croup, epiglottis and CF Flashcards
main cause of epiglottis acute
haemophilus influenza
sx of epiglottis
children aged 1-6
High fevers
Toxic looking child
Intensely painful throat preventing child from speaking or swallowing
saliva drools
Soft inspiratory stridor and rapidly increasing respiratory difficulty over hours
Child sits immobile, upright with open mouth to optimise airway - tripod position
mouth open to keep airway open
Cough minimal or absent
Mx of epiglottis
senior ENT alongisde PICU
do not exmaine or upset the child in absence of senior support
securing the airway first priority
endotrcheal intubation
take cultures once secure and treat with IV abx - cefuroxime
what abx of epiglottis
Iv cefuroxime
croup is a differntial for stridor in children what causes croup
infleunza and parainfluenza viruses
what is laryngomalacia
floppy larynx
congenital abnormality - excessive collapse and indrawing of the supraglottic airways during inspiration producing stridor - noticeable within a few hours of birth - long standing history of breathing difficulties
bronchiltiis casues by
RSV
risk of transmission if both paretns carriers
1/4
risk of parent being a carrier for CF
1/25
neither parent diagnosed with CF yet but boht diagnosed what is the risk to first child
1/25 x 1/25 x 1/4 = 1/2500
Cf auto recessive most common mutations affects
DELTA -F508 - abnormal glycosylation adn subsequent degradation of the CFTR protein before it raches the cell membrane
defects in chloride movements therfore osmosis as secretions very thick
SX in what systems
Respiratory system:
Thick mucus in the lungs causes cough, recurrent infections, and bronchiectasis.
Sinusitis and nasal polyps are also very common.
Patients usually are colonised with Pseudomonas in the lung by about age 20.
Digestive system:
Reduced pancreatic lipase enzyme secretion inhibits fat absorption, causing steatorrhoea
Poor fat absorption consequently contributes to deficiency of fat-soluble vitamins (A, D, E and K)
These factors contribute to poor weight gain
Damage to the pancreas can also result in patients with cystic fibrosis developing diabetes mellitus
Reproductive system:
Seminiferous tubes also get blocked; most men with cystic fibrosis are unable to conceive naturally.
Fertility is also slightly lower than average in women due to thicker cervical mucus.
how does CF present in the neonate
meconium ileus - thick mucus casuing delay in passing of meconium
how is meconium ileus treated
gastrograffin enema
signs of CF
salty sweat
faltering growth
recurrent chest infections
malabsorption syndromes
delayed onset puberty
blood spot test would reveal raised what
have a raised blood immunoreactive trypsinogen
management of CF
Management
Cystic fibrosis is a chronic disease which requires multidisciplinary team management.
Daily chest physiotherapy techniques are necessary to help clear mucus and prevent pneumonias.
Medical management of cystic fibrosis includes prophylactic antibiotics, bronchodilators, and medicines to thin secretions (e.g. dornase alfa).
Pancreatic enzyme replacement (creon) and fat-soluble vitamin supplementation (ADEK) are also useful.
Patients should also have influenza and pneumococcal vaccines
A last-resort for patients with end-stage pulmonary disease in cystic fibrosis may be a bilateral lung transplant.
postive sweat test definded as
chloride over 60
immunoreactive trypsingoen assay is screening
croup typicall affects 6m to 6yr autumn and winter
sx
seal like cough
stridor
fever
icnreased work of breathing
mx of croup
dexamethasone dose
severe - hosp - oxygen nebulised budesonide if wont toelrate PO steriod
nebulised adrenalien for airway too
before referal to ENT for acute epiglottis what shoudl you do
create a secure airway and do an endotracheal intubation
