European Respiratory Society
ERS Handbook of Paediatric Respiratory Medicine

The 18 chapters of the ERS Handbook of Paediatric Respiratory Medicine cover the whole spectrum of paediatric respiratory medicine, from anatomy and development to disease, rehabilitation and treatment. The Editors have brought together leading clinicians to produce a thorough and easy-to-read reference tool. The Handbook is structured to accompany the paediatric HERMES syllabus, making it an essential resource for anyone interested in this field and an ideal educational training guide.

  • European Respiratory Society
  1. Page xxvii
  2. Page xxix
    1. Page 1
      Abstract
      • The anatomy of the thorax can be divided into the lungs, heart, mediastinum, pleura, diaphragm and chest wall.

      • The lungs can be further subdivided into the trachea, bronchi, hila, lobes and preacinar and acinar regions.

      • The mediastinum contains the thymus, the heart and its associated structures, thoracic lymphatics, sympathetic and parasympathetic nerves and the oesophagus.

    2. Page 11
      Abstract
      • Distinguishing obstructive and restrictive disorders is simplistic but a helpful starting point.

      • A combination of spirometry and body plethysmography is most useful.

      • Visual inspection of the flow–volume loop, including the inspiratory limb, is essential.

      • Assessment of inflammation is becoming increasingly recognised as an important part of the overall evaluation.

    3. Page 19
      Abstract
      • Innate immune mechanisms comprise a mechanical, physical and chemical barrier, which act together in the defence against invading microorganisms.

      • The airway epithelium forms a physical barrier against inhaled substances and contributes to host defence by producing mediators of the chemical barrier, including chemokines, cytokines, antimicrobial peptides, proteinase inhibitors and surfactant proteins.

      • Adaptive immune mechanisms include T-cell-mediated responses of different subpopulations and components of the humoral and mucosal immune system.

      • Interaction of innate and adaptive immune regulation is required for specific defence against respiratory diseases, involving prenatal and post-natal factors.

    4. Page 29
      Abstract
      • Exposure to ETS, ambient air pollutants and biomass smoke increases the risk of respiratory disease (e.g. asthma and pneumonia) in children.

      • Protective effects of certain exposures, such as farming lifestyle and some microbes, on asthma and allergy have been observed.

      • Genetic susceptibility and co-exposure to several environmental factors contribute to an overall complex relationship between inhalant allergens and disease development.

  3. Page 33
    1. Page 33
      Abstract
      • Patient history is focused on the respiratory system and is adapted to patient circumstances (emergency situation, chief complaint, chronic problem, age, etc.); however, other pertinent organ systems should not be neglected and structure is important in order to avoid missing helpful clues.

      • Respiratory physical examination of the chest includes inspection, palpation, percussion and auscultation. Nomenclature of lung sounds is a subject of considerable confusion.

      • A structured physical examination of the chest, applied with flexibility in paediatric patients, including the upper respiratory system, evaluation of cyanosis, the digits and other pertinent organ systems, is fundamental to the evaluation of the respiratory patient.

    2. Page 44
      Abstract
      • Cough has different major overlapping constructs based on duration, inflammation type, phenotype or clinical syndromes.

      • It is not logical to try to suppress a cough that has a protective role.

      • It is important to try to make a diagnosis and treat the underlying cause of cough. Potential causes in children are different from those in adults.

      • There is little evidence that either nonspecific isolated cough or post-infectious coughing respond to any currently available treatment.

      • There is good evidence that children with protracted (persistent) productive (moist or wet) cough benefit from treatment with antibiotics to cover the organisms associated with protracted bacterial bronchitis.

    3. Page 50
      Abstract
      • Dyspnoea can be caused by respiratory, cardiac, metabolic, neuromuscular or psychogenic conditions.

      • History taking and physical examination are cornerstones of a proper assessment of a child with dyspnoea, tachypnoea or respiratory distress.

      • A clear diagnosis is mandatory for correct treatment.

    4. Page 57
      Abstract
      • Wheeze is a continuous, usually high-pitched whistling sound that is accompanied by prolongation of the expiratory phase; it is believed to originate from oscillation of large airways in response to turbulent airflow in partially blocked intrathoracic airways.

      • Stridor is a musical, monophonic, high-pitched sound that can be heard without a stethoscope, and it is caused by narrowed large, extrathoracic airways; its presence suggests significant obstruction of airflow in the larynx and proximal trachea.

      • Snoring is produced during sleep and is due to obstructed air movement in the naso- and oropharynx; children who snore tend to have more collapsible airways and/or increased size of adenotonsillar tissue.

      • Rattle is created by the movement of excessive secretions during normal airflow in the central and extrathoracic airways; it has a “rattling”, noncontinuous quality, but quite commonly is mislabelled by parents as wheezing.

      • Hoarseness (or dysphonia) is a disorder of phonation and is used to describe a change in the quality of the voice; it is not usually associated with airway obstruction.

    5. Page 65
      Abstract
      • Participating in and mastering physical exercise is extremely important in children and adolescents.

      • Participation in physical activity improves quality of life, fitness and life expectancy in many respiratory disorders.

      • Treatment of childhood asthma should aim at mastering physical activity and exercise-induced asthma.

      • Several chronic respiratory disorders of childhood may influence the ability to participate in physical activity. Assessment of the ability to participate in physical activity and setting up therapeutic procedures to counteract exercise intolerance should be part of the diagnostic assessment of children with respiratory disease.

    1. Page 70
      Abstract
      • Lung volumes that are not affected by air flow are termed static lung volumes and consist of volumes and capacities (sum of specific volumes).

      • The total lung capacity and the functional residual capacity include a volume of gas that cannot be exhaled (residual volume) and which is important for maintaining continuous gas exchange during profound expiration.

      • Lung volumes that are affected by air flow are termed dynamic lung volumes and are measured during forced expiration.

    2. Page 77
      Abstract
      • Respiratory mechanics are helpful in understanding the cyclic changes in airflow due to pressure differences during breathing and the influence of elastic (compliance) and dynamic properties of the respiratory system (resistance).

      • Both compliance and resistance are volume dependent and display influence of age due to growth and development from infancy throughout childhood to adulthood.

    3. Page 83
      Abstract
      • Bronchodilator reversibility demonstrates reversible bronchial obstruction and is a diagnostic marker of active asthma.

      • Bronchial challenge with methacholine/histamine is a sensitive measure of asthma, but is not so specific.

      • Indirect measures of bronchial responsiveness (exercise, inhaled adenosine monophosphate, hypertonic saline and mannitol, and EVH) are specific, but not sensitive, measures of asthma.

      • Indirect measures of bronchial responsiveness (exercise, etc.) respond rapidly (over 1–3 weeks) to inhaled steroids.

      • Direct measures of bronchial responsiveness (methacholine and histamine) respond slowly to inhaled steroids (over 3 months).

