A scale for the identification of the complex chronic pediatric patient (PedCom Scale): A pilot study.

INTRODUCTION AND OBJECTIVES: The complex chronic condition (CCC) is an increasingly prevalent reality in pediatrics. However, having a CCC does not necessarily mean being a complex chronic patient (CCP). From this perspective, we developed an instrument (PedCom Scale) that would facilitate the identification of the CCP. MATERIAL AND METHODS: Initially, general aspects for the classification of patients as CCP were defined. Subsequently, the items of the scale were developed, scoring them from 0.5 to 4 points. We performed a confirmatory factor analysis (CFA) and the internal consistency was studied using alpha-Cronbach. Concordance was evaluated by intra- and inter-observer study. The gold standard was the classification performed by two evaluators after assessing the patient's medical history. The cut-off point for considering the patient as a CCP was established using the ROC curve. RESULTS: The initial version included 43 items with a global content validity index (CVI) of 0.94. A total of 180 patients were included. After the CFA, one item was eliminated, so the final version consists of 42 items with an CVI of 0.95. The alpha-Cronbach value was 0.723. The intraclass correlation coefficient of the test-retest analysis was 0.998 and 0.996 for the inter-observer study. The cut-off point for considering a patient as a CCP was established at 6.5 points, with this results we obtained a sensitivity of 98% and specificity of 94%. CONCLUSIONS: The PedCom Scale is an easy-to-use tool focused on the identification of the CCP. In our sample, it presented satisfactory levels of internal consistency and adequate levels of intra- and inter-observer agreement, with good sensitivity and specificity for the identification of the CCP.

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).

Influence of pneumococcal vaccination on the hospitalization of healthy pediatric patients due to typical Community-Acquired Pneumonia.

INTRODUCTION: Community-Acquired Pneumonia (CAP) is one of the most frequent causes of hospital admission in children. Our objective is to measure the impact of the introduction of pneumococcal conjugate vaccines on the hospitalization of previously healthy children due to CAP. METHOD: From 2011 to 2016, a partially retrospective, prospective, and descriptive study was carried out on healthy pediatric patients (3 months-14 years old) with CAP, who required hospital admission. Clinical, epidemiological, and demographic characteristics were collected, and vaccination status was obtained from medical records. RESULTS: A total of 292 cases were included, with a mean age of 33.4 months, 54% males. There was a progressive and significant 42% decrease in the number of admissions each year, without significant changes in the annual percentage of parapneumonic pleural effusion (PPE). Fifty-six percent of patients were immunized with a pneumococcal conjugate vaccine (PCV). The percentage of children who were not vaccinated decreased by 14%, and the coverage with PCV-13 increased by 46%. This revealed a significant increase of PPE in vaccinated patients with PCV-7 (63%) compared with unvaccinated (45%) and with PCV-13 (57%), without association with the presence of severe PPE. Moreover, no significant differences in severity or hospital stay were observed in unvaccinated patients, compared to those who were vaccinated. In >2-year-olds, we observed a significant increase in PPE (59%) compared to 45% in younger children. CONCLUSIONS: The increase in vaccination coverage with PCV-13 resulted in a decrease in hospitalizations due to CAP and PPE. Vaccination with PCV-7 is associated in our sample with an increase in PPE but not with severe PPE nor an increase in the hospital stay. There was an epidemiological shift of severe forms of pneumonia and empyema at later ages (>2 years).

Recurrent wheezing during the first 3 years of life in a birth cohort of moderate-to-late preterm infants.

BACKGROUND: Data addressing short- and long-term respiratory morbidity in moderate-late preterm infants are limited. We aim to determine the incidence of recurrent wheezing and associated risk and protective factors in these infants during the first 3 years of life. METHODS: Prospective, multicenter birth cohort study of infants born at 32+0 to 35+0  weeks' gestation and followed for 3 years to assess the incidence of physician-diagnosed recurrent wheezing. Allergen sensitization and pulmonary function were also studied. We used multivariate mixed-effects models to identify risk factors associated with recurrent wheezing. RESULTS: A total of 977 preterm infants were enrolled. Rates of recurrent wheezing during year (Y)1 and Y2 were similar (19%) but decreased to 13.3% in Y3. Related hospitalizations significantly declined from 6.3% in Y1 to 0.75% in Y3. Independent risk factors for recurrent wheezing during Y2 and Y3 included the following: day care attendance, acetaminophen use during pregnancy, and need for mechanical ventilation. Atopic dermatitis on Y2 and male sex on Y3 were also independently associated with recurrent wheezing. Palivizumab prophylaxis for RSV during the first year of life decreased the risk or recurrent wheezing on Y3. While there were no differences in rates of allergen sensitization, pulmonary function tests (FEV0.5 ) were significantly lower in children who developed recurrent wheezing. CONCLUSIONS: In moderate-to-late premature infants, respiratory symptoms were associated with lung morbidity persisted during the first 3 years of life and were associated with abnormal pulmonary function tests. Only anti-RSV prophylaxis exerted a protective effect in the development of recurrent wheezing.

