s prevention of further attacks of acute Chronic rheumatic heart disease Recurrent bouts of ARF with associated carditis result in s carring and \201 brosis of the heart valves (most commonly mitral valve) and may result i n incompetent valves requiring replacement. 08_McClure_08.indd 245 11/14/2012 2:33:25 PM CHAPTER 8 Cardiovascular 246 Pericarditis In \202 ammation of the pericardium may be p or a manifestation of more gener alized illness. The principal causes of pericardial in \202 ammation are: \200 Infections: \200 \200 \200 \200 \200 Connective tissue: \200 \200 \200 \200 Metabolic: \200 \200 Malignancy Radiotherapy viral, e.g. coxsackie B, Epstein \200\223Barr virus (EBV); bacterial, e.g. streptocococcus, mycoplasma; tuberculosis; fungal, e.g. histoplasmosis; parasitic, e.g. toxoplasmosis. rheumatoid arthritis; rheumatic fever; systemic lupus erythematosus (SLE); sarcoidoisis. hyperuricaemia; hypothyroidism. \200 Clinical features The features depend on the extent of involvement of the pericardium. The predominant symptom is precordial pain that is typically sharp, exac- erbated and exaggerat ed by lying down, and relieved by sitting or leaning forward. The pain is often referred to the left shoulder. Other symptoms include cough, dyspnoea, and fever. The accumulation of suf \201 cient \202 uid to cause cardiac tamponade and heart failure is rare. Examination Speci \201 c diagnostic \201 ndings will relate to the amount of \202 uid within the pericardial sac, including pulsus paradoxus, pericardial rub, quiet/distant hear t sounds. Investigations Investigations directed at con \201 rming the diagnosis include: \200 \200 echocardiogram; ECG (typical low voltage QRS complexes). Other investigations should be directed at identifying the underlying cause of the pericard itis and will include: pericardiocentesis, bacterial/viral cul- ture and biochemical analys is; blood serology for viral studies, ASOT, and connective tissue disease. 08_McClure_08.indd 246 11/14/2012 2:33:25 PM PERICARDITIS 247 Management Treatment is directed both at the underlying cause, e.g. antibiotics, and sympto ms, e.g. the following: \200 Analgesia for pain relief. \200 Anti-in \202 ammatory dru gs to reduce pericardial in \202 ammation. \200 Pericardiocentesis for pericardial effus ion causing cardiac tamponade and heart failure. Constrictive pericarditis Previous pericardial in \202 ammation may predispose to this con dition. However, most cases of constrictive pericarditis occur in the absence of any preced ing illness or generalized systemic disease. The \201 brosed restric- tive pericardium im pairs cardiac contractility. Clinical features Include evidence of heart failure, hepatomegaly, and neck vein distention . On auscultation, heart sounds are distant and a characteristic pericardial \200\230knock \200\231 is often heard. CXR may reveal calci \201 cation of the pericardium. Treatment Requires pericardiectomy. 08_McClure_08.indd 247 11/14/2012 2:33:25 PM CHAPTER 8 Cardiovascular 248 Myocarditis Myocarditis may be due to: \200 \200 \200 \200 Infections: Kawasaki disease (see Drugs: adriamycin. Connective tissue disease: b p.774); r heumatic fever (see b p.244); sarcoidosis. viral, e.g. coxsackie B, EBV. b p.716). SLE (see b p.780); rheumatoid arthritis (see Clinical features Variable and will depend on the age of the patient and on the time course of underlying disease. Speci \201 c cardiovascular symptoms include progres- sive worseni ng of dyspnoea and congestive cardiac failure. Sudden onset of ventricular arrhythmia may o ccur. Examination Typical cardiovascular examination includes: \200 \200 \200 \200 weak pulses; tachycardia; gallop heart rhythm; distant heart sounds. Diagnosis Echocardiography shows poor ventricular function. De \201 nitive histological di agnosis is made after percutaneous endomyocardial biopsy. CXR shows cardiomegaly; ECG shows reduced QRS complex size. Treatment This is directed at the underlying cause and at controlling symptoms of congestiv e heart failure. Arrhythmias should be treated. Cardiac transplan- tation is needed in pati ents with refractory heart failure. 