Clinicogenetic characterization and response to disease-modifying therapies in spinal muscular atrophy: real-world experience from a reference center in Southern Brazil.

OBJECTIVE: Spinal Muscular Atrophy linked to chromosome 5q (SMA) is an autosomal recessive neurodegenerative disease characterized by progressive proximal muscle atrophy and weakness. This study addresses the scarcity of research on novel disease-modifying therapies for SMA in Latin America by reporting a real-world experience in Southern Brazil. METHODOLOGY: This is a single-center historical cohort that included all patients diagnosed with spinal muscular atrophy at a Regional Reference Service for rare diseases. RESULTS: Eighty-one patients were included, of whom 7 died during follow-up. Of the remaining 74 patients, 5.4 % were classified as pre-symptomatic, 24.3 % with SMA type 1, 28.4 % with type 2, 36.5 % with type 3, and 5.4 % with type 4. The mean follow-up time ranged from 1.8 years for pre-symptomatic cases to 8.7 years for SMA types 2 and 3. Approximately 42 % of these patients received specific disease-modifying therapy, of these, 96.8 % received Nusinersen, with 19.4 % transitioning to gene therapy using Onasemnogene Abeparvovec, and 6.4 % starting Risdiplam. Most patients with SMA type 1 were on disease-modifying treatment, whereas only slightly over a third of patients with type 2 and about 10 % of type 3 were receiving such treatments. Among treated patients, 80 % demonstrated improvement in motor performance during the follow-up, with a lesser therapeutic response being associated with late initiation of treatment and low motor function scores at baseline. CONCLUSION: This real-world study reinforces the effectiveness of disease-modifying therapies for SMA in Brazil within the context of low- and middle-income countries, which is greater the earlier and the better the patient's functional status.

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.

Hospital physical therapy management in pediatric patients with covid-19: case reports / Manejo da fisioterapia no âmbito hospitalar no paciente pediátrico com covid-19: relato de casos

ABSTRACT Objective: To report the physiotherapeutic management of two pediatric cases with COVID-19 admitted in a reference state hospital to treat the disease in Porto Alegre, Southern Brazil. Cases description: Case 1, female, 10-month-old child, pre-existing chronic disease, hospitalized since birth, mechanical ventilation dependency via tracheotomy, progressed with hypoxemia, requiring oxygen therapy, and increased ventilator parameters, and a diagnosis of COVID-19 was confirmed. Airway clearance and pulmonary expansion maintenance therapies were performed. During hospitalization, the child acquired cephalic control, sitting without support, rolling, holding, and reaching objects. Recommendations were provided to a family member to maintain motor development milestones. Case 2, male, nine years old, previous psychiatric disease and obesity, showed worsening of the sensory state, requiring intensive care and invasive mechanical ventilation, with the diagnosis of SARS-Cov-2 infection. The physical therapy was performed to maintain airway clearance, pulmonary expansion, and early mobilization, showing ventilatory improvement during the intensive care hospitalization and successfully extubated after 17 days. The physical therapy evolved from passive to resistive exercises during the hospitalization, and the patient was able to walk without assistance at discharge, with the same previous functional status. Comments: The COVID-19 showed different manifestations in both cases. Physical therapy treatment was essential to maintain and to recover the functional status of the patients. Future studies are needed to improve the understanding of disease course and its functional consequences to offer an efficient treatment to pediatric patients with COVID-19.

RESUMO Objetivo: Relatar as condutas fisioterapêuticas dos dois casos de pacientes pediátricos com COVID-19 internados em hospital de referência estadual em Porto Alegre para tratamento da doença. Descrição dos casos: Caso 1, sexo feminino, 10 meses de idade com doença crônica preexistente, internada desde o nascimento, utilizava ventilação mecânica via traqueostomia, evoluiu com hipoxemia, necessidade de oxigenoterapia e aumento dos parâmetros ventilatórios, sendo confirmada COVID-19. Foram realizadas técnicas de desobstrução brônquica e manutenção da expansão pulmonar. Além disso, a criança durante a internação adquiriu controle cefálico, sedestação sem apoio, rolar e alcance de objetos e durante infecção por coronavírus foram passadas orientações ao familiar para manutenção dos marcos motores adquiridos. Caso 2, sexo masculino, 9 anos, com doença psiquiátrica prévia e obesidade, evoluiu com quadro de rebaixamento do sensório e necessidade de tratamento intensivo, sendo o paciente colocado em ventilação mecânica invasiva na chegada à unidade e confirmada a infecção por SARS-CoV-2. Realizou fisioterapia para desobstrução brônquica, reexpansão pulmonar e mobilização precoce, apresentando melhora ventilatória ao longo da internação, e após 17 dias foi extubado com sucesso. Evoluiu de cinesioterapia passiva para assistida e resistida na internação pediátrica, conseguindo deambular sem auxílio, e teve alta hospitalar com condição funcional prévia à internação hospitalar. Comentários: A COVID-19 apresentou-se de forma distinta nos casos, todavia a fisioterapia foi essencial para a manutenção e recuperação do quadro funcional dos pacientes. Estudos futuros são necessários para melhor compreensão do curso da doença e suas repercussões funcionais, a fim de traçar um tratamento eficiente para os pacientes pediátricos acometidos pela COVID-19.

