Respiratory

Question 1

croup

Question 2

asthma

Question 3

bronchiectasis

Question 4

COVID 19

Question 5

whooping cough notifiable

Answer 5

yes

Question 6

epidemiology whooping cough

Answer 6

cases in older children and adults tend to be much milder, whilst infants remain most at risk of hospitalisation and death due to pertussis (5). The incidence of pertussis tends to peak every 3-4 years for reasons that are poorly understoo

Question 7

vaccines whooping cough

Answer 7

pertussis vaccines - 2, 3 and 4 mths of age, booster at 3 yrs and 4 mths

Question 8

bacteria whooping cough

Answer 8

bordatella pertussis
gram negative bacillus
spreads by aerosolised droplets
bacteria attaches to respiratory epithelium and produce toxins which paralyse cilia and promote inflammation, impairing clearance of secretions

Question 9

risk factors whooping cough

Answer 9

non vaccination
exposure to an infected individual

Question 10

clinical features whooping cough

Answer 10

1. catarrhal phase - 1-2 weeks
   Rhinitis
   Conjunctivitis
   Irritability
   Sore throat
   Low-grade fever
   Dry cough
2. paroxysmal phase - 2-8 weeks
   severe paroxysms of coughing followed by inspiratory gasp, can result in vomiting
   if <3mths apnoea more common
3. convalescent phase - 3 mths
   decreases in frequency and severity

Question 11

ddx whooping cough

Answer 11

bronchiolitis
mycoplasma pneumonia
bacterial pneumonia

Question 16

management whooping cough

Answer 16

duration of cough <21 days= macrolide
Clarithromycin for those under 1 month, azithromycin or clarithromycin for those over 1 month
Co-trimoxazole is the 2nd line antibiotic where macrolides are contra-indicated or poorly tolerated
Further management is supportive, including paracetamol and / or ibuprofen for symptomatic relief and adequate fluid intake to prevent dehydration
can take up to 3 mths to reolve

Question 16

hospital admission whooping cough indications

Answer 16

Who are under 6 months of age and acutely unwell
With significant breathing difficulties, e.g. apnoeic episodes, cyanosis, respiratory distress or severe paroxysms of coughing
Feeding difficulties
With significant complications, e.g. pneumonia or seizures

Question 16

whooping cough avoiding school ?

Answer 16

The child should avoid nursery or school until they have had the cough for 21 days or have had antibiotics for 5 days

Question 16

investigations whooping cough

Answer 16

<2 weeks - culture of nasopharngeal aspirate or swab…PCR testing
>2 weeks - anti pertussis toxin IgG serology, oral fluid testing for anti pertussis toxin
FBC - lymphocytosis

Question 17

whooping cough complications

Answer 17

Secondary bacterial pneumonia (up to 20% of infants)
Seizures
Encephalopathy (rare)
otitis medis

Question 18

bronchiolitis definition

Answer 18

viral infection of the bronchioles
most commonly caused by RSV

Question 19

RSV

Answer 19

respiratory syncytial virus

Question 20

bronchiolitis epidemiology

Answer 20

children <2 yrs
1/3 will develop in first year of life
occurs during winter and spring months

Question 21

pathophysiology bronchiolitis

Answer 21

Proliferation of goblet cells causing excess mucus production
IgE-mediated type 1 allergic reaction causing inflammation
Bronchiolar constriction
Infiltration of lymphocytes causing submucosal oedema
Infiltration of cytokines and chemokines

Question 22

risk factors bronchiolitis

Answer 22

Being breast fed for less than 2 months Smoke exposure (eg. parents’ smoke) Having siblings who attend nursery or school (increased risk of exposure to viruses) Chronic lung disease due to prematurit

Question 23

clinical features bronchiolitis

Answer 23

Low-grade fever Nasal congestion Rhinorrhoea Cough Feeding difficulty

Question 24

examination bronchiolitis

Answer 24

Tachypnoea Grunting Nasal flaring Intercostal, subcostal or supraclavicular recessions Inspiratory crackles Expiratory wheeze Hyperinflated chest Cyanosis or pallo

