Early lung ultrasound affords little to the prediction of bronchopulmonary dysplasia.

OBJECTIVE: To test the hypothesis that lung ultrasound (LUS) performed in the first week of life would predict bronchopulmonary dysplasia (BPD). Secondary outcomes included the utility of LUS in predicting interim respiratory interventions. DESIGN: A prospective observational cohort study in preterm infants born <28 weeks' gestation in the single tertiary statewide neonatal intensive care unit in Western Australia. METHODS: A rigorous protocol for LUS acquisition on day 1, day 3-4, day 7, day 28 and 36 weeks' postmenstrual age (PMA) was implemented with blinded analysis using a modified, previously validated LUS score. BPD was defined by both recent National Institute of Child Health and Human Development categorical criteria and a continuous physiological variable using a modified Shift test. RESULTS: Of the 100 infants studies, primary outcome data were available for the 96 infants, surviving to 36 weeks' PMA. In a univariate logistic regression analysis, LUS on days 3-4 and day 7 accurately predicted BPD (day 3-4 OR (95% CI)=1.54 (1.03 to 2.42), p=0.044; day 7 OR (95% CI)=1.66 (1.07 to 2.70), p=0.031). The predictive value of LUS was insignificant in a multivariate model in which gestational age was the dominant predictor. LUS accurately predicted interim respiratory outcomes including surfactant administration, duration of intubation and extubation to non-invasive support at 48 hours. CONCLUSIONS: LUS in the first week of life predicted BPD. However, LUS offers little additive accuracy to current gestational age-based models. TRIAL REGISTRATION NUMBER: ACTRN12617000208303.

[Palliative Sedation at the End of Life: A Comparative Study of Chronic Obstructive Pulmonary Disease and Lung Cancer Patients].

BACKGROUND: Although patients with chronic obstructive pulmonary disease (COPD) receive poor-quality palliative care, information about the use of palliative sedation (PS) in the last days of life is very scarce. OBJECTIVES: To compare the use of PS in hospitalized patients who died from COPD or lung cancer and identify factors correlating with PS application. METHODS: In a retrospective observational cohort study, from 1,675 patients died at a teaching hospital between 2013 and 2015, 109 patients who died from COPD and 85 from lung cancer were compared. Sociodemographic data, clinical characteristics, health care resource utilization, application of PS and prescribed drugs were recorded. RESULTS: In the last 6 months of life, patients who died from COPD had more hospital admissions due to respiratory causes and less frequent support by a palliative home care team (PHCT). Meanwhile, during their last hospitalization, patients who died from COPD had fewer do-not-resuscitate orders and were subjected to more intensive care unit admissions and cardiopulmonary resuscitation maneuvers. PS was applied less frequently in patients who died from COPD than in those who died from lung cancer (31 vs. 53%, p = 0.002). Overall, previous use of opioid drugs, support by a PHCT, and a diagnosis of COPD (adjusted odds ratio 0.48, 95% CI: 0.26-0.89, p = 0.020) were retained as factors independently related to PS. In COPD patients, only previous use of opioid drugs was identified as a PS-related factor. CONCLUSION: During their last days of life, hospitalized COPD patients receive PS less frequently than patients with lung cancer.

Prophylactic Flexible Bronchoscopy Immediately following Open Airway Reconstruction in Children.

OBJECTIVES: Prophylactic flexible bronchoscopy immediately following open airway reconstruction allows for directed clearance of the distal airways, potentially reducing the rate of certain postoperative respiratory complications. In this investigation, we sought to determine if prophylactic flexible bronchoscopy at the conclusion of pediatric open airway reconstruction has any benefit over blind flexible suctioning of the trachea. METHODS: A retrospective, single-center study at an urban tertiary care hospital was completed. From January 2010 to April 2013, patients underwent open airway reconstruction, immediately followed by blind flexible suctioning of the trachea for distal airway clearance. From May 2013 through December 2016, sequential patients underwent prophylactic flexible bronchoscopy immediately following airway reconstruction. RESULTS: A total of 29 patients (age: 3.6 months-6.2 years) met inclusion criteria. Sixteen sequential patients underwent simple blind flexible suctioning and 13 sequential patients underwent directed, prophylactic flexible bronchoscopy. Demographics and comorbidities between the groups were equivalent other than slightly older age in the prophylactic bronchoscopy group. All clinical outcomes analyzed were equivalent other than faster time to room air (P < .002) and a decrease in the number of chest physical therapy sessions (P < .02) in a subset of patients who did not undergo prophylactic bronchoscopy. CONCLUSION: This investigation suggests that the use of prophylactic flexible bronchoscopy immediately following open airway reconstruction may not be superior to blind flexible suctioning of the trachea in limiting postoperative pulmonary complications. Further studies of greater power are needed to better elucidate any small differences that may exist between these two interventions.

