Valor del óxido nítrico nasal en el diagnóstico de la discinesia ciliar primaria / Value of nasal nitric oxide in the diagnosis of primary ciliary dyskinesia

Objetivo: Comunicar los valores de óxido nítrico nasal (ONn) en niños con discinesia ciliar primaria (DCP) comparados con los niveles de ONn en niños sanos y en niños afectos de asma, fibrosis quística (FQ) y bronquiectasias pos infecciosas. Pacientes y métodos: Se realizó la determinación de ONn en 9 niños con DCP, 36 niños asmáticos, 31 niños con FQ, 8 niños con bronquiectasias post infecciosas y 37 niños sanos. Se compararon los valores de ONn en las diferentes patologías y se determinó la sensibilidad y la especificidad de la prueba para el diagnóstico de DCP. Resultados: Todos los niños con DCP excepto uno (ONn 348 ppb) mostraron un valor de ONn inferior a 112 ppb, siendo la media de 88 ppb (IC95% 9,6–166). En los niños sanos, la media del ONn fue de 898 ppb (IC95% 801–995), en los asmáticos 1023 ppb (IC95% 911–1137), en los niños con FQ 438 ppb (IC95% 367–508) y en los pacientes con bronquiectasias pos infecciosas de 361 ppb (IC95% 252–470). El valor medio de ONn fue significativamente inferior (p<0,05) en los niños afectos de DCP respecto a todos los demás grupos. Un punto de corte de NOn ≤112 ppb mostró una sensibilidad del 88,9% y una especificidad del 99,1% para el diagnóstico de DCP [área bajo la curva ROC 0,98 (IC95% 0,94–0,99); p<0,0001; razón de probabilidad 95,1]. Conclusiones: Un valor de ONn ≤ 112 ppb en niños es altamente sugestivo de DCP aunque un valor superior no descarta por completo la enfermedad (AU)

Objective: The aim of this study is to report nasal nitric oxide (nNO) values in children with primary ciliary dyskinesia (PCD) and to compare them with nNO values in healthy children, asthmatic children, children with cystic fibrosis and children with post infectious bronchiectasis. Patients and methods: We determined nNO values in 9 children with PCD, 36 asthmatic children, 31 children with cystic fibrosis, 8 children with post infectious bronchiectasis and 37 healthy children. We compared nNO values between these different conditions and calculated sensitivity and specificity of nNO to diagnose PCD. Results: All children with PCD - except one (nNO 348 ppb) – had nNO values below 112 ppb, mean 88 ppb (95%CI 9.6–166). The nNO mean was 898 ppb (95%CI 801-995) in healthy children, 1023 ppb (95%CI 911–1137) in asthmatic children, 438 ppb (95%CI 367–508) in cystic fibrosis children and 361 ppb (95%CI 252–470) in children with post infectious bronchiectasis. The mean concentration of nNO was lower (P<0.05) in PCD patients, compared to the other groups. The measurement of nasal NO in our study population showed, at a cut-off level of ≤112 ppb, a sensitivity of 88.9% and a specificity of 99.1% in the diagnosis of PCD [ROC 0.98 (95%CI 0.94–0.99); P<0.0001; probability ratio 95.1]. Conclusions: The measurement of nasal NO appears to be a useful tool for screening children for PCD, in which a cut-off level of ≤112 ppb suggests the disease, although nNO above 112 ppb does not exclude PCD (AU)

[Value of nasal nitric oxide in the diagnosis of primary ciliary dyskinesia]. / Valor del óxido nítrico nasal en el diagnóstico de la discinesia ciliar primaria.

OBJECTIVE: The aim of this study is to report nasal nitric oxide (nNO) values in children with primary ciliary dyskinesia (PCD) and to compare them with nNO values in healthy children, asthmatic children, children with cystic fibrosis and children with post infectious bronchiectasis. PATIENTS AND METHODS: We determined nNO values in 9 children with PCD, 36 asthmatic children, 31 children with cystic fibrosis, 8 children with post infectious bronchiectasis and 37 healthy children. We compared nNO values between these different conditions and calculated sensitivity and specificity of nNO to diagnose PCD. RESULTS: All children with PCD - except one (nNO 348 ppb) - had nNO values below 112 ppb, mean 88 ppb (95%CI 9.6-166). The nNO mean was 898 ppb (95%CI 801-995) in healthy children, 1023 ppb (95%CI 911-1137) in asthmatic children, 438 ppb (95%CI 367-508) in cystic fibrosis children and 361 ppb (95%CI 252-470) in children with post infectious bronchiectasis. The mean concentration of nNO was lower (P<0.05) in PCD patients, compared to the other groups. The measurement of nasal NO in our study population showed, at a cut-off level of < or =112 ppb, a sensitivity of 88.9% and a specificity of 99.1% in the diagnosis of PCD [ROC 0.98 (95%CI 0.94-0.99); P<0.0001; probability ratio 95.1]. CONCLUSIONS: The measurement of nasal NO appears to be a useful tool for screening children for PCD, in which a cut-off level of < or =112 ppb suggests the disease, although nNO above 112 ppb does not exclude PCD.

