Is neurodevelopment impaired in Brazilian children with intestinal failure on prolonged parenteral nutrition? A single center study.

PURPOSE: To assess the neurodevelopment outcomes of children younger than 42 months of age with intestinal failure (IF) using prolonged parenteral nutrition (PN) followed by a Pediatric Multidisciplinary Intestinal Rehabilitation Program from a public tertiary hospital in Brazil. METHODS: Bayley III scale was administered in children aged 2 to 42 months with IF and receiving PN for more than 60 days. Composite scores in cognitive, motor, and language domains were analyzed. Developmental delay was defined as a performance 2 standard deviations (SD) below the average at the 3 domains. Association between Bayley III composite scores and clinical variables related to IF were tested. RESULTS: Twenty-four children with median (IQR) age of 17.5 months (9-28.5) were studied, 58.3% were male. Developmental delay was found in 34%, 33% and 27% of the patients in cognitive, motor, and language domains, respectively. There was no significant association between the Bayley-III composite scores and length of hospitalization, prematurity, and number of surgical procedures with anesthesia. CONCLUSION: The study demonstrated impairments in the cognitive, motor and language domains in approximately one-third of young patients with IF on prolonged PN.

One-year follow-up of children hospitalized with COVID-19: a prospective cohort study.

OBJECTIVE: Currently, little is known about the long-term outcomes of COVID-19 in the pediatric population. The aim of this study was to investigate the long-term clinical outcomes of pediatric patients hospitalized with COVID-19. METHODS: This was a prospective cohort study involving unvaccinated children and adolescents admitted to a tertiary hospital in southern Brazil with a COVID-19 diagnosis. Data were collected from electronic medical records for one year after the diagnosis. RESULTS: A total of 66 children were included: the median age was 2.9 years; 63.6% were male; and 48.5% were under 2 years of age. Over 70% had at least one comorbidity prior to the COVID-19 diagnosis. During the one-year follow-up period, 59.1% of the children revisited the emergency department, 50% required readmission, and 15.2% died. Younger children with longer hospital stays were found to be at greater risk of readmission. Having cancer and impaired functionality were found to increase the risk of death within one year. CONCLUSIONS: Our findings indicate that most children hospitalized with COVID-19 have comorbidities. Younger age at admission and a longer hospital stay seem to be risk factors for readmission. In addition, the presence of cancer and impaired functionality are apparently associated with the poor outcome of death within the first year after the diagnosis of COVID-19.

Functional status of hospitalized pediatric patients with COVID-19 in southern Brazil: a prospective cohort study.

OBJECTIVE: The present study aimed to assess the functional status of children diagnosed with COVID-19 at the time of hospitalization and the associations with clinical features. METHODS: This prospective cohort study was carried out with children diagnosed with COVID-19 admitted to a tertiary hospital. The patients' functioning was assessed using the pediatric Functional Status Scale (FSS). RESULTS: A total of 62 children with a median age of 3 years old were included in the study, and 70% had some comorbidity prior to the diagnosis of COVID-19. The median length of stay was nine days, during which period five patients died. The FSS assessment of the sample showed that approximately 55% had some functional alteration. The group of patients with the highest FSS scores presented a lengthier hospital stay (p = 0.016), required more oxygen therapy (p < 0.001), mechanical ventilation (p = 0.001), and intensive care unit admissions (p = 0.019), and had more cardiac (p = 0.007), neurological (p = 0.003), and respiratory (p = 0.013) comorbidities. In the multivariate analysis, there was an association between the dependent variable length of stay and the total FSS score (b = 0.349, p = 0.004) and the presence of comorbidities (b = 0.357, p = 0.004). CONCLUSIONS: We observed that more than half of the children hospitalized due to COVID-19 had some level of functional change. Greater alterations in functional status were associated with the presence of previous comorbidities, a greater need for ventilatory support, and longer hospital stays.

