Respiratory (2s + 3s)

Question 1

Epiglottitis is a cellulitis of the supraglottis with the potential to cause airway compromise, and should be treated as a surgical emergency until the airway is examined and secured.

What organism most commonly causes epiglottitis?

Answer 1

• haemophilus influenza type B
• therefore a risk factor is non-vaccination with HiB vaccine

Question 2

What is the clinical presentation of epiglottitis?

Answer 2

• sore throat
• dysphagia
• drooling
• toxic appearance
• acute distress
• fever
• tripod position
• difficulty breathing
• muffled voice, stridor, irritability

Question 3

If you suspect epiglottitis, what should you not do?

Answer 3

• no action should be taken that could stimulate a child w/ suspected epiglottitis
• do not examine throat, lie pt down, separate from parent or take blood as this may evoke complete airway obstruction
• it’s a clinical diagnosis
• lab or other interventions should not preclude or delay timely control of the airway in a suspected case of epiglottitis

Question 4

What are the relevant investigations for epiglottitis?

Answer 4

• laryngoscopy → key to confirming diagnosis, but also therepeautic as an airway can be established in same setting if a direct laryngoscopy comences, this should be performed in the OR as an emergency surgical airway can be obtained if endotracheal intubation is not possible
• lateral neck radiograph → only to be obtained w/ HCP capable of securing airway with proper equipment available during the test

Question 5

What is the management of epiglottitis?

Answer 5

• secure airway + supplemental oxygen
  
  • if intubation not possible during laryngoscopy then tracheostomy
• intravenous antibiotics → co-amoxiclav or ceftriaxone
• adjuncts → corticosteroids, racemic epinephrine, prolonged intubation

The tracheal tube can normally be removed after 24hrs and the child has normally totally recovered in 2-3 days, Abx treatment continues for 3-5 days

Question 6

Acute otitis media (AOM) is part of a spectrum of inflammatory conditions affecting the middle ear. These range from a single episode of AOM, recurrent episodes of AOM, otitis media with effusion (OME or ‘glue ear’) and chronic suppurative otitis media.

What are the risk factors for AOM?

Answer 6

• day care attendance -> inc exposure to resp viruses
• older siblings -> inc exposure to resp viruses
• young age
• family history
• absence of breastfeeding
• immunological deficiency

AOM is generally preceded by a viral URTI that causes congestion of the respiratory mucosa of the nasopharynx + eustachian tube.

Question 7

80% of children experience at least one episode of AOM before the age of 2 years w/ a peak incidence between 6-11 months.

What are the most common causative organisms for Acute Otitis Media?

Answer 7

• streptococcus pneumonia
• haemophilus influenzae
• moraxella catarrhalis
• streptococcus pyogenes

Question 8

What are the clinical features of acute otitis media?

Answer 8

• ?preceding viral resp illness
• acute onset otalgia (ear pain)
• fever
• irritability
• crying
• sleep disturbance
• vomiting
• poor appetite
• physical examination → bulging, erythematous or opaque tympanic membrane, with impaired mobility, perforation can occur +/- purulent ear discharge

Question 9

What investigations can be done for AOM?

Answer 9

• rarely required
• but culture of fluid taken directly from middle ear via tympanocentesis may be useful in immunocompromised children + those <6months, where AOM may be associated w/ an unusual or more invasive pathogen

Question 10

What is the treatment for acute otitis media?

Answer 10

• natural course of AOM is 3 days but can be up to 1 week
• use analgesia + antipyretics for symptom control → paracetamol, NSAIDs, calpol
• IF antibtioics then 5-day course of amoxicillin (*)
  
  • erythromycin or clarithromycin for penicillin allergies

(*) coming up on next cards

Question 11

For acute otitis media, the use of antibiotics is more difficult given the self-limiting nature of the condition in most cases. Three separate antibiotic prescribing strategies may be used according to the age + clinical assessment of the child.

When should antibiotics be prescribed immediately?

Answer 11

• if under 2 years w/ bilateral AOM
• if presenting w/ AOM + ear discharge
• if initially managed without abx but show no improvement after 4 days
• if systemically unwell
• if at high risk of complications due to immunocompromise or other PMHx

Question 12

For AOM, when should there be no antibiotic prescribed?

