Translation and cross-cultural adaptation of the anchor points of the Cornell Assessment of Pediatric Delirium scale into Portuguese.

OBJECTIVE: To translate and cross-culturally adapt the Cornell Assessment of Pediatric Delirium anchor points from English to Brazilian Portuguese. METHODS: For the translation and cross-cultural adaptation of the anchor points, all steps recommended internationally were followed after authorization for use by the lead author. The stages were as follows: translation of the original version into Portuguese by two bilingual translators who were native speakers of the target language, synthesis of the versions, reverse translation by two translators who were native speakers of the source language, review and synthesis of the back-translation, review by a committee of experts and preparation of the final version. RESULTS: The translation and cross-cultural adaptation of the anchor points was conducted in accordance with recommendations. The linguistic and semantic issues that arose were discussed by a committee of judges, with 91.8% agreement, as determined using a Likert scale, after changes by consensus. After reanalysis by the authors, there were no changes, resulting in the final version, which was easy to understand and administer. CONCLUSION: The translation and cross-cultural adaptation of the anchor points of the Cornell Assessment of Pediatric Delirium scale into Portuguese spoken in Brazil were successful, maintaining the linguistic and semantic properties of the original instrument. The table of anchor points is easy to understand and will be helpful during the assessment of children younger than 24 months using the Cornell Assessment of Pediatric Delirium scale.

Translation and cross-cultural adaptation of the anchor points of the Cornell Assessment of Pediatric Delirium scale into Portuguese / Tradução e adaptação transcultural dos pontos âncoras da escala Cornell Assessment of Pediatric Delirium para língua portuguesa

ABSTRACT Objective: To translate and cross-culturally adapt the Cornell Assessment of Pediatric Delirium anchor points from English to Brazilian Portuguese. Methods: For the translation and cross-cultural adaptation of the anchor points, all steps recommended internationally were followed after authorization for use by the lead author. The stages were as follows: translation of the original version into Portuguese by two bilingual translators who were native speakers of the target language, synthesis of the versions, reverse translation by two translators who were native speakers of the source language, review and synthesis of the back-translation, review by a committee of experts and preparation of the final version. Results: The translation and cross-cultural adaptation of the anchor points was conducted in accordance with recommendations. The linguistic and semantic issues that arose were discussed by a committee of judges, with 91.8% agreement, as determined using a Likert scale, after changes by consensus. After reanalysis by the authors, there were no changes, resulting in the final version, which was easy to understand and administer. Conclusion: The translation and cross-cultural adaptation of the anchor points of the Cornell Assessment of Pediatric Delirium scale into Portuguese spoken in Brazil were successful, maintaining the linguistic and semantic properties of the original instrument. The table of anchor points is easy to understand and will be helpful during the assessment of children younger than 24 months using the Cornell Assessment of Pediatric Delirium scale.

RESUMO Objetivo: Traduzir e realizar a adaptação transcultural dos pontos âncoras da escala Cornell Assessment of Pediatric Delirium do inglês para a língua portuguesa do Brasil. Métodos: O processo de tradução e adaptação transcultural dos pontos âncoras seguiu todas as etapas recomendadas internacionalmente após a autorização de uso pela autora principal. As etapas foram: tradução da versão original para língua portuguesa por dois tradutores bilíngues nativos do idioma-alvo; síntese das versões; tradução reversa por dois tradutores nativos do idioma de origem; revisão e síntese da retradução; revisão por um comitê de juízes formado por especialistas e elaboração da versão final. Resultados: O processo de tradução e adaptação transcultural dos pontos âncoras seguiu as recomendações. As questões linguísticas e semânticas que surgiram foram discutidas pelo comitê de juízes, no qual se observou concordância de 91,8% pela escala de Likert com pequenas alterações de forma consensual. Após reanálise dos autores, não houve alterações, resultando na versão final, de fácil compreensão e administração. Conclusão: A tradução e a adaptação transcultural dos pontos âncoras da escala de Cornell Assessment of Pediatric Delirium para a língua portuguesa falada no Brasil foram bem-sucedidas com manutenção das propriedades linguísticas e semânticas do instrumento original. A tabela dos pontos âncoras mostrou ser de fácil compreensão e auxílio durante a avaliação das crianças abaixo de 24 meses por meio da escala de Cornell Assessment of Pediatric Delirium.

Analyses of the soluble levels of sCD40L, sCD62P and sCD40 in pediatric sickle cell anemia patients with abnormal transcranial Doppler.

INTRODUCTION: Serum levels of sCD40L, sCD40 and sCD62P were evaluated in sickle cell anemia (SCA) patients aged between 2 and 16 years with normal transcranial Doppler (TCD) and no stroke (G1, n = 24); in SCA patients with abnormal TCD (G2, n = 16); in SCA patients with a previous history of stroke (G3, n = 8), and; in healthy controls (aged 2 to 13 years; n = 26). RESULTS: The levels of sCD40L were significantly higher in the G1, G2 and G3 groups, compared to controls (p = 0.0001, p < 0.0002 and p = 0.004, respectively). Among patients with SCA, higher levels of sCD40L were found in the G3 group, compared to the G2 group (p = 0.03). In the sCD62P analysis, high levels in G3, compared to G1 (p = 0.0001), G2 (p = 0.03) and G4 (p = 0.01), and G2 also had high levels, compared to G1 (p = 0.04). The G1 patients had a higher sCD40L/sCD62P ratio, compared to G2 (p = 0.003) and controls (p < 0.0001). The sCD40L/sCD40 ratios were higher in G1, G2 and G3, compared to controls (p < 0.0001, p = 0.008 and p = 0.002, respectively). CONCLUSION: It was concluded that the combination of TCD abnormality, associated with levels of sCD40L and sCD62P, may contribute to a better assessment of the risk for stroke in pediatric SCA patients. Favor pedir para os autores corrigirem esta frase, pois NÃO é uma sentença completa em inglês: Our data suggest that decreased values of the s[LSM1] CD40L/sCD62P ratio involving two inflammatory mediators produced in platelet activation, being unprecedented in the literature.