One-year follow-up of children hospitalized with COVID-19: a prospective cohort study / Acompanhamento de um ano de crianças hospitalizadas com COVID-19: estudo prospectivo de coorte

ABSTRACT Objective: Currently, little is known about the long-term outcomes of COVID-19 in the pediatric population. The aim of this study was to investigate the long-term clinical outcomes of pediatric patients hospitalized with COVID-19. Methods: This was a prospective cohort study involving unvaccinated children and adolescents admitted to a tertiary hospital in southern Brazil with a COVID-19 diagnosis. Data were collected from electronic medical records for one year after the diagnosis. Results: A total of 66 children were included: the median age was 2.9 years; 63.6% were male; and 48.5% were under 2 years of age. Over 70% had at least one comorbidity prior to the COVID-19 diagnosis. During the one-year follow-up period, 59.1% of the children revisited the emergency department, 50% required readmission, and 15.2% died. Younger children with longer hospital stays were found to be at greater risk of readmission. Having cancer and impaired functionality were found to increase the risk of death within one year. Conclusions: Our findings indicate that most children hospitalized with COVID-19 have comorbidities. Younger age at admission and a longer hospital stay seem to be risk factors for readmission. In addition, the presence of cancer and impaired functionality are apparently associated with the poor outcome of death within the first year after the diagnosis of COVID-19.

RESUMO Objetivo: Atualmente, pouco se sabe sobre os desfechos em longo prazo da COVID-19 na população pediátrica. O objetivo deste estudo foi investigar os desfechos clínicos em longo prazo de pacientes pediátricos hospitalizados com COVID-19. Métodos: Trata-se de um estudo prospectivo de coorte com crianças e adolescentes não vacinados internados em um hospital terciário do Sul do Brasil com diagnóstico de COVID-19. Os dados referentes ao período de um ano após o diagnóstico foram extraídos dos prontuários médicos eletrônicos. Resultados: Foram incluídas 66 crianças: a mediana da idade foi de 2,9 anos; 63,6% eram do sexo masculino; 48,5% tinham menos de 2 anos de idade. Mais de 70% tinham pelo menos uma comorbidade antes do diagnóstico de COVID-19. Durante o período de um ano de acompanhamento, 59,1% das crianças retornaram ao pronto-socorro, 50% necessitaram de readmissão e 15,2% morreram. O risco de readmissão foi maior em crianças mais novas que permaneceram internadas durante mais tempo. Câncer e funcionalidade prejudicada aumentaram o risco de morte até um ano depois. Conclusões: Nossos achados indicam que a maioria das crianças hospitalizadas com COVID-19 apresenta comorbidades. Ser mais jovem no momento da internação hospitalar e permanecer internado durante mais tempo parecem ser fatores de risco de readmissão. Além disso, câncer e funcionalidade prejudicada são fatores aparentemente relacionados com o mau desfecho de óbito no primeiro ano após o diagnóstico de COVID-19.

Associação entre área de secção transversa do reto femoral e excursão diafragmática com o desmame de pacientes com traqueostomia na unidade de terapia intensiva / Association between rectus femoris cross-sectional area and diaphragmatic excursion with weaning of tracheostomized patients in the intensive care unit

