N. Cobos - Academia.edu (original) (raw)
Papers by N. Cobos
Archivos de Bronconeumología ((English Edition)), 2007
OBJECTIVE: The aim of this study was to determine whether variability in peak expiratory flow (PE... more OBJECTIVE: The aim of this study was to determine whether variability in peak expiratory flow (PEF) could be used to classify the level of severity of asthma in children.
Archivos de Bronconeumología, 1999
... Este círculo vicioso de obstrucción-infec-ción-inflamación daña el pulmón, produciendo bron-q... more ... Este círculo vicioso de obstrucción-infec-ción-inflamación daña el pulmón, produciendo bron-quiectasias quísticas y fibrosis pulmonar. ... Hospitals Vall d'Hebron. Informaciones e inscripciones: Sras. Rosa Llòria y Montse Murillo. Servei de Pneumologia. ...
Medicina clínica, Jan 24, 1998
Cross-sectional study of bone mineral density (BMD) in children and adolescents with cystic fibro... more Cross-sectional study of bone mineral density (BMD) in children and adolescents with cystic fibrosis of the pancreas. The relationship of BMD values with nutritional status, respiratory function and the cystic transmembrane regulator genotype was also evaluated. BMD expressed as grams of hydroxyapatite/cm2 was measured by dual-energy X-ray absorptiometry in the lumbar spine (L2-L4) in 41 patients (21 males and 20 women; age range: 4-21 years) with cystic fibrosis of the pancreas and compared with that of 471 normal controls (256 males and 215 women; age range: 1-20 years). Twenty patients were prepubertal, 9 pubertal and 12 young adults. Clinical repercussion of the disease evaluated by clinical and anthropometric data (weight, height and body mass index) and respiratory function was considered moderate. Height z score (mean [MSE]) was -0.53 (0.28), weight -0.81 (0.21) and body mass index -0.82 (0.12) BMD z score values (mean [MSE]) were -1.14 (0.17) and differed significantly (p &l...
Cirugía pediátrica : organo oficial de la Sociedad Española de Cirugía Pediátrica, 1990
The authors believe that the greater part of chronic cervicofacial adenitis actually observed in ... more The authors believe that the greater part of chronic cervicofacial adenitis actually observed in our hospitals, are not caused by M. tuberculosis or M. bovis but by scrofulaceum mycobacterium, M. avium, M. fortuitum and M. Kansasii, and above all, by the first two of these. They present their experience with 16 cases of cervico-facial adenitis due to atypical mycobacterium (CAAM) treated in our centre during the last years, in which period no case of cervical tuberculosis (CT) was observed. It is important to establish an early differential diagnosis between both etiologies, seeing as treatment is different. Whilst tuberculostatics can solve the phymic infection, surgical extirpation is the only solution for CAAM. The diagnosis of these types of infection is achieved by means of a very characteristic clinical procedure and by cutaneous tests specific for each bacteria. Faced with the clinical suspicion, the total extirpation should be effected of the adenopathic block affected. The ...
Enfermedades infecciosas y microbiología clínica, 1992
We have studied the bacteriology of mucoviscidosis cases from 1985 to 1989. A total of 336 sample... more We have studied the bacteriology of mucoviscidosis cases from 1985 to 1989. A total of 336 samples from 50 patients (median age: 7 year, range: 1 day-18 years) with mucoviscidosis were studied. The most frequently isolated microorganisms was Pseudomonas aeruginosa (59.2%), followed by Staphylococcus aureus (19%) and Haemophilus influenzae (18.4%). In patients younger than 1 year of age other different microorganisms were identified in 61.1% of cases, but S. aureus (11.1%) and Pseudomonas aeruginosa (5.5%) were also isolated. The incidence of Pseudomonas cepacia (0.6%) is low in our environment. We did not isolate any Legionella sp. strain.
Anales de pediatría (Barcelona, Spain : 2003), 2004
To assess the efficacy and tolerability of oral deflazacort versus oral prednisolone in acute mod... more To assess the efficacy and tolerability of oral deflazacort versus oral prednisolone in acute moderate asthma in children. We performed a prospective, randomized, parallel group trial of children aged 6 to 14 years old with a diagnosis of asthma who presented to the pediatric emergency department for moderate asthma exacerbation. All patients were administered short-acting beta2-adrenergic agonists. The intervention groups received either oral deflazacort (1.5 mg/kg) or prednisolone (1 mg/kg) for 7 days. The primary outcome measure was forced expiratory volume in 1 second (FEV1) and secondary outcome measures were pulmonary symptom score index, peak expiratory flow rate (PEFR), hospitalization rate and the use of rescue beta2-agonists. Patients were evaluated at the start of treatment (visit 1), on day 2 (visit 2) and on day 7 (visit 3). Of the 54 children enrolled, two were hospitalized on visit 2 (one from each group). Baseline clinical data were similar in both groups: FEV1: 53 a...