Clinical Characteristics and Mortality Profile of COVID-19 Patients Aged less than 20 years Old in Pernambuco - Brazil.

COVID-19 in children and adolescents has low frequency, severity, and fatality rate all over the world. A cross-sectional study was conducted to assess the epidemiological and clinical aspects of COVID-19 in patients younger than 20 years in Pernambuco (Brazil), with cases confirmed by reverse-transcriptase-PCR SARS-CoV-2 between 13 February and June 19, 2020, reported on information systems. Data regarding age (< 30 days, 1-11 months, 1-4 years, 5-9 years, 10-14 years, and 15-19 years), gender, color/race, symptoms, pregnancy or puerperium, comorbidities, hospitalization, and death were investigated. Fatality rate and mortality coefficient were calculated, and a multiple logistic regression analysis was performed to determine if gender, age, and comorbidities were factors associated with death. Of 682 pediatric cases, 52.8% were female, with a mean age of 9 ± 7.2 years. The most frequent symptoms were fever (64.4%), cough (52.4%), and respiratory distress (32.4%). Hospitalization was reported in 46.2% of cases, mainly among neonates (80.3%) and infants (73.8%). Thirty-eight deaths were notified, and a fatality rate of 5.6% (95% CI: 3.9-7.3) was found, with higher fatality rates among neonates 11.5% (7 of 61) and 9.5% (8 of 84) infants. The mortality coefficient was 10.9 per 100,000 inhabitants < 1 year of age, whereas comorbidities (Odds ratio [OR] = 14.13, 95% CI: 6.35-31.44), age < 30 days (OR = 5.17, 95% CI: 1.81-14.77), and age 1-11 months (OR = 3.28, 95% CI: 1.21-8.91) were independent factors associated with death. The results demonstrate the vulnerability of neonates and infants with severe conditions, need hospitalization, and high fatality rate, indicating the necessity to adapt public health policies for these age-groups.

Temporal Validation of a Predictive Score for Death in Children with Visceral Leishmaniasis.

OBJECTIVES: To perform a temporal validation of a predictive model for death in children with visceral leishmaniasis (VL). METHODS: A temporal validation of a children-exclusive predictive model of death due to VL (Sampaio et al. 2010 model), using a retrospective cohort, hereby called validation cohort. The validation cohort convenience sample was made of 156 patients less than 15 years old hospitalized between 2008 and 2018 with VL. Patients included in the Sampaio et al. 2010 study are here denominated derivation cohort, which was composed of 546 patients hospitalized in the same hospital setting in the period from 1996 to 2006. The calibration and discriminative capacity of the model to predict death by VL in the validation cohort were then assessed through the procedure of logistic recalibration that readjusted its coefficients. The calibration of the updated model was tested using Hosmer-Lemeshow test and Spiegelhalter test. A ROC curve was built and the value of the area under this curve represented the model's discrimination. RESULTS: The validation cohort found a lethality of 6.4%. The Sampaio et al. 2010 model demonstrated inadequate calibration in the validation cohort (Spiegelhalter test: p=0.007). It also presented unsatisfactory discriminative capacity, evaluated by the area under the ROC curve = 0.618. After the coefficient readjustment, the model showed adequate calibration (Spiegelhalter test, p=0.988) and better discrimination, becoming satisfactory (AUROC = 0.762). The score developed by Sampaio et al. 2010 attributed 1 point to the variables dyspnea, associated infections, and neutrophil count <500/mm3; 2 points to jaundice and mucosal bleeding; and 3 points to platelet count <50,000/mm3. In the recalibrated model, each one of the variables had a scoring of 1 point for each. CONCLUSION: The temporally validated model, after coefficient readjustment, presented adequate calibration and discrimination to predict death in children hospitalized with VL.

Noninvasive ventilation as the first choice of ventilatory support in children. / Ventilação não invasiva como primeira escolha de suporte ventilatório em crianças.

OBJECTIVE: To describe the use of noninvasive ventilation to prevent tracheal intubation in children in a pediatric intensive care unit and to analyze the factors related to respiratory failure. METHODS: A retrospective cohort study was performed from January 2016 to May 2018. The study population included children aged 1 to 14 years who were subjected to noninvasive ventilation as the first therapeutic choice for acute respiratory failure. Biological, clinical and managerial data were analyzed by applying a model with the variables that obtained significance ≤ 0.20 in a bivariate analysis. Logistic regression was performed using the ENTER method. The level of significance was set at 5%. RESULTS: The children had a mean age of 68.7 ± 42.3 months, 96.6% had respiratory disease as a primary diagnosis, and 15.8% had comorbidities. Of the 209 patients, noninvasive ventilation was the first option for ventilatory support in 86.6% of the patients, and the fraction of inspired oxygen was ≥ 0.40 in 47% of the cases. The lethality rate was 1.4%. The data for the use of noninvasive ventilation showed a high success rate of 95.3% (84.32 - 106). The Pediatric Risk of Mortality (PRISM) score and the length of stay in the intensive care unit were the significant clinical variables for the success or failure of noninvasive ventilation. CONCLUSION: A high rate of effectiveness was found for the use of noninvasive ventilation for acute episodes of respiratory failure. A higher PRISM score on admission, comorbidities associated with respiratory symptoms and oxygen use ≥ 40% were independent factors related to noninvasive ventilation failure.

