Pediatric Asthma Therapy Assessment Questionnaire for the Brazilian population: Cross-cultural adaptation and measurement properties.

OBJECTIVE: To cross-culturally adapt the Pediatric Asthma Therapy Assessment Questionnaire (ATAQ) into Brazilian Portuguese and analyze its measurement properties. METHODS: This exploratory methodological study included eight experts and 30 caregivers in the translation and cross-cultural adaptation steps. Thereafter, 118 caregivers of pediatric patients with asthma aged between 5 and 17 years were involved in the analysis of measurement properties. We analyzed the content, structural (exploratory and confirmatory factorial), construct (convergent and discriminant), and known-groups validities; floor and ceiling effects; and determined the cut-off point (receiver operator characteristic curve) to identify pediatric patients with uncontrolled asthma. Intraclass correlation coefficient (ICC) analyzed test-retest reliability with 54 caregivers, whereas Cronbach's α and composite reliability verified the internal consistency of the items. RESULTS: The committee of experts and caregivers found the instructions and response options relevant, understandable, and clear (K > 0.75). During the cross-cultural adaptation, three items (2, 4, and 12) were slightly modified by including terms to facilitate understanding. A two-factor structure (asthma control and patient-provider communication) was identified. Internal consistency (α > .67; composite reliability > 0.73) and test-retest reliability (ICC > 0.80) were acceptable. For construct and know-groups validities, 85.71% of the hypothesis were confirmed. A cut-off point of >3 for the control domain was considered adequate to identify pediatric patients with uncontrolled asthma (sensitivity: 86.21%; specificity: 80.90%). CONCLUSION: The Pediatric ATAQ was adequately adapted for Brazilian pediatric patients with asthma and produced valid and reliable measures for assessing asthma control. Therefore, it may be considered an adequate instrument for monitoring asthma control in the Brazilian pediatric population.

Effects of home healthcare for adults with chronic respiratory diseases and post-COVID-19 syndrome on hospital bed turnover rate: a protocol of systematic review with meta-analysis.

INTRODUCTION: Chronic respiratory diseases (CRDs) have a high prevalence, morbidity and mortality worldwide. After the COVID-19 pandemic, the number of patients readmitted after hospital discharge increased. For some populations, early hospital discharge and home healthcare may reduce health costs in patients treated at home when compared with those hospitalised. This study aims to systematically review the effectiveness of home healthcare for patients with CRDs and post-COVID-19 syndrome. METHODS AND ANALYSIS: We will search on MEDLINE, CENTRAL, Embase and PsycINFO. We will include randomised controlled trials (RCTs) and non-RCT studies reported in full text and abstracts. No language restriction will be applied. We will include studies related to adults with a diagnosis of CRDs or post-COVID-19 syndrome that compared in-patient hospital care with any home healthcare. We will exclude studies with participants with neurological, mental diseases, cancer or pregnant women. Two review authors will screen abstracts and select the eligible studies. To investigate the risk of bias, we will use the Cochrane 'Risk of Bias' tool for RCT, and the Risk of Bias In Non-randomised Studies-of Interventions for non-RCT. We will use the five Grading of Recommendations, Assessment, Development, and Evaluations (GRADE) considerations to assess the quality of the evidence. Patients and the public will be involved in the preparation, execution and implementation phases of the review. ETHICS AND DISSEMINATION: No ethical approval is required because only published data will be analysed. The publication of the results in peer-reviewed journals and at relevant conferences will guide the direction of future research in the field and healthcare practice. The results will also be disseminated in plain language on social media to disseminate the knowledge to society and the public interested in the topic.

Germline Compound Heterozygous Variants Identified in the STXBP2 Gene Leading to a Familial Hemophagocytic Lymphohistiocytosis Type 5: A Case Report.

Familial hemophagocytic lymphohistiocytosis (FHL) is a rare, potentially fatal autosomal-recessive immunodeficiency, and STXBP2 mutations have been associated with FHL type 5 (FHL-5). Here, we report a case of a 2-year-old boy who presented with recurrent fever, hepatosplenomegaly, pancytopenia, hyperferritinemia, and hypofibrinogenemia since 4 months of age. His genetic analysis revealed a compound heterozygosity of the STXBP2 gene with a described pathogenic mutation, c.1247-1G>C (splicing acceptor site), harbored by his father and a likely pathogenic variant of uncertain significance (VUS), c.704G>A (p.Arg235Gln), harbored by his mother. He was diagnosed as compound heterozygous for FHL-5 and was treated with the HLH-2004 protocol. Since treatment, this patient has been in remission, and he is being evaluated for a hematopoietic stem cell transplantation (HSCT).