08_McClure_08.indd 248 11/14/2012 2:33:25 PM CARDIOMYOPATHY 249 Cardiomyopathy Cardiomyopathy may be primary, or secondary to systemic or metabolic disease (see b p.962). Primary cardiomyopathy may be classi \201 ed as: \200 \200 \200 b p.240); hypertrophic (obstructive; see dilated (congestive); restrictive (see b p.249). Dilated (congestive) cardiomyopathy This condition is rare and characterized by massive dil atation of the ven- tricles and cardiomegaly. The cause is unknown in most cases, although it is seen in association with other conditions: \200 \200 post-viral infection phenomenon; metabolic (e.g. mitochondrial disease, b p.971). Clinical features \200 \200 Insidious onset of progressive congestive cardiac failure is common. The course is usually progressive and the prognosis poor. Management Mainly directed at treating heart failure and, where possible, any underly- ing cause. Heart transplantation if severe heart failure. Restrictive cardiomyopathy This condition is rare and characterized by poor ventricular com pliance and inadequate ventricular \201 lling. The clinical features are similar to con- strictive pericarditis (b p.247). It is sometimes seen in the L f \202 er hypere- osinoph ilic syndrome (multisystem disorder of skin, lungs, nervous system, and liver) and results in endocardial \201 brosis of the AV valves and the ventricles. Prognosis is poor and car diac transplantation often required. 08_McClure_08.indd 249 11/14/2012 2:33:25 PM CHAPTER 8 Cardiovascular 250 Cardiac arrhythmias See b pp.42 \200\22343, 55 \200\22357. Sinus arrhythmia is normal in ch ildren and ado- lescents. Other arrhythmias are rare in childhood and may be transient or p ermanent. Congenital arrhythmias may occur in structurally normal or abnormal hearts. They may be secondary to myocardial disease (e.g. rheu- matic fever, myocarditis) or follow expo sure to toxins, drugs or surgery to the heart. Children with suspected arrhythmia require a detailed history and examination. The arrhythmia should be identi \201 ed and characteriz ed by ECG. Intermittent arrhythmias may be detected by 24hr ECG. Underlying congenital hear t disease should be excluded by echocardiography. Supraventricular tachycardia The most common abnormal arrhythmia in childhood. Re-entry wit hin the A \200\223V node is the most common mechanism of SVT. Clinical features Sudden onset (and cessation) lasting from seconds to hours; heart rate 25 0 \200\223300beats/min. SVT is well-tolerated in older children, but heart failure may occu r in the young infant. Often precipitated by febrile illness. Wolff \200\223Parkinson \200\223White syndrome Pre-excitation syndrome predisposing to SVT. This is due to an abnormal re-entry circuit of the AV node and an accessory conduction pat hway con- necting atrium to ventricle on the right or left lateral cardiac border, or withi n the ventricular septum. It may be associated with Ebstein anomaly, post-surgical repair, and cardiomyopathy. ECG shows short PR interval and delta wave (slow upstroke of QRS comple x). Treatment \200 Medical: amiodarone. Interventional: when a teenager. adenosine (emergency) -blocking medication, \202 ecainide, electrophysiological studies and intracardiac ablation Ventricular tachycardia Long QT syndrome may be associated with sudden loss of conscious- n ess during exercise, stress or emotion, usually in late childhood. If un- recognized, sudde n death from VT may occur. Inheritance is autosomal dominant; there are several phenotypes. Prolongation of the QT interval on ECG is associated with many drugs, electrolyte disorder s, and head injury. Long QT syndrome is a channelopathy caused by speci \201 c gene muta- tions with gain or l oss of function. There is a range of effect from Long QT, Short QT, Brugada syndrome and ca rdiomyopathy. Anyone with a family history of sudden unexplained death, or syncope on exert ion must be assessed for these. Congenital complete heart block Rare. Mothers of affected children are usually positive for serum anti-Ro or anti-La antibodies and have underlying connective tissue disorder. 08_McClure_08.indd 250 11/14/2012 2:33:25 PM CARDIAC ARRHYTHMIAS 251 Clinical features \200 \200 \200 Fetal hydrops and intrauterine death. Neonate: Childhood: heart failure. asymptomatic; syncope. Management Endocardial/epicardial pacemaker. 08_McClure_08.indd 251 11/14/2012 2:33:25 PM This page intentionally left blank 253 Chapter 9 Respiratory medicine Introduction 254 Common presentation: wheeze 255 Common presentation: stridor 256 Common pr esentation: cough 257 Common presentation: breathlessness 258 Common presentation: snoring 259 Investigations 260 Asthma 262 Asthma: drug delivery devices 264 Asthma: clinic manageme nt (1) 266 Asthma: clinic management (2) 268 Cystic \201 brosis 270 Cystic \201 brosis: problems 271 Cystic \201 brosis: management (1) 272 Cystic \201 brosis: management (2) 274 Chronic lung disease of prematurity 276 Congenital respiratory tract disorders 278 Sle ep apnoea 279 Allergic rhinitis 280 Upper airway infections 282 Laryngeal and tracheal in \202 ammation 284 Bronchial disease 286 Bronchiolitis 288 Pneumonia 290 Pneumonia: treatmen t 292 Pneumonia: effusion, empyema 293 Pulmonary tuberculosis 294 Other disorders 295 09_McClure_09.indd 253 11/14/2012 2:34:53 PM CHAPTER 9 Respiratory medicine 254 Introduction Disorders of the respiratory tract account for a major part of pediatric medic ine both in hospital and in general practice. There are 5 key common presentations