Eosinophilic Granulomatosis with Polyangiitis - description of a pediatric patient with severe disease.

INTRODUCTION: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare vasculitis of small and medium sized blood vessels. CASE DESCRIPTION: Thirteen-year-old male, with history of rhinitis and asthma, who presented to the emergency room with one week of asthenia, arthralgias and myalgias and two days of fever. A diffuse petechial rash, palpable purpura and polyarthritis were detected on examination. Leukocytosis (34990/µL) with eosinophilia (66%) and elevated C-reactive protein were identified. The patient was admitted and ceftriaxone and doxycycline were started. The clinical status deteriorated in the following days. The patient developed myopericarditis, bilateral pulmonary infiltrates and pleural effusion, requiring mechanical ventilation and aminergic support. Non-clonal eosinophils were detected on the bone marrow aspiration and the skin biopsy showed leukocytoclastic vasculitis with eosinophils. Antineutrophil cytoplasmic antibodies and genetic analysis for hypereosinophilic syndrome mutations were negative. After treatment with methylprednisolone for three days a fast clinical, laboratory and radiological improvement occurred. The patient started azatiophrine and reduced steroids progressively. No relapses occurred since diagnosis five years ago. DISCUSSION: Clinical suspicion and early treatment of EGPA are crucial to improve prognosis.

Symptoms of anxiety and depression in patients with persistent asthma: a cross-sectional analysis of the INSPIRERS studies.

OBJECTIVES: Anxiety and depression are relevant comorbidities in asthma, but, in Portugal and Spain, data on this topic are scarce. We assessed, in patients with asthma, the frequency of anxiety and depression using the Hospital Anxiety and Depression Scale (HADS) and the European Quality of Life Five Dimension Questionnaire (EQ-5D); the level of agreement between these questionnaires, and the factors associated with these symptoms. METHODS: This is a secondary analysis of the INSPIRERS studies. A total of 614 adolescents and adults with persistent asthma (32.6±16.9 years, 64.7% female) were recruited from 30 primary care centres and 32 allergy, pulmonology and paediatric clinics. Demographic and clinical characteristics, HADS and EQ-5D were collected. A score ≥8 on Hospital Anxiety and Depression Scale-Anxiety/Hospital Anxiety and Depression Scale-Depression or a positive answer to EQ-5D item 5 indicated the presence of these symptoms. Agreement was determined by Cohen's kappa. Two multivariable logistic regressions were built. RESULTS: According to HADS, 36% of the participants had symptoms of anxiety and 12% of depression. According to EQ-5D, 36% of the participants had anxiety/depression. The agreement between questionnaires in identifying anxiety/depression was moderate (k=0.55, 95% CI 0.48 to 0.62). Late asthma diagnosis, comorbidities and female gender were predictors of anxiety/depression, while better asthma control, health-related quality of life and perception of health were associated with lower odds for anxiety/depression. CONCLUSION: At least 1/3 of the patients with persistent asthma experience symptoms of anxiety/depression, showing the relevance of screening these disorders in patients with asthma. EQ-5D and HADS questionnaires showed a moderate agreement in the identification of anxiety/depression symptoms. The identified associated factors need to be further investigated in long-term studies.

ERS statement on paediatric long-term noninvasive respiratory support.

Long-term noninvasive respiratory support, comprising continuous positive airway pressure (CPAP) and noninvasive ventilation (NIV), in children is expanding worldwide, with increasing complexities of children being considered for this type of ventilator support and expanding indications such as palliative care. There have been improvements in equipment and interfaces. Despite growing experience, there are still gaps in a significant number of areas: there is a lack of validated criteria for CPAP/NIV initiation, optimal follow-up and monitoring; weaning and long-term benefits have not been evaluated. Therapeutic education of the caregivers and the patient is of paramount importance, as well as continuous support and assistance, in order to achieve optimal adherence. The preservation or improvement of the quality of life of the patient and caregivers should be a concern for all children treated with long-term CPAP/NIV. As NIV is a highly specialised treatment, patients are usually managed by an experienced paediatric multidisciplinary team. This statement written by experts in the field of paediatric long-term CPAP/NIV aims to emphasise the most recent scientific input and should open up new perspectives and research areas.