HOSPITAL PHYSICAL THERAPY MANAGEMENT IN PEDIATRIC PATIENTS WITH COVID-19: CASE REPORTS.

OBJECTIVE: To report the physiotherapeutic management of two pediatric cases with COVID-19 admitted in a reference state hospital to treat the disease in Porto Alegre, Southern Brazil. CASES DESCRIPTION: Case 1, female, 10-month-old child, pre-existing chronic disease, hospitalized since birth, mechanical ventilation dependency via tracheotomy, progressed with hypoxemia, requiring oxygen therapy, and increased ventilator parameters, and a diagnosis of COVID-19 was confirmed. Airway clearance and pulmonary expansion maintenance therapies were performed. During hospitalization, the child acquired cephalic control, sitting without support, rolling, holding, and reaching objects. Recommendations were provided to a family member to maintain motor development milestones. Case 2, male, nine years old, previous psychiatric disease and obesity, showed worsening of the sensory state, requiring intensive care and invasive mechanical ventilation, with the diagnosis of SARS-Cov-2 infection. The physical therapy was performed to maintain airway clearance, pulmonary expansion, and early mobilization, showing ventilatory improvement during the intensive care hospitalization and successfully extubated after 17 days. The physical therapy evolved from passive to resistive exercises during the hospitalization, and the patient was able to walk without assistance at discharge, with the same previous functional status. COMMENTS: The COVID-19 showed different manifestations in both cases. Physical therapy treatment was essential to maintain and to recover the functional status of the patients. Future studies are needed to improve the understanding of disease course and its functional consequences to offer an efficient treatment to pediatric patients with COVID-19.

Functional deficit in children with congenital heart disease undergoing surgical correction after intensive care unit discharge. / Déficit funcional em crianças com cardiopatias congênitas submetidas à correção cirúrgica após alta da unidade de terapia intensiva.

OBJECTIVE: To evaluate the functional status of pediatric patients undergoing congenital heart surgery after discharge from the intensive care unit, and to evaluate the correlations among clinical variables, functional status and surgical risk. METHODS: Cross-sectional study including patients aged 1 month to less than 18 years undergoing congenital heart surgery between October 2017 and May 2018. Functional outcome was assessed by the Functional Status Scale, surgical risk classification was determined using the Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1), and clinical variables were collected from electronic medical records. RESULTS: The sample comprised 57 patients with a median age of 7 months (2 - 17); 54.4% were male, and 75.5% showed dysfunction, which was moderate in 45.6% of the cases. RACHS-1 category > 3 was observed in 47% of the sample, indicating higher surgical risk. There was a correlation between functional deficit and younger age, longer duration of invasive mechanical ventilation and longer intensive care unit stay. Moreover, greater functional deficit was observed among patients classified as RACHS-1 category > 3. CONCLUSION: The prevalence of functional deficit was high among children and adolescents with congenital heart disease after cardiac surgery. Higher surgical risk, longer duration of invasive mechanical ventilation, longer intensive care unit stay and younger age were correlated with worse functional status.

Functional Status Scale: Cross-Cultural Adaptation and Validation in Brazil.