that the practitioner should be familiar with and we will focus on these \201 rst. Next, we will discuss the investigations we use in the care of children. Last, we provide a mini-catalog of diseases and condi- tions that you will need to know about, and be able to treat. We have discussed in Chapters 3 (b p.31) and 5 (b pp.48 \200\22350) the general approach to history and examination for patients with respiratory illness. Here are some additional po ints that you will need to take note of during your consultation. History \200 General information: neonatal period and any prior endotracheal intubations; growth and general body proportions; weight loss; immunizations. Age of onset of symptoms or problem. Have there been any triggers to this illness? What makes the problem worse? Exe rcise (e.g. asthma), sleep (e.g. adenotonsillar hypertrophy and snoring)? What makes the pr oblem better? (Bronchodilators in asthma.) Other symptoms: gastro-oesophageal re \202 ux; apnoea; coryza; chest/abdominal pain. \200 \200 \200 haemoptysis; cough; sputum production; choking; Examination See also b p.31. \200 General temperature; level of consciousness; color; and arterial pulse oximetry saturation; pulse rate. Rate, pattern of breathing (episodic, periodic, apnoea), duration of expiratio n, and use of accessory muscles (+/ \200\223 recession). Nose and speech: discharge (e.g. a llergic rhinitis); hyponasal speech (e.g. palate and nasal problems); nasal or mouth breath er; nasal \202 aring. Facial appearance: syndrome). Tonsillar hypertrophy. Cough (paroxys ms, barking, high-pitched). Neck retraction and external compressive mass. Breathing cycle: obstruction; expiratory prolongation or wheeze indicates intrathoracic airway obstruction. Breath sounds. Chest appearance (Harrison \200\231s sulcus), and percussion. Sputum pot. information: growth parameters; clubbing; lymphadenopathy; crease across the bridge of the nose and nasal size of midface, lower jaw, tongue (e.g. craniofacial \200 \200 \200 inspiratory stridor indicates extrathoracic airway \200 \200 09_McClure_09.indd 254 11/14/2012 2:34:54 PM COMMON PRESENTATION: WHEEZE 255 Common presentation: wheeze Wheeze is a breath sound that is heard during expiration. It is often associated with prolongation of the expiratory phase of the breathing cycle. Wheeze indicates obstruction to air \202 ow within the thorax. It can be high- or low-pitched; th is differentiation indicates that the obstruction is likely to be in the smaller and larger airways, respectively. Also, wheezes can be monophonic or polyphonic; identifying these so unds will tell you whether the obstruction is likely to be in one or multiple airways. Differential diagnosis Extrinsic lower airway compression \200 Lung cyst. Vascular: pulmonary artery vascular ring. Lymphatic: Chest parenchyma: e.g. pneumonia, pulmonary oedema, bronchogenic e.g. enlarged left atrium compressing left mainstem bronchus, \200 e.g. enlarged hilar lymph nodes (b pp.290 \200\223291, 654). deformity: e.g. scoliosis (b p.746). Intrinsic change in lower airway dimension \200 \200 \200 \200 \200 \200 \200 \200 b p.262). Asthma ( Bronchiolitis ( Bronchitis and bronchiectasis ( b pp.270 \200\223273). Cystic \201 brosis ( Ciliary disease. Haemangioma. Polyps. Tracheobronchomalacia. b p.288). b pp.257, 291). Intraluminal lower airway obstruction \200 b pp.276, Aspiration of food or milk from gastro-oesophageal re \202 ux ( 295, 326). Foreign body in halation ( Mucus, pus, and blood. \200 b p.51). Each of the conditions in the list above will require speci \201 c investigation and treat ment. Wheeze due to asthma will require both acute and chronic treatment (see b pp.264 \200 \223269). 09_McClure_09.indd 255 11/14/2012 2:34:54 PM CHAPTER 9 Respiratory medicine 256 Common presentation: stridor Stridor is a noise heard during the inspiratory phase of breat hing. It indicates either dynamic or \201 xed extrathoracic airway obstruction (anywhere from the nose to the thoracic inlet). When it arises acutely and is associated with respira tory distress, immediate attention is required (b pp.48 \200\22350, 284 \200\223285). The d ifferential diagnosis of stridor is as follows. Nose and nasopharynx \200 \200 b p.846). Congenital obstruction, e.g. choanal atresia ( In \202 ammation, e.g. rhinitis and sinusit is ( b p.280). Mouth, oropharynx, and hypopharynx \200 \200 \200 \200 e.g. macroglossia and glossoptosis. Congenital obstruction: In \202 ammation: Masses: Foreign body e.g. tonsillar hypertrophy (b p.279). e.g. cystic hygroma or other malformation. (b p.51). Larynx \200 e.g. laryngomalacia, laryngeal web or cleft, vocal Congenital obstruction: cord paralysis. In \202 ammation: Infection: Masses: Trauma: \200 \200 \200 e.g. gastro-oesophageal re \202 ux. e.g. epiglottitis, laryngotracheobronchitis (b p.284). e.g. haemangiomas, abscess. e.g. sub glottic stenosis, foreign body inhalation (b p.51). Trachea \200 \200 Congenital obstruction: Infection: e.g. tracheomalacia, tracheo-oesophageal \201 stula. e.g. bacterial tracheitis. There are speci \201 c treatments for many of these conditions. In persist- ent, non-medic al causes of stridor, airway surgery may be required after endoscopy. 