Medicina clínica, Jan 20, 2001
This was a study aimed to know the knowledge about asthma. 933 teachers of 27 schools from 7 citi... more This was a study aimed to know the knowledge about asthma. 933 teachers of 27 schools from 7 cities (children aged between 4 and 15 years) using a self-administered questionnaire Half of them knew asthma prevalence and its hereditary nature. Nearly all related asthma with allergy, 38% with exercise, 25%had students who suffered from asthma attacks in the school, and 91% admitted to have limited information and wish to improve it. Our survey reveals that Spanish teachers have a limited information about asthma.
Archivos de bronconeumología
Medicina clínica, Jan 28, 2001
To analyze the clinical utility of the quality of life questionnaire for asthmatic children (PAQL... more To analyze the clinical utility of the quality of life questionnaire for asthmatic children (PAQLQ) and to validate it for use in clinical practice. 1,012 children between 6 and 14 years of age, with a diagnosis of mild to moderate asthma attending 48 Spanish hospitals were included in the study. The patients' socio-demographic and clinical characteristics were recorded and all patients were administered the PAQLQ and EQ-5D questionnaires on two occasions: at the baseline visit and at 2-3 months from baseline in patients with uncontrolled asthma(group A) and at baseline and 15 days from baseline in patients with controlled asthma (group B). The feasibility, validity, reliability and sensitivity to change of the PAQLQ were assessed. The PAQLQ proved to be feasible for use in children over 7 years of age with mild or moderate asthma. The PAQLQ did not show a statistically significant relationship with socio-demographic variables, nor with the majority of clinical variables, with t...
Pediatric Pulmonology, 2009
To develop and validate a questionnaire to assess asthma control in children (CAN). Two versions ... more To develop and validate a questionnaire to assess asthma control in children (CAN). Two versions of the CAN (for carers and children) were developed. Both versions were validated in an observational, prospective, multicenter study performed in 38 hospital outpatient clinics throughout Spain. Four hundred fifteen patients and their carers agreed to participate. Of these, 414 patients under 14 years old with frequent episodic or persistent (moderate to severe) asthma completed the questionnaire on 3 occasions (baseline, week 2, and week 12). For patients aged 2-8 the questionnaire was only completed by the carers, but for patients aged 9-14 the questionnaire was completed by the carers and the children. Clinician ratings of asthma control were used as a gold standard to assess the sensitivity, specificity, PPV and NPV of the new measure. Evaluable responses were obtained from 215 carers for children aged 2-8 years and 199 children aged 9-14 years, and their parents. Using a questionnaire total score cut-off of 8 the patient version had a sensitivity of 76.3% and a specificity of 62.9%. For carer version these values were 73% and 69.7%, respectively. A cut point of 8 was selected to maximize the screening accuracy of the CAN questionnaire. Effect sizes in patients with clinician-rated improvements in asthma control were 0.33 and 0.57 for the carer and child versions, respectively. The screening accuracy and validity of the CAN questionnaire make it suitable for use in research and clinical practice. The sensitivity and specificity were close to 70%, which is acceptable for the study objective: obtain a tool to measure the level of asthma control.
Journal of Cystic Fibrosis, 2006
Aims: To determine whether antibiotic treatment of early/~ aeruginosa infection in children and a... more Aims: To determine whether antibiotic treatment of early/~ aeruginosa infection in children and adults with cystic fibrosis eradicates the organism and improves clinical and microbiological outcome. Methods: A systematic review and meta-analysis of relevant trials identified by searching the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register. We included randomised controlled trials of people with cystic fibrosis, in whom 1~ aeruginosa had recently been isolated from respiratory secretions. We
Journal of Cystic Fibrosis, 2010
Journal of Cystic Fibrosis, 2010
Journal of Computer Assisted Tomography, 1998
The clinical and imaging findings of children with Swyer-James-MacLeod syndrome (SJMS) were revie... more The clinical and imaging findings of children with Swyer-James-MacLeod syndrome (SJMS) were reviewed to determine the incidence and type of bronchiectasis and analyze whether the clinical course of patients with bronchiectasis differed from that of patients without bronchiectasis. Our study population consisted of 13 patients. All had inspiratory/ expiratory chest X-ray films, chest CT, and lung scans. Eight underwent pulmonary function test. The results of these studies at diagnosis and during follow-up were analyzed and compared with the clinical features. Bronchiectasis was demonstrated in nine patients, being saccular in five and cylindrical in four. Expiratory slices were helpful for demonstrating bilateral lung involvement that had not been suspected on inspiratory CT scans or conventional radiographs. The clinical features of the five patients with saccular bronchiectasis resembled those of patients with classic postviral bronchiectasis who suffered recurrent pulmonary infections; three of them underwent lobectomy. The remaining patients presented mild respiratory symptoms, with a spontaneous tendency toward improvement. SJMS should be considered as a spectrum disease. Bronchiectasis is not a universal finding. The presence and type of bronchiectasis will influence clinical manifestations and prognosis. Patients without bronchiectasis or with cylindrical bronchiectasis had a lower incidence of pneumonia episodes than those with saccular bronchiectasis.