OBJECTIVE: The goal of the present study was to perform a cross-cultural adaptation and clinical validation of the Functional Status Scale for use in the Brazilian population. DESIGN: Cross-cultural adaptation study followed by a cross-sectional validation study. SETTING: Single-center PICU at a hospital in Porto Alegre, Brazil. PATIENTS: Children and adolescents of both sexes, 1 month and under 18 years old, who had been treated at the PICU. INTERVENTIONS: The cross-cultural adaptation consisted of the following stages: translation, synthesis of the translated versions, back translations, synthesis of the back translations, committee review, and pretesting. For the clinical validation stage, the Brazilian Functional Status Scale was applied within 48 hours after discharge from the PICU. The Brazilian Functional Status Scale's reliability and validity properties were tested. MEASUREMENTS AND MAIN RESULTS: A total of 314 patients were evaluated. Median age was 24 months (7.0-105.0 mo), 54.1% were males, and their overall functional score was 9 ± 2.8. The Brazilian Functional Status Scale demonstrated excellent interobserver reliability, with an intraclass correlation coefficient of 0.98, and κ coefficients between 0.716 and 1.000 for the functional domains, which indicated good to excellent agreement. Using the Bland-Altman method, we confirmed low variability among the evaluator's responses (0.93 to -1.06 points). Regarding the Brazilian Functional Status Scale's content validity, there was a correlation between length of PICU stay (r = 0.378; p < 0.001) and time on invasive mechanical ventilation (r = 0.261; p < 0.05), and the test could discriminate between groups with different comorbidity levels (p < 0.001). CONCLUSIONS: The Functional Status Scale has been culturally adapted and validated for use in Brazil and is now available for use in the assessment of functionality in Brazilian children and adolescents.

Early mobilization protocols for critically ill pediatric patients: systematic review. / Protocolos de mobilização precoce no paciente crítico pediátrico: revisão sistemática.

OBJECTIVE: To describe the existing early mobilization protocols in pediatric intensive care units. METHODS: A systematic literature review was performed using the databases MEDLINE®, Embase, SciELO, LILACS and PeDRO, without restrictions of date and language. Observational and randomized and nonrandomized clinical trials that described an early mobilization program in patients aged between 29 days and 18 years admitted to the pediatric intensive care unit were included. The methodological quality of the studies was evaluated using the Newcastle-Ottawa Scale, Methodological Index for Non-Randomized Studies and the Cochrane Collaboration. RESULTS: A total of 8,663 studies were identified, of which 6 were included in this review. Three studies described the implementation of an early mobilization program, including activities such as progressive passive mobilization, positioning, and discussion of mobilization goals with the team, in addition to contraindications and interruption criteria. Cycle ergometer and virtual reality games were also used as resources for mobilization. Four studies considered the importance of the participation of the multidisciplinary team in the implementation of early mobilization protocols. CONCLUSION: In general, early mobilization protocols are based on individualized interventions, depending on the child's development. In addition, the use of a cycle ergometer may be feasible and safe in this population. The implementation of institutional and multidisciplinary protocols may contribute to the use of early mobilization in pediatric intensive care units; however, studies demonstrating the efficacy of such intervention are needed.

OBJETIVO: Descrever os protocolos existentes de mobilização precoce nas unidades de terapia intensiva pediátrica. MÉTODOS: Trata-se de uma revisão sistemática da literatura cuja busca foi realizada nas bases MEDLINE®, Embase, SciELO, LILACS e PeDRO, sem restrição para data e idioma. Foram incluídos estudos observacionais e ensaios clínicos randomizados e não randomizados, que descrevessem um programa de mobilização precoce em pacientes admitidos na unidade de terapia intensiva pediátrica, com idades entre 29 dias a 18 anos. A qualidade metodológica dos estudos foi avaliada por meio das ferramentas Newcastle-Ottawa, Methodological Index for Non-Randomized Studies e da colaboração Cochrane. RESULTADOS: Foram identificados 8.663 estudos, sendo 6 incluídos nesta revisão. Três estudos descreveram a implementação de programa de mobilização precoce, incluindo atividades como mobilização passiva progressiva, posicionamento, discussão das metas de mobilização com a equipe, além de contraindicações e critérios de interrupção. Cicloergômetro e jogos de realidade virtual também foram usados como recursos para mobilização. Quatro estudos consideram a importância da participação da equipe multiprofissional na implementação dos protocolos de mobilização precoce. CONCLUSÃO: De modo geral, os protocolos de mobilização precoce são baseados em intervenções individualizadas, conforme o desenvolvimento da criança. Além disso, o uso do cicloergômetro pode ser viável e seguro nesta população. A implementação de protocolos institucionais e multiprofissional pode contribuir para a prática da mobilização precoce nas unidades de terapia intensiva pediátrica, no entanto são necessários estudos que comprovem a eficácia da intervenção.

