Resp Flashcards

Question

What is the aetiology of persistent pulmonary HTN of the newborn?

Answer 1

0.5% live births Usually occurs in term or late preterm infants

Answer 2

* Usually presents in first 24hrs of life * Signs of respiratory distress * Cyanosis * Signs of cardiogenic shock (due to myocardial ischaemia and papillary muscle dysfunction) * Hypotension, oliguria, prolonged cap refill

Answer 3

* **Pulse oximetry** * Hypoxia * Out of proportion to parenchymal lung disease on CXR; doesn’t respond to 100% O2 * \>10% difference between pre- and postductal (R thumb and either big toe) * Due to R to L shunting through PDA (but not always present) * **ABG** * **CXR** * Usually normal, may show findings of an associated condition * **_Echocardiogram_** * Urgent → diagnostic * To exclude congenital heart disease, identify signs of pulmonary HTN (e.g. raised pulmonary pressures, tricuspid regurgitation), assess extent to shunting

Answer 4

**Ventilatory and circulatory support:** * ABCDE approach * Give oxygen * Mechanical ventilation (high-frequency) if needed * ECMO (extracorporeal membrane oxygenation) if other treatments fail * IV fluids, vasopressors (e.g. dopamine) * Correct acidosis **Inhaled nitric oxide** * Potent vasodilator * Sildenafil (Viagra) is another vasodilator which can sometimes be used

Answer 5

* HIE, CP, developmental delay etc. * NEC * ARF

Answer 6

Prognosis depends on underlying pathology; high morbidity and mortality

Answer 7

A common, self-limiting tachypnoea in the absence of other causes

Answer 8

Caused by delay in the reabsorption of foetal alveolar fluid * The process of clearing foetal alveolar fluid begins before term birth and continues through labour/delivery * In late gestation the mature lung epithelium switches from actively secreting liquid into the air spaces, to actively resorbing liquid (partly due to Na+ resorption) * If there is short delivery or C-section (without labour), mechanisms to reabsorb alveolar fluid are not established * Leads to pulmonary oedema, decreased pulmonary compliance, decreased tidal volume and increased dead space

Answer 9

Most common cause of respiratory distress in term infants (0.5%)

Answer 10

* Signs of respiratory distress * Usually at the time of birth, within 2hrs of delivery * Most prominent feature is tachypnoea (\>60 in neonates); hypoxia and cyanosis are rare

Answer 11

* **Clinical diagnosis** * **CXR** * To support diagnosis * Prominent perihilar streaking (distended pulmonary veins and lymphatics), patchy infiltrates, fluid in the horizontal fissure, flat diaphragms * **Exclude other causes** * **ABG** (exclude metabolic acidosis, degree of decreased pO2 depends on amount of fluid in lungs) * **Blood cultures** (exclude infection)

Answer 12

Supportive management * Respiratory support * Additional ambient O2; may need nasal cannula, occasionally CPAP * Maintain hydration, IV fluids * NBM until RR \<60 to decrease aspiration risk * Prophylactic antibiotics (until negative blood cultures)

Answer 13

Benign and self-limiting; usually no complications Usually settles within 1^st day of life; can take several days to resolve completely

Answer 14

Congenital defect in the formation of the diaphragm, allowing protrusion of abdominal contents into the chest cavity

Answer 15

There are 3 types: * **Posterolateral Bochdalek’s hernia (main focus):** * Most common, usually L sided (85%); occurs at 6wks gestation * L sided hernias allow herniation of small bowel, large bowel, and intra-abdominal solid organs * R sided hernias allow herniation of liver and a portion of large bowel * Leads to pulmonary hypoplasia, persistent pulmonary HTN of the newborn, L ventricular hypoplasia (if very severe) * **Anterior Morgani’s hernia:** * 3%; small and easily repaired * **Hiatus hernia:** * Stomach herniates through oesophageal hiatus à GORD * Only needs repair if severe symptoms

Answer 16

* previous affected sibling, * cardiac abnormalities (25%), * renal abnormalities * Karyotype abnormalities are seen in 4% infants → associated with trisomy 13, 18, 21, Turner’s syndrome

Answer 17

Usually discovered on antenatal USS screening or following polyhydramnios in the mother * If not detected antenatally, presents at or very soon after birth with: * **Signs of respiratory distress** * **Absent breath sounds on one side of the chest**, usually the L with heart shifted to R * **Bowel sounds audible over chest wall** * Smaller defects may present later with ‘**wheezy child**’ or **recurrent chest infection**

Answer 18

* Antenatal USS * Allows detailed planning of delivery and immediate care of the neonate * **CXR** * Shows loops of bowel in the chest * Confirms diagnosis if not previously diagnosed * **Assess for other abnormalities:** * Echo * Renal USS * Karyotyping

Answer 19

**_Immediate management:_** * **ABCDE approach** * **Endotracheal intubation** and mechanical ventilation if severe; ECMO if necessary * Avoid bag-and-mask ventilation in delivery room because stomach/intestines become distended with air à further impaired lung function * **NG tube** passed and suction applied à **decompression of** stomach * Consider **surfactant** * **Monitor blood gases**, put indwelling venous catheter in to enable drug administration * **BP support** with volume expansion/inotropic agents/colloid **_Surgical management (after stabilisation):_** * Abdo organs are replaced into abdo cavity and diaphragmatic defect is repaired

Answer 20

Complications: * Pulmonary hypoplasia, persistent pulmonary HTN, surfactant deficiency → chronic lung disease * Pneumothorax in normal lung (due to resuscitation) * Intestinal malrotation, volvulus * Neurodevelopmental delay (due to hypoxia) Overall survival is 50%; high mortality if related to pulmonary hypoplasia

Answer 21

Aspiration of meconium (the first faeces of a neonate) into the respiratory system Meconium aspiration syndrome (MAS): spectrum, leading to various degrees of neonatal respiratory distress

Answer 22

* Meconium is passed due to in-utero peristalsis * Usually occurs when GI maturation is sufficient (i.e. in term babies) * Also passed in response to foetal distress (hypoxia, head compression, cord compression, etc.) * Foetal distress/hypoxia causes foetal gasping à meconium aspiration into the lungs (from meconium-stained amniotic fluid) * May happen antenatally or perinatally * In the lungs, meconium leads to: * Mechanical airway obstruction (partial/total) * Leads to decreased ventilation of small airways and air trapping à pneumothorax, increased pulmonary vascular pressure (à R to L shunt) * Pulmonary inflammation (pneumonitis) * Due to pro-inflammatory cytokines in meconium * Surfactant dysfunction * The inflammatory reaction deactivates surfactant à reduced gas exchange à exacerbates hypoxia * This leads to: * Foetal hypoxia * Infection * Persistent pulmonary HTN (due to remodelling of pulmonary vasculature due to hypoxia)

Answer 23

* gestational age \>42wks, * foetal distress, * intrapartum hypoxia, * Apgar score \<7, * chorioamnionitis, * PROM, * oligohydramnios, * IUGR, * maternal HTN/DM/pre-eclampsia/smoking/drug abuse Risk is highest in infants who are post term and suffered birth asphyxia Rare if \<34wks gestation (GI tract not mature enough)

Answer 24

Meconium is passed by 10-20% babies; incidence increases with gestation (25% by 42wks) Of the babies that pass meconium, 10% develop meconium aspiration syndrome