Amino Acid Transport Defects in Human Inherited Metabolic Disorders.

Amino acid transporters play very important roles in nutrient uptake, neurotransmitter recycling, protein synthesis, gene expression, cell redox balance, cell signaling, and regulation of cell volume. With regard to transporters that are closely connected to metabolism, amino acid transporter-associated diseases are linked to metabolic disorders, particularly when they involve different organs, cell types, or cell compartments. To date, 65 different human solute carrier (SLC) families and more than 400 transporter genes have been identified, including 11 that are known to include amino acid transporters. This review intends to summarize and update all the conditions in which a strong association has been found between an amino acid transporter and an inherited metabolic disorder. Many of these inherited disorders have been identified in recent years. In this work, the physiological functions of amino acid transporters will be described by the inherited diseases that arise from transporter impairment. The pathogenesis, clinical phenotype, laboratory findings, diagnosis, genetics, and treatment of these disorders are also briefly described. Appropriate clinical and diagnostic characterization of the underlying molecular defect may give patients the opportunity to avail themselves of appropriate therapeutic options in the future.

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.

Risk factors for bronchiolitis, recurrent wheezing, and related hospitalization in preterm infants during the first year of life.

BACKGROUND: Airway diseases are highly prevalent in infants and cause significant morbidity. We aimed to determine the incidence and risk factors for respiratory morbidity in a Spanish cohort of moderate-to-late preterm (MLP) infants prospectively followed during their first year of life. METHODS: SAREPREM is a multicenter, prospective, longitudinal study. Preterm infants born at 32-35 weeks of gestation with no comorbidities were enrolled within 2 weeks of life and followed at 2-4 weeks, 6, and 12 months of age. Multivariate mixed-models were performed to identify independent risk factors associated with (i) development of bronchiolitis, (ii) recurrent wheezing, or (iii) related hospital admissions. RESULTS: Overall, 977 preterm infants were included, and 766 (78.4%) completed follow-up. Of those, 365 (47.7%) developed bronchiolitis during the first year, 144 (18.8%) recurrent wheezing, and 48 (6.3%) were hospitalized. While low birthweight, day care attendance (DCA) and school-age siblings were significantly and independently associated with both the development of bronchiolitis and recurrent wheezing, lower maternal age increased the risk for bronchiolitis and respiratory-related hospitalizations. Lastly, mechanical ventilation was associated with a higher risk of bronchiolitis and history of asthma in any parent increased the likelihood of developing recurrent wheezing. CONCLUSIONS: In this study, several non-modifiable parameters (family history of asthma, low birthweight, need for mechanical ventilation) and modifiable parameters (young maternal age, DCA, or exposure to school-age siblings) were identified as significant risk factors for the development of bronchiolitis and recurrent wheezing during the first year of life in MLP infants.

Paediatric patients with a tracheostomy: a multicentre epidemiological study.

Changes in the indications for tracheostomy in children have led to the progressively greater involvement of the paediatric pulmonologist in the care of these patients. The aim of this study was to review the current profile of tracheostomised children in Spain. We undertook a longitudinal, multicentre study over 2 yrs (2008 and 2009) of all patients aged between 1 day and 18 yrs who had a tracheostomy. The study, involving 18 Spanish hospitals, included 249 patients, of whom 150 (60.2%) were <1 yr of age. The main indications for the procedure were prolonged ventilation (n=156, 62.6%), acquired subglottic stenosis (n=34, 13.6%), congenital or acquired craniofacial anomalies (n=25, 10%) and congenital airway anomalies (n=24, 9.6%). The most frequent underlying disorders were neurological diseases (n=126, 50.6%) and respiratory diseases (n=98, 39.3%). Over the 2-yr study period, 92 (36.9%) children required ventilatory support, and decannulation was achieved in 59 (23.7%). Complications arose in 117 patients (46.9%). Mortality attributed to the underlying condition was 12.5% and that related directly to the tracheostomy was 3.2%. Respiratory complexity of tracheostomised children necessitates prolonged, multidisciplinary follow-up, which can often extend to adulthood.

[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.