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.

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.

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.

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.

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.

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.

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.

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.

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.

Relationship between nutritional status and maximum inspiratory and expiratory pressures in cystic fibrosis.

BACKGROUND: Malnutrition might be expected to result in reduced maximum respiratory pressure and pulmonary function in cystic fibrosis (CF). OBJECTIVE: To assess the relationship between nutritional status and maximum respiratory pressures in patients with CF. METHODS: We performed a prospective cross-sectional study of patients > or = 16 y old attending the Adult CF Program at Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil. Maximum inspiratory and expiratory pressures (MIP and MEP) were measured as indexes of respiratory muscle strength. Nutritional status was assessed via body mass index (BMI), triceps-skin-fold thickness and mid-upper-arm-muscle circumference. The patients were classified into 2 groups according to BMI: normal and nutritional depletion. Spirometry was performed by all subjects. RESULTS: The study included 39 patients (23 female/16 male) with a mean age of 23.7 +/- 6.4 y. The mean +/- SD percent-of-predicted MIP was 88.0 +/- 28.5% in the normal group and 83.2 +/- 27.3% in the nutritional-depletion group (p = 0.605). The mean +/- SD percent-of-predicted MEP was 84.7 +/- 24.2% in the normal group and 86.1 +/- 26.3% in the nutritional-depletion group (p = 0.874). The mean +/- SD percent-of-predicted forced expiratory volume in the first second (FEV(1)) was 55.2 +/- 27.5% in the normal group and 50.0 +/- 25.6% in the nutritional-depletion group (p = 0.568). MEP and MIP had no significant correlation to BMI or FEV(1). CONCLUSION: MEP and MIP had no significant relationship to nutritional status, clinical score, chest radiograph score, and pulmonary function.

Association between lung function, physical activity level and postural evaluation variables in adult patients with cystic fibrosis.

INTRODUCTION: With the evolution of the cystic fibrosis (CF) disease, the decline of lung function associated with metabolic disorders and malnutrition, causes alterations in respiratory mechanics, musculoskeletal disorders and thoracic deformities, bringing injury to the individual's quality of life. OBJECTIVE: To verify the association between lung function, physical activity level and postural evaluation variables in adults with CF. METHODS: All patients underwent clinical evaluation and spirometry. The International Physical Activity Questionnaire (IPAQ) and an accelerometer were used to verify the physical activity level (PA). Photogrammetry was used with the aid of the Software of postural evaluation (SAPO) and, for complementary comparisons, the 6-minute walk test (6MWT) was used. RESULTS: Twenty-eight adult subjects with CF, mean age of 25.1 ± 6.3 years and mean expiratory volume in the first second (FEV1 ) of 47.1 ± 20.9% of the predicted group participated in the study. The FEV1 correlated with the parameters obtained by the accelerometer (r = 0.723, P = .000), postural evaluation (r = -0.483, P = .005) and 6MWT (r = 0.439, P = .019), but there was no correlation with the data obtained by IPAQ (r = -0.282; P = .073). The time in which each individual remained in moderate to vigorous physical activity correlated with parameters of postural evaluation (thoracic kyphosis r = -0.484, P = .031, cervical lordosis r = 0.531, P = .016), 6MWT (r = 0.564; P = .010) and with the total METS obtained by IPAQ (r = 0.451, P = .046). CONCLUSIONS: Lung function in patients with CF disease is associated with higher thoracic kyphosis, shorter time in moderate and vigorous PA, and shorter distance covered in 6MWT. The accelerometer has been shown to be the best instrument for assessing PA in this public.

Pulmonary hypertension as estimated by Doppler echocardiography in adolescent and adult patients with cystic fibrosis and their relationship with clinical, lung function and sleep findings.