Answer 25

* **Antenatal/perinatal:** * Foetal distress on CTG * Meconium-stained amniotic fluid * **Signs of respiratory distress** * At birth or within 4hrs * **Signs of post-maturity and meconium staining** * Yellow/green skin, long stained nails, dry/scaling skin

Answer 26

* Pulse oximetry (dual) * CXR * Confirm diagnosis * Patchy infiltrations, consolidation, pleural effusion, flattened diaphragms/increased lung volume * Bloods: * FBC, CRP, blood cultures (for infection) * ABG: hypoxaemia, hypercarbia, metabolic acidosis

Answer 27

Depends on severity; may need NICU **1.Supportive care:** * Place under infant warmer * Oxygen therapy: * Nasal canula, CPAP, mechanical ventilation * Nutritional support (IV fluids, switch to NG/oral when possible) **2. Antibiotics**: * IV ampicillin + gentamycin * If clinical suspicion of infection (e.g. chorioamnionitis, PROM, foetal heart-rate abnormalities) * Can be stopped if 48hr cultures and clinical examination findings are negative **3. Surfactant:** * May be given in moderate MAS or if pneumothorax is present **4. Inhaled nitric oxide:** * If pulmonary hypertension is also present

Answer 28

Complications: * Pneumothorax or pneumomediastinum (due to alveolar overdistention from obstruction) * Persistent pulmonary hypertension of the newborn (1/3) * Bronchopulmonary dysplasia * Neurodevelopmental delay (due to hypoxia) Prognosis depends on degree of hypoxia and complications * 80% have a 3-4d illness and are then discharged * 20% require NICU due to increased respiratory and cardiovascular requirements

Answer 29

Self-limiting inflammation of the upper respiratory tract mucosa that may involve nose, throat, sinuses and larynx Coryza: acute inflammation of the mucous membranes of the upper respiratory tract (i.e. a cold; also caused by hay fever) Coryzal symptoms: the symptoms seen in the common cold

Answer 30

Most common pathogen is rhinovirus (\>100 different serotypes) – 50% * Also caused by coronavirus (10-15%), influenza (5-15%), parainfluenza (5%), RSV (5%) Infection leads to influx of polymorphonuclear leukocytes into the nasal submucosa à inflammation Reinfection can occur after re-exposure to the same vial subtype (usually milder and of shorter duration) RFs are exposure to infected people, winter

Answer 31

The most common infection in childhood à children have 6-8/yr; adults have 2-4 Peaks when starting nursery and primary school

Answer 32

* Onset over 1-2d * Rhinorrhoea (clear or mucopurulent) * Sneezing * Nasal blockage * Sore throat (red on examination) * Cough (may persist for up to 4wks) * Low-grade fever (variable) * Lethargy, poor feeding

Answer 33

clinical diagnosis

Answer 34

Educate patients * Colds are self-limiting and have no specific curative treatment; antibiotics do not work (viral infection) * Hygiene measures to limit the spread to others Symptomatic management * Rest, maintain fluid intake * Paracetamol or ibuprofen (for pain/fever) * Intranasal decongestant or antihistamine (not for \<6yo; caution in 6-12yo) * Little evidence of effect but SEs (e.g. drowsiness) * Limit decongestant use to 3-5d (rebound congestion)

Answer 35

Complications: * Otitis media * Acute exacerbation of asthma * Sinusitis Self-limiting → symptoms clear in 7-10d * Children may get back-to-back infections → may last longer

Answer 36

A highly contagious respiratory tract infection caused by **Bordatella pertussis**

Answer 37

Bordatella pertussis is a gram-negative bacillus which spreads through aerosolised droplets * The bacteria attach to the respiratory epithelium and produce toxins à toxins paralyse the cilia and cause inflammation à leads to impaired clearance of respiratory secretions à cough * Highly contagious à up to 90% household contacts develop the disease

Answer 38

* non-vaccination * exposure to an infected individual (esp during catarrhal phase) * Also babies born to mothers who became infected at 34wks or later

Answer 39

It is an endemic, with epidemics every 3-4yrs There is a vaccine but is still reasonably common * Most common in \>15yo but most severe in infants

Answer 40

* **Catarrhal phase:** * Lasts 1-2wks * Coryzal symptoms → mimics other URTIs so rarely diagnosed at this stage * **Paroxysmal phase:** * Lasts for 2-8wks; can last up to 3 months * There are episodes of severe paroxysms of coughing followed by an inspiratory gasp (against a closed glottis), producing the classic ‘whoop’ sound * Often worse at night; may cause vomiting; occasionally severe enough to cause cyanosis * During a paroxysm, the child goes red/blue in the face; mucus flows from the nose/mouth * Epistaxis & subconjunctival haemorrhages can occur after vigorous coughing * In infants, the whoop may be absent but apnoea is common * This is the time that complications frequently occur * **Convalescent phase:** * Symptoms gradually decrease, but cough may persist for many months * **Older children/adolescents may not have distinct stages** * Symptoms include uninterrupted coughing, feelings of suffocation, headaches

Answer 41

* **_If cough is \<2wks from onset:_** * **Culture of a nasopharyngeal aspirate or nasopharyngeal swab** * **PCR testing** of a nasopharyngeal swab can also be done in severe illness → quicker results * *Sensitivity decreases if \>2wks from cough onset* * **_If cough is \>2wks from onset:_** * **Anti-pertussis toxin IgG serology** * *NB pertussis vaccine can produce a false positive* * **FBC** * Characteristically there is **marked** **lymphocytosis** (\>15x10⁹/L)

Answer 42

Indications for hospital admission: * \<6 months of age and acutely unwell * Significant breathing difficulties, e.g. apnoeic episodes, cyanosis, respiratory distress, severe paroxysms * Feeding difficulties, vomiting with dehydration and weight loss * Significant complications, e.g. pneumonia, seizures Antibiotics: * Eradicate the organism and reduce infectivity; only decrease symptoms if started during the catarrhal phase * Macrolides * Azithromycin or clarithromycin * Co-trimoxazole (trimethoprim/sulfamethoxazole) is 2^nd line (if macrolides not tolerated/resistance) * Close contacts should receive macrolide prophylaxis Further management is supportive: * Paracetamol and ibuprofen * Adequate fluid intake * Isolate children (if admitted to hospital); avoid nursery/school until they have had cough for 21d or antibiotics for 5d * Notifiable disease

Answer 43

* Given at 2, 3 and 4 months of age, with a booster at 3yrs and 4months * Unimmunised infant contacts should be vaccinated * Does not guarantee protection but reduces risk and severity; level of protection declines during childhood (but pertussis is much milder in adolescents and adults) * Reimmunization during pregnancy in UK à reduces risk in the infant during the first few months of life

Answer 44

Complications: * **Otitis media** (most common) * **Pneumonia** (20%) * May be primary infection from Bordatella pertussis or secondary to other organisms * **Seizures** * Due to cerebral hypoxia secondary to severe paroxysms of cough * **Apnoea** (esp in infants) * **Bronchiectasis** * **Rib fracture** (more common in adolescents/adults) **_Higher risk of complications and mortality in unvaccinated children \<6mo (3.5% mortality)_**

