Paeds Flashcards
How does erythema toxicum neonatorum present?
aka newborn rash
around day 2-3, lasts about 24 hours
benign
white pinpoint papules at the centre of an erythematous base
looks like red blotches (baby acne) concentrated on trunk (and face)
How does strawberry naevus present?
aka cavernous hemangioma
grows until 6-9 months then regresses
benign
small flat red area > raised dimpled strawberry like lesion
mostly on head/neck
What tx can speed regression of a strawberry naevus?
topical propanolol
What is congenital dermal melanocytosis? How does it present?
mongolian blue spot
flat irregular blue/blue gray spots mostly on back/buttocks
can be mistaken for bruises
benign + spontaneous resolution
How does a stork bite/salmon patch present?
collection of blood vessels
pink patches on face/back of neck
blanch on pressure
get darker when crying/temp changes
fade over a few months
What is a caput succedaneum? What is it caused by?
boggy superficial scalp swelling that can cross suture line
(CA = CS - cap succ = cross suture)
caused by:
- pressure against cervix during birth (traumatic/prolonged birth)
- ventouse delivery
What is a cephalohaematoma? How is it managed?
subperiosteal haemorrhage due to damage to blood vessels during traumatic/prolonged/instrumental delivery
periosteum stuck tightly to skull = does not cross suture lines
usually does not require intervention
risk of anaemia + jaundice - monitor
What are the differentials for a superficial scalp swelling in a newborn? What is the possible complication of both?
caput succedaneum = crosses suture lines
cephalohaematoma = does not cross suture lines, more likely to cause discolouration of skin in area
jaundice = key complication > bruising and blood break down overwhelming newborn liver
When is neonatal jaundice abnormal?
very common
<24hrs old = always abnormal
2-14 days = normal
>2wks = can be either
Neonatal jaundice is caused by raised levels of what?
bilirubin
What are the causes of neonatal jaundice in neonates <24 hours old?
Sepsis :(
rhesus haemolytic disease
ACO incompatibility
TORCH infections
G6PD deficiency (x-linked recessive)
Hereditary spherocytosis (AD)
Cephalohematoma
Crigler-najjar syndrome (no UGT enzyme)
When does rhesus haemolytic disease occur?
mum is rhesus D -ve and baby is rhesus D +ve
What is the pathophysiology of rhesus haemolytic disease?
if mother is rhesus D -ve and baby is +ve
blood from the baby enters mother’s bloodstream > mother recognises baby’s rhesus D antigens on the rbcs as foreign and produces abs against them = mother is sensitised
sensitisation = usually fine in 1st pregnancy unless occurs early on
subsequent pregnancies = mother’s anti-D abs can cross placenta into fetus
if fetus is rhesus +ve > abs attach and cause immune system of fetus to attack own rbcs
= haemolysis > anaemia, high bilirubin
How does normal physiological jaundice occur in the newborn?
- high conc of rbc in fetus + neonate
- rbcs are more fragile (70 day lifespan) > release lots of bilirubin
- less developed liver function > normally excreted by placenta > no placenta > normal rise in bilirubin shortly after birth = mild yellowing of skin and sclera from 2-7 days
Causes of neonatal jaundice can be split into increased production and decreased clearance of bilirubin. Give some examples of each.
increased prod:
haemolytic disease, ABO incompat, sepsis, G6PD def, haemorrhage, cephalohaematoma
decreased clearance: prematurity, breast milk jaundce, cholestasis, extrahepatic biliary atresia, endocrine disorders, gilbert
What is kernicterus? How does it happen?
bilirubin can cross BBB > unconj bil deposition in abasal ganglia + brainstem after it exceeds the albumin binding capacity
excessive levels can lead to
brain damage
What are the causes of prolonged jaundice? When is jaundice considered prolonged?
> 14 days
21 days in a premature baby
biliary atresia
hypothyroidism
breast milk
CF
galactosaemia
UTI
gilbert syndrome
How is neonatal jaundice managed?
total bilirubin on tx threshold chart
phototherapy
severe jaundice - exchange transfusion
What is phototherapy? How does it correct jaundice?
light-box shines blue light on baby’s skin
converts unc bil into isomers that can be excreted in the bile/urine without needing conjugation in the liver
How does kernicterus present?
less responsive, floppy, drowsy baby with poor feeding
can cause seizures, hypotonia, opisthotonos
Is brain damage caused by kernicterus permanent?
yes
> cerebral palsy, LD, deafness
Why are babies who are breastfed more likely to become jaundiced?
components inhibit the ability of liver to process bilirubin
more likely to become dehydrated if not feeding adequately > slow passage of stools > increases absorption of bilirubin in intestines
How should neonatal jaundice be investigated?
FBC (anaemia, haemolysis)
blood film (G6PD, spherocytosis
unconjugated (extra-hepatic source)
conjugated (hepatobiliary source)
blood type of mother + baby (ABO/rhesus incompatability)
direct combs test (haemolysis)
thyroid function (hypo)
LFTs (hepatitis)
What is a tx threshold chart for neonatal jaundice?
age versus total bilirubin
How is haemolytic disease of the newborn prevented?
anti D immunoglobulin at 28 weeks + after sensitising events + 1 dose after birth if mother is rhesus neg
What bloods groups are mother and baby for ABO incompatability to occur?
when mother is type O and baby is type A or B
What are possible sensitisation events during pregnancy?
antepartum haemorrhage
amniocentesis procedures
abdo trauma
What is Kleinhauer test?
checks how much fetal blood has passed into the mother’s blood during a sensitisation event
What are the 4 ABO blood groups and their antigens?