Exploring the implications of different approaches to estimate centre-level adherence using objective adherence data in an adult cystic fibrosis centre - a retrospective observational study.

BACKGROUND: Accurate centre-level medication adherence measurement allows identification of highly performing CF centres, drives shared learning and informs quality improvement. Self-reported adherence is unreliable but data-logging nebulisers can capture objective data. However, adherence levels in current literature are limited by the use of agreed prescriptions and convenience sampling. In this single-centre retrospective study, we quantified the differences in centre-level adherence with different methods of calculating adherence (unadjusted vs normative adherence) and different data sampling frames (convenience sampling vs including difficult to obtain data). METHODS: Adherence data were objectively captured using I-neb® from 2013-2016 in Sheffield Adult CF Centre. Adults on non data-logging devices, on ivacaftor or with previous lung transplantation were excluded. Adherence was calculated based on agreed regimen ('unadjusted adherence') or minimum required regimen ('normative adherence'). I-nebs® not brought to clinic were downloaded during home visits. Adults not on any inhaled therapy but with chronic Pseudomonas aeruginosa infection were included by counting their adherence as "0". RESULTS: Of the 131 included adults, 126 provided I-neb® data. Calculating unadjusted adherence from I-nebs® brought to clinics resulted in the highest centre-level adherence (median 41.8% in 2013). Median adherence reduced after sequentially accounting for minimum required regimen (40.0% in 2013), I-nebs® not brought to clinics (32.9% in 2013) and adults not on any inhaled therapy (31.0% in 2013). CONCLUSIONS: Different approaches of calculating adherence produced different adherence levels. Adherence levels based only on agreed regimen among adults who readily brought their nebulisers to clinics can over-estimate the effective adherence of CF centres.

Management of Australian Adults with Bronchiectasis in Tertiary Care: Evidence-Based or Access-Driven?

PURPOSE: Australian data regarding the management of patients with bronchiectasis is scarce. We sought to compare the management of adults with bronchiectasis attending tertiary Australian centres with recent national and international guidelines. METHODS: The Australian Bronchiectasis Registry is a centralised database of patients with radiologically confirmed bronchiectasis unrelated to cystic fibrosis recruited from 14 tertiary Australian hospitals. We excluded children (<18 years) and those with incomplete data, leaving 589 adults for cross-sectional analyses. We compared the proportion of patients receiving certain therapies, as compared to the proportion eligible for those treatments according to the current guidelines and baseline clinical information available from the registry. RESULTS: Pulmonary rehabilitation was attended by 22%, although it was indicated in 67% of the cohort. Airway clearance was undertaken in 52% of patients, although 71% reported chronic productive cough. Sputum bacterial culture results were available for 59%, and mycobacterial culture results were available for 29% of the cohort. Inhaled antibiotics were used in half of potentially eligible patients. Despite guideline recommendations against routine use, inhaled corticosteroids were used in 48% of patients. Long-term macrolides were used in 28% of participants. CONCLUSIONS: Discrepancies exist between guideline recommendations and real-world treatment of bronchiectasis in Australia, even in tertiary centres. These findings suggest the need for increased patient referral to pulmonary rehabilitation, increased attention to airway clearance, increased collection of sputum samples (especially for mycobacterial culture) and rationalisation of inhaled corticosteroid use. These findings encourage a review of treatment access and will inform ongoing education to promote evidence-based care for people living with bronchiectasis.

Low rates of participation and completion of pulmonary rehabilitation in patients with chronic obstructive pulmonary disease in primary health care / Participación y adherencia a programas de rehabilitación pulmonar en pacientes con EPOC en Chile

ABSTRACT Background: Only 6% of patients with chronic obstructive pulmonary disease (COPD) participate in pulmonary rehabilitation programs (PR) and only 50% of those who participate, complete these programs. Aim: To determine rates of PR program participation and completion among patients with COPD in Chile. Material and Methods: Analysis of a database available at the Ministry of Health, which included data of 277491 patients with COPD (55% females) and their participation in PR programs, between 2014 and 2016. Results: Forty percent of patients were over 75 years of age. Participation rates in PR programs ranged from 2.4 to 2.9%. Rates of completion ranged from 26 to 36%. Conclusions: There is a low rate of participation in PR programs among patients with COPD. Approximately one third of participants complete these programs.