      • Direct measures of bronchial responsiveness (methacholine and histamine) are presently the most exact monitoring tool for asthma and reflect airway remodelling.

      • Indirect measures of bronchial responsiveness reflect airway inflammation.

      • BHR in childhood may predict later asthma.

    4. Page 93
      Abstract
      • Acid–base disturbances can be classified using a three step systematic approach: pH, PaCO2, bicarbonate.

      • If pH is abnormal, determine if acidaemia or alkalaemia.

      • If the measured pH and PaCO2 are both abnormal, assess the direction of change; if they change in opposite directions the primary acid–base abnormality is respiratory, otherwise it is metabolic.

      • When an acid–base imbalance is diagnosed look for compensation or mixed disorders.

    5. Page 100
      Abstract
      • At present, FeNO is the only biomarker in exhaled air that has been standardised, developed and validated for clinical application.

      • Increased FeNO is suggestive of eosinophilic airway inflammation and FeNO has a role in the diagnosis and management of asthma after preschool age. Low FeNO is seen in suppurative airways disease, including CF, and low nasal nitric oxide is typical for primary ciliary dyskinesia or sinusitis.

      • Dose titration of inhaled steroids on the basis of induced sputum eosinophilia has been shown to reduce exacerbations in adult asthmatics, but studies in children are few and inconclusive. The methodology of sputum induction in children is demanding and unlikely to become useful in clinical routine.

      • A large number of other potential biomarkers in exhaled air, or in EBC, await further standardisation. Careful evaluation is needed before these can be applied in clinical practice.

    6. Page 107
      Abstract
      • Measuring lung function in infants and preschool children is possible because of standardised techniques that require minimal cooperation from the child.

      • Sedation is usually required in infants and young children up to 2 years of age (generally chloral hydrate 80–100 mg·kg−1, maximum 1 g) for most PFTs, limiting the use of infant PFTs in routine clinical care.

      • In preschool children, the feasibility of the interruptor technique and plethysmographic sRaw, which are performed during tidal breathing, is usually >80%.

      • Spirometry is also feasible in preschool children when appropriate criteria are used.

    7. Page 113
      Abstract
      • IGW is based on washing in and out inert tracer gases to assess the gas mixing efficiency of the lung.

      • The most important outcome parameter of MBW is the LCI, calculated as the cumulative expired volume needed to clear the lungs of the tracer gas divided by the lung size (FRC).

      • IGW seems most sensitive in children with CF for detecting early lung disease; its role in other disease groups is currently less well examined.

      • After early studies demonstrating the feasibility and usefulness of washout measurements in children, commercially available equipment, and new guidelines for standardisation will now enable the implementation of MBW in routine clinical practice.

    8. Page 118
      Abstract
      • The FOT can be used to measure respiratory mechanics with oscillatory signals superimposed over tidal breathing in awake children as young as 2 years of age.

      • Alterations in respiratory mechanics have been evaluated in several commonly encountered paediatric diseases including asthma, CF and BPD; however, further work is required to cement a place for the FOT in the routine clinical management of such diseases.

    9. Page 122
      Abstract
      • Clinicians should enquire whether the child or adolescent snores and, if so, obtain a PSG.

      • PSG is the gold standard for the diagnosis of sleep-disordered breathing in children.

      • If PSG is not available, then clinicians may order alternative diagnostic tests, such as nocturnal video recording, nocturnal oximetry, daytime nap PSG or ambulatory PSG.

      • Paediatric PSG should be performed in a sleep laboratory equipped for children and staffed by qualified personnel following the American Thoracic Society standards for testing.

      • Age-adjusted rules for the scoring and interpretation of PSGs should be used for children.

  4. Page 132
    1. Page 132
      Abstract
      • Cleaning and disinfection of flexible bronchoscopes are a major concern to prevent cross infection and contamination.

      • Stridor is a common indication for flexible bronchoscopy in infants and laryngomalacia is the most frequent observed abnormality. Direct visualisation of the airways is necessary in all children with persistent stridor because lower airway lesions are frequently associated with upper airway lesions.

      • In older children all pathological respiratory situations, especially persistent or recurrent clinical symptoms and/or radiological abnormalities, should lead to endoscopic airway exploration.

      • Measures to prevent complications include: detection of high-risk children; nebulisation of β2-agonists if there is pre-existing bronchial hyperresponsiveness; careful local anaesthesia to prevent laryngospasm; large oxygen supplementation; use of the lowest flexible bronchoscope diameter; appropriate anaesthesia; and training.

      • Post-BAL fever may occur in up to 50% of cases.

    2. Page 140
      Abstract
      • BAL is a procedure used to recover cellular and noncellular components from the alveolar and bronchial airspaces.

      • Clinical applications involve microbiological studies and/or evaluation of cellular components.

      • BAL is performed for diagnostic and therapeutic indications.

    3. Page 146
      Abstract
      • Bronchial brushing is a useful complementary method for assessing cytological changes in the superficial mucosal layer.

      • Endobronchial biopsy allows more detailed evaluation of inflammation and structural damage of the bronchial wall. It also allows direct histological analysis of an endobronchial lesion, e.g. a tumour.

      • TBLB samples lung parenchyma without the need for thoracoscopy or thoracotomy.

    4. Page 151
      Abstract
      • Rigid bronchoscopy is indicated for foreign body retrieval and pre-operative diagnostic workup of subglottic lesions.

      • With the necessary precautions the procedure is quite safe.

      • Teaching the necessary skills to future generations of paediatric bonchoscopists is a challenge.

    5. Page 156
      Abstract
      • Appropriate sedation is important for a well-tolerated bronchoscopic procedure; available techniques include general anaesthesia and moderate sedation.

      • Various protocols may be used during flexible bronchoscopy that involve the administration of a single drug or drug combination (midazolam, meperidine, propofol, ketamine, remifentanil, etc.), or inhalation agents (premixed 50% nitrous oxide and oxygen or sevoflurane).

      • Rigid bronchoscopy should always be performed under general anaesthesia.

      • Whatever the choice of sedation and technique of oxygen delivery (nasal prongs, face mask, laryngeal mask or endotracheal intubation) it is essential to maintain and preserve spontaneous ventilation.

  5. Page 161
    1. Page 161
      Abstract
      • Chest radiography is the backbone of the radiological diagnosis of chest diseases. The use of fluoroscopy is restricted to special clinical indications.

      • The advent of digital imaging and pulsed fluoroscopy significantly improved the imaging quality of chest radiography and allowed a tremendous reduction of radiation dose.

      • Careful attention is necessary for consideration of radiation protection and necessity of imaging (role of routine follow-up examinations).

    2. Page 166
      Abstract
      • Use of chest CT in children requires special expertise of the radiologist to follow the “As Low As Reasonably Achievable” (ALARA) principle.