Human Genetics, 1995
We present the genotype/phenotype correlation analysis for 16 cystic fibrosis (CF) patients who c... more We present the genotype/phenotype correlation analysis for 16 cystic fibrosis (CF) patients who carry mutation R334W. Current age and age of diagnosis was significantly higher in the R334W/any-mutation group (P < 0.05 and P < 0.01), compared with the delta F508/delta F508 group. A slightly, but not significantly, worse lung function was found in the R334W/any-mutation group, when compared with the delta F508/delta F508 patients. The proportion of patients with lung colonization with bacterial pathogens was slightly, but not significantly, higher in the R334W/any-mutation group (71.4%), compared with the delta F508/delta F508 or R334W/delta F508 groups (55.5%). None of the R334W patients had meconium ileus but 60% were pancreatic insufficient (PI), a significantly lower proportion (P < 0.001) than delta F508/delta F508 patients. Two R334W/N1303K compound heterozygous sisters of three sibs with genotype R334W/delta F508 showed interfamilial discordant clinical data for lung and pancreatic function. The data provided here for mutation R334W demonstrate that this mutation is responsible for a less severe form of CF than delta F508. Interfamilial differences for PI and lung function suggest that other factors, viz. genetic, environmental and medical, contribute to the wide spectrum of clinical differences observed in CF patients with the same CF transmembrane conductance regulator genotypes.
European Journal of Pediatrics, 2000
Short-term clinical trials with DNase have shown minor to moderate benefits in cystic fibrosis pa... more Short-term clinical trials with DNase have shown minor to moderate benefits in cystic fibrosis patients. This study was performed to analyse the effectiveness of DNase use in daily practice and to obtain information on its effects in the long term and at different disease stages. Patients being treated in 13 specialised units were included if they started DNase treatment before June 1996. Baseline data before DNase use and data during the DNase treatment period were recorded. Of the 199 patients included in the study 166 continued on DNase treatment while the data were being collected. The mean age (95% CI) was 14.5 (13.7; 15,2) years; 103 (51.8%) patients were female. The mean maximum change in forced expiratory volume in 1 s (FEV(1)) was observed during the first month of treatment [11.1% (6.1; 16.1)]. By the end of the first and the second year of treatment mean changes in FEV(1) were 3.3% (-1.1; 7. 6) and 5.1% (-0.7; 10.9) respectively; at the end of the same periods 34% of patients had improved their baseline FEV(1) by 10% or more but in around 50% of patients the level fell below the baseline. A large inter-individual variability in changes in pulmonary function after the start of DNase treatment was documented. In addition, the medium-term response to treatment was correlated with early response during the first 3 months. No consistent changes in exacerbation pattern were found during the first year of treatment. The benefits of DNase use in daily practice are limited but apparently can be maintained in the medium term in some patients. A large inter-individual variability in response to DNase treatment has been documented and the benefits are doubtful in around 50% of patients. This observation points to the need to set up a withdrawal trial in these patients, using as an eligibility criterion the early response observed during the first 3 months of treatment.
Clinical Microbiology and Infection, 2005
Pseudomonas aeruginosa colonisation has a negative effect on pulmonary function in cystic fibrosi... more Pseudomonas aeruginosa colonisation has a negative effect on pulmonary function in cystic fibrosis patients. The organism can only be eradicated in the early stage of colonisation, while reduction of bacterial density is desirable during chronic colonisation or exacerbations. Monthly, or at least 3-monthly, microbiological culture is advisable for patients without previous evidence of P. aeruginosa colonisation. Cultures should be performed at least every 2-3 months in patients with well-established colonisation, and always during exacerbations or hospitalisations. Treatment of patients following the first isolation of P. aeruginosa, but with no clinical signs of colonisation, should be with oral ciprofloxacin (15-20 mg/kg twice-daily for 3-4 weeks) plus inhaled tobramycin or colistin (intravenous treatment with or without inhaled treatment can be used as an alternative), while patients with acute infection should be treated for 14-21 days with high doses of two intravenous antimicrobial agents, with or without an inhaled treatment during or at the end of the intravenous treatment. Maintenance treatment after development of chronic P. aeruginosa infection/colonisation (pathogenic colonisation) in stable patients (aged&amp;amp;amp;amp;amp;amp;amp;amp;amp;gt;6 years) should be with inhaled tobramycin (300 mg twice-daily) in 28-day cycles (on-off) or, as an alternative, colistin (1-3 million units twice-daily). Colistin is also a possible choice for patients aged&amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;6 years. Treatment can be completed with oral ciprofloxacin (3-4 weeks every 3-4 months) for patients with mild pulmonary symptoms, or intravenously (every 3-4 months) for those with severe symptoms or isolates with ciprofloxacin resistance. Moderate and serious exacerbations can be treated with intravenous ceftazidime (50-70 mg/kg three-times-daily) or cefepime (50 mg/kg three-times-daily) plus tobramycin (5-10 mg/kg every 24 h) or amikacin (20-30 mg/kg every 24 h) for 2-3 weeks. Oral ciprofloxacin is recommended for patients with mild pulmonary disease. If multiresistant P. aeruginosa is isolated, antimicrobial agents that retain activity are recommended and epidemiological control measures should be established.