Determinants of the Use of Health and Fitness Mobile Apps by Patients With Asthma: Secondary Analysis of Observational Studies.

BACKGROUND: Health and fitness apps have potential benefits to improve self-management and disease control among patients with asthma. However, inconsistent use rates have been reported across studies, regions, and health systems. A better understanding of the characteristics of users and nonusers is critical to design solutions that are effectively integrated in patients' daily lives, and to ensure that these equitably reach out to different groups of patients, thus improving rather than entrenching health inequities. OBJECTIVE: This study aimed to evaluate the use of general health and fitness apps by patients with asthma and to identify determinants of usage. METHODS: A secondary analysis of the INSPIRERS observational studies was conducted using data from face-to-face visits. Patients with a diagnosis of asthma were included between November 2017 and August 2020. Individual-level data were collected, including age, gender, marital status, educational level, health status, presence of anxiety and depression, postcode, socioeconomic level, digital literacy, use of health services, and use of health and fitness apps. Multivariate logistic regression was used to model the probability of being a health and fitness app user. Statistical analysis was performed in R. RESULTS: A total of 526 patients attended a face-to-face visit in the 49 recruiting centers and 514 had complete data. Most participants were ≤40 years old (66.4%), had at least 10 years of education (57.4%), and were in the 3 higher quintiles of the socioeconomic deprivation index (70.1%). The majority reported an overall good health status (visual analogue scale [VAS] score>70 in 93.1%) and the prevalence of anxiety and depression was 34.3% and 11.9%, respectively. The proportion of participants who reported using health and fitness mobile apps was 41.1% (n=211). Multivariate models revealed that single individuals and those with more than 10 years of education are more likely to use health and fitness mobile apps (adjusted odds ratio [aOR] 2.22, 95%CI 1.05-4.75 and aOR 1.95, 95%CI 1.12-3.45, respectively). Higher digital literacy scores were also associated with higher odds of being a user of health and fitness apps, with participants in the second, third, and fourth quartiles reporting aORs of 6.74 (95%CI 2.90-17.40), 10.30 (95%CI 4.28-27.56), and 11.52 (95%CI 4.78-30.87), respectively. Participants with depression symptoms had lower odds of using health and fitness apps (aOR 0.32, 95%CI 0.12-0.83). CONCLUSIONS: A better understanding of the barriers and enhancers of app use among patients with lower education, lower digital literacy, or depressive symptoms is key to design tailored interventions to ensure a sustained and equitable use of these technologies. Future studies should also assess users' general health-seeking behavior and their interest and concerns specifically about digital tools. These factors may impact both initial engagement and sustained use.

Profiling Persistent Asthma Phenotypes in Adolescents: A Longitudinal Diagnostic Evaluation from the INSPIRERS Studies.

We aimed to identify persistent asthma phenotypes among adolescents and to evaluate longitudinally asthma-related outcomes across phenotypes. Adolescents (13-17 years) from the prospective, observational, and multicenter INSPIRERS studies, conducted in Portugal and Spain, were included (n = 162). Latent class analysis was applied to demographic, environmental, and clinical variables, collected at a baseline medical visit. Longitudinal differences in clinical variables were assessed at a 4-month follow-up telephone contact (n = 128). Three classes/phenotypes of persistent asthma were identified. Adolescents in class 1 (n = 87) were highly symptomatic at baseline and presented the highest number of unscheduled healthcare visits per month and exacerbations per month, both at baseline and follow-up. Class 2 (n = 32) was characterized by female predominance, more frequent obesity, and uncontrolled upper/lower airways symptoms at baseline. At follow-up, there was a significant increase in the proportion of controlled lower airway symptoms (p < 0.001). Class 3 (n = 43) included mostly males with controlled lower airways symptoms; at follow-up, while keeping symptom control, there was a significant increase in exacerbations/month (p = 0.015). We have identified distinct phenotypes of persistent asthma in adolescents with different patterns in longitudinal asthma-related outcomes, supporting the importance of profiling asthma phenotypes in predicting disease outcomes that might inform targeted interventions and reduce future risk.