Question 25

ddx bronchiolitis

Answer 25

pneumonia HF croup CF

Question 26

investigations bronchiolitis

Answer 26

nasopharyngeal aspirae or throat swab blood and urine culture FBC ABG CXR - Hyperinflation Focal atelectasis Air trapping Flattened diaphragm Peribronchial cuffing

Question 27

urgent referral bronchiolitis

Answer 27

Apnoea Child looks seriously unwell Severe respiratory distress eg. grunting, marked recessions, respiratory rate >70 Central cyanosis Oxygen sats < 92% Hospital referral should be considered if any of the following are seen (4): Respiratory rate > 60 Inadequate fluid intake (50-75% of usual volume) Clinical dehydration

Question 28

bronchiolitis hospital management

Answer 28

Give oxygen if sats < 92% in room air Give fluids via nasogastric or orogastric tube if inadequate oral intake Consider CPAP if there is impending respiratory failure Perform upper airway suctioning if there are upper airway secretions or apnoea

Question 29

discharge bronchiolitis

Answer 29

Clinically stable Taking adequate oral fluids Maintaining sats > 92% for more than 4 hours

Question 30

complications bronchiolitis

Answer 30

Hypoxia Dehydration Fatigue Respiratory failure Persistent cough or wheeze (very common and parents should be counselled that their child may cough for several weeks) Bronchiolitis obliterans – Airways become permanently damaged due to inflammation and fibrosis

Question 31

prognosis bronchiolitis

Answer 31

lasts for 7-10 days cough can last for 6 weeks

Question 32

CF definition

Answer 32

an autosomal recessive disease caused by a mutation in the CF transmembrane conductance regulator gene (CFTR) resulting in multisystem dysfunction. In 2000 the life expectancy of a child born in 2000 was 50 years

Question 33

epidemiology CF

Answer 33

approximately 1 in 25 Caucasian Europeans are carriers of a CF gene with approximately 1 in 2500 live births having CF. p.Phe508del (ΔF508) is the commonest mutation in the UK with this accounting for at least one mutation in 90% of people with CF in the UK, with 50% of those with CF in the UK are homozygous for this mutation

Question 34

CF respiratory tract

Answer 34

The CTFR gene encodes the CFTR protein – a chloride channel that is present in numerous epithelial tissues. Chloride is driven against its concentration gradient using ATP. In the airway, CFTR is present on airway epithelial cells and submucosal glands and when defective results in disruption to chloride ion movement and also affects sodium reabsorption (by disturbing the function of ENaC) which reduces the amount of water in secretions. This results in reduced airway surface liquid. The airway surface liquid is an important component of the mucociliary escalator and also has key immunological functions. The effects of reduced airway surface liquid serve to impede mucus clearance. The altered lung environment provides a niche for bacterial growth with the biofilm mode of growth providing ideal conditions to protect bacteria from the host immune system and the actions of antibiotics. The pro-inflammatory cascade contributes to tissue damage.

Question 35

CF pancreas

Answer 35

In the pancreas the pancreatic duct is usually occluded in-utero causing permanent damage to the exocrine pancreas rendering patients with CF ‘pancreatic insufficient’. Pancreatic insufficiency is closely related to genotype. Over time, endocrine pancreas is affected with 28% of those older than 10 years requiring treatment for CF-related diabetes mellitus.