BioFactors, 1998
Antioxidant depletion and increased free radical production by inflammatory cells have been descr... more Antioxidant depletion and increased free radical production by inflammatory cells have been described in cystic fibrosis (CF) patients. To evaluate oxidative damage intensity, we measured plasma concentrations of malondialdehyde, hydroperoxides and protein carbon groups as markers of oxidative injury to lipids and proteins in a group of 101 CF patients free of acute exacerbation, and in 43-112 controls. Moreover, we estimated antioxidant function by measuring activities of erythrocyte superoxide dismutase, glutathione reductase and vitamin E concentrations. In CF patients, malondialdehyde and hydroperoxide plasma levels were significantly higher than in controls (p &amp;lt; 0.001). Increased lipid peroxidation was documented by these two markers. Parallel rises in protein carbonyls in plasma of CF patients were observed (p &amp;lt; 0.0001). These patients presented biochemical but not clinical vitamin E deficiency. Glutathione reductase and superoxide dismutase activities were significantly higher than in controls. These results show a serious imbalance of CF patients between oxidant-antioxidant status leading to oxidative stress.
Archivos de Bronconeumología, 2005
Page 1. Introducción La fibrosis quística (FQ) es una enfermedad genética autosómica recesiva. Se... more Page 1. Introducción La fibrosis quística (FQ) es una enfermedad genética autosómica recesiva. Se produce como consecuencia de la alteración del gen CFTR (cystic fibrosis transmem-brane conductance regulator), situado en el brazo largo del cromosoma 71. ...
Anales de Pediatría, 2004
Evaluar la eficacia y tolerancia del deflazacort frente a la prednisolona en el tratamiento de la... more Evaluar la eficacia y tolerancia del deflazacort frente a la prednisolona en el tratamiento de la agudización moderada de asma en niños. Pacientes y métodos Estudio de intervención, multicéntrico, prospectivo, abierto, aleatorizado, grupos paralelos en niños de 6 a 14 años diagnosticados de asma, en situación de agudización moderada tratados todos con agonistas  2-adrenérgicos de corta acción. Los grupos de intervención recibieron deflazacort (1,5 mg/kg) o prednisolona (1 mg/kg) durante 7 días. La medida principal de eficacia fue el volumen espiratorio forzado en el primer segundo (FEV 1) y como medidas secundarias se evaluaron la escala clínica de gravedad, el flujo espiratorio máximo (PEF), el índice de hospitalización y la utilización de medicación  2-agonista de rescate. Todos los sujetos fueron controlados al inicio del tratamiento (visita 1), al segundo día (visita 2) y al séptimo día (visita 3) del estudio. Resultados Se incluyeron en el estudio 54 pacientes, de los cuales dos requirieron hospitalización (uno de cada grupo). Los valores iniciales fueron similares para ambos grupos: FEV 1 , 53 y 51 %; test de broncodilatación, ؉19 y ؉21 %; PEF, 169 y 165 l/min; escala de gravedad, 6,1 y 6,5 para los grupos deflazacort y prednisolona, respectivamente. En la visita 2, todos los parámetros mostraron mejoría: FEV 1 , ؉22,2 y ؉26,5 % (p < 0,05); PEF, ؉64 y ؉49 l/min (p < 0,05); escala de gravedad-4,4 y-3,8 (p < 0,05), sin diferencias significativas entre ambos grupos. En la visita 3 todos los parámetros continuaron mejorando: FEV 1 , ؉33,2 y ؉32,5 % (p < 0,05); PEF, ؉115,7 y ؉87,6 l/min (p < 0,05); escala de gravedad-5,4 y-5,9 (p < 0,05), también sin diferencias significativas entre los dos grupos. No se registraron efectos adversos en ningún paciente. Conclusiones En el tratamiento de la agudización moderada de asma en niños, deflazacort tiene una eficacia similar a prednisolona como se refleja tanto en la mejoría clínica de los pacientes como en la función pulmonar.