Answer 45

A common **viral** respiratory disease of childhood, characterised by the **sudden** **onset** of a **barking cough**, often accompanied by **stridor**, voice **hoarseness** and **respiratory distress** Aka laryngotracheobronchitis

Answer 46

Most commonly caused by **parainfluenza virus (types 1 or 3)** * Other causes are **rhinovirus, RSV & influenza** There is generalised inflammation and oedema of the airways, starting at the larynx, followed by the trachea and bronchi à upper airway obstruction * Children (but not adults) develop croup because children have narrower airways * This leads to croup in children (due to upper airway obstruction) because children have narrower airways than

Answer 47

Common Usually 6mo-3yo (peak incidence in 2yo; can occur in older children) Most common in late Autumn

Answer 48

* Coryza and fever * Followed by: * Barking seal-like cough (due to tracheal oedema and collapse) à cardinal feature * Hoarseness (due to inflammation of vocal cords) * Harsh stridor * Variable degree of respiratory distress * Symptoms often start, and worsen, at night; also worsen with agitation

Answer 49

* Clinical diagnosis * Occasionally AP and lateral neck X-ray → **steeple sign**

Answer 50

Determined by severity * When deciding whether to admit, also consider : * time of day, * ease of access to hospital, * child’s age (low threshold if \<12mo), * parental understanding/confidence * **Steroids are 1^st line for all severities** **_Ensure child is as calm as possible → symptoms exacerbated by crying_**

Answer 51

* Single dose of **_oral dexamethasone_** * Reduces the signs of croup within 2hrs; lasts up to 10hrs * Child can be managed at home * Advice to parents: * Observe child closely for signs of increasing severity (stridor at rest, resp failure, high temperature) * Symptoms usually resolve in 48hrs but can last 1wk * Croup is a viral illness and antibiotics are not needed * Paracetamol or ibuprofen can control pain/fever * Ensure adequate fluid intake

Answer 52

* Admit * **_Oral dexamethasone_** * If severe hypoxia, persistent vomiting or resp distress (preventing oral dose): nebulised budesonide * IM dexamethasone is another alternative * **_Nebulised adrenaline_** * For children with stridor, sternal indrawing at rest, persistent/increasing agitation * Rapid but transient improvement à gives relief while waiting for effects of corticosteroid treatment * Observe closely for 2-3hrs until effects wear off

Answer 53

* Admit * **_Oral dexamethasone or nebulised budesonide_** * **_Nebulised adrenaline_** * **Supplemental O2** (can be given with adrenaline) * **Intubation** if necessary * Indicated in children progressing to respiratory failure

Answer 54

Complications: * Upper airway obstruction * Dehydration (due to inadequate fluid intake) * Respiratory failure * Bacterial tracheitis or pneumonia (due to bacterial super-infection) Usually mild and self-limiting; steroids reduce duration

Answer 55

Swelling of the epiglottis and surrounding tissues, with the potential to cause airway compromise

Answer 56

Caused by **H. influenzae type B (Hib)** * Rarely caused b**y S. pneumoniae, S. aureus, MRSA, viruses** (e.g. parainfluenza) Inflammation → localised airway oedema → exponentially increasing airway resistance whilst narrowing the effective supraglottic aperture * The process affects the supraglottic structures (the glottis is usually not inflamed) * Further compromise can occur from secretions, potentially resulting in complete airway obstruction → life-threatening emergency

Answer 57

* non-vaccination with Hib vaccine * immunocompromise

Answer 58

Very rare in UK due to Hib vaccine (but still possible) Most common in 3-5yo; can affect all ages

Answer 59

* It is important to distinguish clinically between epiglottitis and croup (as they need different treatment) * Acute onset of symptoms (hrs) * High fever * Toxic appearance (child looks very ill) * Intensely painful throat → prevents child speaking or swallowing * Drooling saliva * Soft inspiratory stridor and rapidly increasing respiratory difficulty * Tripod position * Child sits immobile and upright, with head anterior, hands on knees and mouth open à optimises the airway

Answer 60

* Do not lie child down or examine throat with a spatula → can cause total airway obstruction (laryngospasm) * **Clinical diagnosis** (don’t delay treatment with investigations) * **Laryngoscopy** * To confirm diagnosis; also therapeutic (airway can be established at the same time) * Performed in operating theatre as an emergency * Shows swelling of supraglottic structures * **Blood cultures** **after stabilising airway** (see management)

Answer 61

**ABCDE approach**, senior help, **transfer to ITU** **MDT approach** → senior anaesthetist, paediatrician, ENT surgeon called immediately **Supplemental O2** (facemask) until intubation **_Steroids + nebulised adrenaline_** sometimes used as temporary treatment **Intubate with GA** to secure the airway * In theatre * Laryngoscopy often performed before intubation * If this is impossible à tracheotomy **After securing airway:** * **FBC** * **Blood culture** * **IV antibiotics** (**_cefotaxime or ceftriaxone_**) Tracheal tube can usually be removed after 24hrs; antibiotics usually given for 3-5d **Prophylaxis** with **_rifampicin_** is offered to **close household contacts**

Answer 62

Complications: * Airway obstruction → death * Pneumonia (mostly due to intubation; could possibly be due to spread of infection seeding the lower airway) With treatment, most children recover in 2-3d; high mortality and complications if untreated

Answer 63

Congenital abnormality of the larynx cartilage that predisposes to dynamic supraglottic collapse during the inspiratory phase of respiration

Answer 64

Supraglottic collapse during inspiration leads to intermittent upper airway obstruction and stridor * Aetiology is not fully understood, but may involve: * Anatomical abnormalities (e.g. short aryepiglottic folds, curled ‘omega-shaped’ epiglottis) * Immature neural pathways, resulting in neuromuscular incoordination or hypotonia of the supralaryngeal airway * Inflammation and oedema from GORD

Answer 65

* GORD, * neurological dysfunction (hypotonia, cerebral palsy), * laryngeal anatomical abnormalities, * Down’s

Answer 66

Most common source of stridor in infants, and most common congenital laryngeal abnormality

Answer 67

* **Stridor** * Present intermittently and variable in intensity; worsens with crying, activity, lying supine, feeding * Loudness does not correlate with clinical severity * Becomes apparent within 2wks of birth; gradually resolves by 2yrs of age * **Signs of airway obstruction** → nasal flaring, intercostal/subcostal recession, abdominal effort, tracheal tug * NB patients are usually systemically well and comfortable; cyanosis is not usually a feature * **Feeding difficulties** * May develop increased obstruction during feed (seem to run out of breath/cough) à limits intake * May lead to failure to thrive and poor weight gain * **Normal cry**

Answer 68

* Mild * Audible stridor and endoscopic features of laryngomalacia, but no respiratory distress or failure to thrive * Moderate * Stridor, increased work of breathing, progressive feeding difficulties and weight loss/inadequate weight gain * Severe * Considered if patient becomes compromised by airway obstruction or if normal growth is prevented

Answer 69

* **Flexible laryngoscopy** * **_Done in all patients_** * To assess laryngeal anatomy and related comorbidity (e.g. mucosal inflammation due to GORD) * May show collapse of epiglottis into glottic airway on inspiration, narrowing/obstruction of supraglottic airway, or short aryepiglottic folds * **Rigid laryngoscopy can be performed if subglottic views are needed → exclude co-existing conditions**

