Effects of music therapy as an adjunct to chest physiotherapy in children with cystic fibrosis: A randomized controlled trial.

Airway clearance therapy (ACT) is considered an important approach to improve airway clearance in children with cystic fibrosis (CF). Daily ACT administration requires substantial commitments of time and energy that complicate ACT and reduce its benefits. It is crucial to establish ACT as a positive routine. Music therapy (MT) is an aspect of integrative strategies to ameliorate the psycho-emotional consequences of chronic diseases, and a MT intervention could help children with CF between the ages of 2 and 17 develop a positive response. The aim of this randomized controlled trial was to evaluate the effects of specifically composed and recorded instrumental music as an adjunct to ACT. We compared the use of specifically composed music (Treated Group, TG), music that the patient liked (Placebo Group, PG), and no music (Control Group, CG) during the usual ACT routine in children with CF aged from 2 to 17. The primary outcomes, i.e., enjoyment and perception of time, were evaluated via validated questionnaires. The secondary outcome, i.e., efficiency, was evaluated in terms of avoided healthcare resources. Enjoyment increased after the use of the specifically composed music (children +0.9 units/parents +1.7 units; p<0.05) compared to enjoyment with no music (0 units) and familiar music (+0.5 units). Perception of time was 11.1 min (±3.9) less than the actual time in the TG (p<0.05), 3.9 min (±4.2) more than the actual time in the PG and unchanged in the CG. The potential cost saving related to respiratory exacerbations was €6,704.87, while the cost increased to €33,524.35 in the CG and to €13,409.74 in the PG. In conclusion, the specifically composed, played and compiled instrumental recorded music is an effective adjunct to ACT to establish a positive response and is an efficient option in terms of avoided costs. Trial registered as ISRCTN11161411. ISRCTN registry (www.isrctn.com).

Initial Evaluation of Prospective and Parallel Assessments of Cystic Fibrosis Newborn Screening Protocols in Eastern Andalusia: IRT/IRT versus IRT/PAP/IRT.

Identifying newborns at risk for cystic fibrosis (CF) by newborn screening (NBS) using dried blood spot (DBS) specimens provides an opportunity for presymptomatic detection. All NBS strategies for CF begin with measuring immunoreactive trypsinogen (IRT). Pancreatitis-associated protein (PAP) has been suggested as second-tier testing. The main objective of this study was to evaluate the analytical performance of an IRT/PAP/IRT strategy versus the current IRT/IRT strategy over a two-year pilot study including 68,502 newborns. The design of the study, carried out in a prospective and parallel manner, allowed us to compare four different CF-NBS protocols after performing a post hoc analysis. The best PAP cutoff point and the potential sources of PAP false positive results in our non-CF newborn population were also studied. 14 CF newborns were detected, resulting in an overall CF prevalence of 1/4, 893 newborns. The IRT/IRT algorithm detected all CF cases, but the IRT/PAP/IRT algorithm failed to detect one case of CF. The IRT/PAP/IRT with an IRT-dependent safety net protocol was a good alternative to improve sensitivity to 100%. The IRT × PAP/IRT strategy clearly performed better, with a sensitivity of 100% and a positive predictive value (PPV) of 39%. Our calculated optimal cutoffs were 2.31 µg/L for PAP and 167.4 µg2/L2 for IRT × PAP. PAP levels were higher in females and newborns with low birth weight. PAP false positive results were found mainly in newborns with conditions such as prematurity, sepsis, and hypoxic-ischemic encephalopathy.

Tracheal bronchus diagnosed in children undergoing flexible bronchoscopy.

