempo de sedação e do tempo de uso de ventilação mecânica nos seus status funcionais.

ABSTRACT Objective: To evaluate the effects of critical illness on the functional status of children aged zero to 4 years with or without a history of prematurity after discharge from the pediatric intensive care unit. Methods: This was a secondary cross-sectional study nested in an observational cohort of survivors from a pediatric intensive care unit. Functional assessment was performed using the Functional Status Scale within 48 hours after discharge from the pediatric intensive care unit. Results: A total of 126 patients participated in the study, 75 of whom were premature, and 51 of whom were born at term. Comparing the baseline and functional status at pediatric intensive care unit discharge, both groups showed significant differences (p < 0.001). Preterm patients exhibited greater functional decline at discharge from the pediatric intensive care unit (61%). Among patients born at term, there was a significant correlation between the Pediatric Index of Mortality, duration of sedation, duration of mechanical ventilation and length of hospital stay with the functional outcomes (p = 0.05). Conclusion: Most patients showed a functional decline at discharge from the pediatric intensive care unit. Although preterm patients had a greater functional decline at discharge, sedation and mechanical ventilation duration influenced functional status among patients born at term.

Intradialytic periodized exercise improves cardiopulmonary fitness and respiratory function: A randomized controlled trial.

BACKGROUND: Chronic kidney disease (CKD) is a factor that predisposes to gradual physical deconditioning from its early stages leading to cardiorespiratory fitness and musculoskeletal system impairment. We evaluated the effects of combined and periodized intradialytic exercise training on cardiopulmonary fitness and respiratory function in HD subjects. METHODS: A randomized controlled trial with HD subjects was allocated into two groups: exercise group (EXG) and usual care group (UCG). EXG performed a 12-week combined and periodized intradialytic training. UCG maintained the HD routine. RESULTS: Thirty-nine HD subjects were analyzed (EXG = 20; UCG = 19). The EXG in comparison with the UCG showed improvements in peak oxygen consumption (Δ3.1[0.4-5.5] vs. -0.2[-2.0-1.5] ml/kg/min; p = 0.003), forced expiratory volume in the first second (Δ0.1[-0.0-0.1] vs. -0.0[-0.1-0.0] L; p = 0.022), forced vital capacity (Δ0.1[0.0-0.2] vs. -0.1[-0.2-0.0] L; p = 0.005), peak expiratory flow (Δ0.4[-0.7-1.2] vs. -0.1[-0.5-0.2] L; p = 0.046), and maximal inspiratory pressure (Δ7.35[-8.5-17.5] vs. -4.0[-18.0-12.0] cmH2 O; p = 0.028). The EXG, different from the UCG, did not worsen the maximal expiratory pressure (Δ0.1[-8.8-7.5] vs. -2.5[-15.0-9.0] cmH2 O; p = 0.036). Besides, EXG showed a significant improvement in quadriceps strength (32.05 ± 10.61 vs. 33.35 ± 11.62 kg; p = 0.042). CONCLUSIONS: The combined and periodized intradialytic exercise training improved cardiopulmonary fitness, respiratory function, inspiratory muscle strength, and quadriceps strength, beyond maintaining the expiratory muscle strength in HD subjects.

Repeatability of the 6-min walk test in non-cystic fibrosis bronchiectasis.

Non-cystic fibrosis bronchiectasis (NCFB) is a chronic lung disease characterized by progressive and irreversible changes of the bronchial tree. The evaluation of exercise capacity is essential to manage this disease. This study aims to determine the within-subject repeatability of two Six Minute Walk Test (6MWT) in adults with NCFB. NCFB. This cross-sectional observational study included 66 NCFB subjects above 18 years-old (mean of 55 ± 17 years old, 68% women). 73% of the participants presented moderate to severe clinical condition classified by Bronchiectasis Severity Index. It showed that these participants walked 16.6 m less (95%CI 3.8 to 29.4; p < 0.01) in the second 6MWT when compared to the first test, with a within-subject coefficient variation of 9.4% (95%CI 7.2-11.2%) and an intra-test reliability with a high intraclass correlation coefficient of 0.88 (95%CI 0.80-0.93). Bland-Altman plot showed an agreement regarding test repeatability, besides presented a large limit of agreement (- 85 to 116 m). Respiratory rate and systolic blood pressure were significantly higher before starting the second test. In conclusion, 6MWT seems to be reproducible in NCFB subjects and vital sign verification should be attentively checked to assess if the patient is fully recovered to perform a second test, as well as the disease severity score. Other studies on this matter should be conducted with a larger number of participants to confirm the findings of the present study.