Archivos de Bronconeumología ((English Edition)), 2007
OBJECTIVE: The aim of this study was to determine whether variability in peak expiratory flow (PE... more OBJECTIVE: The aim of this study was to determine whether variability in peak expiratory flow (PEF) could be used to classify the level of severity of asthma in children.
Archivos de Bronconeumología, 1999
... Este círculo vicioso de obstrucción-infec-ción-inflamación daña el pulmón, produciendo bron-q... more ... Este círculo vicioso de obstrucción-infec-ción-inflamación daña el pulmón, produciendo bron-quiectasias quísticas y fibrosis pulmonar. ... Hospitals Vall d&#x27;Hebron. Informaciones e inscripciones: Sras. Rosa Llòria y Montse Murillo. Servei de Pneumologia. ...
Medicina clínica, Jan 24, 1998
Cross-sectional study of bone mineral density (BMD) in children and adolescents with cystic fibro... more Cross-sectional study of bone mineral density (BMD) in children and adolescents with cystic fibrosis of the pancreas. The relationship of BMD values with nutritional status, respiratory function and the cystic transmembrane regulator genotype was also evaluated. BMD expressed as grams of hydroxyapatite/cm2 was measured by dual-energy X-ray absorptiometry in the lumbar spine (L2-L4) in 41 patients (21 males and 20 women; age range: 4-21 years) with cystic fibrosis of the pancreas and compared with that of 471 normal controls (256 males and 215 women; age range: 1-20 years). Twenty patients were prepubertal, 9 pubertal and 12 young adults. Clinical repercussion of the disease evaluated by clinical and anthropometric data (weight, height and body mass index) and respiratory function was considered moderate. Height z score (mean [MSE]) was -0.53 (0.28), weight -0.81 (0.21) and body mass index -0.82 (0.12) BMD z score values (mean [MSE]) were -1.14 (0.17) and differed significantly (p &l...
Cirugía pediátrica : organo oficial de la Sociedad Española de Cirugía Pediátrica, 1990
The authors believe that the greater part of chronic cervicofacial adenitis actually observed in ... more The authors believe that the greater part of chronic cervicofacial adenitis actually observed in our hospitals, are not caused by M. tuberculosis or M. bovis but by scrofulaceum mycobacterium, M. avium, M. fortuitum and M. Kansasii, and above all, by the first two of these. They present their experience with 16 cases of cervico-facial adenitis due to atypical mycobacterium (CAAM) treated in our centre during the last years, in which period no case of cervical tuberculosis (CT) was observed. It is important to establish an early differential diagnosis between both etiologies, seeing as treatment is different. Whilst tuberculostatics can solve the phymic infection, surgical extirpation is the only solution for CAAM. The diagnosis of these types of infection is achieved by means of a very characteristic clinical procedure and by cutaneous tests specific for each bacteria. Faced with the clinical suspicion, the total extirpation should be effected of the adenopathic block affected. The ...
Enfermedades infecciosas y microbiología clínica, 1992
We have studied the bacteriology of mucoviscidosis cases from 1985 to 1989. A total of 336 sample... more We have studied the bacteriology of mucoviscidosis cases from 1985 to 1989. A total of 336 samples from 50 patients (median age: 7 year, range: 1 day-18 years) with mucoviscidosis were studied. The most frequently isolated microorganisms was Pseudomonas aeruginosa (59.2%), followed by Staphylococcus aureus (19%) and Haemophilus influenzae (18.4%). In patients younger than 1 year of age other different microorganisms were identified in 61.1% of cases, but S. aureus (11.1%) and Pseudomonas aeruginosa (5.5%) were also isolated. The incidence of Pseudomonas cepacia (0.6%) is low in our environment. We did not isolate any Legionella sp. strain.
Anales de pediatría (Barcelona, Spain : 2003), 2004
To assess the efficacy and tolerability of oral deflazacort versus oral prednisolone in acute mod... more To assess the efficacy and tolerability of oral deflazacort versus oral prednisolone in acute moderate asthma in children. We performed a prospective, randomized, parallel group trial of children aged 6 to 14 years old with a diagnosis of asthma who presented to the pediatric emergency department for moderate asthma exacerbation. All patients were administered short-acting beta2-adrenergic agonists. The intervention groups received either oral deflazacort (1.5 mg/kg) or prednisolone (1 mg/kg) for 7 days. The primary outcome measure was forced expiratory volume in 1 second (FEV1) and secondary outcome measures were pulmonary symptom score index, peak expiratory flow rate (PEFR), hospitalization rate and the use of rescue beta2-agonists. Patients were evaluated at the start of treatment (visit 1), on day 2 (visit 2) and on day 7 (visit 3). Of the 54 children enrolled, two were hospitalized on visit 2 (one from each group). Baseline clinical data were similar in both groups: FEV1: 53 a...