Antecedentes: solo el 6% de los pacientes con enfermedad pulmonar obstructiva crónica.(EPOC) participa en programas de rehabilitación pulmonar. (RP) y solo el 50% de los que participan, completan estos programas. Objetivo: Determinar los índices de participación y adherencia a programas de RP entre los pacientes con EPOC en Chile. Material y Métodos: Análisis de una base de datos disponible en el Ministerio de Salud, que incluía datos de 277,491 pacientes con EPOC (55% mujeres) y su participación en programas de RP, entre 2014 y 2016. Resultados: el cuarenta por ciento de los pacientes tenían más de 75 años de edad. Tasas de participación en programas de RP osciló entre 2,4 y 2,9%. Las tasas de finalización oscilaron entre el 26 y el 36%. Conclusiones: Existe una baja tasa de participación en programas de RP entre pacientes con EPOC. Aproximadamente un tercio de los participantes completan estos programas.

[Characteristics of non-pharmacological interventions in the elderly with COPD. Smoking cessation, pulmonary rehabilitation, nutritional management and patient education]. / Particularités de la prise en charge non médicamenteuse de la BPCO chez les sujets âgés. Réhabilitation, sevrage tabagique, nutrition et éducation thérapeutique.

Chronic obstructive pulmonary disease (COPD) is a respiratory disorder responsible for a high mortality and disability. People older than 65 years are more commonly affected than younger people and tend to present with more symptoms and a greater level of disability. Non-pharmacological interventions play an important role in the management of all patients with COPD and this is particularly true in the elderly. Given the improvement in quality of life and risk of hospitalization, smoking cessation should be promoted to patients of all ages. Increased physical activity is associated with reduced respiratory symptoms. Tests such as the "Short Physical Performance Battery" can be useful in frailer older people with COPD, while walking tests such as the 6-minute walk test are used as an assessment before pulmonary rehabilitation. Increased physical activity should be combined with nutritional management. Screening for undernutrition by questionnaire, body mass index and albumin quantification is recommended in the elderly. In case of undernutrition, oral supplementation seems to reduce the risk of re-admission. All these measures must be included in an education program adapted to the elderly comorbidities (hearing loss, isolation…).

Surgical Ligation Versus Percutaneous Closure of Patent Ductus Arteriosus in Very Low-Weight Preterm Infants: Which are the Real Benefits of the Percutaneous Approach?

Percutaneous treatment of patent ductus arteriosus (PDA) in extreme premature infants is technically difficult, and therefore, often not consider as an alternative to surgery. The main objective of our work was to compare respiratory status prior and post ductal closure and morbi-mortality, in our series of preterm infants with percutaneous PDA closure versus surgical ligation in the same time-period. Retrospective review of all premature infants submitted to percutaneous and surgical PDA closure from January 2011 to December 2016. All the antenatal, perinatal, and postnatal characteristics were collected. The main outcome was the assessment of the pulmonary status before and after ductal closure using a pulmonary score. Secondary outcomes included moderate-severe disability in neurodevelopment, death before discharge, moderate-severe chronic lung disease, and morbidity at discharge. 25 patients with a mean weight of 1330 g (± 280) underwent percutaneous closure of PDA with ADO-II-AS, and a total of 53 underwent surgical ligation. 28/53 with similar gestational age, birth weight, and procedure weight to those in the percutaneous group, were selected to perform the comparative study. Ductal closure (percutaneous and surgical) resulted in improved respiratory status. However, percutaneous group achieved a fastest respiratory improvement, than surgical group. The surgical closure group associated higher morbidity among survivors (HIV, number of sepsis, need, and duration of inotropics post-interventionism). The incidence of recurrent laryngeal nerve palsy among the surgical group was 17%. Percutaneous closure of PDA in carefully selected low-weight preterm infants is a safe and reliable alternative to surgical ligation.

Race Effects of Inhaled Nitric Oxide in Preterm Infants: An Individual Participant Data Meta-Analysis.

