Paediatric Common Conditions 1 Flashcards
What is achondroplasia?
Achondroplasia is an autosomal dominant disorder associated with short stature
mutation in the fibroblast growth factor receptor 3 (FGFR-3) gene
This results in abnormal cartilage giving rise to:
short limbs (rhizomelia) with shortened fingers (brachydactyly)
large head with frontal bossing and narrow foramen magnum
midface hypoplasia with a flattened nasal bridge
‘trident’ hands
lumbar lordosis
What is acute epiglottitis? Presentation?
rare but serious infection caused by Haemophilus influenzae type B
Causes inflammation of the epiglottis
Features:
rapid onset
high temperature, generally unwell
stridor
drooling of saliva
‘tripod’ position
How can acute epiglottitis be diagnosed?
direct visualisation (only by senior/airway trained staff)
x-rays may be done, particularly if there is concern about a foreign body:
a lateral view in acute epiglottis will show swelling of the epiglottis - the ‘thumb sign’
PA view in croup will show subglottic narrowing, commonly called the ‘steeple sign’
How can acute epiglottitis be managed?
immediate senior involvement, including those able to provide emergency airway support (e.g. anaesthetics, ENT)
endotracheal intubation may be necessary to protect the airway
if suspected do NOT examine the throat due to the risk of acute airway obstruction
(the diagnosis is made by direct visualisation but this should only be done by senior staff who are able to intubate if necessary)
oxygen, IV antibiotics (ceftriaxone) , dexamethasone
What is the main complication to be aware of with epiglottitis?
epiglottic abscess, which is a collection of pus around the epiglottis
What is the most common malignancy affecting children?
Acute lymphoblastic leukaemia ( ALL)
How does ALL present?
anaemia: lethargy and pallor
neutropaenia: frequent or severe infections
thrombocytopenia: easy bruising, petechiae
Poor prognostic factors in ALL?
age < 2 years or > 10 years
WBC > 20 * 109/l at diagnosis
T or B cell surface markers
non-Caucasian
male sex
What are the key differentials for acute scrotal pain in children?
Testicular torsion - Most common around puberty
Irreducible inguinal hernia - Most common in children < 2 years old
Epididymitis - Rare in prepubescent children
Alpha-thalassaemia is due to a deficiency of alpha chains in haemoglobin.
Clinical severity depends on the number of alpha globulin alleles affected:
1/ 2 alpha globulin alleles affected = hypochromic and microcytic, Hb level is typically normal
3 alpha globulin alleles affected = hypochromic microcytic anaemia with splenomegaly, Hb H disease
If all 4 alpha globulin alleles are affected (i.e. homozygote) = death in utero (hydrops fetalis)
The Apgar score is used to assess the health of a newborn baby. At what times should it be used?
at 1, and 5 minutes of age
If the score is low then it is again repeated at 10 minutes
What are the APGAR criteria?
What are the normal values?
Activity (muscle tone)
Pulse
Grimace (reflex irritability)
Appearance (skin colour)
Respiratory effort
score of 0-3 is very low, between 4-6 is moderate low and between 7 - 10 means the baby is in a good state
How would you score activity (muscle tone) in the APGAR assessment?
active movement = 2
limb flexion = 1
flaccid = 0
How would you score pulse in the APGAR assessment?
> 100 = 2
< 100 = 1
absent = 0
How would you score grimace (reflex irritability) in the APGAR assessment?
Cries on stimulation/sneezes, coughs = 2
Grimace = 1
Nil = 0
How would you score appearance (skin colour) in the APGAR assessment?
pink = 2
body pink, extremities blue = 1
blue all over = 0
How would you score respiratory effort in the APGAR assessment?
strong, crying = 2
weak, irregular = 1
nil = 0
Which children with appendicitis are more likely to present atypically?
Children who are younger or have a retrocaecal/pelvic appendix
Appendicitis is uncommon in children under 4 years old but in this group often presents with perforation
What is classified as a severe asthma attack in children?
SpO2 < 92%
PEF 33-50% best or predicted
Too breathless to talk or feed
Heart rate
>125 (>5 years)
>140 (1-5 years)
Respiratory rate
>30 breaths/min (>5 years)
>40 (1-5 years)
Use of accessory neck muscles
What is classified as a life threatening asthma attack in children?
SpO2 <92%
PEF <33% best or predicted
Silent chest
Poor respiratory effort
Agitation
Altered consciousness
Cyanosis
Children with severe or life threatening asthma should be transferred immediately to hospital. Which children should you measure PEF in?
Children over 5
How should children with mild to moderate acute asthma be managed?
Bronchodilator therapy:
give a beta-2 agonist via a spacer (for a child < 3 years use a close-fitting mask)
give 1 puff every 30-60 seconds up to a maximum of 10 puffs
if symptoms are not controlled repeat and refer to hospital
Steroid therapy:
should be given to all children with an asthma exacerbation
treatment should be given for 3-5 days
What dose of prednisolone is given in kids with acute asthma?
1-2 mg/kg od (max 40mg)
OR
2 - 5 years = 20 mg od
> 5 years = 30 - 40 mg od
How should newly diagnosed asthma v newly diagnosed asthma with symptoms >= 3 / week or night-time waking be managed?
Newly diagnosed asthma = SABA
More symptomatic = SABA + paediatric low-dose inhaled corticosteroid (ICS)