09_McClure_09.indd 256 11/14/2012 2:34:54 PM COMMON PRESENTATION: COUGH 257 Common presentation: cough Cough is a protective response for removing secretions and parti culate matter from the airway. Main feature sudden expulsion of air from lungs. Differential diagnosis of acute cough Upper airway disease \200 \200 Common Other laryngitis, croup Allergy cord dysfunction. Vocal cold: e.g., rhinovirus. infections: e.g. viral in \202 uenza and parain \202 uenza, sinusitis, tonsillitis, \200 Lower airway disease (b p.262). \200 \200 e.g. respiratory syncytial virus bronchioliti s, bronchitis due to Asthma Infection: adenovirus, in \202 uenza, and parain \202 uenza. Lung parenchymal disease \200 \200 e.g. viral and bacterial pneumonia, empyema (b p.293). pneumonia: e.g. Mycoplasma pneumonia e infection. Infection: Atypical Treatment In general, we do not treat cough, but focus on underlying cause. Differential diagnosis of chronic cough A chronic cough is one that has persisted for more than 8wks (British Thoracic Society 2008 guideline1 for the assessment and management of ch ildren with chronic cough). Its causes are as follows: \200 Upper airway disease: \200 infection \200\224 (b p.286); \200 in \202 ammation \200\224 Lower airway disease: \200 e.g. chronic sinusitis, tonsillitis, Bordetella pertussis e.g. gastro-oesophageal re \202 ux (b p.276). congenital abnormalities \200\224 larynx, pulmonary artery sling; asthma ; infection: forei gn bronchiectasis: tuberculosis, or immunode \201 ciency; cystic e.g. tracheo-oesophageal \201 stula, cleft \200 \200 \200 e.g. post-bronchiolitis symptoms, atypical infections; body e.g. damage to the airway from chronic infection and \201 brosis (b p.270). \200 Lung parenchymal disease: Central causes: \200 \200 Tre atment: try and identify the underlying cause and focus on its treatment. \200 infection, e.g. pneumonia and empyema. Psychogenic cough. Tourette disease: with a tic involving throat clearing or cough. in common with acute cough, when treating chronic cough, Reference 1 Shields Bush A, Everard ML, et al. (2008). BTS guidelines: recommendation for t he asssessment and management of cough in children. Thorax 63(Suppl. 3): 1 \200\22315. 09_McClure_09.indd 257 11/14/2012 2:34:54 PM CHAPTER 9 Respiratory medicine 258 Common presentation: breathlessness Being short of breath can be due to heart or lung disea se. In lung disease, this sensation arises because of lack of oxygen, dif \201 culty breat hing due to airway obstruction, or abnormal lung mechanics. Infants may not be able to expr ess their discomfort \200\224in this instance you will need to be able to identify signs of respiratory distress. The causes include the following. Differential diagnosis Airway obstruction \200 \200 airway (see b pp.48, 282). airway (see b p.48). Upper Lower Abnormal lung mechanics \200 Restrictive kyphoscoliosis (b pp.744 \200\223747). Parenchymal Muscle paralysis (b pp.546 \200\223548). lung disease: e.g. chest wall (obesity), chest deformity, \200 lung disease: e.g. pneumonia, pulmonary hypertension. weakness: e.g. Duchenne muscular dyst rophy, diaphragmatic Hypoxia \200 perfusion mismatch: e.g. lung disease, pneumonia, Ventilation pneumothorax, pulmonary embolism. Heart myocarditis (b pp.231, 246 \200\223248) . disease: e.g. cyanotic congenital heart disease, pericarditis and Emergency treatment and care Discussed in Chapter 5 (b p.50). 09_McClure_09.indd 258 11/14/2012 2:34:55 PM COMMON PRESENTATION: SNORING 259 Common presentation: snoring We are concerned about snoring in children when it indicates t hat the child has obstructive sleep apnoea (OSA), i.e. snoring in association with periods of ineffective breathing lasting longer than 2 breaths (e.g. breath- ing at a rate of 20/mi n, this would be 6s). This is as opposed to central apneas, which are a pause >20s in an ot herwise well child. The most com- mon cause of OSA is adenotonsillar hypertrophy. The other causes are as follows. Differential diagnosis Congenital anatomical \200 \200 \200 Midface: Choanal Tongue: (b p.936). Lower Syndromes: Goldenhar, Apert. e.g. hypoplasia in achondroplasia. atresia (b p.846). e.g. macroglossia in Beckwith syndrom e (b p.949), trisomy 21 \200 jaw: e.g. retro- and micrognathia. e.g. Pierre \200\223Robin sequence (b p.847), Treacher \200\223Collins, In \202 ammation \200 \200 \200 \200 Adenotonsillar hypertrophy. Allergic rhinosinusitis. Nasal