Physical activity and quality of life of children and adolescents with cystic fibrosis: a cross-sectional study / Atividade física e qualidade de vida de crianças e adolescentes com fibrose cística: um estudo transversal

Abstract Introduction: Regular physical activity (PA) is part of outpatient care offered to most people with cystic fibrosis (CF). Objective: To determine the frequency of the self-reported PA and, secondarily evaluate associations between PA with clinical variables and health-related quality of life (HRQoL) in pediatric and adolescent CF subjects. Method: In this cross-sectional study, subjects between 6 and 17 years of age with a confirmed diagnosis of CF were recruited in the outpatient clinic. The subjects answered questions about the practices of PA, HRQoL questionnaire and clinical variables were collected. Subjects were classified into two groups according to weekly PA practice: PA performed ≥ 3 times/week and performed PA ≤ 2 times/week. Results: 66 subjects completed the study, 72.7% (n=48) had PA ≥ 3 times/week and 27.2% (n=18) had PA ≤ 2 times/week. Only twelve children (18,2%) reached the recommendations of the World Health Organization (WHO) to practice moderate to vigorous physical activities daily. The mean age of the subjects evaluated was 12.3 ± 3.2 years, forced expiratory volume in one second 90 ± 24.1% and forced vital capacity 95 ± 20.4%. The group that performed PA ≥ 3 times/week had a better clinical score (p=0.033), a lower number of hospitalizations in the year (p=0.002), a lower number of days hospitalized in the last year (p=0.020) and better score for the physical (p=0.003) and emotion (p=0.048) domains in HRQoL questionnaire. Conclusion: This study concluded that most subjects did not reach the WHO recommendations for the practice of PA. However, individuals who practice PA at least 3 times/week have better HRQoL, clinical score and fewer hospitalizations.

Resumo Introdução: A atividade física regular (AF) faz parte do tratamento ambulatorial oferecido à maioria das pessoas com fibrose cística (FC). Objetivo: Determinar a frequência da AF autorreferida e secundariamente avaliar as associações entre AF e variáveis clínicas e de qualidade de vida relacionada à saúde (QVRS) em crianças e adolescentes com FC. Método: Neste estudo transversal, indivíduos entre 6 e 17 anos com diagnóstico confirmado de FC foram recrutados no ambulatório. Os sujeitos responderam perguntas sobre as práticas de AF, questionário de QVRS e variáveis clínicas. Os indivíduos foram classificados em dois grupos de acordo com a prática semanal de AF: AF realizada ≥3 vezes/semana e AF≤2 vezes/semana. Resultados: 66 indivíduos completaram o estudo, 72,7% (n=48) tinham AF ≥ 3 vezes/semana e 27,2% (n=18) tinham AF ≤ 2 vezes/semana. Apenas doze crianças (18,2%) alcançaram as recomendações da Organização Mundial da Saúde (OMS) para praticar diariamente atividades físicas moderadas a vigorosas. A média de idade dos sujeitos avaliados foi de 12,3 ± 3,2 anos, volume expiratório forçado no primeiro segundo 90 ± 24,1% e capacidade vital forçada 95 ± 20,4%. O grupo que realizou AF ≥ 3 vezes/semana apresentou melhor escore clínico (p=0,033), menor número de internações no ano (p=0,002), menor número de dias hospitalizados no último ano (p=0,020) e melhor escore para os domínios físico (p=0,003) e emoção (p=0,048) no questionário de QVRS. Conclusão: Este estudo concluiu que a maioria dos indivíduos não alcançou as recomendações da OMS para a prática de AF. No entanto, indivíduos que praticam AF pelo menos 3 vezes/semana apresentam melhor QVRS, escore clínico e menos hospitalizações.

Peripheral muscle strength is associated with lung function and functional capacity in patients with cystic fibrosis.

PURPOSE: Cystic fibrosis (CF) is an inherited disease that causes important multisystemic impairments. The present study aimed to evaluate the association of peripheral muscle strength with lung function and functional capacity in adolescents and adults with CF. METHODS: Cross-sectional study with prospective data of patients enrolled in the Program for Adults with CF at Hospital de Clínicas de Porto Alegre. The testing procedures included peripheral muscle strength testing, pulmonary function tests, and the 6-minute walk test. RESULTS: The sample consisted of 41 subjects (27 women) with a mean age of 24.6. Upper extremity muscle strength was associated with forced vital capacity and forced expiratory volume in the first second, and lower extremity muscle strength was associated with the distance covered in the 6-minute walk test, oxygen saturation, forced expiratory volume in the first second, and forced vital capacity. CONCLUSIONS: Muscle strength was positively associated with lung function variable and functional capacity in patients with CF.