OBJECTIVE: To assess whether inhaled nitric oxide (iNO) improves survival without bronchopulmonary dysplasia (BPD) for preterm African American infants. STUDY DESIGN: An individual participant data meta-analysis was conducted, including 3 randomized, placebo-controlled trials that enrolled infants born at <34 weeks of gestation receiving respiratory support, had at least 15% (or a minimum of 10 infants in each trial arm) of African American race, and used a starting iNO of >5 parts per million with the intention to treat for 7 days minimum. The primary outcome was a composite of death or BPD. Secondary outcomes included death before discharge, postnatal steroid use, gross pulmonary air leak, pulmonary hemorrhage, measures of respiratory support, and duration of hospital stay. RESULTS: Compared with other races, African American infants had a significant reduction in the composite outcome of death or BPD with iNO treatment: 49% treated vs 63% controls (relative risk, 0.77; 95% CI, 0.65-0.91; P = .003; interaction P = .016). There were no differences between racial groups for death. There was also a significant difference between races (interaction P = .023) of iNO treatment for BPD in survivors, with the greatest effect in African American infants (P = .005). There was no difference between racial groups in the use of postnatal steroids, pulmonary air leak, pulmonary hemorrhage, or other measures of respiratory support. CONCLUSION: iNO therapy should be considered for preterm African American infants at high risk for BPD. iNO to prevent BPD in African Americans may represent an example of a racially customized therapy for infants.

The recording and characteristics of pulmonary rehabilitation in patients with COPD using The Health Information Network (THIN) primary care database.

Pulmonary rehabilitation is recommended for patients with COPD to improve physical function, breathlessness and quality of life. Using The Health Information Network (THIN) primary care database in UK, we compared the demographic and clinical parameters of patients with COPD in relation to coding of pulmonary rehabilitation, and to investigate whether there is a survival benefit from pulmonary rehabilitation. We identified patients with COPD, diagnosed from 2004 and extracted information on demographics, pulmonary rehabilitation and clinical parameters using the relevant Read codes. Thirty six thousand one hundred and eighty nine patients diagnosed with COPD were included with a mean (SD) age of 67 (11) years, 53% were male and only 9.8% had a code related to either being assessed, referred, or completing pulmonary rehabilitation ever. Younger age at diagnosis, better socioeconomic status, worse dyspnoea score, current smoking, and higher comorbidities level are more likely to have a record of pulmonary rehabilitation. Of those with a recorded MRC of 3 or worse, only 2057 (21%) had a code of pulmonary rehabilitation. Survival analysis revealed that patients with coding for pulmonary rehabilitation were 22% (95% CI 0.69-0.88) less likely to die than those who had no coding. In UK THIN records, a substantial proportion of eligible patients with COPD have not had a coded pulmonary rehabilitation record. Survival was improved in those with PR record but coding for other COPD treatments were also better in this group. GP practices need to improve the coding for PR to highlight any unmet need locally. CHRONIC LUNG DISEASE: ROLLING OUT THE REHAB: Analysis of recent UK data suggests that more patients with chronic lung disease could benefit from lung rehabilitation programmes. During pulmonary rehabilitation (PR), patients with chronic obstructive pulmonary disease (COPD) work with specialists to learn exercises and optimise breathing techniques. The programmes are recommended under current guidelines, particularly for patients with a high breathlessness score. Despite this, when Charlotte Bolton and co-workers at the University of Nottingham analysed 36,189 patient primary care records gathered since 2004, they found only 9.8% of COPD patients had ever had a coded record of being assessed, referred for, or undertaken PR. Those patients who completed PR were 22% less likely to die that those who didn't, although appeared they had also received better overall COPD care. Current smokers, those suffering from co-morbidities and younger patients were more likely to receive PR than other patient groups.

Social Determinants of Adherence to Pulmonary Rehabilitation for Chronic Obstructive Pulmonary Disease.

Adherence to pulmonary rehabilitation (PR) is low. Previous studies have focused on clinical predictors of PR completion. We aimed to identify social determinants of adherence to PR. A cross-sectional analysis of a database of COPD patients (N = 455) in an outpatient PR program was performed. Adherence, a ratio of attended-to-prescribed sessions, was coded as low (<35%), moderate (35-85%), and high (>85%). Individual-level measures included age, sex, race, BMI, smoking status, pack-years, baseline 6-minute walk distance (6MWD: <150, 150-249, ≥250), co-morbidities, depression, and prescribed PR sessions (≤20, 21-30, >30). Fifteen area-level measures aggregated to Census tracts were obtained from the U.S. Census after geocoding patients' addresses. Using exploratory factor analysis, a neighborhood socioeconomic disadvantage index was constructed, which included variables with factor loading >0.5: poverty, public assistance, households without vehicles, cost burden, unemployment, and minority population. Multivariate regression models were adjusted for clustering on Census tracts. Twenty-six percent of patients had low adherence, 23% were moderately adherent, 51% were highly adherent. In the best fitted full model, each decile increase in neighborhood socioeconomic disadvantage increased the risk of moderate vs high adherence by 14% (p < 0.01). Smoking tripled the relative risk of low adherence (p < 0.01), while each increase in 6MWD category decreased that risk by 72% (p < 0.01) and 84% (p < 0.001), respectively. These findings show that, relative to high adherence, low adherence is associated with limited functional capacity and current smoking, while moderate adherence is associated with socioeconomic disadvantage. The distinction highlights different pathways to suboptimal adherence and calls for tailored intervention approaches.