Question 36

CF gi tract

Answer 36

In the gastrointestinal tract, the small intestine secretes viscous mucus which can cause bowel obstruction in-utero which can cause meconium ileus. In the biliary tree in-utero, CF can cause cholestasis which can result in neonatal jaundice. Later in life the same pathology can result in distal intestinal obstruction syndrome (DIOS) and CF-related liver disease (14% of patients)

Question 37

CF repro tract

Answer 37

98% of men with CF are infertile due to a congenital absence of the vas deferens. In women nutrition is likely to be an important predictor of successful pregnancy. It is suggested that the timing of pregnancy be carefully planned, as pregnancy is often associated with a deterioration in lung health

Question 38

risk factors

Answer 38

aut recessive lots of mutations to teh CFTR gene Different organs have different sensitivity to loss of CFTR protein function, with the vas deferens particularly sensitive in contrast to the lungs, which are less sensitive. Instead the lungs are more susceptible to other factors, like stochastic, environmental, and genetic modifiers-genes that modify the expression of CFTR protein

Question 39

clinical features CF

Answer 39

Neonates Meconium ileus – 10% of children present with abdominal distension, delayed passage of meconium and bilious vomiting in the first days of life. Since the introduction of screening for CF in neonates (part of the Guthrie test) the majority of cases of CF are identified here (however it is important to note that it is a screening test and still needs diagnostic testing)4 Failure to thrive Prolonged neonatal jaundice Infancy Failure to thrive Recurrent chest infections Pancreatic insufficiency: steatorrhoea Childhood Rectal prolapse Nasal polyps (N.B. strongly suspect CF in children presenting with nasal polyps) Sinusitis Adolescence Pancreatic insufficiency: diabetes mellitus Chronic lung disease DIOS, gallstones, liver cirrhosis

Question 40

examination CF

Answer 40

Hands: finger clubbing Face: nasal polyps Chest: hyperinflated, crepitiations, portacath (indwelling vascular access device) Abdomen: faecal mass (if constipated/DIOS), may have a scar from ileostomy (meconium ileus)

Question 41

diagnosis of CF

Answer 41

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

Question 42

investigations CF

Answer 42

Chest radiograph: to assess for hyperinflation, there may be evidence of bronchial thickening (undertaken annually as part of annual assessment) Some units undertake CT scanning. Chloride sweat test (at diagnosis and annually if in receipt of CFTR potentiator/corrector therapy) Microbiological assessment e.g. cough swab/sputum sample (at every clinical encounter) Glucose tolerance test (at annual assessment at teenage and beyond) Liver function test and coagulation (at annual assessment) Bone profile (at annual assessment) Lung function testing – spirometry / lung clearance index

Question 43

chloride sweat test

Answer 43

This test measures the electrolyte concentration in sweat Sweat sample is collected by pilocarpine iontophoresis. Careful technique must be employed to ensure and adequate sample is collected to avoid inaccurate results. A common reason for failure of the test is an insufficient sample in a small baby (limit 3kg). A sweat chloride >60mmol/L is suggestive of CF. A sweat chloride of 40-60mmol/L is borderline and should be repeated A single sweat test is not sufficient to diagnose CF, a second test or identification of genetic mutation Faecal elastase to assess pancreatic function Chest CT (high resolution) to assess for bronchiectasis (where bronchial diameter would exceed that of accompanying pulmonary vessels) Genetic analysis

Question 44

management CF

Answer 44

patient and family education twice daily physio mycolytics - DNase, hypertonic saline exercise creon fat soluble vitamins - A, D, E, K monitor growth nutrition as higher metabolic demands - build up milkshakes sputum cultures - monitor for infections, with 2 weeks of abx if pseudomonas - eradication regular azithromycin active segregation minimise complications annual review

Question 45

organisms CF

Answer 45

Staphyloccocus aureus, Haemophilus influenzae and Pseudomonas aeruginosa

Question 46

complications CF resp

Answer 46

allergic bronchopulmonary aspergillosis bronchiectasis haemoptysis pulmonary HTN pneumothorax resp failure nasal polyps

Question 47

complications GI CF

Answer 47

rectal prolapse distal intestinal obstruction syndrome CF releated liver disease

Question 48

complications endocrine CF

Answer 48

CF related diabetes - associated with rapid decline in lung function and disease progression - Clinical presentation can differ to typical type 1 DM and comprises weight loss, anorexia and fall in lung function delayed puberty - avg of 2 yrs, reduced bone minteral density

Question 49

other complications CF

Answer 49

arthritis reduced bone mineral density sub or infetility