Monitoring Adherence to Asthma Inhalers Using the InspirerMundi App: Analysis of Real-World, Medium-Term Feasibility Studies.

Background: Poor medication adherence is a major challenge in asthma and objective assessment of inhaler adherence is needed. InspirerMundi app aims to monitor inhaler adherence while turning it into a positive experience through gamification and social support. Objective: We assessed the medium-term feasibility of the InspirerMundi app to monitor inhaler adherence in real-world patients with persistent asthma (treated with daily inhaled medication). In addition, we attempted to identify the characteristics of the patients related to higher app use. Methods: Two real-world multicenter observational studies, with one initial face-to-face visit and a 4-month telephone interview, were conducted in 29 secondary care centers from Portugal. During an initial face-to-face visit, patients were invited to use the app daily to register their asthma medication intakes. A scheduled intake was considered taken when patients took a photo of the medication (inhaler, blister, or others) using the image-based medication detection tool. Medication adherence was calculated as the number of doses taken as a percentage of the number scheduled. Interacting with the app ≥30 days was used as the cut-off for higher app use. Results: A total of 114 patients {median 20 [percentile 25 to percentile 75 (P25-P75) 16-36] years, 62% adults} were invited, 107 (94%) installed the app and 83 (73%) completed the 4-month interview. Patients interacted with the app for a median of 18 [3-45] days, translated on a median use rate of 15 [3-38]%. Median inhaler adherence assessed through the app was 34 [4-73]% when considering all scheduled inhalations for the study period. Inhaler adherence assessed was not significantly correlated with self-reported estimates. Median adherence for oral and other medication was 41 [6-83]% and 43 [3-73]%, respectively. Patients with higher app use were slightly older (p = 0.012), more frequently taking medication for other health conditions (p = 0.040), and more frequently prescribed long-acting muscarinic antagonists (LAMA, p = 0.024). After 4 months, Control of Allergic Rhinitis and Asthma Test (CARAT) scores improved (p < 0.001), but no differences between patients interacting with the app for 30 days or less were seen. Conclusions: The InspirerMundi app was feasible to monitor inhaler adherence in patients with persistent asthma. The persistent use of this mHealth technology varies widely. A better understanding of characteristics related to higher app use is still needed before effectiveness studies are undertaken.

Interfaces, Circuits and Humidifiers.

Long-term non-invasive ventilation (LTNIV) has been increasingly used in children to manage chronic respiratory failure and airway obstruction. Interfaces are of paramount importance for non-invasive ventilation (NIV) effectiveness and patient compliance. However, historically, the choice of pediatric mask has been limited by the scarce availability of commercial interfaces. In recent years, an increasing number of different masks have been commercialized for children, allowing to increase the number of patients who could benefit from LTNIV. Factors such as the age of the child, disease, craniofacial conformation, type of ventilator and mode of ventilation, and children's and family's preferences should be taken into account when selecting the appropriate mask. Adverse events such as skin lesions, facial growth impairment, and leaks must be prevented and promptly corrected. Humidification is a controversial issue on NIV, but it may be useful in certain circumstances. Regular cleaning and disinfection of interfaces and equipment must be addressed. During follow-up, educational programs, close supervision, and continuous support to children and families are crucial to the success of LTNIV therapy.

Children's Sleep Habits Questionnaire in Two Subpopulations from Cape Verde and Mozambique: Exploratory and Regression Analysis.