Answer 70

Depends on severity Mild disease: * Observation and reassure of high likelihood of spontaneous improvement (1^st line) * GORD treatment if necessary Moderate disease: * Observation and reassurance (1^st line) * Monitor closely for signs of airway obstruction/feeding difficulties (growth chart) * Treat GORD and minor feeding difficulties * Surgical treatment * Considered if patient becomes compromised by airway obstruction or if normal growth is prevented * May involve partial removal of epiglottis, or removal of aryepiglottic cords Severe disease: * Surgery (1^st line) * GORD therapy * BiPAP for obstructive sleep apnoea if surgery failed to resolve

Answer 71

Complications: * GORD exacerbation * Life-threatening airway obstruction (rare) * Failure to thrive (due to feeding difficulties) * Aspiration (due to feeding difficulties) * Pulmonary hypertension (in severe disease with hypoxaemia) Most severe at 6-8mo; improves spontaneously by 12-24mo

Answer 72

An acute viral infection of the bronchioles (the smallest air passages in the lungs)

Answer 73

Usually caused by **respiratory syncytial virus (RSV) (**80%) * Less commonly, parainfluenza virus, rhinovirus, adenovirus, influenza virus, human metapneumovirus * Co-infection with more than one virus, esp RSV and human metapneumovirus, leads to a more severe illness

Answer 74

* Bronchioles are the airways which are \<2mm wide and do not contain cartilage or submucosal glands * The virus infects the respiratory epithelial cells of the bronchioles à leads to mucus secretion, inflammation, oedema and necrosis (so increased cells in the bronchioles) * This leads to a ball-valve effect (obstruction of small airways) à hyperinflation, increased airway resistance, atelectasis (lung collapse) and V/Q mismatch à symptoms * Almost all infants have been infected with RSV by 3yo but only a minority develop bronchiolitis à host and environmental factors play a role in pathogenesis

Answer 75

* infants \<3yo, * November-May, * passive tobacco exposure, * not breastfed, * immunodeficiency RFs for severe disease: * prematurity, * bronchopulmonary dysplasia, * other chronic lung disease (e.g. CF), * congenital heart disease, * immunodeficiency

Answer 76

Most common LRTI in infants à 3% are admitted to hospital each year in annual winter epidemics It almost exclusively occurs in infants (90% are 1-9mo); very rare after 3yo

Answer 77

* **Coryzal symptoms**, followed by **dry wheezy cough and increasing breathlessness** * Coryzal symptoms usually precede bronchiolitis by several days * **Low-grade fever** (usually in first few days of illness) * **Signs of respiratory distress** * **Feeding difficulty, dehydration** * **Recurrent apnoea** (if severe) – may be the only presentation Signs: * **Fine end-expiratory crackles**, high-pitched wheeze * Clinical findings often fluctuate within short time periods à hallmark of bronchiolitis

Answer 78

* Pulse oximetry * No other Ix are routinely recommended * Rapid antigen test for RSV * If resp failure is suspected à CXR, blood gases

Answer 79

Hospital admission is indicated if any of the following are present: * Severe respiratory distress à grunting, marked chest recession, RR \>70, cyanosis * Persistent O2 sats \<90% on air * Apnoea (observed or reported) * Inadequate oral fluid intake (50-75% of usual volume) * Bradycardia (because hypoxaemia can inhibit the CVS) * Excessive sleepiness (may indicate impending resp arrest if due to hypoxia/hypercapnia) * (Concerns re social history)

Answer 80

**Supportive management:** * Monitor for apnoea * Humidified O2 via nasal cannulae or head box * Fluids if needed (NG or IV) * CPAP or mechanical ventilation (if needed) **_Ribavirin_**: * Used for severe disease or RFs (e.g. severe chronic lung disease, immunodeficiency) **Prophylactic** **_palivizumab_**: * Monoclonal antibody against RSV * Given to infants at high risk of RSV infection (premature, BPD, CF, immunodeficiency

Answer 81

Complications: * Recurrent apnoea during infection * Fluid overload (in hospitalised infants, due to inappropriate ADH secretion) * Recurrent episodes of cough and wheeze (50%) * Repeat infection (primary infection with RSV does not confer protective immunity) * Bronchiolitis obliterans: rare complication causing permanent damage to airways, usually following adenovirus infection In general it is a mild, self-limiting condition with recovery within 2wks

Answer 82

Inflammation of the lung parenchyma with consolidation or interstitial lung infiltrates

Answer 83

Pathogens: * Newborn: * Organisms from mother’s genital tract: GBS, E. coli, chlamydia trachomatis, gram-negative bacilli * Infants and young children: * Respiratory viruses (most common): RSV, influenza, parainfluenza, adenovirus, rhinovirus * Bacterial causes: S. pneumoniae, Chlamydia trachomatis, S. aureus (uncommon but serious – can cause superinfection after viral pneumonia) * Hib and Bordatella pertussis if unvaccinated * Children \>5yo: S. pneumoniae, Mycoplasma pneumoniae, Chlamydophila pneumoniae * Mycoplasma pneumoniae is seen in older children/adolescents in close settings (boarding school, university) * All ages: Mycobacterium tuberculosis * Immunocompromised: * Viral: CMV, VZV, HSV, measles, adenovirus * Bacterial: PCP * NB viruses are most common in younger children; bacteria are most common in older children Pneumonia develops due to the invasion and overgrowth of a pathogenic micro-organism in the lung parenchyma, which produces intra-alveolar exudates * Pathogens can reach the lower respiratory tract by inhalation, aspiration of oropharyngeal secretions into trachea, haematogenous spread and direct extension from adjacent infected foci

Answer 84

* chronic lung disease (preterm, BPD, CF), * congenital heart disease, * chronic aspiration (cerebral palsy, TOF, GORD)

Answer 85

Incidence peaks in infancy and old age, but is relatively high in childhood Incidence in UK has decreased since Hib vaccine

Answer 86

* Signs of respiratory distress * Wheeze * Cough (may be productive in older children) * Fever * “Unwell child”, lethargy * Poor feeding * On examination: * End-expiratory crackles * Classic signs of consolidation (dull percussion, decreased breath sounds, bronchial breathing) are often absent in young children Clinical features cannot reliably distinguish between viral or bacterial, but bacterial should be considered especially when there is persistent fever \>38.5⁰C, chest recession and raised RR * There may be some features associated with individual causative agents: * **Pneumococcal**: starts with fever and tachypnoea, cough is not usually an initial feature, rusty-coloured sputum * **Mycoplasma**: cough, chest pain and wheezing * Non-respiratory symptoms are more common, e.g. arthralgia, headache * **Staphylococcal** pneumonia: may follow influenza infection

Answer 87

* Pulse oximetry * Further Ix are only needed for children admitted to hospital (not if managed in community) * CXR * Confirms diagnosis à consolidation * Can’t differentiate between viral or bacterial, but focal consolidation suggests bacterial and diffuse suggests viral * Nasopharyngeal aspirate/swabs * Then culture and PCR to determine cause (but often just shows what is in the nose – unhelpful) * Used because sputum culture cannot be done in young children * Bloods: blood culture, FBC, CRP * Urine antigen tests for pneumococcus