OBJETIVO: Descrever o uso da ventilação não invasiva na prevenção da intubação traqueal em crianças em unidade de terapia intensiva pediátrica e analisar os fatores relacionados à falha. MÉTODOS: Coorte retrospectiva referente ao período de janeiro 2016 a maio 2018. População composta por crianças entre1mês a14 anos, submetidas à ventilação não invasiva como primeira escolha terapêutica para insuficiência respiratória aguda. Analisaram-se os dados biológicos, clínicos e gerenciais, sendo aplicado um modelo com as variáveis que obtiveram significância ≤ 0,20 na análise bivariada. Foi realizada regressão logística pelo método de ENTER. Considerou-se nível de significância de 5%. RESULTADOS: As crianças tiveram média de idade de 68,7 ± 42,3 meses, 96,6% tiveram como diagnóstico principal doença respiratória e 15,8% apresentavam comorbidades. Do total de 209, a ventilação não invasiva foi realizada como primeira opção de suporte ventilatório em 86,6% dos pacientes e a fração inspirada de oxigênio ≥ 0,40 em 47% dos casos. A letalidade foi de 1,4%. O gerenciamento dos dados do uso da ventilação não invasiva demonstrou alta taxa de sucesso, sendo esta de 95,3% (84,32 - 106). As variáveis clínicas significantes no sucesso ou na falha da ventilação não invasiva foram o Pediatric Risk of Mortality (PRISM) e o tempo de estadia na unidade de terapia intensiva. CONCLUSÃO: Observou-se alta taxa de efetividade no uso da ventilação não invasiva para episódios agudos de insuficiência respiratória. PRISM de admissão mais altos, comorbidades associadas ao quadro respiratório e uso de oxigênio ≥ 40%foram fatores independentes relacionados à falha da ventilação não invasiva.

Ventilação não invasiva como primeira escolha de suporte ventilatório em crianças / Noninvasive ventilation as the first choice of ventilatory support in children

RESUMO Objetivo: Descrever o uso da ventilação não invasiva na prevenção da intubação traqueal em crianças em unidade de terapia intensiva pediátrica e analisar os fatores relacionados à falha. Métodos: Coorte retrospectiva referente ao período de janeiro 2016 a maio 2018. População composta por crianças entre1mês a14 anos, submetidas à ventilação não invasiva como primeira escolha terapêutica para insuficiência respiratória aguda. Analisaram-se os dados biológicos, clínicos e gerenciais, sendo aplicado um modelo com as variáveis que obtiveram significância ≤ 0,20 na análise bivariada. Foi realizada regressão logística pelo método de ENTER. Considerou-se nível de significância de 5%. Resultados: As crianças tiveram média de idade de 68,7 ± 42,3 meses, 96,6% tiveram como diagnóstico principal doença respiratória e 15,8% apresentavam comorbidades. Do total de 209, a ventilação não invasiva foi realizada como primeira opção de suporte ventilatório em 86,6% dos pacientes e a fração inspirada de oxigênio ≥ 0,40 em 47% dos casos. A letalidade foi de 1,4%. O gerenciamento dos dados do uso da ventilação não invasiva demonstrou alta taxa de sucesso, sendo esta de 95,3% (84,32 - 106). As variáveis clínicas significantes no sucesso ou na falha da ventilação não invasiva foram o Pediatric Risk of Mortality (PRISM) e o tempo de estadia na unidade de terapia intensiva. Conclusão: Observou-se alta taxa de efetividade no uso da ventilação não invasiva para episódios agudos de insuficiência respiratória. PRISM de admissão mais altos, comorbidades associadas ao quadro respiratório e uso de oxigênio ≥ 40%foram fatores independentes relacionados à falha da ventilação não invasiva.

ABSTRACT Objective: To describe the use of noninvasive ventilation to prevent tracheal intubation in children in a pediatric intensive care unit and to analyze the factors related to respiratory failure. Methods: A retrospective cohort study was performed from January 2016 to May 2018. The study population included children aged 1 to 14 years who were subjected to noninvasive ventilation as the first therapeutic choice for acute respiratory failure. Biological, clinical and managerial data were analyzed by applying a model with the variables that obtained significance ≤ 0.20 in a bivariate analysis. Logistic regression was performed using the ENTER method. The level of significance was set at 5%. Results: The children had a mean age of 68.7 ± 42.3 months, 96.6% had respiratory disease as a primary diagnosis, and 15.8% had comorbidities. Of the 209 patients, noninvasive ventilation was the first option for ventilatory support in 86.6% of the patients, and the fraction of inspired oxygen was ≥ 0.40 in 47% of the cases. The lethality rate was 1.4%. The data for the use of noninvasive ventilation showed a high success rate of 95.3% (84.32 - 106). The Pediatric Risk of Mortality (PRISM) score and the length of stay in the intensive care unit were the significant clinical variables for the success or failure of noninvasive ventilation. Conclusion: A high rate of effectiveness was found for the use of noninvasive ventilation for acute episodes of respiratory failure. A higher PRISM score on admission, comorbidities associated with respiratory symptoms and oxygen use ≥ 40% were independent factors related to noninvasive ventilation failure.

Is oxidized low-density lipoprotein the connection between atherosclerosis, cardiovascular risk and nephrolithiasis?

Nephrolithiasis is considered a systemic disease. A link has been established between nephrolithiasis, cardiovascular disease (CVD), the metabolic syndrome and atherosclerosis. A significant correlation has been found between the high levels of oxidized low-density lipoprotein (oxLDL) and CVD and atherosclerosis, including coronary and femoral artery disease. To the best of our knowledge, oxLDL has not been evaluated in patients with nephrolithiasis. This study aimed to evaluate serum levels of oxLDL, anti-oxLDL antibodies (oxLDL-ab) and other markers of atherosclerosis in patients with nephrolithiasis, according to the severity of the disease. The population sample consisted of 94 patients of 30-70 years of age with no symptoms of CVD who presented with renal calculi documented by ultrasonography, abdominal X-ray or computed tomography. The patients were divided into two groups: Group 1 (≥ 3 stones) and Group 2 (1-2 stones). A comparison control group was formed with 21 healthy individuals. Enzyme-linked immunosorbent assays were used to assess oxLDL and oxLDL-ab. Lipid peroxidation indexes were also analyzed. Median serum oxLDL values were higher in Groups 1 and 2 compared to the control group (≥ 3 stones, p = 0.02; 1-2 stones, p = 0.03). Median serum anti-oxLDL antibody levels were lower in the patients in Group 1 compared to the controls (p = 0.03). There was no significant difference in the oxLDL/oxLDL-ab ratio between patients and controls. These findings suggest that this may be the link between nephrolithiasis and the greater incidence of atherosclerosis and cardiovascular disease in patients with kidney stones.