Associação da função pulmonar e capacidade funcional com o tempo de ventilação mecânica invasiva após cirurgia de revascularização do miocárdio / Association of pulmonary function and functional capacity with invasive mechanical ventilation time after coronary artery bypass grafting

Introdução: A cirurgia de revascularização do miocárdio (CRM) é realizada para diminuir os sintomas característicos da doença arterial coronariana (DAC) e após esse procedimento os pacientes necessitam de ventilação mecânica invasiva (VMI). Um tempo prolongado de VMI resulta no aumento de complicações pulmonares, mortalidade e prolonga o tempo de internação hospitalar. Objetivo: Verificar a associação entre a função pulmonar e a capacidade funcional pré-operatória com o tempo de VMI após CRM. Casuística eMétodos: Estudo decoorte prospectivo realizado entre 2011 e 2015 com inclusão de40 indivíduos. Foram avaliadas a capacidade funcional e função pulmonar no pré-operatório da CRM, além do tempo de VMIno momento após o procedimento cirúrgico. Resultados: Asvariáveis da função pulmonar apresentaram correlação inversacom o tempo de VMI pós CRM, capacidade vital forçada(CVF) (r = -0,51; p = 0,001); o volume expiratório forçadono 1º segundo (VEF1) (r = -0,49; p = 0,001), o pico de fluxoexpiratório (PFE) (r = -0,42; p = 0,008) e a capacidade vitalinspiratória (CVin) (r = -0,51; p = 0,001). A mesma correlaçãonão foi observada entre a capacidade funcional (r = -0,22; p =0,166) e o tempo de VMI pós CRM. Conclusão: Os pacientesque apresentaram melhor função pulmonar no pré-operatóriode CRM, necessitaram um menor tempo de VMI após essacirurgia. O mesmo não foi observado em relação a capacidadefuncional.

Introduction: The coronary artery bypass grafting (CABG) is widely utilized to decrease the symptoms of coronary artery disease. After this surgery the patients will need mechanical ventilation. Prolonged mechanical ventilation results in higher rates of pulmonary complications and mortality, as well as higher hospital length of stay. Objective: To evaluate the association between pulmonary function, functional capacity, and the duration of invasive mechanical ventilation after CABG. Patients andMethods: The prospective cohort study was carried out during 2011 and 2015. In total, 40 patients undergoing CABG were evaluated for preoperative pulmonary function and functional capacity. Results: Preoperative pulmonary function was found to be inversely proportional to duration of invasive mechanical ventilation after CABG, forced vital capacity (FVC) (r = -0,5; p = 0,001), forced expiratory volume in 1 second (FEV1) (r = -0,49; p = 0,001), peak expiratory flow (PEF) (r = -0,42; p = 0,008), inspiratory vital capacity (IVC) (r = -0,51; p = 0,001). However, there was no correlation between functional capacity (r = -0,22; p = 0,166) and duration of invasive mechanical ventilation after CABG. Conclusion: The patients whose pulmonary function were significantly higher remained less time under ventilation than the patients with poorer pulmonary function. However, there was no relation between functional capacity and duration of invasive mechanical ventilation in these patients.

Respiratory therapy: a problem among children and adolescents with cystic fibrosis.

OBJECTIVE: To evaluate the level of self-reported adherence to physical therapy recommendations in pediatric patients (6-17 years) with cystic fibrosis (CF) and to ascertain whether the different levels of adherence correlate with pulmonary function, clinical aspects, and quality of life. METHODS: This was a cross-sectional study. The patients and their legal guardians completed a questionnaire regarding adherence to physical therapy recommendations and a CF quality of life questionnaire. We collected demographic, spirometric, and bacteriological data, as well as recording the frequency of hospitalizations and Shwachman-Kulczycki (S-K) clinical scores. RESULTS: We included 66 patients in the study. Mean age, FEV1 (% of predicted), and BMI were 12.2 ± 3.2 years, 90 ± 24%, and 18.3 ± 2.5 kg/m2, respectively. The patients were divided into two groups: high-adherence (n = 39) and moderate/poor-adherence (n = 27). No statistically significant differences were found between the groups regarding age, gender, family income, and total S-K clinical scores. There were statistically significant differences between the high-adherence group and the moderate/poor-adherence group, the latter showing lower scores for the "radiological findings" domain of the S-K clinical score (p = 0.030), a greater number of hospitalizations (p = 0.004), and more days of hospitalization in the last year (p = 0.012), as well as lower scores for the quality of life questionnaire domains emotion (p = 0.002), physical (p = 0.019), treatment burden (p < 0.001), health perceptions (p = 0.036), social (p = 0.039), and respiratory (p = 0.048). CONCLUSIONS: Low self-reported adherence to physical therapy recommendations was associated with worse radiological findings, a greater number of hospitalizations, and decreased quality of life in pediatric CF patients.