Answer 88

Children with mild-moderate pneumonia can be managed in the community; indications for admission are: * O2 sats \<92% * Cyanosis * Tachypnoea (\>70/min in an infant, \>50 in an older child), recurrent apnoea, grunting * Significant tachycardia for level of fever * Inability to maintain adequate fluid/feed intake * Other chronic conditions, e.g. congenital heart disease, CF, chronic lung disease of prematurity

Answer 89

Supportive management: * Give parents info and safety net if managing at home; ensure they have access to hospital if needed * Paracetamol or ibuprofen * Prevent dehydration à maintain fluid intake, IV fluids if needed * Maintain O2 \>92% (nasal cannulae, face mask, head box) Antibiotics: * For all children with pneumonia (as bacterial and viral cannot be reliably distinguished clinically) à start broad-spectrum antibiotics early until microbiology results are available * Antibiotic choice: * Amoxicillin is 1^st line for all ages * Broader-spectrum, e.g. co-amoxiclav, given for complicated or unresponsive pneumonia * Macrolide (e.g. erythromycin) can be added at any age if there is no response to 1^st line therapy, severe disease, or suspicion of Mycoplasma/Chlamydia pneumonia * Co-amoxiclav is recommended in pneumonia associated with influenza * Metronidazole for aspiration pneumonia * Oral antibiotics for mild-moderate * E.g. oral amoxicillin +/- oral erythromycin * IV antibiotics if oral cannot be tolerated, or signs of severe pneumonia/septicaemia E.g. IV co-amoxiclav or cefuroxime

Answer 90

Complications: * Pleural effusion * Small parapneumonic effusions occur in 1/3 * May resolve with antibiotics, but persistent fever despite 48hrs of antibiotics suggests collection that requires drainage à may develop into empyema * Lung abscess (rare) * Septicaemia and metastatic infection (e.g. osteomyelitis) * HUS * Bronchiectasis Prognosis is very good for children with simple consolidation on CXR and who recover fully * Those with evidence of lobar collapse/atelectasis should have a repeat CXR in 4-6wks

Answer 91

Autosomal recessive disease caused by a mutation in the CFTR gene, resulting in multisystem dysfunction

Answer 92

Caused by a defective protein called the CF transmembrane conductance regulator (CFTR) * The gene for CFTR is located on ch7 à there are over 900 different gene mutations * Mutations are classified into 6 classes (1 is most severe) * Ranges from no complete CRTR proteins (I) to shortened half-life on the membrane (VI) * Phe508del (ΔF508) is the most common mutation in UK * It is class II: misfolded CFTR proteins are formed à most are degraded

Answer 93

* The CFTR protein is a chloride channel found in numerous epithelial tissues * It is cyclic AMP-dependent à drives chloride against its concentration gradient using ATP * Mutations lead to reduced chloride transport, accompanied by reduced sodium and water transport across epithelial cells à thick, sticky secretions à obstruction * Mutations in CFTR affect multiple systems: * Respiratory tract: * Lung is normal at birth, but as it matures, thick mucus is formed in the small airways and mucous gland hyperplasia occurs * This impedes mucus clearance à mucus retention, chronic infection (as provides a niche for bacterial growth), inflammation à destruction of lung tissue * Children with CF are predisposed to infection à most commonly S. aureus, H. influenzae, Pseudomonas * Pseudomonas can cause chronic infection (due to biofilm formation à protected from immune system/antibiotics) * Ultimately leads to bronchial damage, bronchiectasis an abscess formation * Pancreas: * Pancreatic duct is usually occluded in-utero à permanent damage to exocrine pancreas * Patients with CF are usually ‘pancreatic insufficient’ (90%) * Deficient in lipase, amylase and proteins à malabsorption à faltering growth * Over time, the endocrine pancreas is affected à CF-related DM (30% of those \>10yo) * GI tract: * Viscous secretions can cause bowel obstruction in utero à meconium ileus (blockage due to thick meconium) * CF can cause cholestasis in the biliary tree in-utero à neonatal jaundice * Later in life, can cause distal intestinal obstruction syndrome (DIOS) and CF-related liver disease (from the same mechanisms) * Reproductive tract: * 98% men with CF are infertile due to congenital absence of the vas deferens * Women are not necessarily infertile, but pregnancy can causes deteriorating lung health

Answer 94

* Neonates: * Meconium ileus (10%) * Abdo distension, delayed passage of meconium, bilious vomiting in the first days of life * May progress to bowel perforation * Failure to thrive * Prolonged neonatal jaundice * Infancy/young children: * Failure to thrive * Recurrent chest infections * Steatorrhoea, increased appetite (if pancreatic insufficiency) * Older children/adolescents: * Recurrent chest infections, ABPA, asthma * Chronic lung disease (e.g. bronchiectasis) * Nasal polyps * DM, steatorrhoea, increased appetite (if pancreatic insufficiency) * DIOS, gallstones, liver cirrhosis * There may be no signs, or they may include: * Finger clubbing * Hyperinflated chest (due to air trapping), coarse inspiratory crepitations, expiratory wheeze * Signs of malnutrition, reduced muscle mass

Answer 95

* Neonates: * Meconium ileus (10%) * Abdo distension, delayed passage of meconium, bilious vomiting in the first days of life * May progress to bowel perforation * Failure to thrive * Prolonged neonatal jaundice * Infancy/young children: * Failure to thrive * Recurrent chest infections * Steatorrhoea, increased appetite (if pancreatic insufficiency) * Older children/adolescents: * Recurrent chest infections, ABPA, asthma * Chronic lung disease (e.g. bronchiectasis) * Nasal polyps * DM, steatorrhoea, increased appetite (if pancreatic insufficiency) * DIOS, gallstones, liver cirrhosis * There may be no signs, or they may include: * Finger clubbing * Hyperinflated chest (due to air trapping), coarse inspiratory crepitations, expiratory wheeze * Signs of malnutrition, reduced muscle mass

Answer 96

* Newborn screening: immunoreactive trypsinogen (IRT) test * For all newborns in UK in Guthrie test * NB this is just screening à not diagnostic * High IRT suggests CF à do gene mutations and sweat test * Chloride sweat test * Measures electrolyte (Cl-) concentration in sweat à \>60 suggests CF; 40-60 is borderline (repeat) * Single sweat test is not sufficient to diagnose CF (errors common due to inadequate volume) * 2^nd test or identification of a genetic mutation confirms diagnosis * Genetic analysis * To confirm diagnosis * If no mutations identified à 2 sweat tests and fitting history used for diagnosis * Monitoring for complications (see management)