RESUMO Objetivo: Verificar a relação entre área de secção transversa do reto femoral e excursão diafragmática com sucesso no desmame da ventilação mecânica em pacientes críticos crônicos com traqueostomia. Métodos: Este foi um estudo de coorte observacional prospectivo. Incluímos pacientes críticos crônicos (aqueles submetidos à colocação de traqueostomia após 10 dias de ventilação mecânica). A área de secção transversa do reto femoral e a excursão diafragmática foram obtidas por ultrassonografia realizada dentro das primeiras 48 horas após a traqueostomia. Medimos a área de secção transversa do reto femoral e a excursão diafragmática para avaliar sua associação com o desmame da ventilação mecânica, incluindo sua capacidade de prever o sucesso no desmame e a sobrevida durante toda a internação na unidade de terapia intensiva. Resultados: Foram incluídos 81 pacientes. Quarenta e cinco pacientes (55%) foram desmamados da ventilação mecânica. A mortalidade foi de 42% e 61,7% na unidade de terapia intensiva e hospitalar, respectivamente. O grupo que falhou em relação ao grupo que obteve sucesso no desmame apresentou menor área transversa do reto femoral (1,4 [0,8] versus 1,84 [0,76]cm2, p = 0,014) e menor excursão diafragmática (1,29 ± 0,62 versus 1,62 ± 0,51cm, p = 0,019). Quando a área de secção transversa do reto femoral ≥ 1,80cm2 e a excursão diafragmática ≥ 1,25cm era uma condição combinada, apresentava forte associação com sucesso no desmame (RC ajustada de 20,81; IC95% 2,38 - 182,28; p = 0,006), mas não com sobrevida na unidade de terapia intensiva (RC ajustada de 0,19; IC95% 0,03 - 1,08; p = 0,061). Conclusão: O sucesso no desmame da ventilação mecânica em pacientes críticos crônicos foi associado a medidas maiores de área de secção transversa do reto femoral e da excursão diafragmática.

ABSTRACT Objective: To verify the relationship between the rectus femoris cross-sectional area and diaphragmatic excursion with successful weaning from mechanical ventilation in chronic critically tracheostomized patients. Methods: This was a prospective observational cohort study. We included chronic critically ill patients (those who underwent tracheostomy placement after 10 days under mechanical ventilation). The rectus femoris cross-sectional area and diaphragmatic excursion were obtained by ultrasonography performed within the first 48 hours after tracheostomy. We measured rectus femoris cross-sectional area and diaphragmatic excursion to assess their association with weaning from mechanical ventilation, including their potential to predict successful weaning and survival throughout the intensive care unit stay. Results: Eighty-one patients were included. Forty-five patients (55%) were weaned from mechanical ventilation. The mortality rates were 42% and 61.7% in the intensive care unit and hospital, respectively. The fail group in relation to the success group at weaning presented a lower rectus femoris cross-sectional area (1.4 [0.8] versus 1.84 [0.76]cm2, p = 0.014) and lower diaphragmatic excursion (1.29 ± 0.62 versus 1.62 ± 0.51cm, p = 0.019). When rectus femoris cross-sectional area ≥ 1.80cm2 and diaphragmatic excursion ≥ 1.25cm was a combined condition, it had a strong association with successful weaning (adjusted OR = 20.81, 95%CI 2.38 - 182.28; p = 0.006) but not with intensive care unit survival (adjusted OR = 0.19, 95%CI 0.03 - 1.08; p = 0.061). Conclusion: Successful weaning from mechanical ventilation in chronic critically ill patients was associated with higher measurements of rectus femoris cross-sectional area and diaphragmatic excursion.

Clinical characteristics and outcomes of incident cases of COVID-19 in unvaccinated adult cystic fibrosis patients in southern Brazil: a prospective cohort study conducted during the first year of the COVID-19 pandemic.

OBJECTIVE: There is still limited information on the clinical characteristics and outcomes of cystic fibrosis (CF) patients with COVID-19 in Brazil. The objective of this study was to describe the cumulative incidence of COVID-19 in CF patients, as well as their clinical characteristics and outcomes. METHODS: This was a prospective cohort study involving unvaccinated adult CF patients and conducted during the first year of the SARS-CoV-2 pandemic in the city of Porto Alegre, in southern Brazil. The clinical course of the disease was rated on the WHO Ordinal Scale for Clinical Improvement. The primary outcome was the number of incident cases of COVID-19. RESULTS: Between April 30, 2020 and April 29, 2021, 98 CF patients were included in the study. Seventeen patients were diagnosed with COVID-19. For the CF patients, the annual cumulative incidence of COVID-19 was 17.3%, similar to that for the general population, adjusted for age (18.5%). The most common symptoms at diagnosis of COVID-19 were cough (in 59%), dyspnea (in 53%), fatigue (in 53%), and fever (in 47%). Only 6 (35%) of the patients required hospitalization, and 3 (17.6%) required oxygen support. Only 1 patient required mechanical ventilation, having subsequently died. CONCLUSIONS: During the first year of the SARS-CoV-2 pandemic in southern Brazil, the cumulative incidence rate of COVID-19 was similar between CF patients and the general population. More than 50% of the CF patients with SARS-CoV-2 infection had a mild clinical presentation, without the need for hospital admission, and almost the entire sample recovered completely from the infection, the exception being 1 patient who had advanced lung disease and who died.