Respiratory therapy: a problem among children and adolescents with cystic fibrosis / Fisioterapia respiratória: um problema de crianças e adolescentes com fibrose cística

Objective : To evaluate the level of self-reported adherence to physical therapy recommendations in pediatric patients (6-17 years) with cystic fibrosis (CF) and to ascertain whether the different levels of adherence correlate with pulmonary function, clinical aspects, and quality of life. Methods : This was a cross-sectional study. The patients and their legal guardians completed a questionnaire regarding adherence to physical therapy recommendations and a CF quality of life questionnaire. We collected demographic, spirometric, and bacteriological data, as well as recording the frequency of hospitalizations and Shwachman-Kulczycki (S-K) clinical scores. Results : We included 66 patients in the study. Mean age, FEV1 (% of predicted), and BMI were 12.2 ± 3.2 years, 90 ± 24%, and 18.3 ± 2.5 kg/m2, respectively. The patients were divided into two groups: high-adherence (n = 39) and moderate/poor-adherence (n = 27). No statistically significant differences were found between the groups regarding age, gender, family income, and total S-K clinical scores. There were statistically significant differences between the high-adherence group and the moderate/poor-adherence group, the latter showing lower scores for the "radiological findings" domain of the S-K clinical score (p = 0.030), a greater number of hospitalizations (p = 0.004), and more days of hospitalization in the last year (p = 0.012), as well as lower scores for the quality of life questionnaire domains emotion (p = 0.002), physical (p = 0.019), treatment burden (p < 0.001), health perceptions (p = 0.036), social (p = 0.039), and respiratory (p = 0.048). Conclusions : Low self-reported adherence to physical therapy recommendations was associated with worse radiological findings, a greater number of hospitalizations, and decreased quality of life in pediatric CF patients.

Objetivo : Avaliar o grau de adesão autorrelatada às recomendações fisioterapêuticas em pacientes pediátricos (6-17 anos) com fibrose cística (FC) e determinar se os diferentes níveis de adesão se correlacionam com a função pulmonar, aspectos clínicos e qualidade de vida. Métodos : Estudo transversal no qual os pacientes e responsáveis responderam um questionário sobre a adesão à fisioterapia recomendada e um questionário da qualidade de vida em FC. Foram coletados dados demográficos, espirométricos e bacteriológicos, assim como a frequência de internações e resultados do escore clínico de Shwachman-Kulczycki (S-K). Resultados : Participaram 66 pacientes. As médias de idade, VEF1 (em % do previsto) e IMC foram, respectivamente, 12,2 ± 3,2 anos, 90 ± 24% e 18,3 ± 2,5 kg/m2. Os pacientes foram divididos em dois grupos: alta adesão (n = 39) e moderada/baixa adesão (n = 27). Não houve diferenças estatisticamente significativas para idade, sexo, renda familiar e escore clínico de S-K total na comparação dos dois grupos. Houve diferenças estatisticamente significativas entre os grupos alta adesão e moderada/baixa adesão, este último mostrando valores significativamente menores para o domínio "achados radiológicos" do escore clínico de S-K apresentou (p = 0,030), um maior número de hospitalizações (p = 0,004) e de dias de internação no último ano (p = 0,012), assim como menores escores para os seguintes domínios do questionário de qualidade de vida: emocional (p = 0,002), físico (p = 0,019), tratamento (p < 0,001), saúde (p = 0,036), social (p = 0,039) e respiratório (p = 0,048). Conclusões : A baixa adesão autorrelatada às recomendações fisioterapêuticas associou-se com piores achados radiológicos, maior número de hospitalizações e diminuição da qualidade de vida em pacientes pediátricos com FC.

Clinical Outcomes and Prognostic Factors in a Cohort of Adults With Cystic Fibrosis: A 7-Year Follow-Up Study.

BACKGROUND: Due to the heterogeneity of cystic fibrosis (CF), the longer survival observed in cohorts of adult subjects, and inter-population variations, there is a clear need to seek further information about clinical outcomes and prognostic factors in different cohorts of subjects with CF. Our objectives were to evaluate clinical outcomes and prognostic factors in a cohort of adult subjects with CF after a 7-y follow-up period and investigate longitudinal changes in clinical scores, spirometry, 6-min walk test performance, and pulmonary artery systolic pressure as assessed by Doppler echocardiography. METHODS: A cohort of clinically stable subjects (≥16 y old) who were enrolled in an adult CF program in 2004-2005 underwent clinical evaluation. Outcome was classified as good (survival) or poor (survival with lung transplantation or death). In 2011-2012, survivors were re-examined. RESULTS: Of 40 subjects with CF evaluated in 2004-2005, 32 (80%) survived, 2 (5%) survived with lung transplantation, and 6 (15%) died. Logistic regression analysis showed that a low percent-of-predicted FEV1 was associated with poor outcome. An FEV1 cut-off value of ≤30% and pulmonary artery systolic pressure of ≥42 mm Hg predicted poor outcome with high sensitivity, specificity, and positive and negative predictive values. Deterioration was observed in clinical scores (P = .03), FVC (P = .02), FEV1 (P < .001), distance walked in the 6-min walk test (P = .002), baseline SpO2 (P < .001), and final SpO2 (P < .001). CONCLUSIONS: After 7 y of follow-up, 20% of subjects with CF had a poor outcome. Pulmonary artery systolic pressure of ≥42 mm Hg and FEV1 of ≤30% were the most significant prognostic predictors of poor outcome. Clinical and functional deterioration was observed in survivors.