BACKGROUND: In cystic fibrosis (CF) patients, end stage of pulmonary disease is characterized by pulmonary hypertension (PH), hypoxemia, decrease in exercise tolerance, and sleep quality. OBJECTIVE: To evaluate the association between clinical, lung function, sleep quality, and polysomnographic variables with PH in CF patients aged 16 years or older. METHODS: In a cross-sectional study, 51 clinically stable CF patients underwent a clinical evaluation, an overnight polysomnography and answered sleep questionnaires (Pittsburgh Sleep Quality Index and Epworth sleepiness scale). Also, CF patients had their pulmonary function, 6-minute walk test (6MWT) and echocardiography assessed. RESULTS: Fifty-one CF patients participated in the study; 47% were female. The mean age was 25.1 ± 8.8 years. Pulmonary artery systolic pressure (PASP) was greater than 35 mm Hg in 11 (27.5%) patients. Variables associated with PASP>35 mm Hg in univariate analysis were Shwachman-Kulczycki clinical score, forced expiratory volume in 1 second % of predicted, Pseudomonas aeruginosa in sputum culture, at-rest peripheral capillary oxygen saturation (SpO2 ), SpO2 at end of 6MWT and time of oxygen desaturation <90% during sleep. These variables were included in the binary logistic regression. The independent variable associated with the PASP > 35 mm Hg was at-rest SpO2 (OR = 10.8, CI 95% 1.7-67.3, P = .011). The cuttoff SpO2 < 94% had the sensitivity = 7/11 = 64%, specificity = 40/40 = 100%, positive predicted values = 7/7 = 100% and negative predicted values = 40/44 = 91% to the diagnosis of PH. CONCLUSION: the present study showed a high rate of PH in adolescent and adult CF patients. At-rest SpO2 was associated with PH.

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.

Doppler echocardiogram, oxygen saturation and submaximum capacity of exercise in patients with cystic fibrosis.

STUDY OBJECTIVES: To determine the relationship between pulmonary arterial systolic pressure (PASP) and submaximum capacity of exercise, using the six-minute walk test (6MWT) in patients with cystic fibrosis (CF), and to investigate the relation between echocardiographic findings and results of 6MWT, clinical scores, chest radiograph scores and lung function tests. DESIGN: This was a prospective cross-sectional study in patients with CF (16 years and older) with clinical stability, attending the Adult CF Program at the Hospital de Clínicas de Porto Alegre. The patients had Doppler echocardiography and performed a 6MWT. As well as pulmonary function tests and chest roentgenograms, and a clinical score was obtained for all patients. RESULTS: The study included 39 patients with a mean age of 23.7+/-6.3 years. There were no significant correlation between the distance walked and PASP, diameter of the right ventricle (DRV) and pulmonary acceleration time (p>0.05). We observed a significant correlation among PASP and the SpO(2) at rest (r=-0.73; p<0.001), SpO(2) at the end of the 6MWT (r= -0.45; p=0.006), clinical score (r= -0.55; p=0.001), chest radiograph score (r= -0.33; p=0.049), FEV1 (r= -0.63; p< 0.001), and FVC (r=-0.55; p=0.001). Right ventricular outflow tract flow acceleration time (Ac T) was significantly correlated only with the FEV1 (r=0.32; p=0.047). RVD was significantly correlated with SpO(2) at rest (r= -0.44; p=0.005) and clinical score (r= -0.38; p=0.017). The SpO(2) at rest was the single best predictor of PASP and this effect was independent of the relationship between other independent variables (p=0.001). The declining pulmonary function was significantly associated with PASP (p<0.001), SpO(2) at rest (p=0.001), SpO(2) at the end of the 6MWT (p=0.007) and difference between peripheral oxygen saturation at resting and at the end of the 6MWT (p=0.025). CONCLUSION: The PASP was not significantly correlated with the distance walked during the 6MWT in patients with CF. The PASP was strongly correlated with oxygen status at rest. The SpO(2) at rest was the best predictor of PASP. Also, PASP was strongly correlated with Shwachman-Kulczycki score, FEV(1), and FVC in this population.