Predictive factors for premature birth and respiratory exacerbation in pregnancies of women with cystic fibrosis

Abstract Objective: In this present study, the authors evaluated the predictive factors for adverse maternal-fetal outcomes in pregnancies of women with cystic fibrosis (CF). Patients were followed up by a referral center for adults in southern Brazil. Methods: This is a retrospective cohort study that used data from electronic medical records regarding pregnancies of women diagnosed with CF. Results: The study included 39 pregnancies related to 20 different women. The main adverse outcomes were high prevalence rates of premature birth (38.5%) and maternal respiratory exacerbation (84.6%). Lower body mass index (BMI) values (< 20.8) and younger ages of CF diagnosis increased the risk of premature birth. The presence of methicillin-resistant and absence of methicillin-sensitive Staphylococcus aureus, as well as a younger age of diagnosis, increased the risk of maternal respiratory exacerbation during pregnancy. Conclusions: Conception in women with CF is often associated with maternal and fetal complications. Continuous monitoring by a multidisciplinary team should emphasize appropriate nutritional status, investigation of bacterial colonization, and immediate attention to respiratory exacerbations.

Predictive factors for premature birth and respiratory exacerbation in pregnancies of women with cystic fibrosis.

OBJECTIVE: In this present study, the authors evaluated the predictive factors for adverse maternal-fetal outcomes in pregnancies of women with cystic fibrosis (CF). Patients were followed up by a referral center for adults in southern Brazil. METHODS: This is a retrospective cohort study that used data from electronic medical records regarding pregnancies of women diagnosed with CF. RESULTS: The study included 39 pregnancies related to 20 different women. The main adverse outcomes were high prevalence rates of premature birth (38.5%) and maternal respiratory exacerbation (84.6%). Lower body mass index (BMI) values (< 20.8) and younger ages of CF diagnosis increased the risk of premature birth. The presence of methicillin-resistant and absence of methicillin-sensitive Staphylococcus aureus, as well as a younger age of diagnosis, increased the risk of maternal respiratory exacerbation during pregnancy. CONCLUSIONS: Conception in women with CF is often associated with maternal and fetal complications. Continuous monitoring by a multidisciplinary team should emphasize appropriate nutritional status, investigation of bacterial colonization, and immediate attention to respiratory exacerbations.

Association between rectus femoris cross-sectional area and diaphragmatic excursion with weaning of tracheostomized patients in the intensive care unit. / Associação entre área de secção transversa do reto femoral e excursão diafragmática com o desmame de pacientes com traqueostomia na unidade de terapia intensiva.