      • A chest CT investigation requires a well-defined clinical question, detailed patient history, and deliberation with the radiologist prior to the investigation to maximise diagnostic yield and minimise radiation exposure.

      • Careful instruction of the child prior to the investigation is important to reduce anxiety, optimise volume control during the procedure and reduce movement artefacts.

      • Volume control during the chest CT should be considered whenever possible.

    3. Page 176
      Abstract
      • MRI techniques allow for fast and reliable assessment of pulmonary diseases in children.

      • Thoracic MRI is a radiation-free method and can be performed frequently without contrast media application.

      • The diagnostic value of MRI is shown in patients with infectious diseases, immunodeficiency, anatomic abnormalities, acquired chronic diseases and pulmonary tumours.

    4. Page 183
      Abstract
      • A high-resolution linear probe is employed for lung ultrasound examination in children and infants.

      • Lung ultrasound avoids the use of ionising radiation.

      • Lung ultrasound demonstrates very good accuracy in several respiratory diseases.

    5. Page 189
      Abstract
      • The use of radiopharmaceuticals enables information on ventilated volume and regional perfusion of the lungs to be obtained.

      • V′/Q′ scintigraphy enables accurate diagnosis of congenital abnormalities of the lungs, vessels and heart, as well as in patients with bronchiectasis or CF.

      • V′/Q′ scintigraphy is easy to perform, typically without sedation, and causes only low-radiation exposure.

      • A more recent method, 18F-FDG PET/CT, contributes to the diagnosis of malignancies and inflammation.

    6. Page 193
      Abstract
      • Proper and adequate training of the operator along with extensive local sterility measures and anaesthesiology control are prerequisites for safe and effectively performed interventional radiology.

      • In cases where determining a lesion’s nature will alter a patient’s management and benefits outweigh risks, a percutaneous biopsy is indicated; core biopsy is preferred over fine-needle aspiration.

      • Image-guided percutaneous drainage is a safe and efficacious technique for the treatment of pleural effusions, abscesses and empyemas; in the case of complex effusion, abscess or empyema drainage can be combined to fibrinolytic therapy.

      • Tumour localisation or thermoablation can be performed in selected cases of paediatric patients.

      • Transcatheter embolisation seems to be a first-line therapy for the treatment of pulmonary AVM or major haemoptysis in patients with CF.

  6. Page 198
    1. Page 198
      Abstract
      • To match the correct aerosol delivery device to a patient, a clinician should be informed about the disease, drug, deposition and device characteristics, and the ability or disability of the patient to perform an inhalation manoeuvre.

      • A pMDI/VHC with an attached facemask is the first choice in asthma maintenance treatment in young children.

      • DPIs are convenient for older children, who can perform a fast, deep inhalation.

      • New (smart) nebulisers are promising but monitoring of safety and efficacy is important.

      • Correct inhalation technique and adherence are mandatory for successful aerosol therapy.

  7. Page 207
    1. Page 207
      Abstract
      • Acute lung infections have a very high incidence in children.

      • Chronic lung infections (i.e. lasting more than 4 weeks) are also common in children.

      • Sex, age, geography and vaccinations are important determinants of lung infection incidence.

      • The incidence of many infections changes over time and this reflects changes in pathogen and/or host.

    2. Page 214
      Abstract
      • Rapid antigen detection and molecular tests are the methods of choice for the identification of viral infections.

      • Blood culture is positive in <10% of paediatric patients with bacterial LRTIs.

      • IGRAs are the method of choice for discrimination between M. tuberculosis, M. bovis and nontuberculous mycobacteria.

      • BAL GM is a reliable test for the diagnosis of invasive aspergillosis.

    3. Page 221
      Abstract
      • The natural course of RSV infection can be modified by passive immunisation with neutralising antibodies by monthly intramuscular administrations of Palivizumab, a humanised monoclonal IgG1 antibody directed against the F-protein of RSV.

      • There is broad agreement that prematurely born children with chronic lung disease in infancy and children with haemodynamically relevant heart disease during the first 2 years of life may benefit from passive immunisation against RSV.

      • Serious (in particular invasive) pneumococcal diseases can be avoided by active immunisation with either polysaccharide-protein conjugate vaccines during the first 2 years of life or with 23-valent pneumococcal polysaccharide vaccine after the first 2 years of life.

      • Active immunisation against S. pneumoniae serotypes is highly recommended.

    4. Page 227
      Abstract
      • The majority of upper respiratory tract infections are viral in aetiology and self limiting.

      • Consider epiglottitis or bacterial tracheitis in a child with stridor who looks unwell.

      • Decisions regarding antibiotics for otitis media and tonsillitis are difficult and involve pros and cons for the patients and for society; these should be openly discussed when making treatment choices.

    5. Page 233
      Abstract
      • Around 2 million children <5 years of age die from pneumonia each year.

      • While Streptococcus pneumoniae is the “classic” organism it probably accounts for less than half of the cases of pneumonia.

      • Many guidelines do not recommend the use of chest radiographs to make a diagnosis on pragmatic grounds but this is associated with over and under diagnosis.

      • Most children can be treated with oral antibiotics unless they have severe and/or atypical disease or cannot tolerate oral therapy.

    6. Page 242
      Abstract
      • Hospital-acquired LRTIs are associated with significant mortality, morbidity and prolonged hospitalisation.

      • Risk factors include intensive care, immunosuppression and admission of infants to paediatric wards containing patients with LRTIs.

      • Organisms may be part of the patient’s normal flora or maybe transmitted from another patient or healthcare provider.

      • Following recommended infection, control policies significantly reduces rates of nosocomial infection.

    7. Page 248
      Abstract
      • Immunocompromise/immunodeficiency can be broadly divided into congenital (primary immunodeficiency) or acquired (through immunosuppression or infection such as HIV).

      • Preventative measures such as antibiotic prophylaxis along with swift diagnosis and treatment can effectively reduce lung morbidity.

      • Long-term sequelae vary from mild restrictive or obstructive defects to end-stage respiratory failure.

    8. Page 253
      Abstract
      • The diagnosis of non-CF bronchiectasis may be delayed as chronic wet cough can be misdiagnosed as other respiratory diseases.

      • HRCT scanning is necessary as chest radiography is not sensitive for detecting early disease.

      • Prognosis has been related to the extent of disease and the type of bronchiectasis.

      • Treatment is based on optimising airway clearance techniques and intermittent courses of antibiotics for pulmonary exacerbations.

    9. Page 258
      Abstract
      • The incidence of pleural empyema is increasing in many countries.

      • The most common pathogens associated with empyema, necrotising pneumonia and lung abscess are S. pneumoniae and S. aureus.

      • Chest CT is unnecessary for most cases of complicated pneumonia and should be considered in selected cases.

      • Therapeutic choices should be evaluated individually and shared in a multidisciplinary team.

      • Antibiotics remain the mainstay of treatment.