Medicina clínica, Jan 20, 2001
This was a study aimed to know the knowledge about asthma. 933 teachers of 27 schools from 7 citi... more This was a study aimed to know the knowledge about asthma. 933 teachers of 27 schools from 7 cities (children aged between 4 and 15 years) using a self-administered questionnaire Half of them knew asthma prevalence and its hereditary nature. Nearly all related asthma with allergy, 38% with exercise, 25%had students who suffered from asthma attacks in the school, and 91% admitted to have limited information and wish to improve it. Our survey reveals that Spanish teachers have a limited information about asthma.
Archivos de bronconeumología
Medicina clínica, Jan 28, 2001
To analyze the clinical utility of the quality of life questionnaire for asthmatic children (PAQL... more To analyze the clinical utility of the quality of life questionnaire for asthmatic children (PAQLQ) and to validate it for use in clinical practice. 1,012 children between 6 and 14 years of age, with a diagnosis of mild to moderate asthma attending 48 Spanish hospitals were included in the study. The patients' socio-demographic and clinical characteristics were recorded and all patients were administered the PAQLQ and EQ-5D questionnaires on two occasions: at the baseline visit and at 2-3 months from baseline in patients with uncontrolled asthma(group A) and at baseline and 15 days from baseline in patients with controlled asthma (group B). The feasibility, validity, reliability and sensitivity to change of the PAQLQ were assessed. The PAQLQ proved to be feasible for use in children over 7 years of age with mild or moderate asthma. The PAQLQ did not show a statistically significant relationship with socio-demographic variables, nor with the majority of clinical variables, with t...
Pediatric Pulmonology, 2009
To develop and validate a questionnaire to assess asthma control in children (CAN). Two versions ... more To develop and validate a questionnaire to assess asthma control in children (CAN). Two versions of the CAN (for carers and children) were developed. Both versions were validated in an observational, prospective, multicenter study performed in 38 hospital outpatient clinics throughout Spain. Four hundred fifteen patients and their carers agreed to participate. Of these, 414 patients under 14 years old with frequent episodic or persistent (moderate to severe) asthma completed the questionnaire on 3 occasions (baseline, week 2, and week 12). For patients aged 2-8 the questionnaire was only completed by the carers, but for patients aged 9-14 the questionnaire was completed by the carers and the children. Clinician ratings of asthma control were used as a gold standard to assess the sensitivity, specificity, PPV and NPV of the new measure. Evaluable responses were obtained from 215 carers for children aged 2-8 years and 199 children aged 9-14 years, and their parents. Using a questionnaire total score cut-off of 8 the patient version had a sensitivity of 76.3% and a specificity of 62.9%. For carer version these values were 73% and 69.7%, respectively. A cut point of 8 was selected to maximize the screening accuracy of the CAN questionnaire. Effect sizes in patients with clinician-rated improvements in asthma control were 0.33 and 0.57 for the carer and child versions, respectively. The screening accuracy and validity of the CAN questionnaire make it suitable for use in research and clinical practice. The sensitivity and specificity were close to 70%, which is acceptable for the study objective: obtain a tool to measure the level of asthma control.
Journal of Cystic Fibrosis, 2006
Aims: To determine whether antibiotic treatment of early/~ aeruginosa infection in children and a... more Aims: To determine whether antibiotic treatment of early/~ aeruginosa infection in children and adults with cystic fibrosis eradicates the organism and improves clinical and microbiological outcome. Methods: A systematic review and meta-analysis of relevant trials identified by searching the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register. We included randomised controlled trials of people with cystic fibrosis, in whom 1~ aeruginosa had recently been isolated from respiratory secretions. We
Journal of Cystic Fibrosis, 2010
Journal of Cystic Fibrosis, 2010
Journal of Computer Assisted Tomography, 1998
The clinical and imaging findings of children with Swyer-James-MacLeod syndrome (SJMS) were revie... more The clinical and imaging findings of children with Swyer-James-MacLeod syndrome (SJMS) were reviewed to determine the incidence and type of bronchiectasis and analyze whether the clinical course of patients with bronchiectasis differed from that of patients without bronchiectasis. Our study population consisted of 13 patients. All had inspiratory/ expiratory chest X-ray films, chest CT, and lung scans. Eight underwent pulmonary function test. The results of these studies at diagnosis and during follow-up were analyzed and compared with the clinical features. Bronchiectasis was demonstrated in nine patients, being saccular in five and cylindrical in four. Expiratory slices were helpful for demonstrating bilateral lung involvement that had not been suspected on inspiratory CT scans or conventional radiographs. The clinical features of the five patients with saccular bronchiectasis resembled those of patients with classic postviral bronchiectasis who suffered recurrent pulmonary infections; three of them underwent lobectomy. The remaining patients presented mild respiratory symptoms, with a spontaneous tendency toward improvement. SJMS should be considered as a spectrum disease. Bronchiectasis is not a universal finding. The presence and type of bronchiectasis will influence clinical manifestations and prognosis. Patients without bronchiectasis or with cylindrical bronchiectasis had a lower incidence of pneumonia episodes than those with saccular bronchiectasis.