OBJECTIVE: To verify the relationship between the rectus femoris cross-sectional area and diaphragmatic excursion with successful weaning from mechanical ventilation in chronic critically tracheostomized patients. METHODS: This was a prospective observational cohort study. We included chronic critically ill patients (those who underwent tracheostomy placement after 10 days under mechanical ventilation). The rectus femoris cross-sectional area and diaphragmatic excursion were obtained by ultrasonography performed within the first 48 hours after tracheostomy. We measured rectus femoris cross-sectional area and diaphragmatic excursion to assess their association with weaning from mechanical ventilation, including their potential to predict successful weaning and survival throughout the intensive care unit stay. RESULTS: Eighty-one patients were included. Forty-five patients (55%) were weaned from mechanical ventilation. The mortality rates were 42% and 61.7% in the intensive care unit and hospital, respectively. The fail group in relation to the success group at weaning presented a lower rectus femoris cross-sectional area (1.4 [0.8] versus 1.84 [0.76]cm2, p = 0.014) and lower diaphragmatic excursion (1.29 ± 0.62 versus 1.62 ± 0.51cm, p = 0.019). When rectus femoris cross-sectional area ≥ 1.80cm2 and diaphragmatic excursion ≥ 1.25cm was a combined condition, it had a strong association with successful weaning (adjusted OR = 20.81, 95%CI 2.38 - 182.28; p = 0.006) but not with intensive care unit survival (adjusted OR = 0.19, 95%CI 0.03 - 1.08; p = 0.061). CONCLUSION: Successful weaning from mechanical ventilation in chronic critically ill patients was associated with higher measurements of rectus femoris cross-sectional area and diaphragmatic excursion.

OBJETIVO: Verificar a relação entre área de secção transversa do reto femoral e excursão diafragmática com sucesso no desmame da ventilação mecânica em pacientes críticos crônicos com traqueostomia. MÉTODOS: Este foi um estudo de coorte observacional prospectivo. Incluímos pacientes críticos crônicos (aqueles submetidos à colocação de traqueostomia após 10 dias de ventilação mecânica). A área de secção transversa do reto femoral e a excursão diafragmática foram obtidas por ultrassonografia realizada dentro das primeiras 48 horas após a traqueostomia. Medimos a área de secção transversa do reto femoral e a excursão diafragmática para avaliar sua associação com o desmame da ventilação mecânica, incluindo sua capacidade de prever o sucesso no desmame e a sobrevida durante toda a internação na unidade de terapia intensiva. RESULTADOS: Foram incluídos 81 pacientes. Quarenta e cinco pacientes (55%) foram desmamados da ventilação mecânica. A mortalidade foi de 42% e 61,7% na unidade de terapia intensiva e hospitalar, respectivamente. O grupo que falhou em relação ao grupo que obteve sucesso no desmame apresentou menor área transversa do reto femoral (1,4 [0,8] versus 1,84 [0,76]cm2, p = 0,014) e menor excursão diafragmática (1,29 ± 0,62 versus 1,62 ± 0,51cm, p = 0,019). Quando a área de secção transversa do reto femoral ≥ 1,80cm2 e a excursão diafragmática ≥ 1,25cm era uma condição combinada, apresentava forte associação com sucesso no desmame (RC ajustada de 20,81; IC95% 2,38 - 182,28; p = 0,006), mas não com sobrevida na unidade de terapia intensiva (RC ajustada de 0,19; IC95% 0,03 - 1,08; p = 0,061). CONCLUSÃO: O sucesso no desmame da ventilação mecânica em pacientes críticos crônicos foi associado a medidas maiores de área de secção transversa do reto femoral e da excursão diafragmática.

Clinical characteristics and outcomes of incident cases of COVID-19 in unvaccinated adult cystic fibrosis patients in southern Brazil: a prospective cohort study conducted during the first year of the COVID-19 pandemic / Características clínicas e desfechos de casos incidentes de COVID-19 em adultos não vacinados com fibrose cística no sul do Brasil: estudo prospectivo de coorte realizado durante o primeiro ano da pandemia de COVID-19