Protocolos de mobilização precoce no paciente crítico pediátrico: revisão sistemática / Early mobilization protocols for critically ill pediatric patients: systematic review

RESUMO Objetivo: Descrever os protocolos existentes de mobilização precoce nas unidades de terapia intensiva pediátrica. Métodos: Trata-se de uma revisão sistemática da literatura cuja busca foi realizada nas bases MEDLINE®, Embase, SciELO, LILACS e PeDRO, sem restrição para data e idioma. Foram incluídos estudos observacionais e ensaios clínicos randomizados e não randomizados, que descrevessem um programa de mobilização precoce em pacientes admitidos na unidade de terapia intensiva pediátrica, com idades entre 29 dias a 18 anos. A qualidade metodológica dos estudos foi avaliada por meio das ferramentas Newcastle-Ottawa, Methodological Index for Non-Randomized Studies e da colaboração Cochrane. Resultados: Foram identificados 8.663 estudos, sendo 6 incluídos nesta revisão. Três estudos descreveram a implementação de programa de mobilização precoce, incluindo atividades como mobilização passiva progressiva, posicionamento, discussão das metas de mobilização com a equipe, além de contraindicações e critérios de interrupção. Cicloergômetro e jogos de realidade virtual também foram usados como recursos para mobilização. Quatro estudos consideram a importância da participação da equipe multiprofissional na implementação dos protocolos de mobilização precoce. Conclusão: De modo geral, os protocolos de mobilização precoce são baseados em intervenções individualizadas, conforme o desenvolvimento da criança. Além disso, o uso do cicloergômetro pode ser viável e seguro nesta população. A implementação de protocolos institucionais e multiprofissional pode contribuir para a prática da mobilização precoce nas unidades de terapia intensiva pediátrica, no entanto são necessários estudos que comprovem a eficácia da intervenção.

ABSTRACT Objective: To describe the existing early mobilization protocols in pediatric intensive care units. Methods: A systematic literature review was performed using the databases MEDLINE®, Embase, SciELO, LILACS and PeDRO, without restrictions of date and language. Observational and randomized and nonrandomized clinical trials that described an early mobilization program in patients aged between 29 days and 18 years admitted to the pediatric intensive care unit were included. The methodological quality of the studies was evaluated using the Newcastle-Ottawa Scale, Methodological Index for Non-Randomized Studies and the Cochrane Collaboration. Results: A total of 8,663 studies were identified, of which 6 were included in this review. Three studies described the implementation of an early mobilization program, including activities such as progressive passive mobilization, positioning, and discussion of mobilization goals with the team, in addition to contraindications and interruption criteria. Cycle ergometer and virtual reality games were also used as resources for mobilization. Four studies considered the importance of the participation of the multidisciplinary team in the implementation of early mobilization protocols. Conclusion: In general, early mobilization protocols are based on individualized interventions, depending on the child's development. In addition, the use of a cycle ergometer may be feasible and safe in this population. The implementation of institutional and multidisciplinary protocols may contribute to the use of early mobilization in pediatric intensive care units; however, studies demonstrating the efficacy of such intervention are needed.

Effect of vaporized perfluorocarbon on oxidative stress during the cold ischemia phase of lung graft preservation.

Liquid perfluorocarbon (PFC) instillation has been studied experimentally as an adjuvant therapy in the preservation of lung grafts during cold ischemia. The objective of this study was to evaluate whether vaporized PFC is also protective of lung grafts at different cold ischemia times. We performed histological analysis of and measured oxidative stress in the lungs of animals that received only preservation solution with low-potassium dextran (LPD) or vaporized PFC together with LPD. We conclude that vaporized PFC reduces the production of free radicals and the number of pulmonary structural changes resulting from cold ischemia.

Effect of vaporized perfluorocarbon on oxidative stress during the cold ischemia phase of lung graft preservation / Efeito do perfluorocarbono vaporizado sobre o estresse oxidativo no período de isquemia fria durante a preservação pulmonar

ABSTRACT Liquid perfluorocarbon (PFC) instillation has been studied experimentally as an adjuvant therapy in the preservation of lung grafts during cold ischemia. The objective of this study was to evaluate whether vaporized PFC is also protective of lung grafts at different cold ischemia times. We performed histological analysis of and measured oxidative stress in the lungs of animals that received only preservation solution with low-potassium dextran (LPD) or vaporized PFC together with LPD. We conclude that vaporized PFC reduces the production of free radicals and the number of pulmonary structural changes resulting from cold ischemia.

RESUMO O perfluorocarbono (PFC) líquido tem sido estudado experimentalmente como uma substância adjuvante na preservação de enxertos pulmonares durante o período de isquemia fria. O objetivo deste estudo foi avaliar se o PFC vaporizado (e não instilado) também atuaria como protetor de enxertos pulmonares em diferentes tempos de isquemia fria. Realizamos análise histológica e dosamos o estresse oxidativo em pulmões de animais que receberam somente uma solução de preservação com low-potassium dextran (LPD, dextrana com baixa concentração de potássio) ou PFC vaporizado associado a LPD. Concluímos que o PFC vaporizado reduziu a produção de radicais livres e provocou menor número de alterações estruturais pulmonares decorrentes do período de isquemia fria que o uso de LPD isoladamente.