Answer 97

**MDT approach** * GP, respiratory paediatrician, specialist CF nurse, dietitian, physiotherapist, psychologist, social worker * Patient and family education * Explain diagnosis, written support and info * Must understand it is a life-limiting condition that needs frequent follow-up, but can be managed * Annual review: * With respiratory paediatrician, dietician, physiotherapist, CF specialist nurse * Review of clinical symptoms and antibiotics taken * Investigations: * Bloods: FBC, U&Es, LFTs, clotting studies, vitamin A/D/E * Lung function tests (FEV1 declines with disease severity) * CXR * Microbiological assessment (at every clinical encounter) – e.g. sputum sample * OGTT (if \>12yo) * Bone profile * Faecal elastase (low in pancreatic insufficiency) * Prepare to transfer to adult CF services during late adolescence **Respiratory management:** * Twice-daily physiotherapy is usually needed à increases airway secretion clearance * Inhaled mucolytics and DNase: * DNase (dornase alpha) is inhaled à reduces viscosity of mucus by digesting DNA * Hypertonic saline (inhaled) can aid airway clearance, and is often used at the time of physiotherapy to further aid clearance * Salbutamol PRN * Management/prevention of infection: **Nourishment and exercise:** * Encourage exercise à usually able to do as well as their peers if symptoms are controlled * If pancreatic insufficiency: * Pancreatic enzyme supplementation (Creon) * Take with meals which contain fat * Fat-soluble vitamins (A, D, E, K) are poorly absorbed (despite enzyme replacement) à supplement * Monitor growth * Metabolic demands may be greater in CF patients à may lead to growth faltering * High-calorie diet à 150% normal * Build-up milkshakes or overnight gastrostomy (supplemental enteral feeding) if necessary **CFTR modulators:** * Drugs which aim to correct the abnormality in the protein; very expensive so limited availability * CFTR potentiators (e.g. ivacaftor) are helpful in restoring function of CFTR in class II/III mutations * CFTR correctors (e.g. lumifactor) can partially restore number in class II defects (used in ΔF508)

Answer 98

* Continual microbiological assessment to identify organisms colonised * Infections treated with \>2wks antibiotics, even if asymptomatic (high-dose) * If symptomatic, start empirical antibiotics before culture results * Management of mild exacerbation: * Oral co-amoxiclav/co-trimoxazole/ciprofloxacin for 14d * Inhaled tobramycin if no response * Increased chest physiotherapy, inhaled mucolytic (DNase/hypertonic saline), inhaled salbutamol * Management of moderate/severe exacerbation: * IV tobramycin + ticarcillin/tazocin/meropenem/linezolid/vancomycin/ceftazidime * Aminoglycoside (tobramycin) combined with 1 or 2 antibiotics with Staphylococcus or Pseudomonas coverage, depending on suspected colonisation and severity * S. aureus: ticarcillin/clavulanic acid; MRSA: linezolid and vancomycin * Pseudomonas: ticarcillin/clavulanic acid, ceftazidime or tazocin * Increased chest physiotherapy, inhaled mucolytic (DNase/hypertonic saline), inhaled salbutamol * Repeat cultures sent after treatment to ensure organism is eradicated * Chronic pseudomonas treated with long-term inhaled tobramycin to suppress growth * Prophylactic regular azithromycin can be given to reduce exacerbations, even in those not chronically infected with Pseudomonas (has anti-inflammatory properties) * Infection control: * Pseudomonas-naïve patients attend different clinics to those with chronic infection * When admitted, patient should be in a side room * In end-stage CF lung disease, bilateral sequential lung transplantation is the only therapeutic option

Answer 99

* ABCDE approach * Lactulose * NG decompression if still obstructed * If complete obstruction or peritonitis à surgery

Answer 100

**_Complications:_** * **Respiratory tract:** * Allergic bronchopulmonary aspergillosis (ABPA) * Bronchiectasis * Pulmonary HTN and R heart strain à cor pulmonale * Respiratory failure eventually occurs * **GI tract:** * Rectal prolapse (due to frequent passage of bulky stools – manage by ensuring child has adequate pancreatic enzyme replacement and laxatives) * Distal intestinal obstruction syndrome (DIOS): * Obstruction of distal ileum by viscid mucofaeculent material * Affects 10% children with CF * Presents with colicky abdo pain and palpable mass in RLQ * Usually cleared with oral laxatives * Intussusception * CF-related liver disease: * Cholestasis * Gallstones * Liver cirrhosis * May progress to liver failure à liver transplant * **Endocrine:** * CF-related diabetes (CFRD): * Associated with rapid decline in lung function and disease progression * Presents anorexia, weight loss and fall in lung function * Ketoacidosis is rare because there is not a complete lack of insulin * Should be screened annually for CFRD if \>12yo; then annual retinopathy, hypertension and microalbuminuria screening if they have it * Delayed puberty: * Common; average delay 2yrs * May lead to reduced bone mineral density * **Subfertility in males** * Males can have children via intracytoplasmic sperm injection * Psychological repercussions on children and families or frequent hospital appointments, absences from school, etc. **Life expectancy is in the 40s** * Lung disease is the most common cause of morbidity and mortality

Answer 101

Pharyngitis: inflammation of the pharynx and soft palate, causing a sore throat Tonsillitis: inflammation of the palatine tonsils * Often considered a form of pharyngitis (the two are termed ‘acute sore throat’)

Answer 102

Pharyngitis: inflammation of the pharynx and soft palate, causing a sore throat Tonsillitis: inflammation of the palatine tonsils * Often considered a form of pharyngitis (the two are termed ‘acute sore throat’)

Answer 103

Pharyngitis and tonsillitis: * Usually viral à adenoviruses, enteroviruses, rhinoviruses, EBV (infectious mononucleosis – esp tonsillitis), influenza, parainfluenza * Group A Streptococcus (‘strep throat’), esp in older children (15-30%) * Chlamydia or gonorrhoea can also cause pharyngitis in sexually-active adolescents

Answer 104

Pharyngitis and tonsillitis: * Most common in winter/early spring * Bacterial (GAS) is more common in winter/early spring; enterovirus is more common in summer/autumn * GAS represents \<1/3 * Most common in schoolchildren (5-15yo)

Answer 105

* Sore throat * Often causes pain on swallowing (odynophagia) à reduced oral intake * Fever * Rhinorrhoea, nasal congestion, cough * Most common in viral infection; absence of cough predicts GAS aetiology * Headache, nausea, vomiting, abdo pain * Most common in bacterial * Mononucleosis: * Pharyngitis with prolonged malaise and prominent lymphadenopathy * On examination: * Cervical lymphadenopathy * More common in bacterial * Tonsillar enlargement (in tonsillitis) * Pharyngeal/tonsillar exudate * Most common in bacterial Exudates in EBV are more membranous than bacterial (but can’t really tell

Answer 106

* Calculate Centor score * Estimates probability that pharyngitis is due to bacteria à guides use of rapid strep testing and antibiotics * 4/5 make bacterial infection more likely * NB 4/5 points give 50% chance of GAS; 3 gives 35% * Rapid antigen test for GAS * In patients with 4/5 Centor score * Immediate testing à 70-90% sensitive; 95% specific * Positive testing in the absence of symptoms is likely just colonisation * Culture of throat swab for GAS * For children who are negative for GAS via rapid antigen testing, but have clinical signs consistent with GAS * If suspicious of EBV: * FBC (high WCC with lymphocytosis and atypical lymphocytes) * Consider serum monospot

Answer 107

* Supportive management * Paracetamol or ibuprofen * Rest, maintain fluid intake * Antibiotics * If GAS is confirmed on rapid antigen test/throat cultures or severe infection * E.g. penicillin (erythromycin if penicillin allergy) for 10d * Speed recovery from GAS by 1 day (but also prevent rheumatic fever) * Avoid amoxicillin à widespread maculopapular rash if due to infectious mononucleosis * If severe/unable to swallow/airway obstruction: * Hospital admission, IV fluids * IM dexamethasone (for airway obstruction) * Tonsillectomy and adenoidectomy: * Tonsils and adenoids increase in size until 8yo and then gradually regress * Adenoids grow proportionally faster than the airway à effect of narrowing the lumen of airway is greatest from 2-8yrs à may cause enough narrowing to justify adenoidectomy * Indications for tonsillectomy include: * Recurrent severe tonsillitis (as opposed to recurrent URTIs) * \>7/yr for 1yr, \>5/yr for 2yrs or \>3/yr for 3yrs * Tonsillectomy reduces the number of episodes by 1/3; doesn’t help mild symptoms * Peritonsillar abscess (quinsy) * Indications for the removal of both tonsils and adenoids are: * Recurrent otitis media with effusion and hearing loss * Obstructive sleep apnoea