INTRODUCTION: Children's sleep habits are profoundly affected by socio-economic, cultural, and environmental factors. We aim to describe the sleep habits of pediatric sub-populations from Cape Verde and Mozambique using the Children's Sleep Habits Questionnaire, and to ascertain the determinants of the questionnaire's score. MATERIAL AND METHODS: We conducted cross-sectional surveys in surveillance appointments in Cape Verde and in a school in Maputo (Mozambique). The Cape Verde sample included 206 children (mean age: 6.5) and the Mozambique sample 454 children (mean age: 8). The Portuguese version of the Children's Sleep Habits Questionnaire was used to evaluate the children's sleep habits. The distributions of the questionnaire's scores across different levels of the demographic variables were compared using the Mann-Whitney and the Kruskal-Wallis tests. We used regression models to quantify the relationship between the demographic variables and the questionnaire's scores. RESULTS: Cape Verde sample: Questionnaire median score: 50 (range 36 - 81). Prevalence of sleep problems: 29.9% (cut-off = 56). Prevalence of parent-reported sleep problems: 22.8%. Co-sleeping: 63%. Bedtime television: 30%. Daytime nap: 63%. Questionnaire scores are associated with the mother's educational level, the children's age group, and with the frequency of daytime napping. Mozambique Sample: Median Questionnaire score: 48 (range 35 - 77). Prevalence of sleep problems: 28.4% (cut-off: 52). Percentage of parent-reported sleep problems: 6.9%. Co-sleeping: 29%. Bedtime television: 33%. Daytime nap: 23%. Questionnaire scores are associated with the mother's nationality, the father educational level, and with the frequency of bedtime television. DISCUSSION: Parents tend to overestimate the quality of their children's sleep. The prevalence of sleep disturbances and the cut-off values are higher than observed in previous studies, which may be due to the existence of different standards and cultural expectations across populations. Bedtime television, typically associated with more disturbed sleep, is frequent in both samples. Co-sleeping and daytime napping are frequent in the Cape Verde sample; wetting the bed is common and more prevalent than in previous studies. Bed-sharing increases the parents' awareness of their children's sleep quality, contributing to the high prevalence of parent-reported sleep problems. CONCLUSION: Parent-reported sleep problems underestimate the Children's Sleep Habits Questionnaire results. This is not necessarily indicative of more disturbed sleep and might reflect differences in sleep behavior, childcare practice, and cognitions and attitudes towards the concept of 'normal' sleep.

Introdução: Os hábitos de sono são influenciados por diversos factores. Pretendemos descrever os hábitos de sono de duas populações de países africanos aplicando o Children's Sleep Habits Questionnaire e determinar quais são as variáveis que mais influenciam o score do questionário. Material e Métodos: Realizámos dois estudos transversais em consultas de saúde infantil em Cabo Verde e numa escola em Maputo, Moçambique. A amostra de Cabo Verde incluiu 206 crianças (idade média: 6,5 anos) e a amostra de Moçambique 445 crianças (idade média: 8 anos). Aplicámos a versão portuguesa do Children's Sleep Habits Questionnaire. As distribuições dos scores do questionário para diferentes níveis das variáveis demográficas foram avaliadas com os testes de Mann-Whitney e de Kruskal-Wallis. Utilizámos regressões lineares para quantificar a relação entre variáveis demográficas e o score do questionário. Resultados: Cabo Verde: Mediana do score: 50 (36 ­ 81). Problemas de sono identificados pelo questionário: 29,9% (cut-off: 56). Problemas de sono reportados pelos pais: 22,8%. Partilha de cama: 63%. Televisão antes de adormecer: 30%. Sesta: 63%. Moçambique: Mediana do score: 48 (35 ­ 77). Problemas de sono identificados pelo questionário: 28,4% (cut-off: 52). Problemas de sono reportados pelos pais: 6,9%. Partilha de cama: 29%. Televisão antes de adormecer: 33%. Sesta: 23%. Variáveis que influenciam o score: educação da mãe, faixa etária da criança e sestas (Cabo Verde); nacionalidade da mãe, educação do pai, e televisão para adormecer (Moçambique). Discussão: Os pais tendem a sobrestimar a qualidade do sono dos seus filhos. Encontrámos prevalências de problemas do sono e valores de corte mais elevados do que os obtidos em estudos anteriores, o que também se pode dever à existência de diferentes padrões e expectativas em populações com diferentes culturas. Ver televisão para adormecer é um hábito tipicamente associado a maiores perturbações no sono e é frequente em ambas as amostras. Partilha de cama e sestas são frequentes na amostra de Cabo Verde; enurese noturna também é comum e mais prevalente do que em estudos anteriores. A partilha de cama aumenta a perceção que os pais têm da qualidade do sono dos seus filhos, podendo contribuir para a elevada percentagem de problemas de sono reportada pelos pais. Conclusão: Os problemas de sono reportados pelos pais subestimam os resultados do Children's Sleep Habits Questionnaire, o que pode traduzir diferentes expectativas e atitudes face ao sono, e não necessariamente sono patológico. Estudos futuros devem estabelecer valores de corte do Children´s Sleep Habits Questionnaire adaptados a cada cultura.