Translation and cross-cultural adaptation of the Cornell Assessment of Pediatric Delirium scale for the Portuguese language. / Tradução e adaptação transcultural da escala Cornell Assessment of Pediatric Delirium para língua portuguesa.

OBJECTIVE: This study sought to translate the Cornell Assessment of Pediatric Delirium from English into Brazilian Portuguese and cross-culturally adapt it for use in Brazil. METHODS: Following the authorization granted by its main author, the processes of translation and cross-cultural adaptation were performed with regard to the Cornell Assessment of Pediatric Delirium in accordance with the following internationally recommended steps: translation of the original into Portuguese by two native speakers of the target language; synthesis of the translated versions; back-translation by two native speakers of the original language; review and harmonization of the back-translation; a review of the Portuguese version of the Cornell Assessment of Pediatric Delirium by an expert panel composed of specialists; pretesting including assessments of clarity, comprehensibility, and acceptability of the translated version using a sample of the target population; and finishing modifications to achieve the final version. RESULTS: The translation and cross-cultural adaptation of the Cornell Assessment of Pediatric Delirium followed international recommendations. The linguistic and semantic issues that emerged during the process were discussed by the expert panel, which unanimously agreed to slight modifications. During pretesting, the Cornell Assessment of Pediatric Delirium was administered to 30 eligible children, twice per day; the final version was easy to understand, could be completed quickly, and showed a high inter-rater correlation coefficient (0.955). CONCLUSIONS: The translation of the Cornell Assessment of Pediatric Delirium into Brazilian Portuguese and its cross-cultural adaptation were successful and preserved the linguistic and semantic properties of the original instrument. The Cornell Assessment of Pediatric Delirium proved to be easy to understand and could be completed quickly. Additional studies are needed to test the validity and psychometric properties of this version in Brazil.

OBJETIVO: Traduzir e adaptar transculturalmente a escala Cornell Assesment of Pediatric Delirium do inglês para a língua portuguesa do Brasil. MÉTODOS: O processo de tradução e adaptação transcultural da escala Cornell Assesment of Pediatric Delirium seguiu as etapas recomendadas internacionalmente após autorização de uso pela autora principal. As etapas seguidas foram: tradução da versão original para língua portuguesa por dois tradutores bilíngues nativos do idioma alvo; síntese das versões; tradução reversa por dois tradutores nativos do idioma de origem; revisão e harmonização da retradução; revisão da versão em português da Cornell Assesment of Pediatric Delirium por um comitê de juízes formado por especialistas; pré-teste (avaliação de clareza, compreensibilidade e aceitabilidade da versão traduzida em uma amostra da população-alvo) e reconciliação para a elaboração da versão final. RESULTADOS: O processo de tradução e adaptação transcultural da Cornell Assesment of Pediatric Delirium seguiu as recomendações internacionais. As questões linguísticas e semânticas que surgiram durante o processo foram discutidas no comitê de juízes, no qual se observou grande concordância com pequenas alterações. Na etapa de pré-teste, a Cornell Assesment of Pediatric Delirium foi aplicada em 30 crianças elegíveis, duas vezes ao dia, resultando em uma versão de fácil compreensão e rápida administração, com excelente coeficiente de correlação intraobservadores (0,955). CONCLUSÕES: A tradução e a adaptação transcultural da Cornell Assesment of Pediatric Delirium para a língua portuguesa falada no Brasil foram bem-sucedidas e mantiveram as propriedades linguísticas e semânticas do instrumento original. A Cornell Assesment of Pediatric Delirium mostrou ser de fácil compreensão e de rápida aplicação. Novos estudos são necessários para testar a validade e as propriedades psicométricas desta versão no Brasil.

Tradução e adaptação transcultural da escala Cornell Assessment of Pediatric Delirium para língua portuguesa / Translation and cross-cultural adaptation of the Cornell Assessment of Pediatric Delirium scale for the Portuguese language

RESUMO Objetivo: Traduzir e adaptar transculturalmente a escala Cornell Assesment of Pediatric Delirium do inglês para a língua portuguesa do Brasil. Métodos: O processo de tradução e adaptação transcultural da escala Cornell Assesment of Pediatric Delirium seguiu as etapas recomendadas internacionalmente após autorização de uso pela autora principal. As etapas seguidas foram: tradução da versão original para língua portuguesa por dois tradutores bilíngues nativos do idioma alvo; síntese das versões; tradução reversa por dois tradutores nativos do idioma de origem; revisão e harmonização da retradução; revisão da versão em português da Cornell Assesment of Pediatric Delirium por um comitê de juízes formado por especialistas; pré-teste (avaliação de clareza, compreensibilidade e aceitabilidade da versão traduzida em uma amostra da população-alvo) e reconciliação para a elaboração da versão final. Resultados: O processo de tradução e adaptação transcultural da Cornell Assesment of Pediatric Delirium seguiu as recomendações internacionais. As questões linguísticas e semânticas que surgiram durante o processo foram discutidas no comitê de juízes, no qual se observou grande concordância com pequenas alterações. Na etapa de pré-teste, a Cornell Assesment of Pediatric Delirium foi aplicada em 30 crianças elegíveis, duas vezes ao dia, resultando em uma versão de fácil compreensão e rápida administração, com excelente coeficiente de correlação intraobservadores (0,955). Conclusões: A tradução e a adaptação transcultural da Cornell Assesment of Pediatric Delirium para a língua portuguesa falada no Brasil foram bem-sucedidas e mantiveram as propriedades linguísticas e semânticas do instrumento original. A Cornell Assesment of Pediatric Delirium mostrou ser de fácil compreensão e de rápida aplicação. Novos estudos são necessários para testar a validade e as propriedades psicométricas desta versão no Brasil.