Answer 12

• most children + their parents can be reassured that abx are genrally not required in this condition
• their use may be associated w/ side-effects such as n+v, rashes, diarrhoea + can contribute to antibiotic resistance
• parents should be advised to re-present if symptoms worsen or if there is no improvement in 4 days

Question 13

In certain situations it may be more practical to provide a delayed prescription for antibiotics. For AOM, when should there be delayed antibiotic prescibing?

Answer 13

• prescription should be collected if there is no improvement after 4 days
• significant worsening of symptoms at any stage

Any child w/ suspected acute complications of AOM (mastoiditis, meningitis or facial nerve paralysis) should be urgently referred for ENT assessment

Question 14

Tonsillitis is inflammation of the palatine tonsils as a result of either bacterial or viral infection. It will often occur in conjunction w/ inflammation of other areas of the mouth, giving rise to terms tonsillopharyngitis (pharynx also involved) and adenotonsillitis (adenoids also involved).

What are the bacterial and viral organisms responsible for tonsillitis?

Answer 14

• Bacterial →
  
  • group A beta-haemolytic streptococci (GABHS) (strep pyogenes)
  • group C beta-haemolytic streptococci
  • mycoplasma pneumoniae
  • neisseria gonorrhoea
• Viral →​
  
  • adenovirus
  • epstein-barr virus
  • rhinovirus
  • coronavirus
  • enterovirus
  • influenza + parainfluenza

It is difficult to differentiate between viral or bacterial aetiology. Blood testing should include an infectious mononucleosis screen.

Question 15

What are the clinical features of tonsillitis?

Answer 15

• generally lasts 5-7 days
  
  • if >7 days → consider glandular fever
• odynophagia + reduced oral intake
• fever
• halitosis
• new onset snoring (or even apnoeic)
• shortness of breath
• physical examination → red inflamed tonsils, white exudate (pus) spots on tonsils, cervical lymphadenopathy (most commonly lymph nodes in the region of upper 1/3rd SCM)

Question 16

Tonsillitis is a clinical diagnosis. Antibiotics will most likely benefit a patient when their sore throat is caused by streptococcal bacteria. Centor criteria will aid in the diagnosis or exclusion of GABHS-tonsillitis and determine whether antibiotics are an option.

What is the Centor criteria?

Answer 16

Was developed to try and differentiate between bacterial and viral tonsillitis based on clinical symptoms, there are four key criteria:

• tonsillar exudate
• tender anterior cervical lymphadenopathy or lymphadenitis
• fever or history of fever
• absence of cough

a score of 3 or more is highly suggestive of bacterial infection (40-60% likelihood) and a score of 2 or less suggests bacterial infection is unlikely (80% likelihood)

Question 17

The first key decision for tonsillitis is whether the patient requires inpatient admission or not.

What factors suggest severe tonsillitis + an urgent admission and assessment?

Answer 17

• resp compromise (tachypnoea, low sats, use of accessory muscles) or apnoeic episodes -> suggest tonsils are so large that they are affecting the child’s ability to ventilate, alternatively could be epiglottitis
• pts who are unable to eat or drink are at risk of dehydration - they should be admitted for treatment + monitoring until able to drink again
• pts who have been treated w/ appropriate antibiotics in community who are still not getting better should also be admitted for IV therapy + further investigation

Question 18

Paracetamol and iboprufen are effective pain relief in tonsillitis + can be alternated in order to give effective pain relief. Topical analgesia such as difflam (benzydramine) spray/mouthwash can be helpful to reduce pain and allow child to swallow oral analgesic agents.

What is the role of antibiotics in treating tonsillitis?

Answer 18

• NICE recommend antibiotics should not be routinely given for tonsillitis
• in pts fulfulling 3 or more Centor criteria, there is option of antibiotics to cover Group A strep
• also abx for those systemically unwell, with complications (eg quinsy) or at serious risk of complications due to comorbidity
• antibiotic of choice → penicillin
• data suggests cephalosporins may be more effective (?)
• co-amoxiclav often avoided due to small risk of permanent skin rash if tonsilitis is due to glandular fever

Question 19

Tonsillectomy is reserved for patients with recurrent, troublesome tonsillitis. What are the indications for tonsillectomy?

Answer 19

• sore throats caused by tonsillitis
• 5 or more episodes of sore throat per year
• symptoms for at least a year
• episodes of sore throat that are disabling + prevent normal functioning

Question 20

What is cystic fibrosis?