      • Chest drain with fibrinolysis is the preferred primary therapy in empyema; VATS should be reserved for use in patients refractory to medical treatment.

      • The long-term outcome for children with complicated pneumonia and no predisposing conditions is usually good.

    10. Page 266
      Abstract
      • Bacterial bronchitis should be suspected in a child with protracted wet cough.

      • Detailed differential diagnostic protocol aims to exclude other underlying causative factors.

      • Treatment should target the most frequent pathogens.

      • Protracted bacterial bronchitis can be a precursor of chronic suppurative lung disease and bronchiectasis, if left untreated.

      • If detected early and adequately treated, prognosis of protracted bacterial bronchitis is good.

  8. Page 270
    1. Page 270
      Abstract
      • TB remains a major, but often unrecognised, cause of disease and death in children.

      • Children with TB are generally not infectious.

      • TB in children generally reflects active disease in the adult population.

      • Treating TB in children is not straightforward.

    2. Page 284
      Abstract
      • Young children are more likely to develop extrapulmonary manifestations of TB, associated with age-related impairment of cellular immune responses.

      • Extrapulmonary TB can have very severe consequences and thorough investigations and prolonged therapy are required.

      • Conditions of immunosuppression, in particular HIV, are more likely to lead to extrapulmonary severe manifestations. This is proportionate to the level of suppression of T-cell function.

  9. Page 293
    1. Page 293
      Abstract
      • Asthma and wheezing disorders are heterogeneous conditions in terms of risk factors, age of onset, clinical phenotype, severity, response to treatment and long-term course.

      • Despite a large body of research, the factors explaining time trends and international disparities in asthma prevalence remain largely unknown.

      • Both severity of asthma and lung function show a track from early childhood to adulthood.

      • It is important to study phenotype and clinical course of wheezing illness early in life when it might still be possible to modify the natural history of the disease.

    2. Page 298
      Abstract
      • For childhood asthma and wheezing disorders both genes and the environment play a role, which is why they are complex diseases.

      • Genetic studies have identified numerous risk loci for childhood asthma, in particular the GSDMB-ORMDL3 locus on 17q21, which has replicated numerous times and has the strongest effect on childhood-onset asthma found to date.

      • Epidemiological observations have identified both protective environments and risk factors associated with childhood asthma and wheezing disorders. The most robust and consistent finding relating to risk is ETS exposure, particularly maternal smoking during pregnancy.

      • Gene–environment interactions, for example ETS, and the association of the 17q21 locus could be established in relation to childhood asthma.

    3. Page 305
      Abstract
      • Bronchiolitis is the first episode of acute viral infection of terminal and respiratory bronchioli in infants <1 year of age.

      • Symptoms of bronchiolitis are moderate-to-severe respiratory distress with rales at auscultation.

      • Supportive therapies aim to keep the upper airways clear, and the infant oxygenated and hydrated.

      • A bronchodilator mixed with 3% hypertonic saline might be tried and this combination should be continued only if it achieves a documentable clinical benefit.

    4. Page 310
      Abstract
      • Despite the favourable natural history in the majority of children with wheeze during preschool years, symptoms in this age range can be severe or frequent, justifying maintenance treatment.

      • Limited information is available on the pathophysiology of recurrent wheeze in preschool children, which is likely to be complex and multifactorial. As a result, one-dimensional classification systems (e.g. episodic viral versus multiple-trigger wheeze) are of limited value in diagnosis and management.

      • ICS are recommended as first choice maintenance therapy in children with frequent or severe symptoms irrespective of phenotype. Montelukast is an alternative maintenance treatment option, although it is less effective than ICS.

      • If symptoms persist despite maintenance treatment ongoing exposure to relevant inhalant allergens and tobacco smoke, poor adherence or inhalation technique, alternative diagnoses and relevant comorbidity should be excluded before stepping up therapy.

    5. Page 316
      Abstract
      • There is no universally accepted definition of asthma, although reversible airway obstruction, airway hyperresponsiveness and chronic inflammation are key features.

      • There is accumulating evidence that the interaction between respiratory viral infections and atopy is important in the cause and pathogenesis of atopic asthma.

      • Airway remodelling is a common feature in adult severe asthma but this is less clear in childhood, particularly as to when it starts and what elicits the process.

      • The ultimate goal of asthma treatment is to achieve and maintain clinical control with a minimum of side-effects and to reduce future risks to the patient.

      • There is no uniform definition of severe childhood asthma and several criteria are used such as the need for or use of high-dose corticosteroids, severe and/or frequent exacerbations, chronic asthma symptoms, or reduced lung function.

      • Severe childhood asthma should be referred to and managed in specialised paediatric units.

    6. Page 328
      Abstract
      • New treatments based on interventions in the immunopathogenesis of asthma are currently under development and evaluation, especially in subjects whose symptoms are not controlled by current therapy.

      • Omalizumab is a monoclonal human antibody which can antagonise the role of IgE in the pathogenesis of allergic asthma.

      • IL-5 plays an important role in eosinophil activation and airway hyperresponsiveness and is involved in the induction of Th2 responses in the asthmatic airway.

    7. Page 334
      Abstract
      • Although asthma is considered the most common condition presenting with wheezing, not all the children who wheeze are affected by asthma. Recurrent wheezing is a common condition in paediatric practice and the differential diagnosis represents a challenge for paediatricians.

      • The diagnosis of asthma is more difficult in preschoolers.

      • The symptoms of asthma can be associated with other diseases.

  10. Page 339
    1. Page 339
      Abstract
      • Manifestations of allergic diseases may appear at any age, but most commonly occur in childhood.

      • The so-called “allergic march” portrays a succession from atopic eczema, through food allergy to inhalant allergy, often accompanied by asthma and subsequently allergic rhinitis, all of which commonly occur by school age.

      • Viral infections are important triggers of symptoms and exacerbations of asthma in childhood; however, allergic sensitisation appears to precede viral infection in children with viral wheeze and allergic sensitisation who develop asthma.

      • The observed increase in allergic diseases is particularly worrying since it not only affects the subjects with the diseases, but is likely to increase the burden of allergic disease in the offspring of the current younger generation.

    2. Page 345
      Abstract
      • Allergy is a common trigger of asthma.

      • Allergy can start at any age.

      • Allergy testing should be performed in all children with asthma.

    3. Page 349
      Abstract
      • Anaphylaxis is a serious allergic reaction that is rapid in onset and may result in death.

      • The most common causes are food allergens, medications and hymenoptera venoms.

      • Infants are usually not able to describe symptoms; therefore, physicians need to have a high index of suspicion in order to diagnose anaphylaxis.

      • Adrenaline is the medication of choice for anaphylactic episodes.

    4. Page 354
      Abstract
      • Allergic rhinitis is a symptomatic disorder of the nose characterised by itching, nasal discharge, sneezing and nasal airway obstruction induced by an IgE-mediated immune reaction after allergen exposure.