Guía de diagnóstico y tratamiento del asma de control difícil en el niño / Diagnosis and treatment guidelines for difficult-to-control asthma in children

Los niños con asma de control difícil (ACD) requieren frecuentes consultas, reciben complejos regímenes de tratamiento y, a menudo, requieren ingresos en el hospital. Su frecuencia es escasa, y abarca no más del 5% de la población asmática. El ACD requiere un diagnóstico de certeza, por lo que se tendrán que descartar causas de falso ACD, y es necesario hacer un diagnóstico diferencial con factores de enfermedad sobreañadida, medioambientales, psicológicos, y analizar causas que determinen una baja adherencia al tratamiento. Ante un verdadero ACD, el estudio de la inflamación (óxido nítrico exhalado, esputo inducido, lavado broncoalveolar y biopsia bronquial), la función pulmonar y la clínica nos pueden permitir clasificar el ACD en diversos fenotipos que nos facilitarán la toma de decisiones terapéuticas (AU)

Children suffering from difficult-to-control asthma (DCA) require frequent appointments with their physician, complex treatment regimes and often admissions to hospital. Less than 5% of the asthmatic population suffer this condition. DCA must be correctly characterised to rule out false causes of DCA and requires making a differential diagnosis from pathologies that mimic asthma, comorbidity, environmental and psychological factors, and analysing the factors to determine poor treatment compliance. In true DCA cases, inflammation studies (exhaled nitric oxide, induced sputum, broncho-alveolar lavage and bronchial biopsy), pulmonary function and other clinical aspects can classify DCA into different phenotypes which could make therapeutic decision-making easier (AU)

[Diagnosis and treatment guidelines for difficult-to-control asthma in children]. / Guía de diagnóstico y tratamiento del asma de control difícil en el niño.

Children suffering from difficult-to-control asthma (DCA) require frequent appointments with their physician, complex treatment regimes and often admissions to hospital. Less than 5% of the asthmatic population suffer this condition. DCA must be correctly characterised to rule out false causes of DCA and requires making a differential diagnosis from pathologies that mimic asthma, comorbidity, environmental and psychological factors, and analysing the factors to determine poor treatment compliance. In true DCA cases, inflammation studies (exhaled nitric oxide, induced sputum, broncho-alveolar lavage and bronchial biopsy), pulmonary function and other clinical aspects can classify DCA into different phenotypes which could make therapeutic decision-making easier.

Mesa redonda. Asma de difícil control: Introducción / No disponible

No disponible

Complicaciones pulmonares relacionadas con la inmunosupresión en los niños trasplantados de órganos sólidos / No disponible

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[Exhaled and nasal nitric oxide in normal and asthmatic children]. / Oxido nítrico exhalado y nasal en niños normales y asmáticos.

OBJECTIVE: Our aim was to study the concentration of nitric oxide in the exhaled (ENO) and nasal (NNO) air of normal children and asthmatic children who are clinically and functionally stable. PATIENTS AND METHODS: Using a nitric oxide chemiluminescence analyze and a register for CO2, pressure and flow, we studied 73 schoolchildren (6-17 years of age). This included 37 controls and 36 asthmatic children, 21 with mild asthma without antiinflammatory treatment and 15 treated with inhaled corticosteroids. We used the technique of slow exhalation against resistance for (ENO) determination and aspiration with stable flow in nasal cavity while holding the breath for (NNO) determination. RESULTS: The mean ENO was 3.1 ppb (1-6) in the control group, 8.3 ppb (1.7-29.3) in the mild asthma group and 7.7 ppb (2-18.3) in the asthmatics treated with corticosteroids. There were significant differences (p = 0.0001) between the controls and both asthmatic groups. The mean NNO in the controls was 898 ppb and differences between this group and the asthmatic children were found. The ENO and NNO did not change in relation to age or sex. We did not find any relationship between ENO and lung function. There is a significant correlation between ENO and NNO in both asthmatic groups, but not in the control group. CONCLUSIONS: The ENO was higher in asthmatics than in control children. The slow exhalation against resistance technique prevents the contamination of exhaled air with nasal air and this technique can be applied to children over 6 years of age. The NNO was similar in the asthmatic groups and the control group.