Sleep habits in infants: the role of maternal education.

OBJECTIVE: Sleep disturbances are frequent in infants. Early development of sleep routines has a positive effect on sleep quality. The objective of this study was to assess the impact of maternal education on the development of infants' sleep habits (SHs). METHODS: This was an experimental, longitudinal study. Postnatal mothers were allocated into two groups: to receive (intervention group, IG), or not receive (control group, CG) information on sleep hygiene. Individual 15-min sessions were provided and a leaflet was handed to the IG. A questionnaire on infants' SHs was applied by telephone/e-mail at 1, 2, 4, and 6 months of age. RESULTS: At least three questionnaires were answered by 314 mothers. Results were adjusted for maternal age, education, and race. In the IG, infants acquired more independent sleep habits, an effect still present at six months; slept more frequently in their own beds; adjusted odds ratio (ORadj) 3.8, 95% confidence interval (CI) (1.1-13.5); fell asleep more frequently alone (ORadj, 4.29; 95% CI, 2.4-7.6); fell asleep more frequently in their own beds (ORadj, 6.1; 95% CI, 3.5-10.6) and needed less breast/bottle feeding to fall asleep (ORadj, 2.68; 95% CI, 1.5-4.6). The autonomy was greater in IG infants after night awakenings; also until six months they went back to sleep more frequently alone (ORadj, 3.88; 95% CI, 2-7.5) and needed less breast/bottle feeding (ORadj, 2.35; 95% CI, 1.3-4.3). No differences were found regarding the need for light, television or other routines to fall asleep or after night awakenings. CONCLUSION: Maternal education is positively associated with the adoption of autonomous SHs in infants. Early maternal education is an adequate prevention strategy to be considered in neonatal care.

Nocturnal Oximetry-based Evaluation of Habitually Snoring Children.

RATIONALE: The vast majority of children around the world undergoing adenotonsillectomy for obstructive sleep apnea-hypopnea syndrome (OSA) are not objectively diagnosed by nocturnal polysomnography because of access availability and cost issues. Automated analysis of nocturnal oximetry (nSpO2), which is readily and globally available, could potentially provide a reliable and convenient diagnostic approach for pediatric OSA. METHODS: Deidentified nSpO2 recordings from a total of 4,191 children originating from 13 pediatric sleep laboratories around the world were prospectively evaluated after developing and validating an automated neural network algorithm using an initial set of single-channel nSpO2 recordings from 589 patients referred for suspected OSA. MEASUREMENTS AND MAIN RESULTS: The automatically estimated apnea-hypopnea index (AHI) showed high agreement with AHI from conventional polysomnography (intraclass correlation coefficient, 0.785) when tested in 3,602 additional subjects. Further assessment on the widely used AHI cutoff points of 1, 5, and 10 events/h revealed an incremental diagnostic ability (75.2, 81.7, and 90.2% accuracy; 0.788, 0.854, and 0.913 area under the receiver operating characteristic curve, respectively). CONCLUSIONS: Neural network-based automated analyses of nSpO2 recordings provide accurate identification of OSA severity among habitually snoring children with a high pretest probability of OSA. Thus, nocturnal oximetry may enable a simple and effective diagnostic alternative to nocturnal polysomnography, leading to more timely interventions and potentially improved outcomes.