Answer 20

• autosomal recessive condition
• caused by mutatipon in CFTR gene
  
  • this receptor is responsible for the transfer of chloride ions out of the cell + inhibition of the adjacent epithelial sodium channel (ENaC)
  • bc no chloride pumped out of the cell + sodium floods into the cell uninhibited, the cell content becomes v concentrated w/ a high osmolality that draws water in from outside
  • results in thick dehydrated mucus -> builds up in lumens in body
• more than 1600 known genetic mutations for CF w/ delta F508 mutation accounting for more than 70% of mutations in UK population
• 1 in 25 caucasians are carriers + disease affects 1 in 2500 live births

Question 21

What is the pathophysiological impact of CF in the respiratory tract?

Answer 21

• CTFR gene encodes CTFR protein - a chloride channel, present in numerous epithelial tissues
• chloride driven against its concentration gradient using ATP
• in airway, CTFR present on airway epithelial cells + submucosal glands
• when defective → results in disruption to chloride ion movement + also affects sodium reabsorption → reduces amount of water in secretions
• this results in reduced airway surface liquid
• the airway surface liquid is an important component of the mucociliary escalator + has key immunological functions
• effects of reduced airway surface liquid serve to impede mucus clearance
• the altered lung environment provides a niche for bacterial growth
• main infective agents are S. Aureus, H. Influenzae and P aeruginosa

Question 22

What is the pathophysiological impact of CF in the pancreas?

Answer 22

• pancreatic duct usually occluded in-utero causing permanent damage to exocrine pancreas
• rendering pts w/ CF ‘pancreatic insufficient’
• pancreatic insufficiency is closely related to genotype
• over time, endocrine pancreas affeced
• 28% of those older than 10yrs requiring treatment for CF-related diabetes mellitus

Question 23

What is the pathophysiological impact of CF in the GI tract?

Answer 23

• small intestine secretes viscous mucus which can cause bowel obstruction in-utero → can cause meconium ileus
• in the biliary tree in-utero, CF can cause cholestasis → neonatal jaundice
• later in life, same pathology → distal intestinal obstruction syndrome (DIOS) + CF-related liver disease

Question 24

What is the pathophysiological impact of CF in the reproductive tract?

Answer 24

• 98% of men w/ CF are infertile
• due to congenital absence of vas deferens
• in women, nutrition likely to be an important predictor of successful pregnancy
• it is suggested that timing of pregnancy be carefully planned
• as pregnancy often associated w/ deterioration in lung health

Question 25

Clinical presentation of CF can _differ_ depending on the **age** at which patients present. What is the presentation in **neonates**?

Answer 25

Since the introduction of **screening** for CF in neonates (part of the **Guthrie** test) the majority of cases of CF are identified here (however, important to note that it is a screening test + still needs diagnostic testing) * **meconium ileus** - 10% of children present w/ abdominal distension, delayed passage of meconium + bilious vomiting in first few days of life * **failure to thrive** * **prolonged neonatal jaundice**

Question 26

What is the clinical presentation of CF in **infancy**?

Answer 26

* failure to thrive * recurrent chest infections * pancreatic insufficiency: steatorrhoea

Question 27

What is the clinical presentation of CF in **childhood**?

Answer 27

* rectal prolapse * nasal polyps (strongly suspect CF in children presenting w/ nasal polyps) * sinusitis

Question 28

What is the clinical presentation of CF in **adolescence**?

Answer 28

* pancreatic insufficiency: diabetes mellitus * chronic lung disease * DIOS, gallstones, liver cirrhosis

Question 29

Clinical examination may be entirely normal, variably and often related to the severity of lung disease. What are some **signs** that can be elicted on investigation of a patient w/ cystic fibrosis?

Answer 29

* **hands**: finger clubbing * **face**: nasal polyps * **chest**: hyperinflated, creps, portacath (indwelling vascular access device) * **abdo**: faecal mass (if constipated/DIOS), may have scar from ileostomy (meconium ileus)

Question 30

A diagnosis of CF can be made if there is a **fitting clinical history** and a **positive chloride sweat test** which may be supported by the identification of two identified mutations. Patients with cystic fibrosis will end up having a large number of investigations, partly directed by their clinical condition and also as standard routine monitoring of the disease. What should standard investigations include?

