[Guidelines for the follow up of patients with bronchopulmonary dysplasia]. / Protocolo de seguimiento de los pacientes con displasia broncopulmonar.

Bronchopulmonary dysplasia (BPD) is the most common complication of preterm birth, and remains a major problem in pediatric pulmonology units. The decision of discharging from the Neonatal Unit should be based on a thorough assessment of the condition of the patient and compliance with certain requirements, including respiratory and nutritional stability, and caregiver education on disease management. For proper control of the disease, a schedule of visits and complementary tests should be established prior to discharge, and guidelines for prevention of exacerbations and appropriate treatment should be applied. In this paper, the Working Group in Perinatal Respiratory Diseases of the Spanish Society of Pediatric Pulmonology proposes a protocol to serve as a reference for the follow up of patients with BPD among different centers and health care settings. Key factors to consider when planning discharge from the Neonatal Unit and during follow up are reviewed. Recommendations on treatment and prevention of complications are then discussed. The final section of this guide aims to provide a specific schedule for follow-up and diagnostic interventions to be performed in patients with BPD.

[Body plethysmography (I): Standardisation and quality criteria]. / Pletismografía corporal (I): estandarización y criterios de calidad.

Whole body plethysmography is used to measure lung volumes, capacities and resistances. It is a well standardised technique, and although it is widely used in paediatric chest diseases units, it requires specific equipment, specialist staff, and some cooperation by the patient. Plethysmography uses Boyle's law in order to measure the intrathoracic gas volume or functional residual capacity, and once this is determined, the residual volume and total lung capacity is extrapolated. The measurement of total lung capacity is necessary for the diagnosis of restrictive diseases. Airway resistance is a measurement of obstruction, with the total resistance being able to be measured, which includes chest wall, lung tissue and airway resistance, as well as the specific airway resistance, which is a more stable parameter that is determined by multiplying the measured values of airway resistance and functional residual capacity. The complexity of this technique, the reference equations, the differences in the equipment and their variability, and the conditions in which it is performed, has led to the need for its standardisation. Throughout this article, the practical aspects of plethysmography are analysed, specifying recommendations for performing it, its systematic calibration and the calculations that must be made, as well as the interpretation of the results obtained. The aim of this article is to provide a better understanding of the principles of whole body plethysmography with the aim of optimising the interpretation of the results, leading to improved management of the patient, as well as a consensus among the speciality.

[Measurement of CO diffusion capacity (II): Standardization and quality criteria]. / Medición de la difusión de CO (II): estandarización y criterios de calidad.

The diffusion capacity is the technique that measures the ability of the respiratory system for gas exchange, thus allowing a diagnosis of the malfunction of the alveolar-capillary unit. The most important parameter to assess is the CO diffusion capacity (DLCO). New methods are currently being used to measure the diffusion using nitric oxide (NO). There are other methods for measuring diffusion, although in this article the single breath technique is mainly referred to, as it is the most widely used and best standardized. Its complexity, its reference equations, differences in equipment, inter-patient variability and conditions in which the DLCO is performed, lead to a wide inter-laboratory variability, although its standardization makes this a more reliable and reproductive method. The practical aspects of the technique are analyzed, by specifying the recommendations to carry out a suitable procedure, the calibration routine, calculations and adjustments. Clinical applications are also discussed. An increase in the transfer of CO occurs in diseases in which there is an increased volume of blood in the pulmonary capillaries, such as in the polycythemia and pulmonary hemorrhage. There is a decrease in DLCO in patients with alveolar volume reduction or diffusion defects, either by altered alveolar-capillary membrane (interstitial diseases) or decreased volume of blood in the pulmonary capillaries (pulmonary embolism or primary pulmonary hypertension). Other causes of decreased or increased DLCO are also highlighted.

[Respiratory treatments in neuromuscular disease]. / Tratamientos respiratorios en la enfermedad neuromuscular.

In a previous article, a review was presented of the respiratory pathophysiology of the patient with neuromuscular disease, as well as their clinical evaluation and the major complications causing pulmonary deterioration. This article presents the respiratory treatments required to preserve lung function in neuromuscular disease as long as possible, as well as in special situations (respiratory infections, spinal curvature surgery, etc.). Special emphasis is made on the use of non-invasive ventilation, which is changing the natural history of many of these diseases. The increase in survival and life expectancy of these children means that they can continue their clinical care in adult units. The transition from pediatric care must be an active, timely and progressive process. It may be slightly stressful for the patient before the adaptation to this new environment, with multidisciplinary care always being maintained.

[Domiciliary mechanical ventilation in children: a Spanish multicentre study]. / Ventilación mecánica domiciliaria en niños: estudio multicéntrico español.