Sleep disturbance in pediatric patients on automated peritoneal dialysis.

OBJECTIVE: There are few reports describing sleep disturbance (SD) in children on automated peritoneal dialysis (APD), and they are mostly based on the application of questionnaires. As far as we know, only two studies used polysomnography (PSG), the gold standard for the diagnosis of SD. This is the first study assessing SD in children with chronic kidney disease (CKD) on APD in our country. METHODS: This was an observational and descriptive study. Children up to 18 years of age who had undergone APD for a minimum of three months were considered eligible. Exclusion criteria were diabetes, neurologic disorders and sedative/hypnotic therapy. PSG was performed while the children were on their usual cycler nocturnal dialysis regimen. A questionnaire was completed on the same day. Informed, written consent was provided by all participants. RESULTS: Eight children on APD were studied. The median age was 10 years (range, 1-18 years) and the mean body mass index z-score was -0.35 (±0.71). The average duration on dialysis was 8.4 months. SD was noted in five (62.5%) patients. The results of PSG showed decreased sleep efficiency (81.05 ± 0.09%) and latency (13.6 ± 11.6 min), increased awake time (23.08% of total sleep time (TST) ± 14.3), apnea/hypopnea index (1.8/h ± 1.9) and desaturation index (4.5 ± 3.7). Periodic leg movements index average was normal (0.78/h ± 0.77). Subjective data obtained by the sleep questionnaire underestimated PSG findings. CONCLUSION: Our study shows that SD was present in more than half of children on PD. This result, in a small sample of patients, alerts to the need for systematic screening for sleep problems in children with CKD with a low threshold for a formal PSG.

Environment in pediatric wards: light, sound, and temperature.

UNLABELLED: The mutual relationship between sleep and disease is well known, becoming more relevant whenever the disease leads to hospitalization. We intend to describe patterns of environmental factors of some pediatric wards, and to verify if these are in line with those recommended. As a secondary aim, we characterize sleep quality during hospitalization. METHODS: Five pediatric wards of a tertiary-level hospital were included. Light, sound, and temperature were measured and assessed through descriptive statistics. The following recommended values were considered: maximum light 100 Lux, maximum sound 45 dB, and optimal temperature 20-24 °C. A questionnaire was prepared to assess children's sleep, and it was completed by a caregiver. RESULTS: Light values were within the desirable limits for 86% of evaluated time. In all wards, the intensity of sound was much higher than desirable, being above 45 dB during 85% of evaluated time. The temperature was above 24 °C during 78% of total time. Based on 34 answered questionnaires (out of 50 distributed), almost half of the respondents believe that sleep quality and restlessness are worse at the hospital. Most children slept for a longer time at home. Eighteen children awoke more times at the hospital, and those awakenings were mostly attributed to noise. CONCLUSIONS: The sound and temperature were higher than recommended. The different values between these wards may be due to different levels of care, but this shows that there are no standard rules on this matter. A worse quality and shorter duration of sleep at hospital were reported. Comprehensive studies are necessary to evaluate the impact of environmental factors on disease recovery.

[Long-term home oxygen therapy in children: evidences and open issues]. / Oxigenoterapia Domiciliária de Longa Duração na Criança: Evidências e Questões em Aberto.