Answer 30

* **CXR** → assess hyperinflation, bronchial thickening, some units undertake CT scanning * **chloride sweat test** → at diagnosis + annually if in receipt of CFTR potentiator/corrector therapy * **microbiological assessment** → eg. cough swab/sputum sample at every clinical encounter * **glucose tolerance test** → at annual assessment at teenage and beyond * **LFT + coag** → at annual assessment * **bone profile** → at annual assessment * **lung function testing** → spirometry/lung clearance index * faecal elastase → assess pancreatic fxn * chest CT → bronchiectasis * genetic analysis → assess for 1 or more of 32 mutations

Question 31

What is the **chloride sweat test**?

Answer 31

* test measures electrolyte concentration in sweat * sweat sample collected by **pilocarpine iontophoresis** * common reason for failure of test is an insufficient sample in a small baby * a sweat chloride **\>60mmol/L** is suggestive of CF * a sweat chloride of 40-60mmol/L is **borderline** + should be repeated * _a single sweat test is not sufficient to diagnose CF_ * a second test or identification of genetic mutation should confirm diagnosis * there are number of reasons for false positives/negatives

Question 32

How do you manage CF lung disease?

Answer 32

* monitored in young by **observing** symptoms + in older children by recording their **FEV₁** * **physiotherapy** 2x / day to clear secretions * chest percussion * postural drainage * breathing exercises for older children * **prophylactic anti-microbial therapy** * oral abx eg. flucloxacillin → for S. Aureus prevention * _nebulised_ abx eg. colomycin → for _pseudomonas_ prevention * oral antifungals → for aspergillus prevention * acute infections need to be treated aggressively, typically w/ IV abx + supplementary O₂ * **DNase** (dornase alpha) + **hypertonic saline** * **bronchodilators** → airway dilatation, aiding clearance of resp secretions, particularly effective in those w/ concurrent asthma * lung transplant for terminal disease

Question 33

How do you manage **nutrition** in cystic fibrosis?

Answer 33

* **high-calorie diet** (150% of normal) → needed due to malabsorption + raised metabolism due to chronic infection + resp work * **pancreatic enzyme supplementation** (creon) w/ every meal + snack * should encourage children to undertake physical exercise * **fat-soluble vitamins (ADEK)** poorly absorbed in those w/ pancreatic insufficiency, at least vitamins ADE should be supplemented * histamine receptor antagonist (eg **ranitidine**) + **PPIs** → aids absorption of creon by increasing duodenal pH * monitor growth * **enteral feeding** via gastrostomy in extreme cases of malnutrition

Question 34

What is small molecule therapy in CF?

Answer 34

* medication for treating the defective protein itself * **IVACAFTOR** is licensed for individuals w/ gating mutations where CFTR molecule is made + inserted into the cell surface but does not work * Ivacaftor can induce a degree of normal function of protein: * improves weight gain * reduces exacerbation rates * returns sweat chlorides to a normal level * only suitable for roughly 5% of the CF population

Question 35

What are **complications** of cystic fibrosis?

Answer 35

* allergic bronchopulmonary aspergiliosis (ABPA) * bronchiectasis * pneumothorax * rectal prolapse * distal intestinal obstruction syndrome (DIOS) * CF-related: cholestasis, gallstones, liver cirrhosis * CF related diabetes (CFRD) * delayed puberty

Question 36

What is the prognosis of CF?

Answer 36

* death may be from pneumonia or cor pulmonale * 95% die from resp failure * most survive to adulthood (median survival \>35yrs, \>50yrs if born after 2000) * psychological morbidity very prevalent

Question 37

How does **aspiration** cause lung injury?

Answer 37

* **infection** eg. recurrent pneumonia * chemical or physical **trauma** * **obstruction** leading to collapse

Question 38

What investigations need to be done for aspirational lung injury?

Answer 38

* **CXR** → metal object will be radio-opaque ie will appear white on film, an object stuck withina bronchus can act as a ball-valve ie let air but not let it out, so lung will expand + the expanded lung will appear **hyperlucent** on xray and there may also be evidence of mediastinal shift to opposite side of expanded lung * a foreign body is more likely to fall into the _right_ _bronchus_ since it is straighter than left one * **CT** or **MRI** * **bronchoscopy**

Question 39

What is the management of aspirational lung injury?

Answer 39

* removal during bronchoscopy * antibiotics if bacterial pneumonia

Question 40

How do you manage a choking child?

Answer 40

* **infants** → _back blows_ (abdo thrusts may cause intra-abdo injury) * **older children** → _Heimlich manoeuvre_ * 1 hand made into fist, placed on abdo between umbilicus + xiphisternum, other hand placed on top of fist, both hands thurst into abdomen several times