Functional evaluation of pediatric patients after discharge from the intensive care unit using the Functional Status Scale. / Avaliação funcional em pacientes pediátricos após alta da unidade de terapia intensiva por meio da Functional Status Scale.

OBJECTIVE: To evaluate the functional status of pediatric patients after discharge from the pediatric intensive care unit using the Functional Status Scale and to compare the time of invasive mechanical ventilation, length of stay in the pediatric intensive care unit, and Pediatric Index of Mortality 2 results among individuals with different degrees of functional impairment. METHODS: A cross-sectional study was conducted on patients who were discharged from a pediatric intensive care unit. The functional evaluation by the Functional Status Scale was performed on the first day after discharge from the unit, and the Pediatric Index of Mortality 2 was used to predict the mortality rate at the time of admission to the pediatric intensive care unit. RESULTS: The sample consisted of 50 individuals, 60% of which were male, with a median age of 19 [6 - 61] months. The overall score of the Functional Status Scale was 11.5 [7 - 15], and the highest scores were observed in the "motor function" 3 [1 - 4] and "feeding" 4 [1 - 4] domains. Compared to patients who were not readmitted to the pediatric intensive care unit, patients who were readmitted presented a worse overall score (p = 0.01), worse scores in the "motor function" (p = 0.01), "feeding" (p = 0.02), and "respiratory" (p = 0.036) domains, and a higher mortality rate according to the Pediatric Index of Mortality 2 (p = 0.025). CONCLUSION: Evaluation of the functional status using the Functional Status Scale indicated moderate impairment in patients after discharge from the pediatric intensive care unit, mainly in the "motor function" and "feeding" domains; patients who were readmitted to the pediatric intensive care unit demonstrated worse overall functional, motor function, feeding and respiratory scores. Individuals with greater functional impairment had longer times of invasive mechanical ventilation and hospitalization in the pediatric intensive care unit.

OBJETIVO: Avaliar a funcionalidade de pacientes pediátricos após alta da unidade de terapia intensiva pediátrica por meio da Functional Status Scale e comparar o tempo de ventilação mecânica invasiva, tempo de internação e o Pediatric Index of Mortality 2 entre os indivíduos com diferentes graus de comprometimento funcional. MÉTODOS: Estudo transversal, realizado com pacientes egressos de uma unidade de terapia intensiva pediátrica. A avaliação funcional pela Functional Status Scale foi realizada no primeiro dia após a alta da unidade, tendo sido utilizado o Pediatric Index of Mortality 2 como índice preditivo de mortalidade do momento da admissão na unidade. RESULTADOS: A amostra foi composta por 50 indivíduos, sendo 60% do sexo masculino, com mediana de idade de 19 meses [6 - 61]. O escore global da Functional Status Scale foi de 11,5 [7 - 15] e maiores escores nos domínios "função motora" 3 [1 - 4] e "alimentação" 4 [1 - 4]. Os pacientes que reinternaram na unidade de terapia intensiva pediátrica demonstraram, comparativamente aos que não reinternaram, ter pior escore global (p = 0,01), "função motora" (p = 0,01), "alimentação" (p = 0,02), "respiração" (p = 0,036) e maior índice de mortalidade pelo Pediatric Index of Mortality 2 (p = 0,025). CONCLUSÃO: A avaliação da Functional Status Scale indicou disfunção funcional moderada dos pacientes após a alta da unidade de terapia intensiva pediátrica, principalmente na função motora e alimentação; pacientes que reinternaram na unidade de terapia intensiva pediátrica demonstraram ter piores escore funcional global e função motora, alimentação e respiração. Indivíduos com maior comprometimento funcional apresentaram maior tempo de ventilação mecânica invasiva e internação na unidade de terapia intensiva pediátrica.