polyposis. Gastro-oesophageal re \202 ux. Masses \200 \200 Encephalocele. Nasal gliomas. Central causes of pharyngeal hypotonia during sleep \200 \200 \200 \200 b p.550). Cerebral palsy ( Seizures ( Hydrocephalus ( Obesity Treatment When snoring is associated wi th OSA, the underlying cause needs to be treated. b p.505). b p.535). (b p.400) 09_McClure_09.indd 259 11/14/2012 2:34:55 PM CHAPTER 9 Respiratory medicine 260 Investigations Chest X-ray CXR is a key investigation in respiratory disease. It will give you informa- tion about lung volume (e.g. hyperin \202 ation in asthma), signs of chronic in- \202 ammation (e.g. peribronchial cuf \201 ng), and evidence of congenitial lesions (e.g. lung cysts). However, it is an investigation that can be overused. It should not repl ace a thorough clinical examination, and think hard before requesting repeat studies. A CXR is used to glean more clinical evidence about what the child \200\231s underlying pro blem is. So, in each presentation consider why you have requested the test. Here are some e xamples. Wheeze A CXR is not needed every time a patient presents with \200\230asthma \200\231. How ever, if that child has never had an X-ray, or there is something atypical about the histor y, consider other possibilities: \200 Suspected foreign body inhalation: (object often ra diolucent). Suspected gastro-oesophageal re \202 ux with aspiration, or aspiration from ab normal swallowing: i.e. looking for different lobes affected at different times. Monophonic wheeze: the right main-stem bronchus, or a large left atrium compressing the left main-ste m bronchus, or mediastinal mass. i.e. inspiratory and expiratory \201 lm i.e. looking for hilar lymph nodes compressing on Stridor Suspected tracheal lesion around the thoracic inlet. Cough \200 \200 Suspected typical and atypical pneumonia, or empyema. Suspected bronchiectasis. Breathlessness \200 \200 \200 Suspected pulmonary parenchymal disease. Pneumothorax. Suspected heart disease, e.g. heart failure. Snoring with sleep apnoea Suspected cardiopulmonary disease or cor pul- monale from chronic upper airway obstruction. 09_McClure_09.indd 260 11/14/2012 2:34:55 PM INVESTIGATIONS 261 Bedside tests in respiratory medicine Four tests are frequently performed at the bedside or in the laboratory are lung function testing, sweat test, and arterial blood sampling. Lung function testing Spirometry can be achieved in (cid:2)5-yr-olds, but measurements are easier in (cid:2)7 year olds. Peak expiratory \202 ow rate (PEFR) monitoring is useful in asthma. Other measurements include: \200 FEV capacity. Exercise testing. Bronchodilator responsiveness (i.e. reversibility). 1/FVC: the forced expired volume in 1s as a fraction of forced vital \200 Sweat test This test is used in the diagnosis of cystic \201 brosis. Sweating is induced in an area of the forearm using pilocarpine, and a capillary tube is used to collect the sw eat. A minimum of 15 L (and preferably >30 L) of sweat should be collected. In cystic \201 brosis abnormal function of the sweat glands results in higher concentrations of chlor ide in the sweat: \200 \200 Suspicious: Diagnostic: >40mmol/L (>30mmol/L in newborn screened babies). >60mmol/L. Pulse oximetry Assessment of oxygen saturation (SpO2) using a pulse oximeter is a non-invas ive way of assessing a child \200\231s oxygenation using a probe attached to a \201 nger or toe. Arterial blood gas Assessment of blood O2, CO2, and acid \200\223base are impor- ta nt in critically ill children, or in those where you suspect signi \201 cant lung disease. Further investigations Sometimes more detailed investigations are needed before you can sel ect the best treatment for your patient. These include the following: Imaging \200 Chest airways as well as abnormalities in parenchymal tissue density. Thorac ic and mediastinal anatomy. Nuclear perfusion (Q), as well as V/Q matching. computed tomography: useful for assessing abnormalities in MRI: useful for looking at airway \200\223blood interface, and vascular imaging: useful for assessing regional ventilation (V) and Direct visualization of structure \200 used to assess directly the airway from nose to Flexible bronchoscopy: distal bronchus; used to lavage the lung for microscopy and culture 09_McClure_09.indd 261 11/14/2012 2:34:55 PM CHAPTER 9 Respiratory medicine 262 Asthma Asthma is a disease of chronic airway in \202 ammation, bronchial hyper-reac- tivit y, and reversible airway obstruction. It affects 10% of the population and can develop at a ny age, but typically half of the pediatric cases pre- sent before the age of 10yrs. There is often a family history of asthma or atopic disease. Diagnosis History \200 Cough after exercise or sometimes in the early morning, disturbing sleep. Shortness of brea th. Limitation in exercise performance. \200 Examination In the child with chronic problems consistent \201 ndings include: \200 \200 \200 Barrel-shaped chest. Hyperin \202 ation. Wheeze and prolonged expiration. Chest X-ray Not needed if there has been recent imaging. It may show: \200 \200 \200 \200 Hyperin \202 ation. Flattened hemi-diaphragms. Peribronchial cuf \201 ng. Atelectasis. Spirometry \200 \200 \200 \200 Peak expiratory \202 ow rate (PEFR) <80% predicted for height. FEV Concave scooped shape in \202 ow volume curve. Bronchodilator response to FEV1 or PEFR). 