Objective Measurement of Adherence to Out-Patient Airway Clearance Therapy by High-Frequency Chest Wall Compression in Cystic Fibrosis.

BACKGROUND: Objective measures of adherence to high-frequency chest wall compression (HFCWC), a form of airway clearance therapy for patients with cystic fibrosis, are lacking. We used a novel electronic monitoring device integrated into an HFCWC vest to measure adherence compared with self-reported adherence. We determined factors that influenced adherence and how adherence correlated with baseline pulmonary function and pulmonary exacerbations. METHODS: Data were collected by direct measurement of date, time of day, and duration of HFCWC use to determine the number of daily treatments and daily duration of treatments. Chart review provided prescribed airway clearance therapy treatment and demographic and clinical information. Subject and caregiver report of the daily number of airway clearance therapy treatments was obtained by telephone interviews. Analysis used 2-sample and paired t test, analysis of variance, and linear regression. RESULTS: Average adherence was 69%. Adherence was highest in children (82%, P = .02) and those receiving assistance with treatment (82%, P < .001). Subjects overestimated therapy duration from a mean ± SD of 127 ± 169% by adults to 19.2 ± 26.3% by parents or guardians of children. Average adherence decreased with increasing prescribed therapy time (P = .02). Average daily therapy time and adherence had significant positive associations with baseline FEV1 percent of predicted (P = .02 and P = .02, respectively) and negative associations with pulmonary exacerbations during the pre-study period and at baseline (P = .044 and P = .02, respectively). CONCLUSIONS: Greater adherence to HFCWC measured directly by a novel recorder was associated with better baseline pulmonary function and fewer exacerbations in the pre-study and baseline period. Adherence decreased with age and prescribed therapy time and increased with therapy assistance. Self-report overestimation is large and thus not an accurate measure of adherence.

Pulmonary Rehabilitation in COPD According to Global Initiative for Chronic Obstructive Lung Disease Categories.

BACKGROUND: Pulmonary rehabilitation (PR) programs are a mainstay for treatment in COPD. Lung function impairment alone does not predict beneficial effects of PR. The new COPD categories take into account assessment of symptoms, such as dyspnea and exacerbations, which may be important indications for PR. This study evaluates the effect of PR on exercise capacity, symptoms, and health status in different COPD categories. METHODS: Subjects with COPD referred for PR were classified into COPD categories A, B, C, and D. Exercise capacity (6-min walk distance [6MWD] and constant work rate at 80% of peak work rate), symptoms (Mahler's index), and health status (St George Respiratory Questionnaire) were compared before and after PR programs for each COPD category. Changes were analyzed using generalized estimating equations and logistic regression models. RESULTS: One hundred sixty-seven subjects were included (COPD categories A [16%], B [12%], C [31%], and D [41%]). Groups were homogeneous in age, body mass index, smoking pack-years, and comorbidities. Significant improvements in all outcomes were found after adjusting for COPD categories, age, sex, body mass index, and COPD-specific comorbidity index. All COPD categories improved exercise capacity (6MWD and constant work rate). Categories A and C had more pronounced improvements in 6MWD than categories B and D. Symptoms (Mahler's index) also improved significantly in categories A and C, whereas change was not significant in categories B and D. Global health status (St George Respiratory Questionnaire) improved significantly in all COPD categories. Despite these differences, the odds of achieving a minimum clinically important difference in each outcome were similar and without statistical significance for the A, B, and C categories when compared with D. CONCLUSIONS: This study demonstrates that patients in all COPD categories may improve exercise capacity, symptoms, and health status with PR programs, and COPD categories alone may not be sufficient to discriminate which patients may benefit most from them.

Respiratory therapy: a problem among children and adolescents with cystic fibrosis.