ABSTRACT Objective: This study sought to translate the Cornell Assessment of Pediatric Delirium from English into Brazilian Portuguese and cross-culturally adapt it for use in Brazil. Methods: Following the authorization granted by its main author, the processes of translation and cross-cultural adaptation were performed with regard to the Cornell Assessment of Pediatric Delirium in accordance with the following internationally recommended steps: translation of the original into Portuguese by two native speakers of the target language; synthesis of the translated versions; back-translation by two native speakers of the original language; review and harmonization of the back-translation; a review of the Portuguese version of the Cornell Assessment of Pediatric Delirium by an expert panel composed of specialists; pretesting including assessments of clarity, comprehensibility, and acceptability of the translated version using a sample of the target population; and finishing modifications to achieve the final version. Results: The translation and cross-cultural adaptation of the Cornell Assessment of Pediatric Delirium followed international recommendations. The linguistic and semantic issues that emerged during the process were discussed by the expert panel, which unanimously agreed to slight modifications. During pretesting, the Cornell Assessment of Pediatric Delirium was administered to 30 eligible children, twice per day; the final version was easy to understand, could be completed quickly, and showed a high inter-rater correlation coefficient (0.955). Conclusions: The translation of the Cornell Assessment of Pediatric Delirium into Brazilian Portuguese and its cross-cultural adaptation were successful and preserved the linguistic and semantic properties of the original instrument. The Cornell Assessment of Pediatric Delirium proved to be easy to understand and could be completed quickly. Additional studies are needed to test the validity and psychometric properties of this version in Brazil.

Level of independence, functional capacity and respiratory muscle strength of patients with mucopolysaccharidosis type VI in the Northeast of Brazil / Nível de independência, capacidade funcional e força muscular respiratória de pacientes com mucopolissacaridose tipo VI no Nordeste do Brasil

Abstract Objectives: to assess the functional independence, functional capacity and respiratory muscle strength (RMS) in individuals with mucopolysaccharidosis (MPS) type VI. Methods: in this cross-sectional study, the Pediatric Evaluation of Disability Inventory (PEDI) and the Functional Independence Measure (FIM) scales were used to assess the functional independence. The functional capacity and the RMS were assessed by a 6-minute walk test (6MWT) and Manovacuometry, respectively. The associations between RMS and 6MWD were analyzed by using the adjusted simple linear regression models. And besides, the T-test was used to compare the differences among the groups. Results: twenty-four patients with MPS VI were included, the median age was 8 (ranged from 2-38 years old). The PEDI presented a functional performance below the expectations at the self-care and mobility domains of 33.3% when compared to groups of Brazilian children at the same age assessed with typical development. The decreased social function was found in only one single case. According to the FIM, of the 12 patients evaluated (age> 7.5 years), 58.3% were classified as modified dependence and 41.7% as modified independence. The mean distance in the 6MWT was significantly lower than predicted, 263m in G1 (children, n= 7) and 336m in G2 (adolescents and adults, n=6), p<0.001. The RMS was also lower than predicted in both groups, except for the RMS in G1. Conclusions: the functional capacity and the RMS were significantly reduced in individuals with MPS VI, with a decreased functional independence in one-third of the patients. However, a multidisciplinary follow-up in this population is essential to prevent, diagnose and treat early complications.

Resumo Objetivos: avaliar a independência funcional, capacidade funcional e força muscular respiratória (FMR) em indivíduos com mucopolissacaridose (MPS) tipo VI. Métodos: neste estudo transversal, as escalas Inventário de Avaliação Pediátrica de Incapacidade (PEDI) e Medida de Independência Funcional (MIF) foram utilizadas para avaliar a independência funcional. A capacidade funcional e a FMR foram avaliadas por teste de caminhada de 6 minutos (6MWT) e Manovacuometria, respectivamente. As associações entre FMR e 6MWD foram analisadas usando modelos de regressão linear simples. Além disso, t-test foi usado para comparar diferenças entre grupos. Resultados: vinte e quatro pacientes com MPS VI foram incluídos no estudo, a média da idade foi 8 anos (2-38 anos). A PEDI apresentou desempenho funcional abaixo das expectativas nos domínios de autocuidado e mobilidade em 33,3% quando comparada com grupos de crianças brasileiras com desenvolvimento típico da mesma idade avaliada. A diminuição da função social foi encontrada em apenas um único caso. De acordo com a MIF, dos 12 pacientes avaliados (idade> 7,5 anos), 58,3% foram classificados como dependência modificada e 41,7% como independência modificada. A distância média no 6MWTfoi significativamente menor do que o previsto, 263 m em G1 (crianças, n = 7) e 336 m em G2 (adolescentes e adultos, n = 6), p<0,001. A FMR também foi menor do que o previsto em ambos os grupos, com exceção em G1. Conclusões: a capacidade funcional e a FMR foram signif icativamente reduzidas em indivíduos com MPS VI, com diminuição da independência funcional em um terço dos pacientes. Portanto, o seguimento multidisciplinar no acompanhamento dessa população é essencial para prevenir, diagnosticar e tratar complicações precocemente.

Weaning failure and respiratory muscle function: What has been done and what can be improved?