Avaliação funcional em pacientes pediátricos após alta da unidade de terapia intensiva por meio da Functional Status Scale / Functional evaluation of pediatric patients after discharge from the intensive care unit using the Functional Status Scale

RESUMO Objetivo: Avaliar a funcionalidade de pacientes pediátricos após alta da unidade de terapia intensiva pediátrica por meio da Functional Status Scale e comparar o tempo de ventilação mecânica invasiva, tempo de internação e o Pediatric Index of Mortality 2 entre os indivíduos com diferentes graus de comprometimento funcional. Métodos: Estudo transversal, realizado com pacientes egressos de uma unidade de terapia intensiva pediátrica. A avaliação funcional pela Functional Status Scale foi realizada no primeiro dia após a alta da unidade, tendo sido utilizado o Pediatric Index of Mortality 2 como índice preditivo de mortalidade do momento da admissão na unidade. Resultados: A amostra foi composta por 50 indivíduos, sendo 60% do sexo masculino, com mediana de idade de 19 meses [6 - 61]. O escore global da Functional Status Scale foi de 11,5 [7 - 15] e maiores escores nos domínios "função motora" 3 [1 - 4] e "alimentação" 4 [1 - 4]. Os pacientes que reinternaram na unidade de terapia intensiva pediátrica demonstraram, comparativamente aos que não reinternaram, ter pior escore global (p = 0,01), "função motora" (p = 0,01), "alimentação" (p = 0,02), "respiração" (p = 0,036) e maior índice de mortalidade pelo Pediatric Index of Mortality 2 (p = 0,025). Conclusão: A avaliação da Functional Status Scale indicou disfunção funcional moderada dos pacientes após a alta da unidade de terapia intensiva pediátrica, principalmente na função motora e alimentação; pacientes que reinternaram na unidade de terapia intensiva pediátrica demonstraram ter piores escore funcional global e função motora, alimentação e respiração. Indivíduos com maior comprometimento funcional apresentaram maior tempo de ventilação mecânica invasiva e internação na unidade de terapia intensiva pediátrica.

ABSTRACT Objective: To evaluate the functional status of pediatric patients after discharge from the pediatric intensive care unit using the Functional Status Scale and to compare the time of invasive mechanical ventilation, length of stay in the pediatric intensive care unit, and Pediatric Index of Mortality 2 results among individuals with different degrees of functional impairment. Methods: A cross-sectional study was conducted on patients who were discharged from a pediatric intensive care unit. The functional evaluation by the Functional Status Scale was performed on the first day after discharge from the unit, and the Pediatric Index of Mortality 2 was used to predict the mortality rate at the time of admission to the pediatric intensive care unit. Results: The sample consisted of 50 individuals, 60% of which were male, with a median age of 19 [6 - 61] months. The overall score of the Functional Status Scale was 11.5 [7 - 15], and the highest scores were observed in the "motor function" 3 [1 - 4] and "feeding" 4 [1 - 4] domains. Compared to patients who were not readmitted to the pediatric intensive care unit, patients who were readmitted presented a worse overall score (p = 0.01), worse scores in the "motor function" (p = 0.01), "feeding" (p = 0.02), and "respiratory" (p = 0.036) domains, and a higher mortality rate according to the Pediatric Index of Mortality 2 (p = 0.025). Conclusion: Evaluation of the functional status using the Functional Status Scale indicated moderate impairment in patients after discharge from the pediatric intensive care unit, mainly in the "motor function" and "feeding" domains; patients who were readmitted to the pediatric intensive care unit demonstrated worse overall functional, motor function, feeding and respiratory scores. Individuals with greater functional impairment had longer times of invasive mechanical ventilation and hospitalization in the pediatric intensive care unit.

Oxidative Stress and Lung Ischemia-Reperfusion Injury.

Ischemia-reperfusion (IR) injury is directly related to the formation of reactive oxygen species (ROS), endothelial cell injury, increased vascular permeability, and the activation of neutrophils and platelets, cytokines, and the complement system. Several studies have confirmed the destructiveness of the toxic oxygen metabolites produced and their role in the pathophysiology of different processes, such as oxygen poisoning, inflammation, and ischemic injury. Due to the different degrees of tissue damage resulting from the process of ischemia and subsequent reperfusion, several studies in animal models have focused on the prevention of IR injury and methods of lung protection. Lung IR injury has clinical relevance in the setting of lung transplantation and cardiopulmonary bypass, for which the consequences of IR injury may be devastating in critically ill patients.

Lung and liver changes due to the induction of cirrhosis in two experimental models.