Relationship between anxiety symptoms, clinical control and quality of life of children with asthma: A cross-sectional study.

OBJECTIVE: To investigate the relationship between anxiety symptoms and factors related to clinical control and quality of life of children with asthma aged 7-12 years. METHODS: A cross-sectional comparative study involving children recruited from a reference center for asthma treatment in the city of Natal-RN. Diagnosis, control and severity were performed following GINA recommendations (2019). Sociodemographic information and spirometry results from a bronchodilator test were collected. Modified Fisher's scale, clinical control questionnaires (c-ACT and ACT), quality of life assessment (PAQLQ) (total score and domains) and anxiety symptoms assessment (SCAS) (total score and domains) were applied. RESULTS: The sample consisted of 42 children with asthma. Symptoms of social phobia (as identified by SCAS), and household head education were related to clinical asthma control (p = .006; R 2 = .19). Total SCAS score, guardian's education and physical activity were related to total PAQLQ (p < .0001; R 2 = .33). SCAS was related to the activity limitation domain of PAQLQ (p = .004; R 2 = .17). SCAS, gender, and physical activity were related to the PAQLQ symptoms domain (p = .003; R 2 = .32). The guardian's education, physical activity practice, and the symptoms of separation anxiety, panic attack and agoraphobia (as identified by SCAS) were related to the emotional domain of PAQLQ (p = .004; R 2 = .45). CONCLUSION: The present study shows evidence of an association between anxiety symptoms, poorer clinical control, and health-related quality of life in children with asthma.

Vitamin A deficiency alters airway resistance in children with acute upper respiratory infection.

OBJECTIVE: To assess whether vitamin A deficiency alters the recovery of total respiratory resistance (TRR) following acute upper respiratory tract infection (URI). METHODS: This is a case control study of children, age 4-6 years and grouped as: URI, (n = 74), URI and wheezing, (URI-wheezing, n = 52), and healthy controls (n = 51). Vitamin A and total respiratory resistance (TRR) were assessed using the modified relative dose response (MRDR) and forced oscillometry, respectively. RESULTS: Children with URI and URI-wheezing had lower retinol, 32.4 ± 13.12 and 18.3 ± 6.83 µg/dl respectively, compared to controls, 56.9 ± 29.82 µg/dl (ANOVA, P < 0.001). The MRDR was elevated in children in the URI or URI-wheezing groups 0.066 ± 0.045 and 0.021 ± 0.021, respectively, compared to controls 0.007 ± 0.006 (ANOVA, P < 0.0001). The TRR in the URI and URI-wheezing groups differed from controls. During convalescence, the TRR failed to decline in the URI-group only when the MRDR was >0.06. In the URI-wheezing group, TRR declined independently of retinol and MRDR. CONCLUSION: Vitamin A contributes to preservation of airway function during and in recovery after upper respiratory infection in children.

Estudo de uma família portadora de hemoglobina S / Study of a family carrier of hemoglobin S

Foi realizado um estudo em uma família natural do estado do Rio Grande do Norte (Brasil) com o objetivo de pesquisar a presença de hemoglobina S. Nesta Família constituída de 14 membros, a presença dessa hemoglobina foi pesquisada através do teste de falcizaçäo das hemácias e pela eletroforese de hemoglobina em acetato de celulose. Outros dados hematológicos foram obtidos pelos métodos rotineiros e, paralelamente, foram realizados exames clínicos com o objetivo de evidenciar sinais físicos relacionados com a presença da hemoglobina S. Constatou-se que a presença da hemoglobina S estava presente em alguns membros da família na forma heterozigoto (Hb-AS) tais como o avô materno, a avó paterna, ambos os pais e 3 dos filhos do casal e apenas um membro era portador dessa hemoglobina na forma homozigoto (Hb-SS)