1/FVC <80% predicted. -agonist therapy (i.e. 15% increase in Medication The main medications used for maintenance are bronchodilators, which give short- term relief of symptoms, and prophylactic therapy, which reduces chronic in \202 ammation and bronchial hyperreactivity. In the out- patient clinic our aim is to titrate these treat ments so that the child can function normally, yet still avoid any detrimental effect on gr owth and development. See b p.267 for stepwise manner in which these drugs may be used. Bronchodilators \200 Short-acting \200 Long-acting \200 Short-acting anticholinergic: 2-agonists: salbutamol, terbutaline. 2-agonists: salmeterol, formoterol. ipratropium br omide. 09_McClure_09.indd 262 11/14/2012 2:34:55 PM ASTHMA 263 Chronic treatment of in \202 ammation and hyperreactivity \200 \200 \200 \200 \200 Inhaled steroids: Oral steroids: Sodium cromoglicate: Methylxanthines: Leukotriene inhibito rs: amount of steroid therapy that is needed to control symptoms. Combination inhalers cont aining inhaled steroids and long-acting B budesonide, beclometasone, \202 uticasone. prednisolone. rarely used. theophylline. montelukast and za \201 rlukast may reduce the 2-agonists. Side-effects of chronic treatment Steroids When long-term oral steroids or high-dose inhale d steroids are used, spe- cial attention will need to be given to unwanted effects includin g: \200 Impaired growth: can affect growth in height, but also ask about frequency of hai r-cuts, or changing shoe size, as these are early indicators of poor growth. Adrenal suppre ssion Oral candidiasis . Altered bone metabolism \200 \200 Theophylline Now rarely used in children, but you should be aware that there are a number o f problems related to toxic blood levels, including: \200 \200 \200 \200 \200 Vomiting Sleep disturbance or increased sleeping. Headaches. Poor concentration and deterio ration of performance at school. Arrhythmias. 09_McClure_09.indd 263 11/14/2012 2:34:55 PM CHAPTER 9 Respiratory medicine 264 Asthma: drug delivery devices There are a number of drug delivery devices that are availabl e for use in children: \200 Nebulizer: use in emergency treatment at all ages for delivery of bronchodilator (although a spacer device is often used instead). Large or small volume spacer with metered dose inha ler (MDI): infancy to any age (facemask for under 3yrs and mouthpiece for older children). This device uses a plastic \200\230bubble \200\231 with a valve at one end and a place whe re an MDI can be inserted at the other end. The spacer allows the aerosol particles from th e inhaler to be slowed and inhaled on each breath. It stops the drug dose from the MDI depo siting in mouth or stomach, and allows it to go with inspired air all the way down to the s mall airways. Dry salbutamol (Ventolin Accuhaler ). These can be used in children >5yrs. P ropellant >12yrs, although are dif \201 cult to use and generally not advised. The device uses a gas propellant to aerosolize the drug. use in powder device: terbutaline sulfate (Bricanyl Turbohaler ), metered dose inhaler (PMDI): these can be used in children Inhalation technique In the clinic you will need to make sure that your patient is getting and taking the medication prescribed. In all children you will need to see that they have t he appropriate technique and device for their age. Child >3yrs Look for 5-breath tidal volume breathing technique. \200 to allow full use of the diaphragm. Stands \200 MDI. Shake \200 MDI into spacer. Place \200 device in mou th. Place Firm \200 seal with mouth around mouthpiece. \200 Breathe once). Continue in and out tidally: when good rhythm, activate device (only If \200 second dose is needed: then shake MDI and repeat as above. Infant Note that, if th e infant is crying, less drug will be inhaled. Make sure that the person giving the medicat ion: \200 breathing 5 times. Tilt the spacer so that the valve is open (in small volume device you do not need to tip as the valve is low resistance). Let the infant take at least \201 ve breaths from each dose actuated. 