INTRODUCTION: Long-term home oxygen therapy is indicated for patients with chronic hypoxemia. We intend to describe pediatric population on long-term home oxygen therapy followed-up at Pediatric Respiratory Unit of a tertiary care hospital between 2003-2012 and to compare with previous 1991-2000 review; to verify conformity with international and national recommendations and need for specific pediatric national guidelines, non-existent in Portugal. MATERIAL AND METHODS: Retrospective, descriptive and comparative study based on clinical files review. Review the guidelines for oxygen therapy in pediatric population. RESULTS: We studied 86 patients (59.3% males). The median age at the beginning of oxygen therapy was 0.0 (0.0-216.0) months, with a median duration of 15.0 (3.0-223.0) months. The most frequent diagnosis was bronchopulmonary dysplasia (53.5%), followed by bronchiolitis obliterans (14.0%), neurologic disorders (10.5%), cystic fibrosis (8.1%), miscellaneous syndromes (5.8%), sickle-cell disease (3.5%), other neonatal lung diseases (2.3%) and interstitial lung diseases (2.3%). Are maintained on follow-up 53 (61.6%) patients, 38 on oxygen therapy; 12 (13.9%) died. The median time of follow-up was 39.5 (1.0-246.0) months, minim on other neonatal lung diseases and maximum on cystic fibrosis. Comparing with previous review, this shows a relative increase in bronchiolitis obliterans and bronchopulmonary dysplasia patients, with increased duration in the latter, and inclusion of neurologic and hematologic patients. DISCUSSION: Prescription of long-term oxygen therapy in pediatric age mainly occurs in specific diseases of infants and pre-school aged. Neurologic and hematologic patients represent new indications, similarly to international publications. CONCLUSION: The knowledge of national reality and pediatric orientations are needed for care plans and rational prescription.

IntroduçÉo: Oxigenoterapia domiciliária de longa duraçÉo está indicada em doentes com hipoxémia crónica. Pretendemos descrever a populaçÉo em programa de oxigenoterapia domiciliária de longa duraçÉo acompanhada numa Unidade de Pneumologia Pediátrica de Hospital Terciário entre 2003-2012 e comparar com revisÉo de 1991-2000; verificar conformidade com orientações nacionais e internacionais, refletindo sobre necessidade de orientações nacionais especificamente pediátricas, inexistentes em Portugal.Material e Métodos: Estudo retrospetivo, descritivo e comparativo por consulta de processo clínico. Pesquisa de orientações sobre oxigenoterapia em idade pediátrica.Resultados: Incluímos 86 doentes (59,3% rapazes). A idade mediana de início da oxigenoterapia foi 0,0 (0,0-216,0) meses e a duraçÉo mediana de 15,0 (3,0-223,0) meses. O diagnóstico mais frequente foi displasia broncopulmonar (53,5%), seguindo-se bronquiolite obliterante (14,0%), doença neurológica (10,5%), fibrose quística (8,1%), síndromes polimalformativas (5,8%), doença de células falciformes (3,5%), outras doenças pulmonares neonatais (2,3%) e doenças pulmonares intersticiais (2,3%). Mantêm acompanhamento 53 (61,6%) doentes, 38 mantendo oxigenoterapia; 12 (13,9%) faleceram. O tempo mediano de seguimento foi 39,5 (1,0-246,0) meses, mínimo nas outras doenças pulmonares neonatais e máximo na fibrose quística. Comparativamente ao estudo anterior revela aumento relativo dos lactentes com bronquiolite obliterante e displasia broncopulmonar, aumento da duraçÉo nestes últimos e inclusÉode doentes neurológicos e hematológicos.DiscussÉo: A prescriçÉo de oxigenoterapia domiciliária de longa duraçÉo em pediatria ocorre sobretudo em doenças específicas dos lactentes e idade pré-escolar. Doentes neurológicos e hematológicos sÉo novos grupos de prescriçÉo, à semelhança da literatura internacional.ConclusÉo: O conhecimento da realidade nacional e orientações pediátricas sÉo relevantes para organizaçÉo de cuidados eprescriçÉo racional.

Sleep deprivation and accidental fall risk in children.