CONTEXT: To evaluate lung and liver changes in two experimental models using intraperitoneal carbon tetrachloride (CCl4) and bile duct ligation (BDL). methods: Twenty-four male Wistar rats were divided into a control group (CO) and an experimental group (EX). We evaluated the liver transaminases (AST, ALT, AP), arterial blood gases (PaO2, PCO2 and SpO2) and lipid peroxidation by TBARS (substances that react to thiobarbituric acid) and chemiluminescence. We also evaluated the antioxidant enzyme superoxide dismutase (SOD) and histology of lung tissue and liver. RESULTS: There were significant differences in AST, ALT, ALP and PaO2 between CO group and EX group (P<0.05). The levels of TBARS, chemiluminescence and activity of enzyme superoxide dismutase were increased to different degrees in the CCl4 groups: CO and in the BDL -EX (P<0.05, respectively). In the lung histology, an increase in the wall thickness of the pulmonary artery and a diameter reduction in the CCl4 animal model were observed: comparing CO group with EX group, we observed a reduction in thickness and an increase in the diameter of the artery wall lung. CONCLUSION: Both experimental models have caused liver damage and alterations in the artery wall that are associated with major changes in pulmonary gas exchange.

Lung and liver hhanges due to the induction of cirrhosis in two experimental models / Alteracoes hepaticas e pulmonares decorrentes da inducao de cirrose em dois modelos experimentais.

Context To evaluate lung and liver changes in two experimental models using intraperitoneal carbon tetrachloride (CCl4) and bile duct ligation (BDL). Methods Twenty-four male Wistar rats were divided into a control group (CO) and an experimental group (EX). We evaluated the liver transaminases (AST, ALT, AP), arterial blood gases (PaO2, PCO2 and SpO2) and lipid peroxidation by TBARS (substances that react to thiobarbituric acid) and chemiluminescence. We also evaluated the antioxidant enzyme superoxide dismutase (SOD) and histology of lung tissue and liver. Results There were significant differences in AST, ALT, ALP and PaO2 between CO group and EX group (P<0.05). The levels of TBARS, chemiluminescence and activity of enzyme superoxide dismutase were increased to different degrees in the CCl4 groups: CO and in the BDL -EX (P<0.05, respectively). In the lung histology, an increase in the wall thickness of the pulmonary artery and a diameter reduction in the CCl4 animal model were observed: comparing CO group with EX group, we observed a reduction in thickness and an increase in the diameter of the artery wall lung. Conclusion Both experimental models have caused liver damage and alterations in the artery wall that are associated with major changes in pulmonary gas exchange. .

Objetivo Avaliar as alterações pulmonares e hepáticas em dois modelos experimentais de cirrose hepática pelo uso de tetracloreto de carbono intraperitoneal (CCl4) e ligadura de ducto biliar. Métodos Vinte e quatro ratos machos Wistar foram divididos em grupo controle (CO) e experimental (EX). Foram avaliadas as transaminases hepáticas (AST, ALT, FA), gasometria arterial (PaO2, PCO2 e SatO2) e a lipoperoxidação através de TBARS (substâncias que reagem ao ácido tiobarbitúrico) e por quimiluminescência. Também foi avaliada a atividade antioxidante da enzima superóxido dismutase e a histologia do tecido pulmonar e hepático. Resultados Nas enzimas hepáticas (AST, ALT e FA), bem como na PaO2 foram observadas diferenças significativas (P≤0,05) entre os grupos CO vs EX em ambos modelos. Os níveis de TBARS, quimiluminescência e a atividade da enzima superóxido dismutase encontram-se aumentados nos grupos CCl4 e ligadura de ducto biliar: CO vs EX (P≤0,05). Na análise histológica do pulmão observamos um aumento na espessura da parede da artéria pulmonar e uma redução no diâmetro no modelo CCl4: CO vs EX, e no modelo de ligadura de ducto biliar podemos observar uma redução da espessura e aumento no diâmetro da parede da artéria pulmonar. Conclusão Ambos os modelos experimentais provocaram dano hepático, além de causar alterações na parede da artéria pulmonar contribuindo na redução das trocas gasosas. .

[Assessment of functional capacity and pulmonary in pediatrics patients renal transplantation].

INTRODUCTION: Pediatric patients undergoing kidney transplantation can present changes in pulmonary function and functional capacity for exercise. OBJECTIVE: To evaluate the functional capacity and pulmonary function in children and adolescents undergoing kidney transplantation. METHOD: Children and adolescents aged six to 18 years of age were evaluated in Outpatient Clinic of Nephrology, Hospital da Criança Santo Antônio, Santa Casa de Porto Alegre, RS, Brazil in period the june of 2010 the march of 2011. Pulmonary capacity was assessed by spirometry and maximal respiratory pressures and functional capacity through 6-minute walk test (6MWT). RESULTS: The sample comprised 25 patients, 14 (56%) males with a mean age of 13.5 ± 3.3 years. From, 19 (76%) underwent dialysis before transplantation. Mean forced vital capacity (FVC) was 97.91 + 24.32% and forced expiratory volume in one second (FEV1) 100.53 + 17.66% from predicted value. In the 6MWT, the patients walked 229.14 meters less than predicted (p < 0.001). The maximum inspiratory pressure (MIP) was significantly lower than predicted, and the difference in cmH2O -24.63 (p = 0.03), as the maximum expiratory pressure (MEP), with a difference of 49.27 cmH2O (p < 0.001). By correlating, functional capacity, spirometry and maximal respiratory pressures, find an association between FVC and 6MWT (r = 0.52, p = 0.01) and FVC and MIP (r = 0.54, p = 0.01). CONCLUSION: Reduced functional capacity and maximum respiratory pressures were diagnosed in a small cohort of pediatric patients after kidney transplantation. Better the functional capacity and PiMáx better the FVC.

