Paeds: Resp Flashcards
Cystic Fibrosis
what is it
an autosomal recessive genetic condition affecting mucus glands
Cystic Fibrosis
what is it caused by
a genetic mutation of the cystic fibrosis transmembrane conductance regulatory gene
on Ch7
Cystic Fibrosis
what is the most common variant of the cystic fibrosis transmembrane conductance regulatory gene
the delta-F508 mutation: codes for cellular channels, particularly a type of chloride channel
Cystic Fibrosis
what causes a lack of lipase in the digestive tract
thick pancreatic and biliary secretions that cause a blockage of the ducts
Cystic Fibrosis
what causes the susceptibility to airway infections and bacterial colonisation
low volume thick airway secretions that reduce airway clearance
Cystic Fibrosis
what causes male infertility
congenital bilateral absence of the vas deferens
the sperm have no way of getting from the testes to the ejaculate
Cystic Fibrosis
what is the mode of inheritence
autosomal recessive
Cystic Fibrosis
both parents are healthy, one sibling has cystic fibrosis and a second child does not have the disease, what is the likelihood of the second child being a carrier?
2 in 3
Cystic Fibrosis
when is it screened
at birth with the newborn bloodspot test
Cystic Fibrosis
what is often the first sign
meconium ileus (not passing meconium within 24h)
Cystic Fibrosis
sx
- chronic cough
- thick sputum production
- recurrent resp tract infections
- steatorrhoea
- abdo pain and bloating
- salty kisses
- failure to thrive
Cystic Fibrosis
why does steatorrhoea occur
due to the lack of fat digesting lipase enzymes
Cystic Fibrosis
signs
- low weight or height
- nasal polyps
- finger clubbing
- crackle + wheezes on auscultation
- abdo distention
what are causes of clubbing in children
- hereditary clubbing
- cyanotic heart disease
- infective endocarditis
- cystic fibrosis
- TB
- IBD
- liver cirrhosis
Cystic Fibrosis
what are the 3 key methods for establishing a diagnosis
- newborn blood spot testing
- sweat test
- genetic testing for CFTR gene during pregnancy
Cystic Fibrosis
what is the gold standard for diagnosis
sweat test
Cystic Fibrosis
what happens during a sweat test
- pilocarpine applied to skin patch
- electrodes places on either side and current is passed between electrodes
- this causes skin to sweat
- sweat absorbed by filter paper
- sent to lab for Chloride conc
Cystic Fibrosis
what is the diagnostic concentration in the sweat test
> 60mmol/l
Cystic Fibrosis
why does bacteria colonate in these pts easily
they struggle to clear secretions in the airway
perfect environment with moisture and oxygen to colonise
Cystic Fibrosis
what are key microbial colonisers
Staph aureus
pseudomonas
Cystic Fibrosis
how do pts prevent staph aureus infection
take long term prophylactic flucloxacillin
Cystic Fibrosis
why should CF patients stay away from each other
risk of spreading pseudomonas
Cystic Fibrosis
why are pseudomonas difficult
resistant to multiple antibiotics
significant increase in morbidity and mortality
Cystic Fibrosis
trx for Pseudomonas colonisation
long term nebulised abx: tobramycin
PO ciprofloaxin alternatively
Cystic Fibrosis
mnx
- chest physio
- exercise
- high calorie diet
- CREON tablets
- prophylactic flucloxacillin
- treat chest infections
- salbutamol
- neb DNase (dornase alfa)
- neb hypertonic saline
- vaccinations: pneumococcal, influenza, varicella
Cystic Fibrosis
what is Nebulised DNase (dornase alfa)
an enzyme that can break down DNA material in respiratory secretions, making secretions less viscous and easier to clear
Cystic Fibrosis
why do they need follow up every 6m
- sputum culture for pseudomonas
- screening for diabetes, osteoporosis, vitamin D deficiency and liver failure
Bronchiolitis
what is it
inflammation and infection in the bronchioles, the small airways of the lungs
Bronchiolitis
what is it caused by
Respiratory syncytial virus (RSV)
Bronchiolitis
what age is it most common in
<6m
Bronchiolitis
what is heard on auscultation
wheeze and crackles
Bronchiolitis
why does it have a significant effect on infants and not adults
the airways of infants are very small to begin with
so even the smallest amount of inflammation and mucus in the airway has a significant effect on the infant’s abaility to circulate air to the alveoli and back out
Bronchiolitis
presentation
- Coryzal sx
- resp distress signs
- dyspnoea
- tachypnoea
- poor feeding
- mild fever
- apnoeas
- wheeze and crackles on auscultation
Bronchiolitis
what are coryzal sx
- snotty nose
- sneezing
- mucus in throat
- watery eyes
Bronchiolitis
signs of respiratory distress (9)
- raised RR
- use of accessory muscles
- intercostal recessions
- subcostal recessions
- nasal flaring
- head bobbing
- tracheal tugging
- cyanosis
- abnormal airway noises
Bronchiolitis
what is wheezing
whistling sound caused by narrowed airways, typically heard during expiration
what is grunting
caused by exhaling with the glottis partially closed to increase positive end-expiratory pressure
what is stridor
a high pitched inspiratory noise caused by obstruction of the upper airway, for example, in croup
Bronchiolitis
what is the typical RSV course
- starts with URTI: coryzal sx
- half get better, half develop chest sx over the next 1-2d
- sx last 7-10d
- fully recover in 2-3w
Bronchiolitis
what day are sx generally worst on
day 3-4
Bronchiolitis
what are infants more likely to have during childhood if they have had bronchiolitis as an infant
viral induced wheeze
Bronchiolitis
reasons for admission
- <3m
- preexisting condition: prem, Down’s, CF
- 50-75% less of their normal intake of milk
- clinical deyhdration
- RR>70
- O2 sats <92%
- mod-sev resp distress
- apnoeas
- parents not confident
Bronchiolitis
mnx
supportive:
- adequate intake,
- saline nasal drops and suctioning
- O2 if <92%
- ventilatory support if required
Bronchiolitis
mnx: why is it important to avoid overfeeding
a full stomach will restrict breathing
start with small frequent feeds and gradually increase as tolerated
Bronchiolitis
what are the steps in order of severity for ventilatory support
- high flow humidified O2
- CPAP
- intubation + ventilation
Bronchiolitis
what is high flow humidified O2
via tight nasal cannula
delivers air + O2 continuously with some added pressure
it adds positive end-expiratory pressure to maintain the airway at the end of expiration
Bronchiolitis
what is CPAP
sealed nasal cannular performs similarly to high flow (Airvo ot Optiflow)
but can deliver much higher and more controlled pressures
Bronchiolitis
what does intubation and ventilation involve
inserting an endotracheal tube into the trachea to fully control ventilation
Bronchiolitis
what inx is useful in severe resp distress and in monitoring children who are having ventilatory support
capillary blood gas
Bronchiolitis
what are the most helpful signs of poor ventilation
- rising pCo2
- falling pH
Bronchiolitis
what does rising pCO2 show
the airways have collapsed and can’t clear waste CO2
Bronchiolitis
what does a falling pH show
CO2 is building up and not able to buffer the acidosis this creates
this is resp acidosis
Bronchiolitis
which babies are high risk
ex-premature
congenital heart disease