Human Genetics, 1995
We present the genotype/phenotype correlation analysis for 16 cystic fibrosis (CF) patients who c... more We present the genotype/phenotype correlation analysis for 16 cystic fibrosis (CF) patients who carry mutation R334W. Current age and age of diagnosis was significantly higher in the R334W/any-mutation group (P < 0.05 and P < 0.01), compared with the delta F508/delta F508 group. A slightly, but not significantly, worse lung function was found in the R334W/any-mutation group, when compared with the delta F508/delta F508 patients. The proportion of patients with lung colonization with bacterial pathogens was slightly, but not significantly, higher in the R334W/any-mutation group (71.4%), compared with the delta F508/delta F508 or R334W/delta F508 groups (55.5%). None of the R334W patients had meconium ileus but 60% were pancreatic insufficient (PI), a significantly lower proportion (P < 0.001) than delta F508/delta F508 patients. Two R334W/N1303K compound heterozygous sisters of three sibs with genotype R334W/delta F508 showed interfamilial discordant clinical data for lung and pancreatic function. The data provided here for mutation R334W demonstrate that this mutation is responsible for a less severe form of CF than delta F508. Interfamilial differences for PI and lung function suggest that other factors, viz. genetic, environmental and medical, contribute to the wide spectrum of clinical differences observed in CF patients with the same CF transmembrane conductance regulator genotypes.
European Journal of Pediatrics, 2000
Short-term clinical trials with DNase have shown minor to moderate benefits in cystic fibrosis pa... more Short-term clinical trials with DNase have shown minor to moderate benefits in cystic fibrosis patients. This study was performed to analyse the effectiveness of DNase use in daily practice and to obtain information on its effects in the long term and at different disease stages. Patients being treated in 13 specialised units were included if they started DNase treatment before June 1996. Baseline data before DNase use and data during the DNase treatment period were recorded. Of the 199 patients included in the study 166 continued on DNase treatment while the data were being collected. The mean age (95% CI) was 14.5 (13.7; 15,2) years; 103 (51.8%) patients were female. The mean maximum change in forced expiratory volume in 1 s (FEV(1)) was observed during the first month of treatment [11.1% (6.1; 16.1)]. By the end of the first and the second year of treatment mean changes in FEV(1) were 3.3% (-1.1; 7. 6) and 5.1% (-0.7; 10.9) respectively; at the end of the same periods 34% of patients had improved their baseline FEV(1) by 10% or more but in around 50% of patients the level fell below the baseline. A large inter-individual variability in changes in pulmonary function after the start of DNase treatment was documented. In addition, the medium-term response to treatment was correlated with early response during the first 3 months. No consistent changes in exacerbation pattern were found during the first year of treatment. The benefits of DNase use in daily practice are limited but apparently can be maintained in the medium term in some patients. A large inter-individual variability in response to DNase treatment has been documented and the benefits are doubtful in around 50% of patients. This observation points to the need to set up a withdrawal trial in these patients, using as an eligibility criterion the early response observed during the first 3 months of treatment.
Clinical Microbiology and Infection, 2005
Pseudomonas aeruginosa colonisation has a negative effect on pulmonary function in cystic fibrosi... more Pseudomonas aeruginosa colonisation has a negative effect on pulmonary function in cystic fibrosis patients. The organism can only be eradicated in the early stage of colonisation, while reduction of bacterial density is desirable during chronic colonisation or exacerbations. Monthly, or at least 3-monthly, microbiological culture is advisable for patients without previous evidence of P. aeruginosa colonisation. Cultures should be performed at least every 2-3 months in patients with well-established colonisation, and always during exacerbations or hospitalisations. Treatment of patients following the first isolation of P. aeruginosa, but with no clinical signs of colonisation, should be with oral ciprofloxacin (15-20 mg/kg twice-daily for 3-4 weeks) plus inhaled tobramycin or colistin (intravenous treatment with or without inhaled treatment can be used as an alternative), while patients with acute infection should be treated for 14-21 days with high doses of two intravenous antimicrobial agents, with or without an inhaled treatment during or at the end of the intravenous treatment. Maintenance treatment after development of chronic P. aeruginosa infection/colonisation (pathogenic colonisation) in stable patients (aged&amp;amp;amp;amp;amp;amp;amp;amp;amp;gt;6 years) should be with inhaled tobramycin (300 mg twice-daily) in 28-day cycles (on-off) or, as an alternative, colistin (1-3 million units twice-daily). Colistin is also a possible choice for patients aged&amp;amp;amp;amp;amp;amp;amp;amp;amp;lt;6 years. Treatment can be completed with oral ciprofloxacin (3-4 weeks every 3-4 months) for patients with mild pulmonary symptoms, or intravenously (every 3-4 months) for those with severe symptoms or isolates with ciprofloxacin resistance. Moderate and serious exacerbations can be treated with intravenous ceftazidime (50-70 mg/kg three-times-daily) or cefepime (50 mg/kg three-times-daily) plus tobramycin (5-10 mg/kg every 24 h) or amikacin (20-30 mg/kg every 24 h) for 2-3 weeks. Oral ciprofloxacin is recommended for patients with mild pulmonary disease. If multiresistant P. aeruginosa is isolated, antimicrobial agents that retain activity are recommended and epidemiological control measures should be established.