INTRODUCTION: Domiciliary mechanical ventilation (DMV) use is increasing in children. Few studies have analysed the characteristics of patients using this technique. MATERIALS AND METHODS: An observational, descriptive, transversal, multicentre study was conducted on patients between 1 month and 16 years of age dependent on domiciliary mechanical ventilation. RESULTS: A total of 163 patients with a median age of 7.6 years from 17 Spanish hospitals were studied. The main reasons for DMV were neuromuscular disorders. The median age at beginning of DMV was 4.6 years. Almost three-quarters (71.3%) received non-invasive ventilation. Patients depending on invasive ventilation were younger, started DMV at an earlier age, and had more hours of mechanical ventilation per day. The large majority (80.9%) used DMV during sleep time only, and 11.7% during the whole day. Only 3.4% of patients had external health assistance. Just under half (48.2%) were being followed up in specific DMV or multidisciplinary clinics. Almost three-quarters (72.1%) of patients attended school (42.3% with adapted schooling). Only 47.8% of school patients had specific caregivers in their schools. CONCLUSIONS: DMV in children is used in a very heterogeneous group of patients, and in an important number of patients it is started before the third year of life. Despite there being a significant proportion of patients with a high dependency on DMV, few families receive specific support at home or at school, and health care surveillance is variable and poorly coordinated.

[Comparison between conventional ph measurement and multichannel intraluminal esophageal impedance in children with respiratory disorders]. / Comparación entre pH-metría convencional e impedanciometría intraluminal multicanal en niños con patología respiratoria.

INTRODUCTION: Gastroesophageal reflux (GER) is associated with respiratory symptoms. The link between non-acid GER and the pathogenesis of respiratory disease has been demonstrated. Esophageal multichannel intraluminal impedance (MII) is able to detect non-acid and alkaline GER, as well as reflux height. The objective of the study was to compare the diagnostic effectiveness of dual-channel pH-meter and MII. PATIENTS AND METHODS: A prospective study was conducted on patients diagnosed with uncontrolled asthma, persistent cough, or chronic laryngitis. Patients were monitored continuously for 24 hours using a combination of MII and a dual-channel pH-meter. A descriptive and comparative analysis of the techniques was performed using the t test for comparison between groups and McNemar test for non-parametric data. RESULTS: A total of 49 patients with respiratory disease between September 2008 and April 2010 (79.6% uncontrolled asthma, 10.2% persistent cough, and 10.2% chronic laryngitis) were included in the study. The mean number of refluxes detected was 18.3 (range 0-93) using the pH-meter and 39.2 (11-119) using MII (P<.001). Acid GER was detected using pH in 7 children and using MII in 25 children (8 acid, 10 alkaline and 7 mixed). A mean of 21 proximal refluxes were detected using MII. CONCLUSIONS: MII makes it possible to diagnose a greater number of refluxes, whether acid or alkaline, than conventional pH measurement in children with respiratory disease that is poorly controlled with their usual treatment. MII can also detect proximal refluxes.

[Video-polysomnography in preterm infants with birth weight under 1,800 g]. / Estudio vídeo-polisomnográfico en prematuros menores de 1.800g.

INTRODUCTION: Neonatal video-polysomnography (VPSG) in preterm infants is indicated to rule out sleep-related respiratory disturbances before discharge from neonatal units and to study neonatal sleep patterns and associated pathologic conditions. OBJECTIVE: To study the presence of apnea, hypopnea, and periodic breathing in preterm infants with low birth weight (< 1,800 g) and to describe their cardio-respiratory variability, electroencephalogram (EEG) abnormalities, and brain maturity in connection with sleep stages. METHOD: We performed a cross-sectional, descriptive study through review of the clinical histories of preterm infants with a birth weight < 1,800 g who had undergone VPSG (2001-2003). The most common indication for VPSG was the presence of sleep apneas and oxygen desaturation. The data obtained were used for descriptive statistical analysis. RESULTS: Thirty-one infants were studied, with a mean gestational age of 27.8 weeks and a mean birth weight of 1,158 g. The most frequently associated condition was hyalin membrane disease (68 %). Cardiorespiratory impairment was detected in 65 %, altered sleep pattern in 6 %, immature EEG recording in 32 %, abnormal pattern in 13 %, and immature plus abnormal pattern in 3 %. Sixty-nine percent of the infants required home monitoring and 23 % needed oxygen at home. Four infants were readmitted on several occasions due to apnea. CONCLUSION: We wish to stress the importance of carrying out VPSG with suitable methodology in preterm infants with cardiorespiratory or neurological impairment in order to detect abnormalities on EEG and to assess brain maturity and sleep-related abnormal respiratory events.

[Complementary therapies in cystic fibrosis: evidence of therapeutic benefits and treatment recommendations]. / Tratamientos complementarios en fibrosis quística: evidencia de su beneficio terapéutico y recomendaciones sobre su uso.