      • According to ARIA guidelines, allergic rhinitis is divided into intermittent or persistent disease and the severity is classified as mild or moderate/severe depending on the severity of symptoms and their impact on social life, school and work.

      • The diagnosis of allergic rhinitis is based on the concordance between a typical history of allergic symptoms and diagnostic tests.

      • The therapeutic strategies of allergic rhinitis are patient education, pharmacotherapy and allergen-specific immunotherapy.

      • The inflammation of the nasal mucosa may affect the eye mucosa, air sinuses, the ear and the lower airways.

    5. Page 363
      Abstract
      • AD is a multifactorial disease.

      • The environmental factors interact with skin and allergic genetic factors so that the clinical appearance is multifaceted and therapy is complex.

      • Therapy mainstays are emollients, avoidance of irritants and allergens, topical anti-inflammatory drugs (mainly corticosteroids) and control of infections and pruritus.

      • In a subgroup of children, early and severe manifestation of AD has been associated with an increased risk of asthma (the so-called “atopic march”).

    6. Page 370
      Abstract
      • Food allergy is on the rise especially in children.

      • Its symptoms may include respiratory complaints.

      • The presence of asthma is a negative prognostic factor for anaphylactic reactions.

      • Severe asthma may be associated with food allergy.

      • Currently, the treatment for food allergy treatment is avoidance, but OIT is a promising new approach.

    7. Page 376
      Abstract
      • Unlike in adults where asthma with ABPA is common, ABPA is rarely seen in children other than complicating CF.

      • In the context of CF, ABPA is difficult to diagnose because it mimics CF lung disease.

      • The single most useful diagnostic test is an abrupt, four-fold rise in serum IgE.

      • The mainstay of ABPA treatment is systemic corticosteroids, increasingly pulsed methyl prednisolone rather than oral prednisolone.

    8. Page 383
      Abstract
      • Immunotherapy with seasonal and perennial airborne allergens has beneficial effects on allergic asthma.

      • Anti IgE therapy reduces the risk of asthma exacerbations on children with severe allergic asthma.

  11. Page 390
    1. Page 390
      Abstract
      • CF is the most common life-threatening autosomal-recessive disorder in Caucasian populations with an incidence of 1/2500 and a carrier frequency of 1/25.

      • CF is caused by mutations in the CFTR gene on chromosome 7.

      • The CF phenotype is very heterogeneous and depends on both nature and localisation of the underlying CFTR mutations, as well as genetic background and environmental influences.

      • CFTR analysis is indicated for diagnostic purposes in individuals with clinical suspicion of CF or CFTR-related disorders, fetuses with echogenic bowel or whose parents are proven CF carriers, and for carrier testing in persons with a positive family history or in partners of proven CF carriers.

    2. Page 397
      Abstract
      • The gold standard confirmation method for a suspected CF diagnosis is the measurement of sweat chloride using pilocarpine iontophoresis.

      • A borderline or positive result should always be confirmed with a second sweat test or by CFTR mutation analysis.

      • Until recently, the diagnosis has usually been made based on clinical manifestations, but newborn screening for CF has been implemented in many European countries.

      • Once CF diagnosis has been confirmed, other family members should be offered screening for the disease using sweat testing, especially all siblings.

    3. Page 402
      Abstract
      • CF lung disease begins early in life. It is characterised by impaired mucociliary clearance and mucus obstruction, and chronic pulmonary infection and inflammation.

      • There is a progressive decline of lung function with episodes of acute worsening of respiratory symptoms, referred to as pulmonary exacerbations.

      • Pulmonary effects of CF typically have the largest impact on morbidity and mortality, and account for over 80% of fatalities due to the disease.

      • Current management of CF lung disease is predominantly symptomatic. The cornerstones of CF respiratory care are airway clearance and treatment of pulmonary infections.

      • Lung transplantation is the final therapeutic option for patients with end-stage lung disease.

    4. Page 410
      Abstract
      • Nutritional status is strongly associated with pulmonary function and survival in CF.

      • Nutritional management should be started as soon as possible after diagnosis.

      • Patients’ height and weight should be measured at each clinical visit and BMI calculated (percentile or z-score in children, absolute BMI in adults).

      • EPI should be confirmed by a biological test (steatorrhoea or faecal elastase-1) and PERT should be started as soon as possible.

      • Fat-soluble vitamins need to be given in association with pancreatic enzymes in EPI patients.

      • The gastrointestinal tract is a major source of comorbidity in CF patients.

      • Entities such as fibrosing colonopathy, appendiceal mucocoele and DIOS are specific to CF.

      • In the case of acute abdominal pain, surgical conditions need to be sought; all surgical aetiologies may take on the appearance of recurrent pain with mild symptoms.

    5. Page 421
      Abstract
      • Despite the successes of CFTR modulation, treatment of infection and inflammation remain important targets.

      • Therapeutic options in CF are increasing rapidly.

      • CFTR directed pharmacotherapy is moving into the clinic.

    6. Page 427
      Abstract
      • Every effort should be made to optimise standard therapy in patients with apparently end-stage CF lung disease.

      • Prediction of prognosis in end-stage CF lung disease is difficult, and more prolonged survival than previously expected can be anticipated.

      • Timely referral for lung transplant assessment is essential to maximise chances of benefit.

  12. Page 435
    1. Page 435
      Abstract
      • Many children with airway malformations are already symptomatic in the neonatal period or in infancy.

      • Airway anomalies are important differential diagnoses in children with many respiratory abnormalities.

      • Airway abnormalities may be part of complex syndromes, and in many cases are associated with other congenital anomalies.

      • Early, accurate diagnosis and appropriate management is particularly important in severe central airway stenosis.

      • With airway growth, mild-to-moderate stenoses in the first years of life frequently become less prominent.

      • Depending on the type and extent of the malformation, conservative or surgical management options have to be chosen on an individual basis.

      • With adequate management, in most patients the long-term prognosis is favourable.

    2. Page 445
      Abstract
      • The routine introduction of antenatal ultrasound scanning has not only increased our knowledge of CTMs but has resulted in improved antenatal counselling and management of these conditions.

      • Antenatal “resolution” of CCAMs is reported in up to 20% cases but in most cases there is evidence of their persistence on post-natal CT images.

      • Management of asymptomatic CCAMs is controversial with some physicians opting for regular follow-up and imaging to gauge progress whilst others opt for surgical removal.

      • Long-term follow-up studies to assess the natural history, including respiratory and neurodevelopmental outcomes, especially after fetal intervention, are required.

    3. Page 452
      Abstract
      • The incidence of vascular malformations is ∼1% but the true incidence is difficult to assess if less severe abnormalities are included.

      • The most severe forms of vascular rings can be detected during the neonatal diagnostic work-up, cause serious symptoms in the newborn period and require surgery within the first year of life.