OBJECTIVE: To evaluate the level of self-reported adherence to physical therapy recommendations in pediatric patients (6-17 years) with cystic fibrosis (CF) and to ascertain whether the different levels of adherence correlate with pulmonary function, clinical aspects, and quality of life. METHODS: This was a cross-sectional study. The patients and their legal guardians completed a questionnaire regarding adherence to physical therapy recommendations and a CF quality of life questionnaire. We collected demographic, spirometric, and bacteriological data, as well as recording the frequency of hospitalizations and Shwachman-Kulczycki (S-K) clinical scores. RESULTS: We included 66 patients in the study. Mean age, FEV1 (% of predicted), and BMI were 12.2 ± 3.2 years, 90 ± 24%, and 18.3 ± 2.5 kg/m2, respectively. The patients were divided into two groups: high-adherence (n = 39) and moderate/poor-adherence (n = 27). No statistically significant differences were found between the groups regarding age, gender, family income, and total S-K clinical scores. There were statistically significant differences between the high-adherence group and the moderate/poor-adherence group, the latter showing lower scores for the "radiological findings" domain of the S-K clinical score (p = 0.030), a greater number of hospitalizations (p = 0.004), and more days of hospitalization in the last year (p = 0.012), as well as lower scores for the quality of life questionnaire domains emotion (p = 0.002), physical (p = 0.019), treatment burden (p < 0.001), health perceptions (p = 0.036), social (p = 0.039), and respiratory (p = 0.048). CONCLUSIONS: Low self-reported adherence to physical therapy recommendations was associated with worse radiological findings, a greater number of hospitalizations, and decreased quality of life in pediatric CF patients.

Respiratory therapy: a problem among children and adolescents with cystic fibrosis / Fisioterapia respiratória: um problema de crianças e adolescentes com fibrose cística

Objective : To evaluate the level of self-reported adherence to physical therapy recommendations in pediatric patients (6-17 years) with cystic fibrosis (CF) and to ascertain whether the different levels of adherence correlate with pulmonary function, clinical aspects, and quality of life. Methods : This was a cross-sectional study. The patients and their legal guardians completed a questionnaire regarding adherence to physical therapy recommendations and a CF quality of life questionnaire. We collected demographic, spirometric, and bacteriological data, as well as recording the frequency of hospitalizations and Shwachman-Kulczycki (S-K) clinical scores. Results : We included 66 patients in the study. Mean age, FEV1 (% of predicted), and BMI were 12.2 ± 3.2 years, 90 ± 24%, and 18.3 ± 2.5 kg/m2, respectively. The patients were divided into two groups: high-adherence (n = 39) and moderate/poor-adherence (n = 27). No statistically significant differences were found between the groups regarding age, gender, family income, and total S-K clinical scores. There were statistically significant differences between the high-adherence group and the moderate/poor-adherence group, the latter showing lower scores for the "radiological findings" domain of the S-K clinical score (p = 0.030), a greater number of hospitalizations (p = 0.004), and more days of hospitalization in the last year (p = 0.012), as well as lower scores for the quality of life questionnaire domains emotion (p = 0.002), physical (p = 0.019), treatment burden (p < 0.001), health perceptions (p = 0.036), social (p = 0.039), and respiratory (p = 0.048). Conclusions : Low self-reported adherence to physical therapy recommendations was associated with worse radiological findings, a greater number of hospitalizations, and decreased quality of life in pediatric CF patients.

Objetivo : Avaliar o grau de adesão autorrelatada às recomendações fisioterapêuticas em pacientes pediátricos (6-17 anos) com fibrose cística (FC) e determinar se os diferentes níveis de adesão se correlacionam com a função pulmonar, aspectos clínicos e qualidade de vida. Métodos : Estudo transversal no qual os pacientes e responsáveis responderam um questionário sobre a adesão à fisioterapia recomendada e um questionário da qualidade de vida em FC. Foram coletados dados demográficos, espirométricos e bacteriológicos, assim como a frequência de internações e resultados do escore clínico de Shwachman-Kulczycki (S-K). Resultados : Participaram 66 pacientes. As médias de idade, VEF1 (em % do previsto) e IMC foram, respectivamente, 12,2 ± 3,2 anos, 90 ± 24% e 18,3 ± 2,5 kg/m2. Os pacientes foram divididos em dois grupos: alta adesão (n = 39) e moderada/baixa adesão (n = 27). Não houve diferenças estatisticamente significativas para idade, sexo, renda familiar e escore clínico de S-K total na comparação dos dois grupos. Houve diferenças estatisticamente significativas entre os grupos alta adesão e moderada/baixa adesão, este último mostrando valores significativamente menores para o domínio "achados radiológicos" do escore clínico de S-K apresentou (p = 0,030), um maior número de hospitalizações (p = 0,004) e de dias de internação no último ano (p = 0,012), assim como menores escores para os seguintes domínios do questionário de qualidade de vida: emocional (p = 0,002), físico (p = 0,019), tratamento (p < 0,001), saúde (p = 0,036), social (p = 0,039) e respiratório (p = 0,048). Conclusões : A baixa adesão autorrelatada às recomendações fisioterapêuticas associou-se com piores achados radiológicos, maior número de hospitalizações e diminuição da qualidade de vida em pacientes pediátricos com FC.