09_McClure_09.indd 264 11/14/2012 2:34:55 PM ASTHMA: DRUG DELIVERY DEVICES 265 09_McClure_09.indd 265 11/14/2012 2:34:55 PM CHAPTER 9 Respiratory medicine 266 Asthma: clinic management (1) The aim of treatment is to allow the child to lead a normal l ife. In the clinic you will come across children with seemingly distinct clinical pat- tern s of their chronic asthma. Patients with frequent or persistent asthma should be seen in a specialist clinic. Nebulized treatment is used in severe acute asthma. It is not recommende d in mild to moderate severity asthma. Instead, use multidosing (up to 10 puffs) bronchodil ator. Infrequent episodic asthma Characteristics \200 \200 \200 \200 75% of asthmatics. <4 episodes per year. Symptom-free between acute episodes. No regular tr eatment needed. Management strategy \200 \200 B 2-bronchodilators. Treat acute episodes with Use nebulized bronchodilators and short-course prednisolone in mo re severe episodes (i.e. prednisolone 3 days, given once daily in the morning after breakfa st with no need to taper treatment). Frequent episodic asthma Characteristics \200 \200 \200 20% of asthmatics. Episodes every 2 \200\2234 weeks. Regular treatment is needed. Management strategy \200 \200 B 2-bronchodilator as required. Use Use regular, low-dose inhaled steroid. Persistent asthma Characteristics \200 (cid:2) \200 3 episodes/wk, with cough at night/m orning. \200 Less than 5% of asthmatics. Regular treatment is needed. Management strategy \200 \200 \200 \200 Use prophylactic inhaled steroids. Long-acting Oral steroids may be needed. Oral leukotrien e inhibitors may enable reduction in steroid usage. B 2-bronchodilator may be helpful. Exercise-induced asthma Management strategy \200 \200 2-bronchodilator before exercise. Mild: use Severe: low-dose inhaled steroid. 09_McClure_09.indd 266 11/14/2012 2:34:55 PM ASTHMA: CLINIC MANAGEMENT (1) 267 Escalating therapy Having reviewed the history and categorized your patient in terms of cli n- ical pattern and severity, use a logical, stepwise approach to escalating therapy (see B ox 9.1). Box 9.1 The stepwise approach to drugs Before altering a treatment, ensure that treatment is being taken in an effective manner Step 1: occasional use of relief bronchodilators Short-acting B Step 2: regular inhaled pre venter therapy Short-acting B (200 \200\223400micrograms/day) 2-bronchodilator for relief of symptoms 2-bronchodilator as required + low-dose inhaled steroid Step 3: add-on therapy \200 B 2-bronchodilator as required + high-dose inhaled Short-acting steroid or Low-dose inhaled steroid +/ \200\223 long-acting bronchodilator If control is still inadequate use a trial of other therapies, e.g. leukotriene receptor an tagonist or slow release theophylline Step 4: persistent poor control B \200 2-bronchodilator as required + high-dose inhaled Short-acting steroid (up to 800micrograms/day) + long-acting bronchodilator or Theophylline s or ipratropium +/ \200\223 alternate day steroid Step 5: continuous or frequent use of oral steroid \200 \200 \200 Use daily steroid tablet in lowest dose Maintain high-dose inhaled steroid at 800micrograms /day Refer to respiratory specialist 09_McClure_09.indd 267 11/14/2012 2:34:56 PM CHAPTER 9 Respiratory medicine 268 Asthma: clinic management (2) 0 \200\2232yrs In this age group, a spacer device with an app ropriate face mask is used, e.g. a small volume Aerochamber or Ablespacer which can tak e any inhaler; or a large volume Volumatic or Nebuhaler , which only \201 t cer- tain inhalers. Prophylactic therapy with inhaled steroids is more effective than cromoglicate. Acute treatment \200 \200 \200 : <2400micrograms/day (in 6 doses). : <6000micrograms/day (in 6 doses). : <480microgr ams/day (in 4 doses). Salbutamol via Volumatic Terbutaline via Nebuhaler Ipratropium via Volumatic Prophylactic treatment \200 \200 : 100 \200\223400micrograms/day. Budesonide via Nebuhaler Beclometasone via Volumatic : 100 \200\223400micrograms/day. 3 \200\2235yrs Acute treatment \200 \200 : <3600micrograms/day (in 6 doses). : <6000micrograms/day (in 6 doses). Salbutamol via Volumatic Terbutaline via Nebuhaler Prophylactic treatment \200 \200 \200 \200 : 100 \200\223800micrograms/day. Budesonide via Nebuhaler Beclomethasone via Volumatic Fluticasone via Volumatic Salmeterol via Volumatic given alone and only when the child is also taking an inhaled steroid. Combin ation inhaler: salmeterol). : 100 \200\223800micrograms/day. : (>4yrs) 100 \200\223200micrograms/day : (>4yrs) 50micrograms/day. Must never be Seretide (contains \201 xed doses of \202 uticasone and 5 \200\22312yrs Acute treatment \200 \200 \200 : <7200micrograms/day (in 6 doses). Salbutamol Accuhaler Salbutamol inhaler: (>12 years) <7200micrograms/day (in 6 doses). Terb utaline inhaler: (>12 years) <7200micrograms/day (in 6 doses). Prophylactic treatment \200 \200 \200 \200 : 100 \200\223800micrograms/day. Budesonide Turbohaler Beclometasone via Accuhaler Fluticasone via Volumatic Combination inh aler \200\223 Seretide and salmeterol); or Symbicort