      • The less severe abnormalities are detected in later life, when unexplained recurrent respiratory symptoms or occasional mild dysphagia leads to radiographic or endoscopic evaluation. Symptoms such as dyspnoea, wheezing and cough are often misdiagnosed as asthma, particularly if they occur in older children.

      • The vascular malformations that most frequently cause symptoms are: double aortic arch; right aortic arch with a left ligament arising from the descending aorta; aberrant subclavian artery; pulmonary sling; and aberrant innominate artery.

    1. Page 461
      Abstract
      • BPD remains a significant cause of long-term respiratory illness despite major advances in the care of the preterm newborns.

      • The primary pathological process is inflammation, driven through the NF-κB pathway, and triggered by a variety of genetic and environmental factors.

      • Management is directed at minimising lung insults, by limiting oxygen toxicity and ventilator-induced lung trauma.

      • Drugs that may influence development of BPD include caffeine and vitamin A, although new anti-inflammatory drugs are in development.

    2. Page 466
      Abstract
      • BPD is characterised by impaired lung growth and altered lung structure, which may be further aggravated by poor nutritional status.

      • BPD is associated with increased work of breathing and a higher resting metabolic state; therefore, energy expenditure is high, making sufficient nutrition a challenge and growth restriction in preterm infants with BPD a common problem.

      • The nutritional challenge continues after discharge, the growth pattern of infants with BPD must be closely monitored and, when needed, nutrition should be supplemented to ensure adequate catch-up growth.

    3. Page 469
      Abstract
      • BPD is a distinct disease entity of survivors of preterm birth.

      • The prevalence of BPD among survivors of very preterm birth is 20–40%.

      • BPD is associated with a risk for significant neurodevelopmental delay.

      • Compared with non-BPD peers, infants with BPD may exhibit poorer academic achievements and impaired emotional and physical development.

    4. Page 472
      Abstract
      • Survivors of extreme prematurity, and those with BPD in particular, experience high rates of respiratory symptoms (mainly cough and wheeze) and hospital readmission in the early years of life.

      • Into mid-childhood and adolescence, clinical symptoms become milder and less frequent, but spirometric studies show that many of those born very prematurely have scarcely reversible airflow obstruction (mean FEV1 70–80% predicted).

      • Although BPD survivors frequently suffer from asthma-like symptoms, BPD and asthma are distinct clinical entities resulting from different pathogenic mechanisms and caution is needed when recommending asthma treatments to BPD patients.

      • Due to the natural age-related decline in respiratory function, it is reasonable to expect a phenotype resembling COPD to develop in some survivors of BPD, so these patients should be followed up into adulthood and efforts should be made to prevent them from smoking.

  13. Page 477
    1. Page 477
      Abstract
      • All children with parapneumonic pleural effusion or empyema should be admitted to hospital and managed following local or national guidelines.

      • Intravenous antibiotics and careful consideration of pleural drainage procedures are the most important aspects of parapneumonic effusion/empyema management.

      • Chylothorax is a rare condition in children usually caused by injury to the thoracic duct; simple chest drainage and dietary modifications are the mainstay of treatment.

      • When haemothorax is diagnosed, blood should be promptly drained from the pleural cavity with a chest tube.

    2. Page 485
      Abstract
      • The most common cause of pneumothorax in paediatric patients is the rupture of bullae or blebs in the apex of the lung without an underlying predisposing lung disease or history of trauma.

      • When pneumothorax is suspected, standard erect posterior to anterior chest radiograph in inspiration technique represents the diagnostic gold standard.

      • Patients with pneumothorax who experience symptoms should be treated with oxygen supplementation and needle aspiration or chest catheter insertion independent of the size of the pneumothorax.

      • In the setting of recurrent pneumothorax, surgical treatment is indicated. The preferred technique consists of the resection of the causative bleb or bulla and a pleurodesis procedure.

    3. Page 492
      Abstract
      • Neuromuscular disorders are relatively common, with a prevalence of 1:3000.

      • Sleep disordered breathing is likely when vital capacity falls to <60% predicted.

      • NIV is indicated to control symptomatic sleep disordered breathing.

      • Use of NIV in Duchenne muscular dystrophy may double life expectancy.

    4. Page 497
      Abstract
      • Despite obvious deformations, lung function remains surprisingly preserved in most individuals with adolescent idiopathic scoliosis, pectus excavatum and pectus carinatum.

      • In contrast, severe thoracic restriction conveys a high risk of pulmonary morbidity and respiratory failure in early-onset scoliosis and complex syndromal thoracovertebral malformations.

      • Surgical correction rarely improves lung function but may significantly deteriorate the natural history if young individuals undergo early fusion of the thoracic spine.

      • Recent developments in orthopaedic techniques allow timely intervention in early-onset scoliosis promoting spinal and chest wall growth, but the potential for improving lung function remains to be clarified.

  14. Page 503
    1. Page 503
      Abstract
      • The two-process model of sleep and wakefulness predicts the day-to-day synchronisation of an organism to its environment by the interaction of a circadian (C) and a homeostatic process (S).

      • Circadian rhythms are driven by an endogenous circadian pacemaker, located in the suprachiasmatic nucleus (SCN) of the hypothalamus. Circadian sleep rhythm controls the sleep–wake cycle, modulates physical activity and food consumption, and over the course of the day, regulates body temperature, heart rate, muscle tone and hormone secretion.

      • The SCN sets the body clock to ∼24 h. The main influence of the SCN on sleep is due to a series of relays through the dorsomedial nucleus of the hypothalamus, which signals to the sleep–wake systems to coordinate their activity with day–night cycles.

      • There are two types of sleep state, NREM and REM sleep.

      • NREM sleep is conventionally divided into three or four stages, each with its own distinguishing characteristics.

    2. Page 514
      Abstract
      • OSAS in children is a complex, multi-organ syndrome consisting of habitual snoring, witnessed apnoea, sleep fragmentations and diurnal consequences.

      • The most frequent cause is adeno-tonsillar hypertrophy and treatment involves adeno-tonsillectomy; however, many children do not resolve and need further therapy, such as orthodontic therapy.

    3. Page 521
      Abstract
      • Central sleep apnoea and hypoventilation syndromes are causes of morbidity in childhood that may result in severe complications if left untreated.

      • Complications of sleep apnoea include pulmonary hypertension, cor pulmonale, systemic hypertension, cardiac arrhythmias, hypoxic cerebral injury and seizures.

      • PSG is the gold standard test for SDB and is required to diagnose sleep-related disorders.

      • Treatments include supplementary oxygen, caffeine (apnoea of prematurity), diaphragm pacing, CPAP, BiPAP and mechanical ventilation via tracheostomy.

    4. Page 528
      Abstract
      • Obesity is defined as BMI >98th centile according to age- and sex-specific centile charts.

      • Obesity is associated with a change in static and dynamic lung volumes.

      • Obese children experience more respiratory symptoms compared to their normal-weight peers.