[Assessment of 2 portable peak expiratory flow meters and reference values for students 6 to 16 years of age]. / Evaluación de dos medidores portátiles de flujo espiratorio máximo y valores de referencia para escolares de 6 a 16 años.

OBJECTIVE: This study examines the technical characteristics of two different peak expiratory flow meters, of high range, and the reference values of peak expiratory flow (PEF) for schoolchildren. PATIENTS AND METHODS: The gauge accuracy and precision were previously determined in 20 units of each model (PF-Control and Mini-Wright), with a syringe servocontrolled by simulating 4 predetermined PEF fluxes (125, 262, 424 and 587 L/min). Relatives were asked about passive smoking and the childhood background concerning asthma, recurrent bronchitis or recent respiratory infection. The PEF of 1,142 schoolchildren, 669 boys and 473 girls between 6 and 16 years of age and coming from 6 different locations of different demographic and social characteristic of Catalonia, Spain, were measured. RESULTS: Readings of both gauges differed in accuracy, although they presented a good intradevice precision. The PF-control is within the reliance intervals for fluxes of 425 and 587 L/min, with a suprareading of 15.3% for the 262 L/min and infrareading of 19.2% for the 125 L/min controls. Flux with the Mini-Wright shows systematic over-reading of between 17.9% and 30.2%, with an accurate reading only in the 587 L/min control flux. No significant correlation was found between the PEF and family passive smoking (56.3%), pupils with asthma background (7.1%), recurrent bronchitis (11%) or recent respiratory infection (7.7%). CONCLUSIONS: The accuracy difference forces the use of diverse percentile tables for each of the PEF gauge patterns; hence, we present the reference tables for each gauge, in means of 10, 50 and 90 percentiles, which can be used as reference values for our school population according to their age, size and sex.

[Cystic fibrosis: a microbiological study over an 8-year period]. / Fibrosis quística: estudio microbiológico durante un período de 8 años.

OBJECTIVE: To study the microbiology of cystic fibrosis in our hospital for the period from 1985 to 1992. MATERIAL AND METHODS: The number of samples analyzed totalled 1,034, most of which were sputum and nasopharyngeal aspirates belonging to 113 patients (49 women and 64 men). The average age was 10 years (range: 15 days-33 years). RESULTS AND DISCUSSION: Only 1.7% of the samples were negative. Normal flora were found in 10.8% and one or more potentially pathogenic microorganisms were found in the remaining 87.4%. Colonies were over 10(6) UFC/ml in size in 77.8% of the quantified cultures. The most frequently identified microorganisms in the population overall were P. aeruginosa (53.9%), S. aureus (30.3%) and H. influenzae (22.0%). In patients less than 12 months old, however, the most common isolations were of S. pneumoniae and B. catarrhalis; cultures from patients older than 16 years old most often yielded filiform fungi, mainly Aspergillus spp. We found no strains of Legionella spp. and P. cepacia was found in only 3 cases, in which the clinical outcome was good. In addition to the 3 most common organisms, we recorded several consecutive isolations of Proteus mirabilis, Xanthomonas maltophilia and Serratia marcescens in patients older than 11 years old; this finding suggests that given the improved survival of cystic fibrosis patients over the coming years and the antibiotic pressure placed on them, there may be slight changes in the bacterial ecology typical of this disease. No strain of S. aureus proved resistant to methicillin, but P. aeruginosa was shown to be resistant to gentamycin (58.2%) among the aminoglycosides and also to some of the beta-lactams considered to be effective, as follows: 25.2% to piperacillin, 22.6% to ceftazidime and even 19.8% to aztreonam. There was slight resistance of ciprofloxacin (6.3%).

[Adenomatous cystic pulmonary malformations: presentation of 26 cases]. / Malformación adenomatoidea quística pulmonar. Presentación de 26 casos.

We present our experience of 26 cases of cystic adenomatoid malformations of the lung (CAM), treated in the Department of Pediatric Surgery in our hospital between 1967-1991. There were two clinical pictures: one neonatal severe respiratory distress and successive repeated pulmonary infection appearing after the patient's first year of life and requiring both urgent diagnosis and treatment. Embryological development determines the pathologic classification of this entity in 3 types. Basic examinations by image are analyzed, bearing in mind their diagnostic value and the patterns they show. After analysing all the conditioning factors, no explanation has been found to the different course that this affectation (< 1 month and > 1 year of age respectively). Normally, neonatal mortality is closely related to other malformations, particularly to cardiovascular ones. Differential diagnosis is very important in the neonatal period, especially with regard to diaphragmatic hernia, lobar emphysema and pulmonary cysts. All these cases have been verified and classified by means of a pathologic study, which has shown the need for surgical operation. In the follow up of the patients no alteration has been noticed in the pulmonary function.