Exercise programme in patients with cystic fibrosis: a randomized controlled trial.

OBJECTIVES: Assess the effects of a home exercise programme, based on aerobic training and muscle strength training, in patients with cystic fibrosis (CF), for a period of 3 months. METHODS: Randomised controlled clinical experiment, with an analysis of intention to treat including clinically stable patients with CF and of age ≥ 16. Assessments include: a 6 min walk test (6 MWT), one-repetition maximum strength test (1 RM), spirometry and quality of life questionnaires. The patients randomised for the exercise group exercise group followed a home exercise protocol, supervised by telephone, while the control group maintained their usual activities. RESULTS: 41 Patients were included, 22 in the control group and 19 in the exercise group. The exercise group presented a significant increase in muscle strength in upper limbs (UULL) on the 1 RM test. There was no significant difference between groups on the scores for general quality of life and specifically for CF and in the distance walked on the 6 MWT. CONCLUSION: The study demonstrated that a home exercise programme had positive effects in adult patients with CF, including gain in muscle strength in UULL. No increase in tolerance to exercise was shown and improvement in the quality of life of the patients who received intervention.

Adherence to airway clearance therapies by adult cystic fibrosis patients.

BACKGROUND: Airway clearance therapy (ACT) is critical in cystic fibrosis (CF). OBJECTIVES: To determine rates of self-reported adherence to ACT by patients treated in an adult CF program, to identify patient characteristics associated with poor adherence, to typify adherence according to ACT technique, and to indicate reasons for poor adherence. METHODS: Our cross-sectional study included CF subjects age 16 years and older. Enrollees were evaluated via general structured questionnaire, adherence questionnaire, clinical assessment, spirometry, and S(pO(2)) values. Each was stratified by self-reporting protocol as high, moderate, or poor adherence to ACT. Concordance between physiotherapist recommended ACT technique and self-reported subject adherence was subjected to agreement analysis. RESULTS: Of the 63 subjects studied, 38 (60%) qualified as high adherence, 12 (19%) as moderate adherence, and 13 (21%) as poor adherence. Logistic regression identified education level (less than high school) as an independent factor associated with poor adherence (odds ratio 10.2, 95% CI 1.23-84.7, P = .03). Positive expiratory pressure (κ = 0.87) and flutter device (κ = 0.63) usage both corresponded with a high level of agreement, while active cycle of breathing technique (κ = 0.40) and autogenic drainage (κ = 0.39) each showed moderate agreement. Agreement was low for percussion and postural drainage (κ = 0.23). Reasons given most frequently for poor adherence to ACT were "not enough time to do ACT" (28%), "cannot be bothered" (16%), and "do not enjoy ACT technique" (8%). Many (32%) provided no reason. CONCLUSIONS: Study outcomes showed a high rate of ACT adherence in adult CF subjects. Lower level of education was the most important factor in poor adherence to ACT. Self-reported adherence and treatment recommendations were in best agreement with positive expiratory pressure and flutter device techniques.

Glucose intolerance in patients with cystic fibrosis: sex-based differences in clinical score, pulmonary function, radiograph score, and 6-minute walk test.

OBJECTIVE: To determine the sex-specific relationships between glucose intolerance and Shwachman-Kulczycki clinical score, lung function, Brasfield chest-radiograph score, and exercise capacity in patients with cystic fibrosis (CF). METHODS: We used a cross-sectional study design and included CF patients ≥ 10 years old. All patients had clinical and nutritional evaluation, oral glucose tolerance test, spirometry, chest radiograph, and 6-min walk test (6MWT). Patients were classified as having normal glucose tolerance, impaired glucose tolerance, or CF-related diabetes mellitus. RESULTS: We included 88 patients: 59 with normal glucose tolerance, 15 with impaired glucose tolerance, and 14 with CF-related diabetes. Shwachman-Kulczycki clinical score (P = .04), at-rest S(pO(2)) (P = .001), S(pO(2)) difference before versus after 6MWT (P = .001), and Brasfield chest-radiograph score (P = .01) were significantly lower in the impaired-glucose-tolerance group than in the normal-glucose-tolerance group, but did not differ from the CF-related-diabetes group. In female patients only, percent-of-predicted FEV(1) was significantly lower in the impaired-glucose-tolerance group than in the normal-glucose-tolerance group (P = .02), but did not differ from the CF-related-diabetes group (P = .10). There was a significant relationship between glucose intolerance and sex when clinical score, 6-min walk distance, FEV(1), and radiograph score were combined in a multivariate analysis. CONCLUSIONS: In patients with CF, glucose intolerance was associated with poor clinical score, lower at-rest S(pO(2)), greater S(pO(2)) difference before versus after 6MWT, poor lung function, and lower radiograph score. Overall, multivariate analysis indicated poorer performance in the latter variables in female patients with glucose intolerance than in male patients with glucose intolerance.