      • There is a parallel increase in asthma and obesity prevalence, but a true relationship is controversial.

      • Obese children should be screened at routine visits for the presence of snoring, apnoea, sleep disordered breathing and daytime drowsiness.

  15. Page 533
    1. Page 533
      Abstract
      • ALI can be caused by endogenous or exogenous factors.

      • VILI causes additional harm in the presence of lung injury.

      • Protective ventilation strategies have reduced the mortality of ARDS.

    2. Page 538
      Abstract
      • There are a wide range of causes of both acute and chronic respiratory failure in children.

      • Blood gas analysis remains the central investigation when assessing respiratory failure.

      • Methods of support, and particularly noninvasive techniques, have allowed a much greater ability to avoid prolonged intensive care.

      • Improvements in technology, intensive care and the provision of home care for children in chronic respiratory failure have led to increasing numbers of these children being cared for in the community.

      • Early recognition of chronic respiratory failure should lead to interventions that will correct gas exchange as far as possible before secondary complications develop.

    3. Page 545
      Abstract
      • The main objective of oxygen therapy and mechanical ventilation is to restore a normal nocturnal and daytime gas exchange and a normal sleep quality.

      • LTOT is the treatment of choice of chronic hypoxaemia.

      • Mechanical ventilation is the treatment of choice of chronic hypercapnia.

  16. Page 551
    1. Page 551
      Abstract
      • The gold standard for the diagnosis of PCD is a combination of ciliary beat pattern and frequency analysis and electron microscopy in patients with upper or lower airway disease.

      • Specific ultrastructural defects responsible for PCD result in specific abnormalities in beat frequency and pattern; however, a small number of milder phenotypes may appear with subtle or no apparent structural defects or ciliary dysfunction.

      • When diagnosis by ultrastructural analysis or beating patterns analysis is not conclusive, but the suspicion of PCD is high, further testing should be performed in order to find the right diagnosis, such as nasal nitric oxide, immunofluorescent microscopy and genetic analysis.

    2. Page 559
      Abstract
      • CPA constitutes one of the most serious challenges to the normal development of the respiratory tract, and it represents a major health risk throughout infancy and childhood.

      • Several studies have reported an association between GOR and CPA.

      • Both acid and nonacid reflux are implicated in the pathophysiology of parenchymal lung disease.

      • The diagnosis of GOR-related aspiration remains challenging because of the absence of sensitive and specific tests.

    3. Page 566
      Abstract
      • Children <3 years of age are at greatest risk of FBA.

      • FBA must be suspected for any witnessed choking episodes.

      • FBA cannot be excluded on either normal physical examination or chest radiograph.

      • Removal of the foreign body is the primary objective and mainstay of treatment.

    4. Page 570
      Abstract
      • Bronchiolitis obliterans is a rare paediatric chronic obstructive lung disease, which follows a severe insult to the respiratory tract and results in narrowing and/or complete obliteration of the small airways.

      • The most common cause is severe lower airway infection, followed by bone marrow or lung transplantation, drug toxicity, noxious inhalation injury, vasculitis and autoimmune disorders.

      • Open lung biopsy for histological confirmation of the diagnosis is rarely necessary. In the appropriate setting, after the exclusion of other chronic obstructive lung disease, HRCT provides adequate evidence for a correct diagnosis.

      • Lung function is characterised by a moderate-to-severe fixed airflow obstruction unresponsive to bronchodilators that may slowly progress to fatal deterioration even a few months after diagnosis. Mortality rates range from 3.2% to 16.7%, depending on bronchiolitis obliterans severity.

      • The treatment of bronchiolitis obliterans is often unsuccessful because patients are referred to specialised centres when irreversible fibrotic changes and airway obliteration have already occurred.

    5. Page 577
      Abstract
      • Plastic bronchitis in children is usually associated with congenital heart disease post-surgery with Fontan physiology.

      • Cast formation appears to be related to poor cardiac output, lymphatic abnormalities, inflammation, and tissue factor activation.

      • There is no proven therapeutic value in using hypertonic saline, salbutamol, corticosteroids, acetylcysteine, dornase alfa, antibiotics or expectorants.

      • There is some evidence to support the use of low-dose azithromycin and aerosol heparin to prevent casts, and tPA can help mobilise casts in situ but can be very irritating to the airway. Airway clearance therapy, such as high frequency chest wall compression, is strongly recommended.

      • Thoracic duct ligation and cardiac transplantation appear to reduce or eliminate cast formation in some patients.

    6. Page 582
      Abstract
      • Haemangiomas are benign and respond to propranolol.

      • Lymphangiomas may need to be resected if they obstruct the airway; the clinical course is less predictable.

      • Papillomatosis of the larynx may require repeated surgical resection and often responds to cidofivir.

      • Newer therapies are currently being explored for lymphangiomas and papillomas.

    7. Page 587
      Abstract
      • ILD in children represents a heterogeneous group of respiratory disorders that are mostly chronic and impair the respiratory function of the lung.

      • The term ILD has been replaced by the term DPLD, thus covering all pathological entities affecting lung homeostasis and remodelling following injury.

      • The mechanisms underlying disease development are dependent on the type of DPLD; the common basis is the interaction between injurious environmental triggers and genetic predisposition.

      • The presenting clinical manifestations are often subtle and nonspecific, therefore, a two-step diagnosis approach is required: 1) diagnosis of DPLD syndrome; 2) diagnosis of specific DPLD.

      • Treatment protocols remain difficult to produce but the overall strategies include general measures and pharmacological therapy, mainly anti-inflammatory and immunosuppressive molecules.

      • A favourable response to anti-inflammatory therapy can be expected in almost two-thirds of cases, although significant sequelae are often observed.

    8. Page 596
      Abstract
      • Two major groups of disorders are associated with the surfactant system: surfactant dysfunction mutations and PAP.

      • Surfactant dysfunction mutations include diseases caused by mutations in the genes coding for SP-B, SP-C, the lipid transporter ABCA3 and the transcription factor TTF1.

      • Although these entities may show a PAP-like pattern on histology, the extent of alveolar filling is much less than in PAP and this term should be avoided when naming the surfactant dysfunction mutations.

      • Hereditary deficiency of the α-chain of the receptor for GM-CSF is responsible for increased accumulation of surfactant in the alveolar space and resulting PAP.

      • Surfactant dysfunction mutations may present at birth as respiratory distress syndrome or later in infancy as chronic dyspnoea and hypoxia (chILD syndrome), whereas mutations in the α-chain of the receptor for GM-CSF present only as chILD syndrome.

    9. Page 601
      Abstract
      • The symptoms of pulmonary hypertension are nonspecific and the possibility of the condition should always be remembered. Syncope on exercise should never be ignored.

      • If pulmonary hypertension is secondary to lung disease, this is usually obvious from the chest radiograph.