BioFactors, 1998
Antioxidant depletion and increased free radical production by inflammatory cells have been descr... more Antioxidant depletion and increased free radical production by inflammatory cells have been described in cystic fibrosis (CF) patients. To evaluate oxidative damage intensity, we measured plasma concentrations of malondialdehyde, hydroperoxides and protein carbon groups as markers of oxidative injury to lipids and proteins in a group of 101 CF patients free of acute exacerbation, and in 43-112 controls. Moreover, we estimated antioxidant function by measuring activities of erythrocyte superoxide dismutase, glutathione reductase and vitamin E concentrations. In CF patients, malondialdehyde and hydroperoxide plasma levels were significantly higher than in controls (p &amp;lt; 0.001). Increased lipid peroxidation was documented by these two markers. Parallel rises in protein carbonyls in plasma of CF patients were observed (p &amp;lt; 0.0001). These patients presented biochemical but not clinical vitamin E deficiency. Glutathione reductase and superoxide dismutase activities were significantly higher than in controls. These results show a serious imbalance of CF patients between oxidant-antioxidant status leading to oxidative stress.
Archivos de Bronconeumología, 2005
Page 1. Introducción La fibrosis quística (FQ) es una enfermedad genética autosómica recesiva. Se... more Page 1. Introducción La fibrosis quística (FQ) es una enfermedad genética autosómica recesiva. Se produce como consecuencia de la alteración del gen CFTR (cystic fibrosis transmem-brane conductance regulator), situado en el brazo largo del cromosoma 71. ...
Anales de Pediatría, 2004
Evaluar la eficacia y tolerancia del deflazacort frente a la prednisolona en el tratamiento de la... more Evaluar la eficacia y tolerancia del deflazacort frente a la prednisolona en el tratamiento de la agudización moderada de asma en niños. Pacientes y métodos Estudio de intervención, multicéntrico, prospectivo, abierto, aleatorizado, grupos paralelos en niños de 6 a 14 años diagnosticados de asma, en situación de agudización moderada tratados todos con agonistas  2-adrenérgicos de corta acción. Los grupos de intervención recibieron deflazacort (1,5 mg/kg) o prednisolona (1 mg/kg) durante 7 días. La medida principal de eficacia fue el volumen espiratorio forzado en el primer segundo (FEV 1) y como medidas secundarias se evaluaron la escala clínica de gravedad, el flujo espiratorio máximo (PEF), el índice de hospitalización y la utilización de medicación  2-agonista de rescate. Todos los sujetos fueron controlados al inicio del tratamiento (visita 1), al segundo día (visita 2) y al séptimo día (visita 3) del estudio. Resultados Se incluyeron en el estudio 54 pacientes, de los cuales dos requirieron hospitalización (uno de cada grupo). Los valores iniciales fueron similares para ambos grupos: FEV 1 , 53 y 51 %; test de broncodilatación, ؉19 y ؉21 %; PEF, 169 y 165 l/min; escala de gravedad, 6,1 y 6,5 para los grupos deflazacort y prednisolona, respectivamente. En la visita 2, todos los parámetros mostraron mejoría: FEV 1 , ؉22,2 y ؉26,5 % (p < 0,05); PEF, ؉64 y ؉49 l/min (p < 0,05); escala de gravedad-4,4 y-3,8 (p < 0,05), sin diferencias significativas entre ambos grupos. En la visita 3 todos los parámetros continuaron mejorando: FEV 1 , ؉33,2 y ؉32,5 % (p < 0,05); PEF, ؉115,7 y ؉87,6 l/min (p < 0,05); escala de gravedad-5,4 y-5,9 (p < 0,05), también sin diferencias significativas entre los dos grupos. No se registraron efectos adversos en ningún paciente. Conclusiones En el tratamiento de la agudización moderada de asma en niños, deflazacort tiene una eficacia similar a prednisolona como se refleja tanto en la mejoría clínica de los pacientes como en la función pulmonar.