Repeatability of the 6-minute walk test in adolescents and adults with cystic fibrosis.

OBJECTIVE: To determine the repeatability of the 6-minute walk test (6MWT) in adolescents and adults with cystic fibrosis (CF). METHODS: This was a prospective cross-sectional study. We included consecutive patients ages>or=15 years attending an adult CF program. The patients underwent the 6MWT, pulmonary function tests, and clinical evaluation. The second 6MWT was performed following a rest period of 60 min. RESULTS: Thirty-one patients were included. The mean+/-SD age was 23.5+/-6.7 y, and the mean FEV1 was 61+/-28% of predicted. The mean+/-SD walked distance in the first 6MWT was 583.6+/-68.6 m and in the second 6MWT was 590.0+/-72.2 m. The mean difference between the first and second 6MWT was -6.5 m, with limits of agreement between -74.9 m and 61.9 m, and the coefficient of variation was 4.3%. The mean oxygen desaturation in the first 6MWT was 2.5+/-4.5%, and in the second test it was 1.8+/-4.0%. The mean difference between the first and second test was 0.6%, and the coefficient of variation was 104%. CONCLUSIONS: Although the 6MWT distance was reproducible, the wide limits of agreement exceeded the minimum important difference for this test. These findings indicate that, in the routine evaluation of CF patients, at least two 6MWTs are required on any testing occasion to obtain a reliable estimate of the 6MWT distance.

Predictors of oxygen desaturation during the six-minute walk test in patients with cystic fibrosis.

OBJECTIVE: To identify the predictive factors of oxygen desaturation during the six-minute walk test (6MWT) in patients with cystic fibrosis (CF). METHODS: Prospective cross-sectional study involving clinically stable patients with CF aged > or = 10 years. The patients were submitted to nutritional evaluations, oral glucose tolerance tests, pulmonary function tests, chest X-rays and 6MWTs. RESULTS: The study included 88 patients (43 females and 45 males; mean age, 19.9 + or - 7.2 years; mean FEV(1), 65.4 + or - 28.4%). We observed oxygen desaturation in 13 patients (OD+ group) and no oxygen desaturation in 75 (ODmicro group). In comparison with ODmicro group patients, OD+ group patients presented higher mean age (p = 0.004), worse clinical score (p < 0.001), worse radiological score (p < 0.001), higher incidence of glucose intolerance (p = 0.004), lower incidence of methicillin-sensitive Staphylococcus aureus infection (p < 0.001), higher incidence of methicillin-resistant S. aureus infection (p = 0.016), higher incidence of Pseudomonas aeruginosa infection (p = 0.008), lower mean resting SpO(2) (p < 0.001) and lower mean FEV(1) (p < 0.001). In the logistic regression analysis, oxygen desaturation during the 6MWT correlated with resting SpO(2) (OR = 0.305, p < 0.001) and FEV(1) (OR = 0.882, p = 0.025). The parameters maximizing the predictive value for oxygen desaturation were resting SpO(2) < 96% and FEV(1) < 40%. In this sample, 15% of the patients with CF aged > or = 10 years presented oxygen desaturation during the 6MWT. CONCLUSIONS: Resting SpO(2) < 96% and FEV(1) < 40% can predict oxygen desaturation during the 6MWT.

Respiratory patterns in spirometric tests of adolescents and adults with cystic fibrosis.

OBJECTIVE: To evaluate spirometric patterns of respiratory disorders and their relationship with functional severity and maximal expiratory flows at low lung volumes in patients with cystic fibrosis (CF). METHODS: A retrospective cross-sectional study including adolescents and adults with CF. All of the patients were submitted to spirometry. Patients were classified as having preserved respiratory function, obstructive lung disease (OLD), OLD with reduced FVC, presumptive restrictive lung disease (RLD) or mixed obstructive and restrictive lung disease (MORLD). Maximal expiratory flows at low lung volumes were assessed using FEF(25-75%), FEF(75%) and FEF(75%)/FVC. We included 65 normal subjects, also submitted to spirometry, as a control group. RESULTS: The study group included 65 patients: 8 (12.3%) with preserved lung function; 18 (27.7%) with OLD; 24 (36.9%) with OLD and reduced FVC; 5 (7.7%) with presumptive RLD; and 10 (15.4%) with MORLD. The FEV1 was significantly lower in the OLD with reduced FVC group and the MORLD group than in the other groups (p < 0.001). In the patients with preserved respiratory function, FEF(25-75%) and FEF(75%) were significantly reduced in 1 patient, as was FEF(75%)/FVC in 2 patients. CONCLUSIONS: The respiratory pattern was impaired in 88% of the patients with CF. The most common pattern was OLD with reduced FVC. The degree of functional impairment was greater in the OLD with reduced FVC group and in the MORLD group than in the other groups. Maximal expiratory flows at low lung volumes were impaired in a low percentage of patients with preserved respiratory function.

