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Papers by Carmen Luna Paredes

Microbiology Spectrum

In recent years, some techniques have been incorporated for the study of microbial ecosystems, be... more In recent years, some techniques have been incorporated for the study of microbial ecosystems, being 16S rRNA gene sequencing being the most widely used. Metaproteomics provides the advantage of identifying the interaction between microorganisms and human cells, but the available databases are less extensive as well as imprecise.

European Respiratory Journal, 2014

Background and Objective :Stenting may be indicated for the maintenance of airway patency in sele... more Background and Objective :Stenting may be indicated for the maintenance of airway patency in selected cases in children. Metallic and plastic stents are the most frequently used but complications have been reported repeatedly. Herein, we show our experience with a new type of biodegradable polydioxanone stent evaluating its safety and effectiveness in the setting of severe tracheal narrowing in children. Patients and methods : Seven custom-made polydioxanone stents were implanted in two infants with severe airway narrowing due to an extensive tracheal re-stenosis after a failed slide tracheoplasty in one case (6 stents) , and a persistent tracheomalacia after aortopexy in the other. Stent size ranged from 5-8mm diameter and 15-30mm length. All the devices were inserted bronchoscopically under general anaesthesia. Results : There were no complications related to stent placement. Tracheal narrowing relief and clinical improvement were achieved in both patients (age, 5 and 8 months). O...

Enfermedades Infecciosas y Microbiología Clínica, 2019

Introduction: New massive sequencing techniques make it possible to determine the composition of ... more Introduction: New massive sequencing techniques make it possible to determine the composition of airway microbiota in patients with cystic fibrosis (CF). However, the relationship between the composition of lung microbiome and the clinical status of paediatric patients is still not fully understood. Material and methods: A cross-sectional observational study was conducted on induced sputum samples from children with CF and known mutation in the CFTR gene. The bacterial sequences of the 16SrRNA gene were analyzed and their association with various clinical variables studied. Results: Analysis of the 13 samples obtained showed a core microbiome made up of Staphylococcus spp., Streptococcus spp., Rothia spp., Gemella spp. and Granulicatella spp., with a small number of Pseudomonas spp. The cluster of patients with less biodiversity were found to exhibit a greater number of sequences of Staphylococcus spp., mainly Staphylococcus aureus (p 0.009) and a greater degree of lung damage. Conclusion: An airway microbiome with greater biodiversity may be an indicator of less pronounced disease progression, in which case new therapeutic interventions that prevent reduction in non-pathogenic species of the airway microbiota should be studied.

Paediatric Bronchology, 2017

Archivos de Bronconeumología (English Edition), 2018

Background: The most common cystic fibrosis (CF)-causing mutation is deltaF508 (F508del), which i... more Background: The most common cystic fibrosis (CF)-causing mutation is deltaF508 (F508del), which is present in 28% of CF Spanish patients. While the literature based on real-life studies on CF patients homozygous F508del treated with lumacaftor/ivacaftor is limited, it demonstrates the need for better strategies to prevent related adverse events (AEs) as well as the development of newer drugs. Methods: We conducted a multicenter, retrospective, observational study to describe the effects of lumacaftor/ivacaftor treatment in real-life in Spain. 20 CF patients were included, all aged 6 and upwards and presented with ppFEV1 < 40%, chosen from CF units country-wide. For the purposes of the study, they were treated with lumacaftor/ivacaftor 200/125 mg two tablets twice a day on a compassionate use programme throughout 2016. The primary endpoint was measured in all of the sample patients. Data were analysed from ppFEV1 at baseline and was measured every 6 months. Results: The mean age was 26.65 (range of 10-45), while the mean ppFEV1 before the treatment was 32.4% and mean BMI was 19.9 kg/m 2. We analysed the changes in ppFEV1 and BMI from baseline during the treatment with lumacaftor/ivacaftor, but no differences were found. However, a moderate association between days of intravenous antibiotic needed and the use of lumacaftor/ivacaftor (p = 0.001) was established. Indeed, under the lumacaftor/ivacaftor, patients required 5.8 days of intravenous antibiotic treatment compared to 14.9 days prior to study. Also, severe pulmonary exacerbations requiring hospitalisation were statistically fewer under lumacaftor/ivacaftor treatment (p = 0.003). Finally, 75% of the sample presented with AEs, which led 35% of the subjects to discontinue the treatment. Conclusions: While treatment with lumacaftor/ivacaftor resulted in an improvement in the number of pulmonary severe exacerbations, no improvement in ppFEV1 or BMI was found.