ABSTRACT Objective: There is still limited information on the clinical characteristics and outcomes of cystic fibrosis (CF) patients with COVID-19 in Brazil. The objective of this study was to describe the cumulative incidence of COVID-19 in CF patients, as well as their clinical characteristics and outcomes. Methods: This was a prospective cohort study involving unvaccinated adult CF patients and conducted during the first year of the SARS-CoV-2 pandemic in the city of Porto Alegre, in southern Brazil. The clinical course of the disease was rated on the WHO Ordinal Scale for Clinical Improvement. The primary outcome was the number of incident cases of COVID-19. Results: Between April 30, 2020 and April 29, 2021, 98 CF patients were included in the study. Seventeen patients were diagnosed with COVID-19. For the CF patients, the annual cumulative incidence of COVID-19 was 17.3%, similar to that for the general population, adjusted for age (18.5%). The most common symptoms at diagnosis of COVID-19 were cough (in 59%), dyspnea (in 53%), fatigue (in 53%), and fever (in 47%). Only 6 (35%) of the patients required hospitalization, and 3 (17.6%) required oxygen support. Only 1 patient required mechanical ventilation, having subsequently died. Conclusions: During the first year of the SARS-CoV-2 pandemic in southern Brazil, the cumulative incidence rate of COVID-19 was similar between CF patients and the general population. More than 50% of the CF patients with SARS-CoV-2 infection had a mild clinical presentation, without the need for hospital admission, and almost the entire sample recovered completely from the infection, the exception being 1 patient who had advanced lung disease and who died.

RESUMO Objetivo: Ainda não há informações suficientes sobre as características clínicas e desfechos de pacientes com fibrose cística (FC) e COVID-19 no Brasil. O objetivo deste estudo foi descrever a incidência cumulativa de COVID-19 em pacientes com FC, bem como suas características clínicas e desfechos. Métodos: Estudo prospectivo de coorte com adultos com FC não vacinados, realizado na cidade de Porto Alegre, no sul do Brasil, durante o primeiro ano da pandemia de SARS-CoV-2. A evolução clínica da COVID-19 foi avaliada por meio da WHO Ordinal Scale for Clinical Improvement (escala ordinal de evolução clínica, elaborada pela OMS). O desfecho primário foi o número de casos incidentes de COVID-19. Resultados: Entre 30 de abril de 2020 e 29 de abril de 2021, 98 pacientes com FC foram incluídos no estudo. Dezessete pacientes receberam diagnóstico de COVID-19. Nos pacientes com FC, a incidência cumulativa anual de COVID-19 foi de 17,3%, semelhante à observada na população geral, ajustada pela idade (18,5%). Os sintomas mais comuns no momento do diagnóstico de COVID-19 foram tosse (em 59%), dispneia (em 53%), fadiga (em 53%) e febre (em 47%). Apenas 6 (35%) dos pacientes necessitaram de hospitalização, e 3 (17,6%) necessitaram de suporte de oxigênio. Apenas 1 paciente necessitou de ventilação mecânica e, posteriormente, morreu. Conclusões: Durante o primeiro ano da pandemia de SARS-CoV-2 no sul do Brasil, a taxa de incidência cumulativa de COVID-19 foi semelhante nos pacientes com FC e na população geral. Mais de 50% dos pacientes com FC e infecção por SARS-CoV-2 apresentaram manifestações clínicas leves, sem necessidade de internação hospitalar, e quase toda a amostra se recuperou completamente da infecção, à exceção de 1 paciente, que apresentava doença pulmonar avançada e morreu.

Functional status of hospitalized pediatric patients with COVID-19 in southern Brazil: a prospective cohort study / Estado funcional de pacientes pediátricos hospitalizados com COVID-19 no sul do Brasil: um estudo de coorte prospectivo

ABSTRACT Objective: The present study aimed to assess the functional status of children diagnosed with COVID-19 at the time of hospitalization and the associations with clinical features. Methods: This prospective cohort study was carried out with children diagnosed with COVID-19 admitted to a tertiary hospital. The patients' functioning was assessed using the pediatric Functional Status Scale (FSS). Results: A total of 62 children with a median age of 3 years old were included in the study, and 70% had some comorbidity prior to the diagnosis of COVID-19. The median length of stay was nine days, during which period five patients died. The FSS assessment of the sample showed that approximately 55% had some functional alteration. The group of patients with the highest FSS scores presented a lengthier hospital stay (p = 0.016), required more oxygen therapy (p < 0.001), mechanical ventilation (p = 0.001), and intensive care unit admissions (p = 0.019), and had more cardiac (p = 0.007), neurological (p = 0.003), and respiratory (p = 0.013) comorbidities. In the multivariate analysis, there was an association between the dependent variable length of stay and the total FSS score (b = 0.349, p = 0.004) and the presence of comorbidities (b = 0.357, p = 0.004). Conclusions: We observed that more than half of the children hospitalized due to COVID-19 had some level of functional change. Greater alterations in functional status were associated with the presence of previous comorbidities, a greater need for ventilatory support, and longer hospital stays.