OBJECTIVES: To look for an association between sleep deprivation and risk of accidental falls (AF) in children. METHODS: A questionnaire was applied to two groups of children aged 1-14 years, encompassing children observed in an emergency room for AF (G1) and children attending health care visits (HV) (G2). Collected data included demographic characteristics, medical history, previous week's sleep pattern (PWSP), sleep duration and sleep pattern in the preceding 24 h, mechanism of fall, and injury severity. EXCLUSION CRITERIA: acute or chronic disease or exposure to drugs interfering with sleep. Statistical analyses included Fisher's exact test, Pearson Chi-square, Fisher-Freeman-Halton test, T and Mann-Whitney tests for independent samples, and multivariate logistic regression (α=5%). RESULTS: We obtained 1756 questionnaires in G1 and 277 in G2. Of those, 834 in G1 and 267 in G2 were analyzed. We found an increased risk of AF in boys (OR 1.6; 95% CI 1.2-2.4). After controlling for age, gender, summer holidays, parental education and profession, lack of naps and PWSP were associated with increased risk (OR 2.1; 95% CI 1.3-3.3 and OR 2.7; 95% CI 1.2-6.1). In 3-5 year-old children there was an association between AF and a shorter than usual sleep duration in the previous 24 h (p=0.02). CONCLUSIONS: To our knowledge, our study is the largest so far to assess the association between sleep deprivation and childhood injury. It evidences a protective effect of naps in children. Sleep duration of less than 8 h increases risk of AF. Pre-schoolers may be particularly susceptible to sleep deprivation.

Clinical, radiological, and physiological differences between obliterative bronchiolitis and problematic severe asthma in adolescents and young adults: the early origins of the overlap syndrome?

PURPOSE: Few reports have compared chronic obstructive lung diseases (OLDs) starting in childhood. AIMS: To describe functional, radiological, and biological features of obliterative bronchiolitis (OB) and further discriminate to problematic severe asthma (PSA) or to diagnose a group with overlapping features. RESULTS: Patients with OB showed a greater degree of obstructive lung defect and higher hyperinflation (P < 0.001). The most frequent high-resolution computed tomography (HRCT) features (increased lung volume, inspiratory decreased attenuation, mosaic pattern, and expiratory air trapping) showed significantly greater scores in OB patients. Patients with PSA have shown a higher frequency of atopy (P < 0.05). ROC curve analysis demonstrated discriminative power for the LF variables, HRCT findings and for atopy between diagnoses. Further analysis released five final variables more accurate for the identification of a third diagnostic group (FVC%t, post-bronchodilator ΔFEV(1) in ml, HRCT mosaic pattern, SPT, and D. pteronyssinus-specific IgE). CONCLUSIONS: We found that OB and PSA possess identifiable characteristic features but overlapping values may turn them undistinguishable.

Development and validation of a short-form, rapid estimate of adult literacy in medicine.

BACKGROUND: Although prior studies used the 66-item Rapid Estimate of Adult Literacy in Medicine (REALM instrument) for literacy assessment, researchers may require a shorter, validated instrument when designing interventions for clinical contexts. OBJECTIVE: To develop and validate a very brief literacy assessment tool, the REALM-Short Form (REALM-SF). PATIENTS: The model development, validation, and field testing validation samples included 1336, 164, and 50 patients, respectively. SETTING: General medicine and subspecialty clinics and medicine inpatient wards. DESIGN: For development and validation samples, indicator variables for REALM instrument items were evaluated as potential predictors of REALM instrument score by stepwise multiple regression analysis with subsequent bootstrap and confirmatory factor analysis of selected items. Pearson correlations compared REALM-SF and REALM instrument scores and kappa analyses compared grade level assignments. For the field testing validation sample, Pearson correlations compared Wide Range Achievement Test and REALM-SF scores. RESULTS: The REALM-SF included 7 items with stable model coefficients and 1 underlying linear factor. REALM-SF and REALM instrument scores were highly correlated in development (r = 0.95, P < 0.001) and validation (r = 0.94, P < 0.001) samples. There was excellent agreement between REALM-SF and REALM instrument grade-level assignments when dichotomized at the 6th grade (development: 97% agreement, K = 0.88, P < 0.001; validation: 88% agreement, K = 0.75, P < 0.001) and 8th grade levels (development: 94% agreement, K = 0.78, P < 0.001; validation: 84% agreement, K = 0.67, P < 0.001). REALM-SF and Wide Range Achievement Test scores were highly correlated (r = 0.83, P < 0.001) in field testing validation. CONCLUSIONS: The REALM-SF provides researchers a brief, validated instrument for assessing patient literacy in diverse research settings.