croup, epiglottis and CF

Question 1

main cause of epiglottis acute

Answer 1

haemophilus influenza

Question 2

sx of epiglottis

Answer 2

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

Question 3

Mx of epiglottis

Answer 3

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

Question 4

what abx of epiglottis

Answer 4

Iv cefuroxime

Question 5

croup is a differntial for stridor in children what causes croup

Answer 5

infleunza and parainfluenza viruses

Question 6

what is laryngomalacia

Answer 6

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

Question 7

bronchiltiis casues by

Answer 7

RSV

Question 8

risk of transmission if both paretns carriers

Answer 8

1/4

Question 9

risk of parent being a carrier for CF

Answer 9

1/25

Question 10

neither parent diagnosed with CF yet but boht diagnosed what is the risk to first child

Answer 10

1/25 x 1/25 x 1/4 = 1/2500

Question 11

Cf auto recessive most common mutations affects

Answer 11

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

Question 12

SX in what systems

Answer 12

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.

Question 13

how does CF present in the neonate

Answer 13

meconium ileus - thick mucus casuing delay in passing of meconium

Question 14

how is meconium ileus treated

Answer 14

gastrograffin enema

Question 15

signs of CF

Answer 15

salty sweat
faltering growth
recurrent chest infections
malabsorption syndromes
delayed onset puberty

Question 16

blood spot test would reveal raised what

Answer 16

have a raised blood immunoreactive trypsinogen

Question 17

management of CF

Answer 17

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.

Question 18

postive sweat test definded as

Answer 18

chloride over 60

immunoreactive trypsingoen assay is screening

Question 19

croup typicall affects 6m to 6yr autumn and winter
sx

Answer 19

seal like cough
stridor
fever
icnreased work of breathing

Question 20

mx of croup

Answer 20

dexamethasone dose

severe - hosp - oxygen nebulised budesonide if wont toelrate PO steriod
nebulised adrenalien for airway too

Question 21

before referal to ENT for acute epiglottis what shoudl you do

Answer 21

create a secure airway and do an endotracheal intubation