RESUMO Objetivo: O objetivo do presente estudo foi de avaliar o estado funcional de crianças diagnosticadas com COVID-19 no momento da internação e as associações com suas características clínicas. Métodos: Este estudo de coorte prospectivo foi realizado com crianças diagnosticadas com COVID-19 internadas em um hospital terciário. A funcionalidade dos pacientes foi avaliada por meio da Escala de Estado Funcional (FSS) pediátrica. Resultados: Foram incluídas no estudo 62 crianças com idade mediana de 3 anos, das quais 70% apresentavam alguma comorbidade antes do diagnóstico de COVID-19. O tempo mediano de internação foi de nove dias, período no qual cinco pacientes vieram a óbito. A avaliação da FSS da amostra mostrou que aproximadamente 55% apresentavam alguma alteração funcional. O grupo de pacientes com os maiores escores na FSS teve um maior tempo de internação (p = 0,016), necessitou de mais oxigenoterapia (p < 0,001), ventilação mecânica (p = 0,001) e internações em unidade de terapia intensiva (p = 0,019) e tinha mais comorbidades cardíacas (p = 0,007), neurológicas (p = 0,003) e respiratórias (p = 0,013). Na análise multivariada, observou-se uma associação entre a variável dependente tempo de internação e o escore total da FSS (b = 0,349, p = 0,004) e a presença de comorbidades (b = 0,357, p = 0,004). Conclusões: Verificou-se que mais da metade das crianças internadas devido à COVID-19 apresentaram algum nível de alteração funcional. Maiores alterações no estado funcional foram associadas à presença de comorbidades prévias, maior necessidade de suporte ventilatório e maior tempo de internação.

Functional capacity, pulmonary function, and quality of life in hematopoietic stem cell transplantation survivors.

OBJECTIVE: Hematopoietic stem cell transplantation (HSCT) is used as a treatment for several diseases. The objective was to evaluate the functional capacity, pulmonary function, and quality of life (QoL) in HSCT survivors. METHODS: The patients were submitted to spirometry and six-minute walk test (6MWT) and evaluation of QoL (Functional Assessment of Cancer Therapy Bone Marrow Transplantation-FACT-BMT) and the level of physical activity (Human Activity Profile-HAP) in the post-HSCT outpatient ambulatory. RESULTS: The sample was composed of 103 individuals with a mean age of 42.1 ± 12 and a median HSCT time of 5 (2-11) years. The average distance walked in the 6MWT was 524.7 ± 73.5 m; the FACT-BMT score, 82 ± 8.7 points; FEV1, 76.8 ± 22.6%; and HAP, 71.9 ± 11.2 points. There was a significant difference between debilitated and physically active individuals for the variables: forced expiratory volume in the first second (FEV1) in liters (p = 0.008) and % of predicted (p = 0.017), FEV1/FVC (p = 0.032), distance on the 6MWT (p < 0.001), fatigue after the 6MWT (p < 0.001), and physical well-being (p = 0.005). CONCLUSIONS: HSCT survivors have long-term changes in functional capacity, pulmonary function, and QoL. Subjects more active had better results in pulmonary function and functional capacity.

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.

Dyspnea perception during the inspiratory resistive loads test in obese subjects waiting bariatric surgery.