Preditores da dessaturação do oxigênio no teste da caminhada de seis minutos em pacientes com fibrose cística / Predictors of oxygen desaturation during the six-minute walk test in patients with cystic fibrosis

OBJETIVO: Identificar os fatores preditores de dessaturação de oxigênio durante o teste de caminhada de seis minutos (TC6) em pacientes com fibrose cística (FC). MÉTODOS: Estudo transversal e prospectivo em pacientes com FC clinicamente estáveis com idade superior a 10 anos. Os pacientes foram submetidos à avaliação nutricional, teste oral de tolerância à glicose, testes de função pulmonar, exame radiológico do tórax e TC6. RESULTADOS: Foram incluídos 88 pacientes (43 femininos e 45 masculinos) com média de idade de 19,9 ± 7,2 anos e média de VEF1 de 65,4 ± 28,4 por cento. Observamos que 75 pacientes apresentaram-se sem dessaturação de oxigênio (SD) e 13 com dessaturação (CD). Os pacientes do grupo CD apresentaram maior média de idade (p = 0,004), pior escore clínico (p < 0,001), pior escore radiológico (p < 0,001), maior frequência de intolerância à glicose (p = 0,004), menor frequência de infecção bacteriana por Staphylococcus aureus sensível à meticilina (p < 0,001), maior frequência de infecção por S. aureus resistente à meticilina (p = 0,016) e por Pseudomonas aeruginosa (p = 0,008) e menor valor médio de SpO2 em repouso (p < 0,001) e de VEF1 (p < 0,001) do que os pacientes do grupo SD. Na análise de regressão logística, SpO2 em repouso (OR = 0,305, p < 0,001) e VEF1 (OR = 0,882, p = 0,025) se associaram com a dessaturação de oxigênio no TC6. Os parâmetros que maximizaram o valor preditivo para dessaturação de oxigênio foram SpO2 em repouso < 96 por cento e VEF1 < 40 por cento. Nessa amostra, 15 por cento dos pacientes com FC com mais de 10 anos apresentaram dessaturação de oxigênio no TC6. CONCLUSÕES: Os parâmetros SpO2 em repouso < 96 por cento e VEF1 < 40 por cento contribuem como preditores de dessaturação no TC6.

OBJECTIVE: To identify the predictive factors of oxygen desaturation during the six-minute walk test (6MWT) in patients with cystic fibrosis (CF). METHODS: Prospective cross-sectional study involving clinically stable patients with CF aged > 10 years. The patients were submitted to nutritional evaluations, oral glucose tolerance tests, pulmonary function tests, chest X-rays and 6MWTs. RESULTS: The study included 88 patients (43 females and 45 males; mean age, 19.9 ± 7.2 years; mean FEV1, 65.4 ± 28.4 percent). We observed oxygen desaturation in 13 patients (OD+ group) and no oxygen desaturation in 75 (ODµ group). In comparison with ODµ group patients, OD+ group patients presented higher mean age (p = 0.004), worse clinical score (p < 0.001), worse radiological score (p < 0.001), higher incidence of glucose intolerance (p = 0.004), lower incidence of methicillin-sensitive Staphylococcus aureus infection (p < 0.001), higher incidence of methicillin-resistant S. aureus infection (p = 0.016), higher incidence of Pseudomonas aeruginosa infection (p = 0.008), lower mean resting SpO2 (p < 0.001) and lower mean FEV1 (p < 0.001). In the logistic regression analysis, oxygen desaturation during the 6MWT correlated with resting SpO2 (OR = 0.305, p < 0.001) and FEV1 (OR = 0.882, p = 0.025). The parameters maximizing the predictive value for oxygen desaturation were resting SpO2 < 96 percent and FEV1 < 40 percent. In this sample, 15 percent of the patients with CF aged > 10 years presented oxygen desaturation during the 6MWT. CONCLUSIONS: Resting SpO2 < 96 percent and FEV1 < 40 percent can predict oxygen desaturation during the 6MWT.

Padrões ventilatórios na espirometria em pacientes adolescentes e adultos com fibrose cística / Respiratory patterns in spirometric tests of adolescents and adults with cystic fibrosis