Avaliação da capacidade funcional e pulmonar em pacientes pediátricos transplantados renais / Assessment of functional capacity and pulmonary in pediatrics patients renal transplantation

INTRODUÇÃO: Pacientes pediátricos submetidos a transplante renal podem apresentar alterações na função pulmonar, bem como na capacidade funcional para o exercício. OBJETIVO: Avaliar a capacidade funcional e capacidade pulmonar de crianças e adolescentes submetidas a transplante renal. MÉTODO: Foram avaliadas crianças e adolescentes com idade entre 6 e 18 anos em acompanhamento no ambulatório de Nefrologia do Hospital da Criança Santo Antônio, Santa Casa de Porto Alegre-RS, Brasil, no período de junho de 2010 a março de 2011. A capacidade pulmonar foi avaliada por meio da espirometria e das pressões respiratórias máximas e a capacidade funcional pelo Teste da Caminhada dos 6 minutos (TC6). RESULTADOS: A amostra foi composta por 25 pacientes, sendo 14 (56%) do sexo masculino, com média de idade de 13,5 ± 3,3 anos. Destes, 19 (76%) realizaram hemodiálise anterior ao transplante. A média da Capacidade Vital Forçada (CVF) foi 97,91 ± 24,32% e o Volume Expiratório Forçado no primeiro segundo (VEF1) foi 100,53 ± 17,66% do valor predito. No TC6, os pacientes caminharam 229,14 metros menos do que o predito (p < 0,001). A Pressão Inspiratória Máxima (PiMáx) foi significativamente menor que o predito, com uma diferença de -24,63 cmH2O (p = 0,03), assim como a Pressão Expiratória Máxima (Pe-Máx), com uma diferença de 49,27 cmH2O (p < 0,001). Ao correlacionarmos capacidade funcional, espirometria e pressões respiratórias máximas, encontramos associação entre CVF e TC6 (r = 0,52, p = 0,01) e CVF e PiMáx (r = 0,54 e p = 0,01). CONCLUSÃO: Os pacientes transplantados apresentaram diminuição da capacidade funcional, bem como das pressões respiratórias máximas. Quanto melhor a capacidade funcional e a PiMáx, melhor a CVF.

INTRODUCTION: Pediatric patients undergoing kidney transplantation can present changes in pulmonary function and functional capacity for exercise. OBJECTIVE: To evaluate the functional capacity and pulmonary function in children and adolescents undergoing kidney transplantation. METHOD: Children and adolescents aged six to 18 years of age were evaluated in Outpatient Clinic of Nephrology, Hospital da Criança Santo Antônio, Santa Casa de Porto Alegre, RS, Brazil in period the june of 2010 the march of 2011. Pulmonary capacity was assessed by spirometry and maximal respiratory pressures and functional capacity through 6-minute walk test (6MWT). RESULTS: The sample comprised 25 patients, 14 (56%) males with a mean age of 13.5 ± 3.3 years. From, 19 (76%) underwent dialysis before transplantation. Mean forced vital capacity (FVC) was 97.91 + 24.32% and forced expiratory volume in one second (FEV1) 100.53 + 17.66% from predicted value. In the 6MWT, the patients walked 229.14 meters less than predicted (p < 0.001). The maximum inspiratory pressure (MIP) was significantly lower than predicted, and the difference in cmH2O -24.63 (p = 0.03), as the maximum expiratory pressure (MEP), with a difference of 49.27 cmH2O (p < 0.001). By correlating, functional capacity, spirometry and maximal respiratory pressures, find an association between FVC and 6MWT (r = 0.52, p = 0.01) and FVC and MIP (r = 0.54, p = 0.01). CONCLUSION: Reduced functional capacity and maximum respiratory pressures were diagnosed in a small cohort of pediatric patients after kidney transplantation. Better the functional capacity and PiMáx better the FVC.