Journal of Pediatric Surgery, 2018

Introduction: Laryngeal stenosis is infrequent in children and usually secondary to endotracheal ... more Introduction: Laryngeal stenosis is infrequent in children and usually secondary to endotracheal intubation. The aims of this study were to review the outcomes of the distinct endoscopic and surgical procedures and to suggest a technical modification for one of them. Methods: Retrospective review of patients with the diagnosis of laryngeal stenosis treated in an academic tertiary care institution between 2000 and 2017. The following variables were analyzed: demographic data, endoscopic findings including anatomic type and severity of the lesion, associated anomalies, type of treatment, outcomes, and time of follow-up. Results: Seventy-eight children were included in the study (39 boys) with a median age at diagnosis of 9 months, and 33 (42.3%) showed an associated anomaly. Lesions were acquired in 84.6% of cases and the subglottic region was most frequently involved (77%). Thirty patients (38.4%) had a severe stenosis (Myer-Cotton grades III and IV) and a tracheotomy was performed as an initial treatment in 38 patients (48.7%). Overall, 91% of endoscopically or surgically treated patients showed a good outcome and the decannulation rate in the series was 79.4%. Fourteen patients were managed conservatively. Median follow-up was 29 months (I.R. 10-60 m.) Conclusions: Laryngeal stenosis in children is usually acquired and exhibit a wide range of anatomic presentations. Endoscopic therapeutic procedures may be useful in the management of low grade immature stenosis. Reconstructive surgical techniques may provide a high success rate with an appropriate selection of candidates.

Paediatric Respiratory Medicine, 2013

International Journal of Pediatric Otorhinolaryngology, 2012

To assess the incidence of airway obstruction symptoms and the presence of obstructive sleep apne... more To assess the incidence of airway obstruction symptoms and the presence of obstructive sleep apnea in children with severe craniofacial anomalies by a proactive screening program using a standard questionnaire and cardiorespiratory polygraphy. Children with severe craniofacial anomalies referred to our paediatric airway unit from February 2001 to June 2011 were eligible to be included in this retrospective, single centre study. Symptoms of airway obstruction were proactively investigated using the shorter version of the Pediatric Sleep Questionnaire (PSQ). Obstructive sleep apnea was assessed by means of cardiorespiratory polygraphy. Demographic data and reason for referral were also recorded. Primary outcomes were the prevalence of symptoms of airway obstruction and OSA. 44 children (24 girls) with severe craniofacial anomalies (15 Crouzon, 13 Apert, 9 Goldenhar, 5 Treacher-Collins, 2 Pfeiffer) were included, at a mean age of 5 years (range 8 months to 14 years). Reason for referral was routine follow up in 30 patients and overt OSA symptoms and signs in the remaining 14. PSQ results showed symptoms of airway obstruction in 82% of patients, being snoring the most frequent symptom (64.1%) followed by apneas (33.3%). Polygraphic studies showed inconclusive results in 8 children (18.2%), normal apnea-hypopnea index (AHI) in 16 (36.4%), mild obstructive sleep apnea in 9 (20.4%), moderate in 4 (9.1%) and severe obstructive sleep apnea in 7 (15.9%). Children with craniofacial anomalies have a high prevalence of symptoms of airway obstruction and obstructive sleep apnea that support a proactive screening strategy in this highly selected population.

Journal of Pediatric Surgery, 2012

The purpose of this study is to assess the incidence of airway anomalies in children with severe ... more The purpose of this study is to assess the incidence of airway anomalies in children with severe craniofacial syndromes and to establish the role of bronchoscopy in the care of these patients. Consecutive children with craniofacial syndromes, including both bony deformities of the skull and face, in which a bronchoscopy was performed between 1995 and 2010 were retrospectively reviewed. Thirty-six patients (22 boys, 14 girls; mean age, 39 months) were studied. Craniofacial synostosis was present in 21 patients (Crouzen syndrome, 11; Apert syndrome, 7, Pfeiffer syndrome, 3) and craniofacial dysostosis in 15 (Goldenhart syndrome, 8; Treacher Collins syndrome, 7). In 30 patients (83.3%), bronchoscopy was performed because of respiratory symptoms (apneic episodes, 22; respiratory distress, 13; stridor, 6; cyanosis, 1) and, in the remaining 6 (asymptomatic children), during guided tracheal intubation before a surgical procedure. Airway anomalies were found in 69.4% of patients (70% in symptomatic patients). Management consisted of tracheotomy in 13 patients, adenoidectomy/tonsillectomy in 13, glossopexy in 5, antireflux surgery in 3, and supraglottoplasty in 2. Airway anomalies occurred in 70% of children with severe craniofacial syndromes and respiratory symptoms. Bronchoscopy should be performed routinely in this selected group of patients, and the entire airway must be examined. Treatment should be tailored to each individual patient.

Journal of Cystic Fibrosis, 2017

Results: A total of 36 subjects, 17 male and 19 female, with mean [range] age 24.7 years [18-45] ... more Results: A total of 36 subjects, 17 male and 19 female, with mean [range] age 24.7 years [18-45] entered the study. Baseline characteristics: predicted FEV 1 91.4% [67.6-128.7]; BMI 22.4 kg/m 2 [18-28]; sweat chloride 104 mmol/L [76-120]. After a single 50 mg dose of QR-010 (n = 6), median serum C max was 2.0950 ng/mL [1.1100-4.6500] at 0.5 hours with median AUC (0-t last) 12.4825 ng/mL [0.2775-24.7175]. No subjects withdrew from the study. No serious adverse events were reported; all treatmentemergent adverse events were mild or moderate. Conclusions: Single doses up to 50 mg of QR-010 were safe and welltolerated. A maximum tolerated dose was not established. Systemic concentrations of QR-010 were measured following a single dose administration, indicating that QR-010 has the potential to treat both pulmonary and extra-pulmonary manifestations of CF.