OBJETIVO: Avaliar os padrões dos distúrbios ventilatórios observados na espirometria em pacientes com fibrose cística (FC) e suas relações com a gravidade funcional e com o comportamento dos fluxos máximos expiratórios a baixos volumes. MÉTODOS: Estudo transversal e retrospectivo, incluindo pacientes adolescentes e adultos com FC. Todos os pacientes foram submetidos à espirometria. Os pacientes foram classificados como tendo função ventilatória preservada, distúrbio ventilatório obstrutivo (DVO), DVO com CVF reduzida, sugestivo de distúrbio ventilatório restritivo (DVR) ou distúrbio ventilatório combinado (DVC). Os fluxos máximos expiratórios a baixos volumes foram avaliados utilizando-se FEF25-75 por cento, FEF75 por centoe FEF75 por cento/CVF. O grupo controle incluiu 65 indivíduos normais, também submetidos à espirometria. RESULTADOS: Foram incluídos 65 pacientes no grupo de estudo: 8 (12,3 por cento) com função pulmonar preservada, 18 (27,7 por cento) com DVO, 24 (36,9 por cento) com DVO com CVF reduzida, 5 (7,7 por cento) com padrão sugestivo de DVR e 10 (15,4 por cento) com DVC. O VEF1 foi significativamente menor nos grupos DVO com CVF reduzida e DVC, comparados com os outros grupos (p < 0,001). Nos pacientes com função ventilatória preservada, FEF25-75 por cento e FEF75 por cento foram significativamente reduzidos em 1 paciente, assim como FEF75 por cento/CVF em 2 pacientes. CONCLUSÕES: O padrão ventilatório estava alterado em 88 por cento dos pacientes com FC. O distúrbio mais frequente foi DVO com CVF reduzida. Houve maior prejuízo funcional nos pacientes com DVO e CVF reduzida e com DVC. Os fluxos expiratórios máximos a baixos volumes foram alterados em uma pequena percentagem de pacientes com função pulmonar preservada.

OBJECTIVE: To evaluate spirometric patterns of respiratory disorders and their relationship with functional severity and maximal expiratory flows at low lung volumes in patients with cystic fibrosis (CF). METHODS: A retrospective cross-sectional study including adolescents and adults with CF. All of the patients were submitted to spirometry. Patients were classified as having preserved respiratory function, obstructive lung disease (OLD), OLD with reduced FVC, presumptive restrictive lung disease (RLD) or mixed obstructive and restrictive lung disease (MORLD). Maximal expiratory flows at low lung volumes were assessed using FEF25-75 percent, FEF75 percent and FEF75 percent/FVC. We included 65 normal subjects, also submitted to spirometry, as a control group. RESULTS: The study group included 65 patients: 8 (12.3 percent) with preserved lung function; 18 (27.7 percent) with OLD; 24 (36.9 percent) with OLD and reduced FVC; 5 (7.7 percent) with presumptive RLD; and 10 (15.4 percent) with MORLD. The FEV1 was significantly lower in the OLD with reduced FVC group and the MORLD group than in the other groups (p < 0.001). In the patients with preserved respiratory function, FEF25-75 percent and FEF75 percent were significantly reduced in 1 patient, as was FEF75 percent/FVC in 2 patients. CONCLUSIONS: The respiratory pattern was impaired in 88 percent of the patients with CF. The most common pattern was OLD with reduced FVC. The degree of functional impairment was greater in the OLD with reduced FVC group and in the MORLD group than in the other groups. Maximal expiratory flows at low lung volumes were impaired in a low percentage of patients with preserved respiratory function.

Fatores associados ao insucesso no desmame ventilatório de crianças submetidas a cirurgia cardíaca pediátrica / Factors associated with failure in ventilatory weaning of children undergone pediatric cardiac surgery

OBJETIVO: Verificar os fatores associados ao insucesso no desmame de crianças submetidas a correção cirúrgica de cardiopatias congênitas. MÉTODOS: Estudo de coorte. A amostra foi composta de 29 crianças. As cardiopatias foram divididas em cianóticas (nove crianças) e acianóticas (20 crianças). Foram estudadas crianças de 0 a 5 anos, submetidas a cirurgia cardíaca com permanência em ventilação mecânica invasiva. RESULTADOS: O grupo insucesso apresentou valor menor na SpO2 e valores maiores nos dias de permanência em VMI e na FiO2 quando comparados ao grupo sucesso (P<0,05). Observou-se que quatro (14 por cento) crianças tiveram insucesso no desmame, sendo necessário retornar a prótese ventilatória antes de 48 horas após a extubação. CONCLUSÃO: Os dias de VMI, a SpO2 reduzida e a FiO2 elevada foram os principais fatores associados à falha na extubação neste grupo de pacientes.

OBJECTIVE: To assess factors associated with failure in ventilatory weaning of children undergone surgical correction of congenital heart diseases. METHODS: Cohort study with 29 children. The heart diseases were divided into cyanotic (nine children) and acyanotic (20 children). We studied children from 0 to 5 years who underwent heart surgery with invasive mechanical ventilation. RESULTS: The failure group presented smaller value in SpO2 and higher values during days of stay in IMV and FiO2 when compared to the success group (P<0.05). It was observed that 4 children (14 percent) had failed in weaning, being necessary to return the ventilatory prosthesis before 48 hours after extubation. CONCLUSION: The days of IMV, SpO2 and FiO2 were the main factors associated with failed extubation in this group of patients.