Cystic fibrosis (CF) is an autosomal-recessive disorder that predominantly affects the respiratory system. When this disease was described in 1938 the mortality rate was approximately 70 % in the first year of life. Survival has dramatically increased from a median of approximately 4 years in the 1960s to 19 years in the 1970s and 33 years in 2001 according to figures from the American Cystic Fibrosis Foundation. This impressive increase in the life expectancy of individuals with CF is undoubtedly related to recent advances in the organization of specialized CF units and to the use of new therapies against respiratory involvement.The traditional basis of treatment for CF lung disease includes nutritional support, antibiotic therapy, chest physical therapy and aerobic exercise. Preventive measures such as influenza vaccination and avoidance of tobacco smoke are also useful. Several new approaches such as ion transport therapy, protein therapy and gene therapy are currently being developed. Many studies have provided clear evidence of the therapeutic benefits of antibiotics, respiratory physiotherapy, exercise, and nutrition. In this article we review the scientific evidence on the advantages of the use of several therapeutic interventions against inflammation, increased sputum viscoelasticity and adhesiveness, and bronchial obstruction in CF patients.

[Management of parapneumonic pleural effusions]. / Tratamiento de los derrames pleurales paraneumónicos.

Pleural effusion in children is most often due to bacterial pneumonia. Between 0.6 and 2% of pneumonias are complicated by empyema and approximately 40% of children hospitalized with pneumonia have a pleural effusion. In recent years Streptococcus pneumoniae is the most prevalent organism. Treatment is based on the early and judicious use of antibiotics, imaging techniques, thoracocentesis, pleural drainage, fibrinolytics, thoracoscopy and thoracotomy. Indications for early pleural drainage are gross pus, positive Gram stain in pleural fluid, pleural glucose less than 50mg/dL, pleural fluid pH of less than 7 and sonographic evidence of loculations. Local fibrinolytics may decrease the need for surgical treatment, with a success rate between 38 and 100%, according to the effusion stage. Thoracoscopic debridement is useful in the fibrinopurulent stage with loculations, with favorable results in 30-100% of patients, also depending on the effusion stage.

[Reproducibility of the walking test in patients with cystic fibrosis]. / Reproductibilidad del test de la marcha (walking test) en pacientes afectos de fibrosis quística.

OBJECTIVE: The walking test is a useful and objective method for evaluating the tolerance for exercise in patients with chronic bronchopulmonary diseases. Our objective was to check the reproducibility of this test and evaluate whether there are differences between tests of varying duration (2 and 6 minutes) in a group of patients with cystic fibrosis. PATIENTS AND METHODS: We utilized the walking test on 29 patients who were in a stable phase and under care in the Cystic Fibrosis Unit of our hospital. Two tests were carried out, one of 2 minutes and the other of 6 minutes duration, both of which were repeated after a 15-minute interval. RESULTS: The reproducibility of the walking test in this type of patient was very good and we found an excellent correlation between the two-minute test and the six-minute test. We did not observe a training effect when the test was repeated. CONCLUSIONS: The two minute walking test has a high reproducibility and we propose this test, because it is shorter and more comfortable for pediatric patients with cystic fibrosis, in order to evaluate the evolution, progressive deterioration of the of the patient and the response to different types of treatments.

[Assessment of lung function using forced impulse oscillometry in cystic fibrosis patients]. / Valoración de la función pulmonar por medio de la oscilometría forzada de impulsos en pacientes con fibrosis quística.

The aim of this study was to evaluate the usefulness of forced impulse oscillometry to measure airway resistance in patients with cystic fibrosis. Thirty-four patients (20 men) with a mean age of 15 +/- 4 years were studied. All patients underwent forced impulse oscillometry, forced spirometry and body plethysmography. Correlations among spirometric, plethysmographic and oscillometric variables were analyzed. We found a statistically significant relation between both forced expiratory volume in one second (FEV1) and total airway resistance (Raw) and the following oscillometric variables: impedance (Zrs), resonance frequency (Fres), resistance to 5 hertz (Rrs5) and reactance to 5 hertz (Xrs5). The measurements that correlated most highly with classical pulmonary function tests were Zrs and Xrs5. Both resistance (Rrs) and reactance (Xrs) of the respiratory system were dependent on frequency. Their correlation with FEV1 and Raw were therefore lower when frequencies above 5 hertz were used. We conclude that airway resistances of cystic fibrosis patients can be adequately estimated by forced impulse oscillometry. This technique is a promising test of pulmonary function in such patients.

[Spontaneous chylothorax: seven cases of prenatal diagnosis]. / Quilotórax espontáneo: siete casos de diagnóstico prenatal.

Authors present 7 cases of spontaneous congenital chylothorax, all of them diagnosed antepartum echographycally. Six of them needed cardiopulmonary resucitation but none presented fetal anoxia. Three cases had associated malformations. Treatment included thoracocenthesis, pleural drainage, assisted ventilation and appropriate nutrition. Pleural effusion lasted from 14 days to 6 months. Complications were: pneumothorax, metabolic acidosis and bacterial infections. Three patients died by respiratory failure. Pulmonary hypoplasia was present in two of them, and suspected in the third one. Survivors are free of symptoms.