INTRODUCTION: Respiratory muscle dysfunction, being a common cause of weaning failure, is strongly associated with prolonged mechanical ventilation (MV) and prolonged stay in intensive care units. Inspiratory muscle training (IMT) has been described as an important contributor to the treatment of respiratory muscle dysfunction in critically ill patients. Its effectiveness is however yet controversial. OBJECTIVE: To discuss evidence for assessment of readiness and the effectiveness of interventions for liberation from MV, with special attention to the role of IMT. METHODS: PubMed, LILACS, PEDro and Web of Science were searched for papers of assessment and treatment of patients who failed liberation from MV after at least one attempt published in English or Portuguese until June 2016. RESULTS: Weaning predictors are related to weaning success (86%-100% for sensitivity and 7%-69% for specificity) and work of breathing (73%-100% for sensitivity and 56%-100% for specificity). Spontaneous breathing trials (SBT), noninvasive MV and early mobilization have been reported to improve weaning outcomes. Two modalities of IMT were identified in five selected studies: 1) adjustment of ventilator trigger sensitivity 2) inspiratory threshold loading. Both IMT training modalities promoted significant increases in respiratory muscle strength. IMT with threshold loading showed positive effect on endurance compared to control. CONCLUSION: Methods to indentify respiratory muscle weakness in critically ill patients are feasible and described as indexes that show good accuracy. Individualized and supervised rehabilitation programs including IMT, SBT, noninvasive MV and early mobilization should be encouraged in patients with inspiratory muscle weakness.

Effects of pressure support ventilation on ventilator-induced lung injury in mild acute respiratory distress syndrome depend on level of positive end-expiratory pressure: A randomised animal study.

BACKGROUND: Harmful effects of spontaneous breathing have been shown in experimental severe acute respiratory distress syndrome (ARDS). However, in the clinical setting, spontaneous respiration has been indicated only in mild ARDS. To date, no study has compared the effects of spontaneous assisted breathing with those of fully controlled mechanical ventilation at different levels of positive end-expiratory pressure (PEEP) on lung injury in ARDS. OBJECTIVE: To compare the effects of assisted pressure support ventilation (PSV) with pressure-controlled ventilation (PCV) on lung function, histology and biological markers at two different PEEP levels in mild ARDS in rats. DESIGN: Randomised controlled experimental study. SETTING: Basic science laboratory. PARTICIPANTS: Thirty-five Wistar rats (weight ±â€ŠSD, 310 ±â€Š19) g received Escherichia coli lipopolysaccharide (LPS) intratracheally. After 24 h, the animals were anaesthetised and randomly allocated to either PCV (n=14) or PSV (n=14) groups. Each group was further assigned to PEEP = 2 cmH2O or PEEP = 5 cmH2O. Tidal volume was kept constant (≈6 ml kg). Additional nonventilated animals (n=7) were used as a control for postmortem analysis. MAIN OUTCOME MEASURES: Ventilatory and mechanical parameters, arterial blood gases, diffuse alveolar damage score, epithelial integrity measured by E-cadherin tissue expression, and biological markers associated with inflammation (IL-6 and cytokine-induced neutrophil chemoattractant, CINC-1) and type II epithelial cell damage (surfactant protein-B) were evaluated. RESULTS: In both PCV and PSV, peak transpulmonary pressure was lower, whereas E-cadherin tissue expression, which is related to epithelial integrity, was higher at PEEP = 5 cmH2O than at PEEP = 2 cmH2O. In PSV, PEEP = 5 cmH2O compared with PEEP = 2 cmH2O was associated with significantly reduced diffuse alveolar damage score [median (interquartile range), 11 (8.5 to 13.5) vs. 23 (19 to 26), P = 0.005] and expressions of IL-6 and CINC-1 (P = 0.02 for both), whereas surfactant protein-B mRNA expression increased (P = 0.03). These changes suggested less type II epithelial cell damage at a PEEP of 5 cmH2O. Peak transpulmonary pressure correlated positively with IL-6 [Spearman's rho (ρ) = 0.62, P = 0.0007] and CINC-1 expressions (ρ = 0.50, P = 0.01) and negatively with E-cadherin expression (ρ = -0.67, P = 0.0002). CONCLUSION: During PSV, PEEP of 5 cmH2O, but not a PEEP of 2 cmH2O, reduced lung damage and inflammatory markers while maintaining epithelial cell integrity.

Follow-up brain imaging of 37 children with congenital Zika syndrome: case series study.

Objective To compare initial brain computed tomography (CT) scans with follow-up CT scans at one year in children with congenital Zika syndrome, focusing on cerebral calcifications.Design Case series study.Setting Barão de Lucena Hospital, Pernambuco state, Brazil.Participants 37 children with probable or confirmed congenital Zika syndrome during the microcephaly outbreak in 2015 who underwent brain CT shortly after birth and at one year follow-up.Main outcome measure Differences in cerebral calcification patterns between initial and follow-up scans.Results 37 children were evaluated. All presented cerebral calcifications on the initial scan, predominantly at cortical-white matter junction. At follow-up the calcifications had diminished in number, size, or density, or a combination in 34 of the children (92%, 95% confidence interval 79% to 97%), were no longer visible in one child, and remained unchanged in two children. No child showed an increase in calcifications. The calcifications at the cortical-white matter junction which were no longer visible at follow-up occurred predominately in the parietal and occipital lobes. These imaging changes were not associated with any clear clinical improvements.Conclusion The detection of cerebral calcifications should not be considered a major criterion for late diagnosis of congenital Zika syndrome, nor should the absence of calcifications be used to exclude the diagnosis.

Effectiveness of tranexamic acid for reducing intraoperative bleeding in palatoplasties: A randomized clinical trial.

BACKGROUND: Few data are available regarding blood loss during cleft palate surgery. This study assessed the effect of using tranexamic acid in the reduction of intraoperative bleeding. METHODS: A double-blind, randomized clinical trial compared intraoperative bleeding and the incidence of oronasal fistulas after palatoplasties in a control group that was given placebo and an intervention group that was given 10 mg/kg tranexamic acid followed by a continuous infusion of 1 mg/kg/h of the same until the end of surgery. Patients who underwent primary palatoplasty with no known or suspected coagulation disorders at our institution during the study period were included in the study. RESULTS: 70 patients were allocated, 66 received the intervention. Blood loss was reduced by 11.9%, without statistical significance. The incidence of fistulas in the intervention and control groups was 12.9% and 18.75%, respectively. The reduction of 5.8% (CI 95%: 12%-23.8%) was not significant. CONCLUSIONS: The reduction of intraoperative blood loss was lower than expected and not statistically significant; a larger sample is needed to confirm the observed reduction. The drug did not seem to have negative effects on flap viability.