Treatment burden in patients with at least one class IV or V CFTR mutation.

CFTR mutations are grouped according to disease-causing mechanism. Several studies demonstrated that patients having at least one mutation of class IV/V, present with a milder phenotype, but little is known about their relative treatment burden. We compared treatment burden between patients with two class I, II, or III mutations and patients with at least one mutation of class IV/V in the 2010 database of the Belgian CF Registry. We calculated a "Treatment Burden Index" (TBI) by assigning long term therapies to categories low, medium and high intensity, for differential weighing in the total score. There were 779 patients with two known class I/II/III mutations and 94 patients with at least one class IV/V mutation. Compared to class I/II/III, class IV/V patients had a lower median number of clinic visits (4 vs. 5; P < 0.001), a lower risk of hospitalization (24.7% vs. 50.8%; P < 0.001) and intravenous antibiotic treatment (23.5% vs. 46.0%; P < 0.001) and a lower median TBI (6 vs. 9; P < 0.001). These differences remained significant when only class IV/V patients with pancreatic insufficiency (n = 31) were considered. This study clearly demonstrates the significantly lower treatment burden in patients with CF and at least one class IV/V mutation compared to patients with two class I/II/III mutations and contributes to providing better individual counseling at time of diagnosis.

Adherence to treatment in children and adolescents with cystic fibrosis: a cross-sectional, multi-method study investigating the influence of beliefs about treatment and parental depressive symptoms.

BACKGROUND: Adherence to treatment is often reported to be low in children with cystic fibrosis. Adherence in cystic fibrosis is an important research area and more research is needed to better understand family barriers to adherence in order for clinicians to provide appropriate intervention. The aim of this study was to evaluate adherence to enzyme supplements, vitamins and chest physiotherapy in children with cystic fibrosis and to determine if any modifiable risk factors are associated with adherence. METHODS: A sample of 100 children (≤18 years) with cystic fibrosis (44 male; median [range] 10.1 [0.2-18.6] years) and their parents were recruited to the study from the Northern Ireland Paediatric Cystic Fibrosis Centre. Adherence to enzyme supplements, vitamins and chest physiotherapy was assessed using a multi-method approach including; Medication Adherence Report Scale, pharmacy prescription refill data and general practitioner prescription issue data. Beliefs about treatments were assessed using refined versions of the Beliefs about Medicines Questionnaire-specific. Parental depressive symptoms were assessed using the Center for Epidemiologic Studies Depression Scale. RESULTS: Using the multi-method approach 72% of children were classified as low-adherers to enzyme supplements, 59% low-adherers to vitamins and 49% low-adherers to chest physiotherapy. Variations in adherence were observed between measurement methods, treatments and respondents. Parental necessity beliefs and child age were significant independent predictors of child adherence to enzyme supplements and chest physiotherapy, but parental depressive symptoms were not found to be predictive of adherence. CONCLUSIONS: Child age and parental beliefs about treatments should be taken into account by clinicians when addressing adherence at routine clinic appointments. Low adherence is more likely to occur in older children, whereas, better adherence to cystic fibrosis therapies is more likely in children whose parents strongly believe the treatments are necessary. The necessity of treatments should be reinforced regularly to both parents and children.

Adherence to nebulised therapies in adolescents with cystic fibrosis is best on week-days during school term-time.

OBJECTIVES: Treatment regimen for families of children with cystic fibrosis (CF) is considerable, particularly when nebulised therapies for chronic Pseudomonas aeruginosa airway infection are prescribed. Adherence to these regimens is variable, particularly in adolescence. Previously, we reported children to be more adherent in evenings compared to mornings, suggesting an association with time-pressure. The aim of this study was to determine whether adherence would be better in adolescent patients at weekends and during school holidays when time-pressures may be less. STUDY DESIGN: 24 patients (14 male, median [range] age 13.9 [11.1-16.8] years) were enrolled from two regional paediatric CF centres in the United Kingdom. Data for a full scholastic year, were downloaded openly from a breath-activated data logging nebuliser (I-neb™). Adherence (% of doses taken÷expected number) was calculated during term-times, holidays, weekends and weekdays, for each patient. RESULTS: Large variations in adherence were seen between patients. However, adherence during term-time was significantly better than holidays (p<0.001). Weekday adherence was better than weekend adherence in term-time but not holidays. Interestingly, patients prescribed three daily treatments took on average 1.4 treatments/day, a similar number to those prescribed two daily treatments. CONCLUSION: Overall adherence to inhaled therapies was reasonable, but significantly reduced during holiday periods. This suggests a need for families to have not only time, but also structure in their daily routine to maintain optimal adherence to long-term therapies. It is important for CF teams to appreciate these factors when supporting families.