Identification of low dyspnea perception is relevant, since this condition is significantly associated with worse outcomes. We investigated dyspnea perception during the inspiratory resistive loads test on obese subjects waiting bariatric surgery in comparison with normal subjects. Secondarily, we analysed the proportion of obese subjects with low, moderate and high dyspnea perception. This observational study included subjects with body mass index (BMI) ≥ 35 kg/m2, compared to healthy subjects with BMI ≥ 18 and <25 kg/m2. Subject underwent clinical evaluation, inspiratory test with progressive resistive loads and spirometry. We studied 23 obese subjects (mean BMI = 51.9 ± 9.3 kg/m2) and 25 normal subjects (mean BMI = 24.3 ± 2.3 kg/m2). With the increase magnitude of resistive loads there was a significant increase in dyspnea score (p < 0.001) and progressive increase of the generated inspiratory pressure (p < 0.001), but there was no difference between the groups in terms of dyspnea score (p = 0.191) and no interaction effect (p = 0.372). Among the obese subjects, 4 individuals were classified as low perception, 11 as moderate and 8 as high. In conclusion, the degree of dyspnea perception during the inspiratory progressive resistive loads test did not differ between obese and normal subjects. Among obese subjects, only 17% were classified as low dyspnea perception.

Respiratory physical therapy techniques recommended for patients with cystic fibrosis treated in specialized centers.

BACKGROUND: Respiratory therapy is a part of the treatment of patients with cystic fibrosis (CF). However, there is no information about the main factors influencing the recommendation of the use of different techniques or devices by physical therapists from different specialized centers. OBJECTIVE: To determine the respiratory therapy techniques recommended for the treatment of patients with CF seen in specialized treatment centers in Brazil. METHODS: This is a descriptive study including a sample of Brazilian CF specialized treatment centers. Data on demographics, spirometric variables, and recommended respiratory therapy treatment techniques were collected. RESULTS: Twelve specialized treatment centers with a total of 974 patients were included (13.5±11.3 years old and FEV1 (%) 75.7±27.9). The most recommended techniques were huffing (61.1% of patients), high frequency oral oscillation (HFOO) (52.0%), and positive expiratory pressure (PEP) (45.3%). Most often, recommendation was to perform treatment once (54.8% of patients) or twice (34%) a day. There was great variability in the recommendation among the different states. When data were separated by age, there was a predominance of performing conventional and manual techniques in infants and preschool children. There were no significant variations according to pulmonary function. Based on the literature, techniques based on volume, huffing, and PEP were most prevalently performed in international centers. CONCLUSION: The most recommended treatment techniques for patients with CF in Brazil are huffing, HFOO, and PEP, followed by volume-based techniques. There were geographical variations in the preferred treatment techniques, as well as based on patient age, but not based on the level of pulmonary function.

Adherence to inhalation therapy and quality of life in children with cystic fibrosis: a cross-sectional study

Introduction: Inhalation therapy is a crucial part of the cystic fibrosis (CF) treatment regimen. Drugs that assist in mucociliary clearance and inhaled antibiotics are used by most patients. Methods: This is a cross-sectional study where patients with CF and their caregivers answered questionnaires regarding their adherence to inhalation therapy and QoL. Demographic, spirometric, and bacteriological data, as well as S-K scores and hospitalization frequencies were also collected. Results: We included 66 patients in this study; participants had a mean age of 12.3 years and Z-scores of -1.4 for forced expiratory volume in 1 second and 48.6 for body mass index. Patients were divided into 2 groups according to their self-reported adherence to inhalation therapy: high adherence (n = 46) and moderate/low adherence (n = 20). When comparing both groups, there was no statistically significant differences in age, sex, family income, and S-K score (p > 0.05). The high-adherence group had had shorter hospitalization periods in the previous year (p = 0.016) and presented better scores in the following domains of the QoL questionnaire: emotion (p = 0.006), eating (p = 0.041), treatment burden (p = 0.001), health perception (p = 0.001), and social (p = 0.046). Conclusions: A low self-reported adherence to inhalation therapy recommendations was associated with longer hospitalizations in the previous year and with a decrease in QoL in pediatric patients with CF. (AU)

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.