This paper describes the clinical features of paediatric patients with tracheal bronchus (TB) identified with flexible bronchoscopy (FB) in a tertiary care hospital. A retrospective review of every FB with diagnosis of TB carried out in our centre since 1990 was performed which considered specifically: age at diagnosis, gender, semiology, somatic anomalies, tracheal bronchus type, other bronchoscopic findings and clinical progress. Out of 1665 FB in 1337 patients, TB was found in 26 (1.9%). The median age was 15 months (age range 1 month-13 years), with no gender differences. Of 26 patients, a total of 24 had associated congenital pathologies (92.3%) (heart disease 69%, chromosomal abnormality 35% and spinal fusion defect 11%). FB was performed for a number of reasons including: recurrent or persistent wheezing, pneumonia or atelectasis, persistent stridor and refractory cough. The type of TB identified was primarily ectopic lobar of the right upper lobe (73%) and the apical supernumerary (11.5%). In addition to TB, other defects were found by FB in 90% of cases, such as tracheomalacia, bronchomalacia or tracheal stenosis. The review confirmed a higher prevalence of TB than expected in previous series and highlighted its association with other airway or somatic malformations, notably congenital cardiac defects and spinal fusion defects. All patients were managed conservatively for the TB.

[Prevalence of tuberculosis infection in BCG-vaccinated and unvaccinated Mediterranean schoolchildren]. / Prevalencia de infección por tuberculosis en una población escolar mediterránea (con y sin vacunación antituberculosa).

OBJECTIVE: To determine the prevalence of tuberculous infection in schoolchildren in the province of Malaga, Spain, and to evaluate its relation to sociodemographic variables. POPULATION AND METHODS: Tuberculin skin tests were used to screen 6-year-olds in their first year of primary school in this cross-sectional study of the prevalence of infection, with calculation of 95% confidence interval (CI). Children who had not been vaccinated against tuberculosis at birth were considered to have a positive test result if they had an induration of >or=5 mm. Those who had received bacillus Calmette-Guerin (BCG) vaccination at birth were considered to have a positive test result if they had an induration size of >10 mm or >15 mm, depending on whether they came from a country with a high or low prevalence of tuberculosis, respectively. RESULTS: The prevalence of infection in the group of unvaccinated children was 1.16% (95% CI, 0.69%-1.63%). No statistically significant differences were found for any of the sociodemographic variables analyzed. The prevalence of infection in the BCG-vaccinated children was 6.66% (95% CI, 5.55%-7.77%). CONCLUSIONS: The prevalence of tuberculosis infection in our area is slightly higher than that reported after other studies conducted in Spain. This could indicate that tuberculosis continues to be a public health problem in the province of Malaga. Infection in the subgroup of BCG-vaccinated children, however, was lower than that observed in studies of immigrants in Spain who had been immunized at birth. This is probably because the majority of immigrants in our area come from countries with a low incidence of tuberculosis.

[Validity and reliability of the St George's Respiratory Questionnaire in adults with cystic fibrosis]. / Validez y fiabilidad del Cuestionario Respiratorio de St. George en población adulta con fibrosis quística.

OBJECTIVE: To study self-perceived quality of life in adults with cystic fibrosis (CF), and to assess the validity of the St George's Respiratory Questionnaire (SGRQ) for use in these patients. PATIENTS AND METHODS: We studied 37 adults with CF who were in stable condition as indicated by their respiratory and nutritional status. Disease severity was assessed by spirometry in conjunction with a modified National Institutes of Health (NIH) scoring system and the Bhalla scale. Nutritional status was evaluated by measuring height and weight, calculating body mass index, analyzing bioelectric impedance, and performing various laboratory tests. The patients' quality of life was assessed using the SGRQ. RESULTS: SGRQ scores were higher (indicating poorer quality of life) among patients with CF than in the general population or among patients with chronic obstructive pulmonary disease. Internal consistency coefficients indicated the SGRQ had good reliability (Cronbach alpha 0.864). Women with CF tended to score higher than men, although this difference was not statistically significantly. A statistically significant relationship was observed between SGRQ score and severity of pulmonary impairment (the more severe the impairment, the worse the patient's quality of life). Statistically significant positive relationships were found between SGRQ domains and age, body mass index, and body fat percentage. Significant negative relationships were found between these domains and the modified NIH score, the Bhalla score, forced expiratory volume in 1 second (expressed as a percentage of predicted), and somatomedin C and zinc levels. CONCLUSIONS: Self-perceived quality of life is worse among adults with CF than in the general population or among patients with chronic obstructive pulmonary disease. The SGRQ is a valid instrument for analyzing health-related quality of life in adults with CF as it discriminates very well between different degrees of severity of pulmonary impairment and also have an appropriate intern consistency.