Chikungunya infection in infants / Infecção por Chikungunya em lactentes

Abstract Introduction: the infection of chikungunya virus presents clinical manifestations variables, particularly in infants in which may present multiple cutaneous manifestations. Description: a case series study was carried out in an analytical character of 14 infants (>28 days to < 2 years old) admitted in a hospital between November 2015 and January 2016 with suspected case of chikungunya, by a specific IgM reactive serology. Patients positive for dengue fever, Zika virus, bacterial infections and other exanthematic diseases were excluded. Fever and cutaneous alterations were the most frequent clinical manifestations in 100% of the cases, followed by irritability (64.3%), vomits and arthralgia/arthritis in 35.7% each. Three children presented alterations in the cerebrospinal fluid compatible to meningitis. Anemia frequency was 85.7%. The median white blood cells count was 7.700/mm3 (2.600 to 20.300/mm3). High levels of aminotransferases were observed in three cases (230 to 450 U/L). Antibiotic therapy was indicated in 64.3% of the cases. Two infants needed opioid derivatives for analgesia while others took acetaminophen and/or dipyrone. Discussion: the study shows evident multi-systemic involvement of chikungunya infection in infants. The treatment is supportive, giving special attention to hydration, analgesia, skin care, and rational use of antibiotic therapy.

Resumo Introdução: a infecção pelo vírus chikungunya apresenta manifestações clínicas variáveis, particularmente em lactentes, nos quais parece haver uma multiplicidade de manifestações cutâneas. Descrição: foi feito um estudo tipo de série de casos de caráter analítico com 14 lactentes (>28 dias a < 2 anos) internados entre novembro de 2015 e janeiro de 2016 como caso suspeito de infecção por chikungunya com sorologia IgM específica reagente. Excluídos pacientes com exame positivo para dengue, Zika vírus, infecção bacteriana e outras doenças exantemáticas. As manifestações clínicas mais frequentes foram febre e alterações cutâneas em 100% dos casos, irritabilidade (64,3%), vômitos e artralgia/artrite em 35,7% cada. Três crianças apresentaram alterações liquóricas compatíveis com meningite. A frequência de anemia foi de 85,7%. A mediana de leucometria foi de 7.700/mm3 (2.600 a 20.300/mm3). Níveis aumentados de aminotransferases foram observados em três casos (230 a 450 U/L). Antibioticoterapia foi indicada em 64,3% dos casos. Dois lactentes necessitaram de derivados de opioides para analgesia fixa, enquanto os demais, paracetamol e/ou dipirona. Discussão: o estudo evidencia envolvimento multisistêmico da infecção por chikungunya em lactentes. O tratamento permanece de suporte, com atenção a hidratação, analgesia, cuidados com a pele e ao uso racional de antibioticoterapia.

Relative frequency, Possible Risk Factors, Viral Codetection Rates, and Seasonality of Respiratory Syncytial Virus Among Children With Lower Respiratory Tract Infection in Northeastern Brazil.

Few studies, each limited to a single major city, have investigated the prevalence and seasonal patterns of different viruses among children with low respiratory tract infections (LRTI) in Northeastern Brazil. The aim of this study was to determine the frequency of respiratory syncytial virus (RSV) and of 7 other viruses in children for LRTI in 4 capitals from this region, and investigate their association with several risk factors, including meteorological data. From April 2012 to March 2013, 507 children, aged up to 24 months and hospitalized with LRTI in one of the participating centers at Aracajú, Salvador, Recife, and Maceió, had a sample of nasopharyngeal aspirate collected and analyzed for the following viruses by reverse-transcription polymerase chain reaction followed by hybridization on low-density microarrays: RSV, influenza, parainfluenza, adenovirus, rhinovirus, metapneumovirus, bocavirus, and coronavirus. The result was positive in 66.5% of cases, RSV was the most common virus (40.2%). Except for rhinovirus (17%), all other virus had frequency rates lower than 6%. Viral coinfections were detected in 13.8% of samples. Possible related risk factors for RSV infection were low age upon entry, attendance of daycare, low gestational age, and low educational level of the father. The relative frequency of viral infections was associated with increasing temperature and decreasing humidity separately, but the results also suggested both associated with increased frequency of RSV. Some of these findings differ from those reported for other regions in Brazil and may be used to guide policies that address LRTI.

Dispositivos ventilatórios não invasivos em criança portadora de amiotrofia espinhal do tipo 1: relato de caso / Noninvasive ventilation devices in a child with spinal muscular atrophy type 1: a case report

Amiotrofia espinhal do tipo 1 (AME 1) é uma doença genética autossômica recessiva que promove morte celular de neurônios motores localizados no corno anterior da medula e núcleos motores do tronco cerebral. A precoce morbimortalidade está associada à disfunção bulbar e insuficiência respiratória, necessitando de internamento hospitalar e de suporte ventilatório artificial. O objetivo do estudo foi divulgar a relevância da manutenção de paciente com AME 1 sem prótese ventilatória invasiva e com protocolo de fisioterapia individualizado, proporcionando melhor qualidade de vida e integração com seus familiares. Descrição: relato de caso de menor diagnosticado com AME 1 que aos 11 meses foi submetido à ventilação mecânica invasiva (VMI) por 76 dias, obtendo sucesso no desmame após aplicação de um protocolo de fisioterapia respiratória, incluindo a utilização de tosse mecanicamente assistida e ventilação não invasiva (VNI). Discussão: apesar das dificuldades e intercorrências observadas, a assistência proposta alcançou o objetivo de retirada da VMI e transferência para internação domiciliar com dispositivos ventilatórios não invasivos. A VMI por traqueostomia é considerada tratamento de eleição no Brasil, no entanto, as famílias precisam ser esclarecidas da irreversibilidade da doença e das possibilidades estratégicas das terapêuticas atuais (VMI, VNI e paliação) para o manuseio de criança grave com AME 1...