[Congenital lobar emphysema: report of 38 cases]. / Enfisema lobar congénito. Presentación de 38 casos.

We present our experience of 38 patients suffering from congenital lobar emphysema (CLE) treated in the Department of Pediatric Surgery in our hospital between 1966-1991. 22 of these patients had to undergo surgical correction due to the severity of their respiratory symptoms. The other 16 presented mild respiratory symptoms: 8 of them received conservative treatment and the remainin 8 had to be operated on due to broncial compression caused by the vascular malformation. In most cases the main symptoms were dyspnea and cyanosis, which indicate the severity of the process. 20 cases presented during the patient's first month of life, 10 within the first 6 months and the remaining 8 between the first 6 months and 5 years. The different aetiologics forms in our study are analysed and compared with those described in the literature. The main diagnostic tested and revied, specially radiology tested led to a correct differential diagnosis and subsequently to an adequate therapeutic treatment. It is possible to carry out other tests since they do not actually help diagnosis, we have considered them unnecessary. All the surgical operations used are analysed as well as the complications observed. The clinical course of the patients has proved to be particularly good, since there was no mortality and the morbility rate was low.

[Bronchiectasis in early childhood. Its surgical treatment]. / Bronquiectasias en la primera infancia. Tratamiento quirúrgico.

We discuss the measures used, including physical examination, radiology, scintigraphy, T.A.C. and bronchography, to diagnoses 34 patients with bronchiectasis. In addition, the treatment used up until the time where the decision to perform surgery is also presented. Ten patients were submitted to surgical intervention and their operations, as well as the effected lobes, are described. The guidelines for surgical preparation, surgical difficulties encountered, postoperative care and the results of the surgeries are all discussed. Due to the age of some of these patients, obvious difficulties prevented functional testing. In those patients in whom it was possible, no loss in respiratory capacity has been observed.

[Pulmonary sequestration. Apropos of 10 cases]. / Secuestro pulmonar. A propósito de diez casos.

Pulmonary sequestration is a congenital anomaly in which an aberrant systemic artery arising from the thoracic or abdominal aorta supplies part of the lung, usually the lower lobes. The sequestrates part of the lung may be anatomically included in the substance of the lobe (intralobar) or may be separate from the other lobes, being contained within its own pleural investment (extralobar). The cases of ten patients with sequestration of the lung seen during a seven-year period are reviewed, with special consideration of their clinical and radiographic findings. Emphasis is made on the differences of our cases with those of the literature: 1) Eighty per cent of the patients had previous symptoms; 2) The intra/extralobar ratio was 1.5/1; 3) All intralobar sequestrations were indifferently localized in left or right lower lobes, and 4) Associated anomalies were present with the same incidence in the two classic forms.

[Bird breeder's lung in children]. / Pulmón del criador de pájaros en la infancia.

A case of hypersensitivity pneumonitis due to pigeon-dropping antigens is reported in a 9 year old girl, and compared with other seven previous reports in children. The insidious nature of this disease, as well as the importance of detailed environmental information in children with unexplained respiratory disease are emphasized. In this case, lung function tests showed a classic restrictive ventilatory defect, and a serious obstructive ventilatory defect evidenced in the narrowing of the smaller airways, and a reduction in the forced expiratory flow at small lung volumes. Hypersensitivity pneumonitis is a lung disease that results from sensitization by inhalation of a variety of organic dusts. Patients usually have circulating antibodies against the etiologic agents. Most patients with this disease also have sensitized T-cells to these agents. Long-term exposure can lead to irreversible lung disease. The histopathologic features are chronic interstitial and alveolar inflammation frequently accompanied by a granulomatous response. The most common symptomatic features are fever, chills and dyspnea 4 to 8 hours after exposure. Antibody activity to antigens is detected in the serum of both symptomatic and asymptomatic breeder's lung. Cellular hypersensitivity to antigens is demonstrated "in vitro" with peripheral lymphocyte populations in almost all symptomatic patients.

[Cystic adenomatoid malformation of the lung (author's transl)]. / Malformación adenomatoidea quística congénita del pulmón.

Four cases of congenital cystic adenomatoid malformation are presented. The age of the patients ranged from 1 day to 7 years. All cases were treated surgically and 3 of them survived. The only death in the group corresponded to the neonatal case. The clinical, radiological and patological features of congenital cystic adenomatoid malformation are also reviewed. Occasionally the clinical manifestations will be hardly noticeable or non existent and the malformation may be diagnosed beyond the neonatal period in the course of a radiological examination performed as routine or because of repeated respiratory infections. Surgery is the treatment of choice. In the group of neonates where the cystic adenomatoid malformation doesn't cause respiratory difficulty the authors feel that it might be advisable to postpone surgery until a later date.