      • In a child with pulmonary hypertension and a normal chest radiograph, remember OSA and occult interstitial lung disease are possible causes.

      • Children with pulmonary hypertension should be referred to specialist centres for consideration of emerging therapies.

    10. Page 610
      Abstract
      • Eosinophilic lung diseases are a diverse group of disorders characterised by pulmonary opacities associated with tissue or peripheral eosinophilia.

      • Hypersensitivity pneumonitis, or extrinsic allergic alveolitis, is a diffuse granulomatous ILD caused by inhalation of various antigenic organic particles or low molecular weight chemicals.

    11. Page 619
      Abstract
      • Pulmonary haemorrhage can occur at any age.

      • Presentation varies from the acute and life-threatening to “hidden”, with no obvious haemoptysis.

      • Many cases are idiopathic but a number of clear underlying causes can be recognised by selective investigations.

      • Known complications include chronic iron deficiency anaemia and pulmonary fibrosis.

      • Systemic corticosteroids are the most effective treatment in the majority of cases.

    12. Page 625
      Abstract
      • SCD includes HbS-haemoglobinopathies, which lead to haemoglobin polymerisation with subsequent vaso-occlusion, a chronic haemolytic anaemia and endothelial damage in blood vessels, with consequent chronic organ failure.

      • In the lungs and airways, SCD induces acute pulmonary vascular occlusions, ACS, LRTIs, and chronic lung disease with lung fibrosis and pulmonary hypertension.

      • Important pulmonary comorbidities of children with SCD are bronchial hyperresponsiveness, atopy and asthma.

    13. Page 630
      Abstract
      • Primary pulmonary neoplasms are rare in childhood; metastatic disease or inflammatory/congenital diseases are more frequently recognised.

      • The most common primary lung malignancies in children are pleuropulmonary blastoma and carcinoid tumour; bronchogenic carcinomas are exceptionally rare.

      • Symptoms of primary lung tumours in childhood are nonspecific (cough, haemoptysis, chest pain or shortness of breath); persistent symptoms or persistent radiographic findings despite therapy require a CT scan and/or a MRI of the chest.

      • Tumours arising in the anterior mediastinum are most commonly due to lymphoma followed by germ cell tumours; large masses present life-threatening airway compromise, especially during anaesthesia.

    14. Page 636
      Abstract
      • Although individual rare diseases are, by definition, rare, taken together, they are sufficiently common that they need to be considered in paediatric respiratory differential diagnosis.

      • Respiratory paediatricians need to be aware that multisystem diseases may present with respiratory signs and symptoms.

      • Respiratory paediatricians also need to be ready to advise specialists in other fields, especially cardiology, nephrology and hepatology, about respiratory complications of their disease specialities.

      • Ciliary dysfunction, far from being a purely respiratory issue, affects multiple organs. The basic science and clinical aspects of ciliopathy are a huge growth area.

    15. Page 647
      Abstract
      • Short- and long-term outcomes have improved but still lag behind other solid-organ transplant groups. Chronic graft rejection remains a significant barrier to further improvements in survival.

      • Donor organ shortage remains a critical issue. Given this limited resource, optimal timing of transplantation is both essential and challenging, especially in conditions where survival can be difficult to predict.

      • A lifelong regimen of triple immunosuppression is used, consisting of a CNI, a cell cycle inhibitor (or antimetabolite) and a corticosteroid. Important drug interactions exist with CNIs which the physician must be aware of.

      • Post-transplantation management focuses on ongoing rehabilitation, careful surveillance, and treatment of acute and chronic complications, including infection and graft rejection.

  17. Page 656
    1. Page 656
      Abstract

      Rehabilitation programmes in children and adolescents with chronic respiratory disorders:

      • aim at preventing worsening of the disease, improving self-management and restoring quality of life in order to enable full participation in daily life, including school, and social and physical activities,

      • consist of standardised multidisciplinary interventions,

      • include diagnostic procedures, specific medical care and nursing, nutritional and psychological counselling and educational interventions,

      • address a broad spectrum of chronic respiratory conditions and can contribute efficiently to a general improvement in morbidity and mortality, in the context of a complex disease management.

      • Advanced lung disease results in increased energy expenditure, an augmented level of inflammation and diminished appetite, contributing to a loss of body weight and requiring specific nutritional management, including counselling, installation of a daily nutrition plan, high-protein calorie supplementation and substitution of vitamins and micronutrients.

    2. Page 662
      Abstract
      • Environmental triggers, such as in- and outdoor allergens and irritants, can elicit and exacerbate acute attacks in asthmatic children.

      • Children are particularly susceptible to air pollution because of their higher ventilation rate, with an increased risk of medication use and hospitalisation.

      • Interventions must be addressed to multiple triggers through multiple intervention components.

      • Simple measures (mattress covers, HEPA filters, air filtration, home repairs, and limiting levels of exertion in outdoor activities when pollutant concentrations are elevated) may protect children’s respiratory health, reducing asthma symptom-days, school days missed and healthcare utilisation.

    3. Page 665
      Abstract
      • Paediatric respiratory physiotherapy has developed into a wide field, encompassing airway clearance, rehabilitation, aerosol treatment, tracheostomy management and long-term home ventilation programmes.

      • Paediatric respiratory physiotherapy is a special therapeutic approach to respiratory disorders in newborns, infants, children and adolescents. As such it needs a profound understanding of the structure and the function of the growing respiratory tract and the necessary competencies required for working with paediatric patients.

      • An individualised approach to the treatment of a specific patient is preferable to any kind of rigid therapeutic routine.

      • Airway clearance employs basic mechanisms like expiratory airflow, forced expiration and gas–liquid pumping. It might, therefore, be considered as a therapeutic application of respiratory physiology.

    4. Page 670
      Abstract
      • Infants and children with chronic lung disease are at risk of developing hypoxia and respiratory symptoms during air travel.

      • The hypoxia challenge test is the currently recommended tool for the identification of at-risk indviduals.

      • The hypoxia challenge test does not predict the incidence of in-flight hypoxia in infants born preterm and travelling by air at near term.

      • The accuracy of the hypoxia challenge test in infants and young children has not been assessed.

      • Further research into the prediction of in-flight hypoxia is required in infants and young children.

    5. Page 673
      Abstract
      • A physically active lifestyle including sports and supervised conditioning programmes provides physiological improvements in muscle function, cardiopulmonary efficiency, immune system function, obesity prevention and self-esteem.

      • Cardiopulmonary exercise testing provides a global assessment of the integrative exercise responses involving the pulmonary, cardiovascular, and skeletal muscle systems.

      • V′O2max reflects the maximal ability of the body to take in, transport and utilise oxygen and it is the best single measure of aerobic fitness.

      • Exercise prescriptions to improve cardiopulmonary fitness in patients with chronic health diseases are based on the initial level of fitness and include the modalities of physical activity as well as the frequency, intensity and duration of the training.