Answer 108

Laryngitis: inflammation of the larynx, which can lead to oedema of the vocal cords

Answer 109

* Inflammation of endolaryngeal structures causes tissue oedema and erythema à damage to vocal cords and narrowing of airway in children (seen in croup and epiglottitis) * Usually viral (self-limiting, related to a URTI) * Rhinovirus (most common), RSV, adenovirus, influenza, parainfluenza * Can be bacterial (more serious – see epiglottitis) * Hib, Moraxella, S. pneumoniae * Non-infectious causes include reflux laryngitis and vocal strain (e.g. singers)

Answer 110

* Rhinovirus peaks are in autumn and spring; influenza peaks are in winter and early spring

Answer 111

* Hoarseness à most characteristic symptom * Sore throat, dysphagia and odynophagia * Fever * Coryzal symptoms * Redness of oropharynx

Answer 112

* Clinical diagnosis * Laryngoscopy can be considered à gold standard for diagnosis * Oropharyngeal swabs if bacterial infection is suspected

Answer 113

* Supportive management * Paracetamol or ibuprofen * Voice rest (3-7d), increased hydration, humidification * Antibiotics if bacterial suspected (penicillin for GAS) Management of severe symptoms → see epiglottitis

Answer 114

* Sinusitis * Otitis media * Peri-tonsillar/neck abscess (quinsy) – in tonsillitis * Scarlet fever * Rheumatic fever: * Rare complication of GAS * Presents as polyarthritis, carditis, subcutaneous nodules, erythema marginatum and chorea * Glomerulonephritis: * Autoimmune complication occurring 1-3wks after GAS infection * Manifests with haematuria and oedema * Most patients recover without long-term renal impairment * Complications of EBV: hepatosplenomegaly, splenic rupture (avoid contact sports) Usually self-limiting and resolves in a few days Infected individuals are not immune to reinfection

Answer 115

* Occasional complication of group A strep infection * Most common in 5-12yo * Symptoms: * Fever precedes headache and tonsillitis by 2-3d * ‘Sandpaper-like’ maculopapular rash with flushed cheeks and perioral sparing * Tongue is often white and coated; may be sore or swollen * Treat with antibiotics (penicillin or erythromycin) * Complications include glomerulonephritis and rheumatic fever (rare in developed countries)

Answer 116

Symptomatic inflammation of the mucosal lining of the nasal cavity and paranasal sinuses

Answer 117

Usually viral but can be bacterial * Following an episode of viral sinusitis, 1% have secondary bacterial infection (due to oedema and mucous production blocking normal ventilation and drainage of the sinus) * The most common bacterial causes are S. pneumoniae, H. influenzae and Moraxella catarrhalis Usually affects paranasal and maxillary sinuses * Frontal sinuses don’t develop until late childhood à frontal sinusitis is uncommon in the first decade of life

Answer 118

* Facial pain/pressure * Purulent nasal discharge * Nasal congestion * Dental pain (in maxillary sinusitis) * Coryzal symptoms * In secondary bacterial infection, symptoms may worsen after an initial improvement

Answer 119

* Clinical diagnosis

Answer 120

Symptomatic management * Rest and hydration * Paracetamol and ibuprofen * Decongestant (e.g. oxymetazoline nasal) * Intranasal corticosteroid Antibiotics: * If bacterial cause is suspected * Amoxicillin/co-amoxiclav * May wait for up to 10d before starting antibiotics à most cases resolve without them * But have a lower threshold to give antibiotics in children than adults (because higher risk of complications) If recurrent episodes à refer to ENT

Answer 121

Complications: * Chronic sinusitis (\>12wks) * Bacterial meningitis (due to direct extension) * Periorbital or orbital cellulitis * Periorbital occurs most commonly in children \<3yo; does not affect vision * Orbital occurs most commonly in children \>3yo; causes visual impairment Usually self-limiting and resolves within 1 month * Complications are more common in children than adults

Answer 122

Infection involving the middle ear space

Answer 123

May be viral or bacterial * Viral pathogens are most common à include RSV and rhinovirus * Bacterial pathogens include S. pneumoniae, non-typable H. influenzae (not Hib) and Moraxella catarrhalis * Co-infections with virus and bacteria may occur Common complication of viral respiratory illnesses * Upper respiratory tract viruses can infect the nasal passages, eustachian tube and middle ear à inflammation à impaired mucociliary action and ventilatory function in the eustachian tube * Middle ear effusion develops à bacterial co-infection (if they contaminate the effusion) * Inflammatory response causes pressure against the tympanic membrane à pain and fever Infants and young children are prone to otitis media because their Eustachian tubes are short, horizontal and function poorly

Answer 124

Most children will have at least 1 episode Most common at 6-18 months

Answer 125

* Preceding upper respiratory symptoms * Otalgia (ear pain) * Fever * Young children may present with non-specific symptoms: of irritability, sleep disturbance, decreased feeding * Otorrhoea in tympanic membrane perforation

Answer 126

* Every child with fever must have their tympanic membrane examined * Bright red, bulging tympanic membrane, with loss of the normal light reflection * Occasionally there is acute perforation of the tympanic membrane with pus in the external canal

Answer 127

Symptomatic management * Paracetamol or ibuprofen Antibiotics: * Amoxicillin 10d * Not usually needed; may be started after 2-3d if symptoms haven’t improved * May give the parents a prescription but ask them to only use it if child remains unwell after 2-3d * Marginally shorten the duration of pain; don’t reduce the risk of hearing loss Tympanocentesis * Considered if unresponsive to antibiotics or if there is severe pain

Answer 128

Complications: * Otitis media with effusion (aka glue ear) * Due to recurrent acute otitis media * Very common between 2-7yo * Usually asymptomatic apart from possible decreased hearing * Eardrum is dull and retracted, often with a fluid level visible * Usually resolves spontaneously, but may cause conductive hearing loss (interfering with normal speech and development) * Grommets (ventilation tubes) can be inserted for conductive hearing loss * Usually only last 12 months * Perforated tympanic membrane * Treat with antibiotics; usually heals quickly * Bullous myringitis * Bullae may develop on tympanic membrane; treat with antibiotics * Mastoiditis * When bacterial otitis media extends into the mastoid air cells * Treat with parenteral antibiotics Usually resolves spontaneously over 2-3d

Answer 129

Diffuse inflammation of the external ear canal, which may also involve the pinna or tympanic membrane

Answer 130

A form of cellulitis that involves the skin and subdermis of the external auditory canal, with acute inflammation and variable oedema Usually caused by bacterial infection à most commonly Pseudomonas and S. aureus (but often polymicrobial) * May also be caused by trauma (from scratching), swimming or skin disease (eczema)