turbohaler ( \201 xed doses of bud esonide and formoterol). : 100 \200\223800micrograms/day. : 100 \200\223400micrograms/day. (contains \201 xed doses of \202 uticasone 09_McClure_09.indd 268 11/14/2012 2:34:56 PM ASTHMA: CLINIC MANAGEMENT (2) 269 Useful clinic guides Steroids \200 Fluticasone and budesonide are preferable since they have fewer side-effects than beclometa sone Patients on doses of steroids greater than \202 uticasone 500micrograms/day, budeson ide 800micrograms/day, beclometasone 800micrograms/day should be under the supervision of a specialist clinic Long-acting \200 2-agonists Salmeterol may be of value for night-time symptoms or daytime activity Should be used as a prophylactic agent Consider in patients on inhaled beclometasone or budesonide 400microgram s/day, or \202 uticasone 200micrograms/day \200 Allergen avoidance \200 \200 \200 \200 Removal of feather or woolen bedding Wrapping of mattress in plastic Cleaning of carpets an d furniture No pets in the house if the child is allergic to them Passive smoking \200 \200 No smoking in the house or car. Parents/carers must be strongly encouraged to stop smoking completely. Education Older patients will need to learn more about their condition and how it is best t reated. For example: \200 \200 \200 \200 \200 Which medication to use and when Best inhaler technique What to do if asthma is getting wor se Not to smoke Gargle after steroid inhaler use so as to avoid oral thrush 09_McClure_09.indd 269 11/14/2012 2:34:56 PM CHAPTER 9 Respiratory medicine 270 Cystic \201 brosis Cystic \201 brosis (CF) is an autosomal recessive genetic disorder l eading to a defect in the CF transmembrane receptor (CFTR) protein, which results in defect ive ion transportation in exocrine glands. In the lung abnormal sodium and chloride ion tra nsportation causes thickening of respiratory mucus. The lung is therefore prone to inadequa te mucociliary clearance, chronic bac- terial infection, and lung injury. There are also si milar effects \200\224although not with superadded infection \200\224in other organs that l ead to pancreatic insuf \201 ciency, liver disease, and, in the male, infertility. There a re over 1500 mutations in the CFTR gene; the most common is the ZF508 deletion. CF is the m ost common genetic disease in Caucasions (1/2500). Diagnosis Screening Since 2007 in the UK all newborn babies are screened for cystic \201 brosis looking for an abnormally raised immunoreactive trypsinogen (IRT) and 29 CFTR gene m utations from blood-spot analysis on the Guthrie card. History Give particular attention to: \200 \200 \200 \200 \200 \200 Cough and wheeze. Shortness of breath. Sputum production. Haemoptysis. Stool type (e.g. fat ty, oily, pale) and frequency. Weight loss or poor weight gain. About 10 \200\22320% of CF patients present in the neonatal period with meco- nium ileus. H owever, most children with CF present with: \200 \200 \200 malabsorption; failure to thrive; recurrent chest infection. Examination Full assessment of: \200 \200 \200 respiratory system; liver and GI system; growth and development. Investigations \200 \200 Sweat test showing increased chloride levels (>60mmol/L). CXR: dilatation, cysts, linear sh adows, and in \201 ltrates. Lung function: lung volumes. hyperin \202 ation, increased antero-posterior diameter, bronchial obstructive pattern with decreased FVC and increased 09_McClure_09.indd 270 11/14/2012 2:34:56 PM CYSTIC FIBROSIS: PROBLEMS 271 Cystic \201 brosis: problems Lifelong therapy and supervision are required in CF. There i s a variety of problems that can be expected at different ages. Infancy \200 \200 \200 b p.862). Meconium ileus ( Neonatal jaundice (prolonged). Hypoproteinaemia and oedema. Childhood \200 \200 \200 \200 \200 \200 Recurrent lower respiratory tract infections. Bronchiectasis (occasionally) Poor appetite. Rectal prolapse ( Nasal polyps. Sinusitis (rare to have symptoms). b p.871). Adolescence \200 \200 \200 \200 \200 \200 \200 \200 \200 \200 Bronchiectasis. Diabetes mellitus ( Cirrhosis and portal hypertension. Distal intestinal ob struction ( Pneumothorax. Haemoptysis. Allergic bronchopulmonary aspergillosis. Male infert ility. Arthropathy. Psychological problems. b p.406). b p.274). 09_McClure_09.indd 271 11/14/2012 2:34:56 PM CHAPTER 9 Respiratory medicine 272 Cystic \201 brosis: management (1) The management of the child with CF requires close co- operation be- tween local hospitals and regional centers. Patients and their families gain much from expert clinics, and from other patients and their families. Effective management requires a multidisciplinary team approach, which should include: \200 \200 \200 \200 \200 \200 \200 pediatric pulmonologist; physiotherapist; dietician; nurse liaison or practitioner in CF; p rimary care team; teacher; psychologist.