The spinal muscular atrophy type 1 (AME1) is an autosomal recessive genetic disorder that promotes cell death of motor neurons located in the anterior horn of the spinal cord and motor nuclei of the brainstem. Early mortality is linked to the bulbar dysfunction and respiratory failure, requiring hospitalization and invasive mechanical ventilation (IMV). The purpose of this study is to promote the maintenance patients with SMA type 1 without invasive ventilation support and individualized physical therapy protocol, improving child's quality of life and integration with their families. Description: this report relates a child diagnosed with AME1, who at 11 months was admitted and underwent IMV for 76 days, succeeding extubation after respiratory therapy protocol application using cough machine and noninvasive ventilation (NIV). Discussion: despite the difficulties and complications observed, there was persistence of the proposed assistance, reaching goal of withdrawal of IMV and transfer to home care with noninvasive ventilation devices. The IMV for tracheostomy is considered treatment of choice in Brazil. Notwithstanding, families need to be clarified of the irreversibility of the disease and strategic possibilities of therapeutic strategies (IMV, NIV and palliation) for the management of the child with severe SMA 1...

Impact of chronic kidney disease on quality of life, lung function, and functional capacity / Repercussão da doença renal crônica na qualidade de vida, função pulmonar e capacidade funcional

OBJECTIVES: To evaluate the impact of the chronic kidney disease (CKD) on quality of life, from the children's and their parents' perspective, respiratory muscle strength, lung function, and functional capacity in children and adolescents. METHOD: Cross-sectional study of children with CKD aged 8 to 17 years. Those incapable of taking the tests were excluded. After an interview, quality of life by Pediatric Quality of Life Inventory) (PedsQLTM), muscular strength, pulmonary function tests, and the 6-minute walking test (6MWT) were applied. Student's t-test, ANOVA (difference in means), and Pearson's coefficient of correlation were used. The level of significance was set at 5%. RESULTS: Of the 40 patients, the mean distance walked at the 6MWT was 396 meters, and the mean final score at the quality of life test as perceived by the children and parents was 50.9 and 51, respectively. From the children's perspective, the transplanted patients had a higher quality of life score when compared to those undergoing hemodialysis (p < 0.001); those who practiced physical activity had better quality of life when compared to the sedentary children (p < 0.001). From the children's and the parents' perspectives, the male gender had a higher quality of life score (p < 0.05). There was a positive correlation between the distance walked at the 6MWT and age, height, final PedsQLTM, forced vital capacity (FVC), and forced expiratory volume in the first second (FEV1), as well as a negative correlation between FEV1/FVC and the distance walked. CONCLUSION: A significant reduction in the quality of life and the functional capacity was observed in children with CKD, influenced by the type of treatment, gender, and sedentary life style. .

OBJETIVOS: Avaliar repercussões da doença renal crônica (DRC) sobre a qualidade de vida na percepção das crianças e dos pais, força muscular respiratória, função pulmonar e capacidade funcional em crianças e adolescentes. MÉTODO: Estudo transversal de crianças e adolescentes com DRC de oito a 17 anos. Excluídas as incapazes de realizar os testes. Após entrevista, aplicou-se questionário de qualidade de vida (PedsQLTM), testes de força muscular, função pulmonar e teste de caminhada de 6 minutos (TC6 min). Foi utilizado o teste t de Student e ANOVA (diferenças de médias) e o coeficiente de correlação de Pearson. Considerou-se nível de significância de 5%. RESULTADOS: Dentre os 40 pacientes, a média da distância percorrida no TC6 min foi de 396 ± 71 metros, e a média do escore final de qualidade de vida percebida pelas crianças e pelos pais de 50,9 e 51, respectivamente. Na percepção das crianças, os transplantados apresentaram maior escore de qualidade de vida, comparados aos em hemodiálise (p < 0,001), e aos com atividade física e melhor qualidade de vida, comparadas às sedentárias (p < 0,001). Na percepção das crianças e dos pais, o sexo masculino apresentou maior escore de qualidade de vida (p < 0,05). Houve correlação positiva entre a distância percorrida no TC6 min e as variáveis idade, altura, PedsQLTM final da criança, capacidade vital forçada (CVF) e volume expiratório forçado no primeiro segundo (VEF1) e negativa entre VEF1/CVF e a distância percorrida. CONCLUSÃO: Observou-se redução significativa na qualidade de vida e na capacidade funcional em crianças com DRC influenciadas pelo tipo de tratamento, sexo e sedentarismo. .

Impact of chronic kidney disease on quality of life, lung function, and functional capacity.

OBJECTIVES: To evaluate the impact of the chronic kidney disease (CKD) on quality of life, from the children's and their parents' perspective, respiratory muscle strength, lung function, and functional capacity in children and adolescents. METHOD: Cross-sectional study of children with CKD aged 8 to 17 years. Those incapable of taking the tests were excluded. After an interview, quality of life by Pediatric Quality of Life Inventory) (PedsQL(TM)), muscular strength, pulmonary function tests, and the 6-minute walking test (6MWT) were applied. Student's t-test, ANOVA (difference in means), and Pearson's coefficient of correlation were used. The level of significance was set at 5%. RESULTS: Of the 40 patients, the mean distance walked at the 6MWT was 396 meters, and the mean final score at the quality of life test as perceived by the children and parents was 50.9 and 51, respectively. From the children's perspective, the transplanted patients had a higher quality of life score when compared to those undergoing hemodialysis (p<0.001); those who practiced physical activity had better quality of life when compared to the sedentary children (p<0.001). From the children's and the parents' perspectives, the male gender had a higher quality of life score (p<0.05). There was a positive correlation between the distance walked at the 6MWT and age, height, final PedsQL(TM), forced vital capacity (FVC), and forced expiratory volume in the first second (FEV1), as well as a negative correlation between FEV1/FVC and the distance walked. CONCLUSION: A significant reduction in the quality of life and the functional capacity was observed in children with CKD, influenced by the type of treatment, gender, and sedentary life style.