Answer 131

* Ear pain and tragal tenderness * Otorrhoea * Itchiness of the ear * On examination: * Skin of the external auditory canal appears erythematous and swollen * Tympanic membrane may be erythematous à can make differentiation from otitis media difficult

Answer 132

* Clinical diagnosis * Pneumatic otoscopy can be considered * Determines mobility of tympanic membrane in response to pressure changes * Normal in otitis externa, but decreased movement if combined with otitis media * Consider swabs and culture if doesn’t respond to antibiotics

Answer 133

Antibacterial otic drops * Ciprofloxacin/dexamethasone is 1^st line * Prior to use, clear the ear canal of any debris or wax * If no response à oral ciprofloxacin Paracetamol and ibuprofen

Answer 134

Complications: * Contact dermatitis from otic drops * Osteomyelitis of the skull base (rare) Usually resolves within 7-10d of treatment

Answer 135

A chronic respiratory disorder characterised by variable airway inflammation, airway obstruction and airway hyper-responsiveness

Answer 136

* **Viral episodic wheezing/viral induced wheeze** → wheeze only in response to viral infections * Most common cause of wheeze in preschool children * Due to small airway narrowing as a result of immune response to viral infection à wheezing only occurs when child has a cold (episodic) * Usually resolves by 5yo (probably due to increased airway size) * **Multiple trigger wheeze** → wheeze in response to multiple triggers * Occurs in preschool and school aged children * Wheeze is triggered by many stimuli, e.g. viruses, cold air, dust, animal dander, exercise * A useful diagnosis in preschool age group, where a formal diagnosis of asthma is not usually justified * A significant proportion go on to have asthma * **Asthma** * Recurrent wheezing is associated with symptoms between viral infections (interval symptoms), caused by a variety of triggers * Can be atopic (i.e. due to allergens such as house dust-mite) or non-atopic (e.g. due to exercise) * Not usually diagnosed in preschool children

Answer 137

* Susceptible individuals have an exaggerated response to various stimuli * Common triggers are URTIs, cold air, exercise, atmospheric pollution, NSAIDs, beta-blockers, allergens (house dust mite, grass pollen, etc.), emotional upset * Classical allergic asthma is driven by Th2 T cells * Allergens are presented to Th2 T cells by dendritic cells à activation of Th2 cells * This leads to a disproportionate immune response à cytokine release activates the humoral immune system à proliferation of mast cells, eosinophils and dendritic cells * Cytokines released by these cells contribute to inflammation and bronchoconstriction * Other mediators further exacerbate the situation by causing exudate production, e.g. histamine released from mast cells * Bronchial inflammation consists of: * Oedema * Excessive mucous production * Infiltration with cells (eosinophils, mast cells, neutrophils, lymphocytes) * The bronchial inflammation leads to bronchial hyperresponsiveness à exaggerated ‘twitchiness’ to inhaled stimuli à causes reversible bronchoconstriction à airway narrowing * The combination of oedema, mucous, cellular infiltration and bronchoconstriction lead to the symptoms (wheeze, cough, breathlessness, chest tightness) * Late phase response can occur (after 2-4hrs, effect may last 3-6 months) * Persistent bronchoconstriction due to vicious cycle of inflammation, oedema and excess mucus

Answer 138

* FHx of asthma/atopy, * prematurity, * parental smoking, * viral bronchiolitis in early life

Answer 139

* **Wheeze** * Parents have varying definitions of wheeze à ask them to describe it * “whistling in chest when breathing out” * **Dry cough** * **Breathlessness, chest tightness** * **Symptoms worse at night and in early morning →** may cause difficulty sleeping * **Coryzal symptoms** (in viral induced wheeze)

Answer 140

* Usually normal between acute episodes, but look for: * Generalised **polyphonic expiratory wheeze** with a **prolonged expiratory phase** * Younger children may have **widespread expiratory creps** due to mucous * **Hyperinflated chest →** suggests long-standing poorly-controlled asthma * **Harrison’s sulci** (depressions at the base of the thorax associated with muscular insertion of the diaphragm) à associated with long-standing poorly-controlled asthma * **Evidence of eczema and allergic rhinitis**

Answer 141

* **Clinical diagnosis** * Parental description and response to treatment (i.e. if suspecting asthma à start bronchodilators to see if they work) * **Peak expiratory flow rate** * Most children \>5yo can use a peak flow meter or do spirometry * Less sensitive than spirometry but portable so good for serial measurements * Poorly controlled asthma: variability in peak flow, with diurnal variability (morning lower than evening), day-to-day variability * Peak flow diary * **Spirometry:** * In children \>5yo * Usually normal in-between exacerbations, but there may be an obstructive pattern if poorly controlled (FEV1:FVC \<70%) à improvement after bronchodilators (\>12%) suggests asthma * **Plot growth** * Usually normal unless asthma is very severe * **Other investigations less commonly used:** * **Skin prick testing** for common allergens (which may be triggers) * **CXR** (usually normal; performed as baseline/to exclude other DDx)

Answer 142

* Uncontrolled asthma is defined as 3 or more days a week with symptoms/use of SABA or 1 or more nights a week awakening due to asthma

Answer 143

* **SABA: salbutamol** * Rapid onset of action (max effects after 10-15mins), effective for 2-4hrs * Taken as needed * **ICS: budesonide** * Preventer therapy à decrease airway inflammation * Doses: low: \<200ug; medium: 200-400ug; high: \>400ug * No SEs in low-dose * May cause mild reduction in height velocity (usually caught up) * At high doses/oral: impaired growth, adrenal suppression, altered bone metabolism * **LTRA: montelukast** * Oral, once daily * **LABA: salmeterol, formoterol** * Effective for 12hrs * **MART**: * Combined ICS and LABA in a single inhaler, used for maintenance and reliever therapy

Answer 144

Non-pharmacological measures: * Monitor growth with growth charts (esp if regular corticosteroids) * Avoid triggers if possible * Allergen immunotherapy à sometimes used for atopic asthma due to a single allergen * Advise parents not to smoke in the house * Psychological intervention in chronic severe asthma General management points: * Always consider compliance or poor inhaler technique if symptoms are poorly controlled * Inhaler technique should be reviewed * Consider device used (see basics) * All children diagnosed with asthma should have a written asthma management plan

Answer 145

Viral-induced wheeze: * Very similar step-up pathway * SABA PRN for symptoms * Montelukast started at the first sign of a viral cold

Answer 146

**Asthma exacerbation:** * The main complication * Can be life-threatening * Clinical features: * Respiratory distress, wheeze, SOB * Vary depending on severity à see diagram

Answer 147

* **ABCDE approach** * **O2 if sats are \<92%** * **SABA** * Given via inhaler and spacer, unless attack is severe to life-threatening when it is nebulised * Burst therapy: 1 puff, 5-10 deep breaths, repeat up to 10 times à this is 1 burst * **Steroids (oral or IV)** * **Ipratropium (nebulised/inhaled) – in severe** * I**V salbutamol, aminophylline or magnesium sulphate – in severe**

Answer 148

* Bronchodilators are taken as inhaler device with spacer at intervals of 4hrly or more * This is called stretching → first they are stretched to 1hrly, if no symptoms then stretched to 2hrly etc. * SaO2 \>94% in air * Inhaler technique assessed/taught * Written asthma management plan given and explained to parents