Post transfusion lung injury in the neonatal population.

OBJECTIVE: The objective of this study was to examine the changes in level of respiratory support following transfusion in neonates who require intermediate or intensive care. STUDY DESIGN: Data on respiratory support were collected retrospectively from the medical record before, during and after transfusion. Neonatal post transfusion lung injury (NPTLI) was defined as an increase in the highest mean airway pressure (MAP) of ≥2 cm H(2)O or FiO(2) >0.15 in the 6-h after transfusion that persisted from 6 to 18 h post transfusion. RESULT: A total of 373 (330 packed red blood cell) transfusions were given to 108 infants. NPTLI occurred following 31 (8.3%) transfusions in 23 patients. During the first 6 h after transfusion, FiO2 or MAP was increased in 47 transfusions (12.6%) and the changes persisted in 31 transfusions (7.8%). Infants who developed NPTLI were less mature (27.1±0.7 vs 31.0±0.5 weeks; P=0.005) and of lower birth weight (1001±110 vs1692±104 g; P=0.001). Infants who developed NPTLI were more likely to develop necrotizing enterocolitis (6/24 vs 4/85; P=0.002) and die within 24 h of transfusion (5/22 vs 3/85; P=0.003). CONCLUSION: In neonates receiving intensive or intermediate care, blood transfusion was associated with need for increased respiratory support in a significant number of cases. Development of NPTLI was associated with poorer outcomes.

Usefulness of lung-to-head ratio and intrapulmonary arterial Doppler in predicting neonatal morbidity in fetuses with congenital diaphragmatic hernia treated with fetoscopic tracheal occlusion.

OBJECTIVE: To explore the potential value of intrapulmonary artery Doppler velocimetry in predicting neonatal morbidity in fetuses with left-sided congenital diaphragmatic hernia (CDH) treated with fetoscopic tracheal occlusion (FETO). METHODS: Observed/expected lung-to-head ratio (O/E-LHR), and intrapulmonary Doppler pulsatility index and peak early-diastolic reversed flow were evaluated within 24 h before FETO in a consecutive cohort of 51 fetuses with left-sided CDH at between 24 and 33 weeks' gestation. Lung Doppler parameters were converted into Z-scores and defined as abnormal if the pulsatility index had a Z-score of > 1.0 or the peak early-diastolic reversed flow had a Z-score of > 3.5. The association of O/E-LHR and Doppler velocimetry with neonatal outcome was assessed using multiple linear or logistic regression analysis adjusted for gestational age at birth. RESULTS: Among the 26 fetuses that survived, 18 (69.2%) had normal and eight (30.8%) had abnormal Doppler values. O/E-LHR was not associated with neonatal morbidity in surviving fetuses. Compared with the group with normal Doppler parameters, cases with abnormal intrapulmonary Doppler were associated with a significant increase in the duration of mechanical ventilation (average increase of 21.2 (95% CI, 9.99-32.5) days; P < 0.01), conventional ventilation (15.2 (95% CI, 7.43-23.0) days; P < 0.01), high-frequency ventilation (6.34 (95% CI, 0.69-11.99) days; P < 0.05), nitric oxide therapy (5.73 (95% CI, 0.60-10.9) days; P < 0.05), oxygen support (36.5 (95% CI, 16.3-56.7) days; P < 0.01), parenteral nutrition (19.1 (95% CI, 7.53-30.7) days; P < 0.01) and stay in neonatal intensive care unit (42.7 (95% CI, 22.9-62.6) days; P < 0.001), and with significantly higher rates of high-frequency ventilation (87.5 vs. 44.4%;P < 0.05), oxygen requirement at 28 days of age (75.0 vs. 11.1%; P < 0.01), gastroesophageal reflux (62.5 vs. 22.2%; P < 0.05) and tube feeding at discharge (37.5 vs. 5.56%; P < 0.05). CONCLUSION: As previously reported, O/E-LHR did not predict neonatal morbidity. In contrast, intrapulmonary artery Doppler evaluation was predictive of neonatal morbidity in CDH fetuses treated with FETO.