A - A antigen
B - B antigen
AB - both A and B
O - no ABO antigens
When do abs to ABO antigens start to be produced in an individual?
individuals generate IgM ABs to antigens absent from their rbcs without prior exposure
begins at birth, detectable from 3 months ish
Which abs are produced in each ABO blood group?
A - anti-B
B - anti-A
AB - no ABO abs
O - anti-A, anti-B, anti-AB
Which blood types can each blood group receive? Which are the universal recipient and donor?
A - A, O
B - B, O
AB - A, B, AB, O (universal recipient)
O - O (universal donor)
Why does ABO incompatibility occur?
ABO Abs are typically IgM = does not cross placental barrier
Small number of people develop IgG ABO Abs > these mothers can have haemolytic disease of the fetus and newborn due to ABO incompatibility
Which type of ab can cross the placenta?
IgG only
What are the TORCH infections?
toxoplasmosis
other (syphilis, parvovirus, varicella zoster)
rubella
CMV
herpes/hepatitis/HIV
What can neonatal jaundice be exacerbated by?
polycythaemia (gestational diabetes)
bruising (caput succedaneum and cephalohaematoma)
What are the causes of (normal) jaundice between 2 and 14 days?
physiological
breast milk
infection > dehydration
Why can congenital hypothyroidism cause jaundice?
slows everything down > impaired bilirubin excretion
How is congential hypothyroidism found?
newborn blood spot screening test
How do babies with congenital hypothyroidism present?
prolonged neonatal jaundice
poor feeding
constipation
increased sleeping
reduced activity
slow growth + development
What two things can happen to the thyroid gland that causes hypothyroidism?
dysgenesis - underdeveloped gland
dyshormonogenesis - full developed but does not produce enough hormone
What is biliary atresia?
section of the bile duct is narrowed/absent
Why does biliary atresia cause jaundice?
> cholestasis > conj bilirubin still excreted into the bile > bile can’t be transported from liver to bowel and isn’t excreted > jaundice
How does biliary atresia present?
prolonged jaundice
What is the initial investigation for biliary atresia?
conjugated and unconjugated bilirubin
> high proportion of conjugated = liver processing it and not excreting it
How is biliary atresia treated?
surgery - attach small intestine to liver
often require a liver transplant eventually
Why babies are at higher risk of jaundice and kernicterus?
premature babies
due to immature liver
How does oesophageal atresia/tracheo-oesophageal fistula present?
polyhydramnios (cannot swallow AF)
blowing bubbles
salivation/drooling
cyanotic episodes on feeding
resp distress/aspiration
How is oesophageal atresia/tracheo-oesophageal fistula diagnosed?
pass NG tube and x-ray > NG tube will coil in oesophagus
abdo XR - no bubbles = isolated OA
What is gastroschisis?
paraumbilical defect (hole in the abdominal wall beside belly button) - always to right of the cord
intestinal contents often on outside of body
What is gastroschisis caused by?
likely a vascular accident in early embryonic life
How do gastroschisis and omphaloceles present on investigation?
antenatal scans usually
raised AFP
intestines likely to be outside of body at birth
may have comorbid intestinal atresia
How is gastroschisis treated?
requires LSCS delivery
staged corrective surgery
TPN slowly introduced
What is an omphalocele?
ventral defect in umbilical ring with herniation of abdominal contents covered by peritoneum
(similar to gastroschisis but covered by thin sac)
What can cause an omphalocele?
small defect - beckwith-wiedemann syndrome
large defects - pulmonary hypoplasia
How is an omphalocele treated?
may need LSCS if large
protect herniated contents, prevent hypothermia
gastric decompression
staged surgical repair
When is a congenital diaphragmatic hernia likely to present?
prenatal US
How does a CDH usually present?
newborn resp distress (may need resuscitation at birth)
if small - resp distress after feeding
displaced apex beat
bowel sounds in hemithorax
scaphoid abdomen
What are the causes of small bowel obstruction?
duodenal atresia
malrotation + volvulus
meconium ileus in CF
What condition is duodenal atresia a/w?
Down’s (1/3rd)
What XR is duodenal atresia likely to present with?
double bubble
What is a volvulus? When does it present?
loop of intestine twists round itself and the mesentery attached to it > bowel obstruction
When does a volvulus generally present?
presents later (2-30 days) so meconium will have passed normally
What XR sign does volvulus present with?
coffee bean sign
What is meconium ileus? What is it a/w?
pathognomonic for CF, usually the 1st sign
meconium thick + sticky > gets stuck and obstructs bowel
When should meconium be passed by? What should it look like?
24 hours
black
How does meconium ileus present?
not passing meconium within 24hrs
abdo distention
vomiting
SBO
What test should be done if a baby has meconium ileus?
heel prick for CF
How can meconium ileus be treated?
therapeutic contrast enema (gastrografin) to pass it
How can meconium ileus be differentiated from Hirschsprung’s after an enema?
will pass after enema in MI, not after hirschsprung
What is hirschsprung’s disease?
congenital condition where nerve cells of myenteric + submucosal plexus absent in a segment of the colon (usually rectosigmoid)
What is the pathophysiology of Hirschsprung’s?
in fetal development parasympathetic ganglion cells start high in GI tract and migrate down > in hirschsprung’s they don’t travel all the way so a section if left without them
responsible for stimulating peristalsis > bowel looses motility + stops passing food along
Does the aganglionic section of colon in Hirschsprung’s relax or constrict?
constricts > obstruction
proximal to obstruction > distended and full
How does Hirschsprung’s present?
functional LBO with failure to pass meconium within 24hrs
chronic constipation
abdo distention
late bilious vomiting
poor weight gain/failure to thrive
What would happen after a PR in a newborn with Hirschsprung’s?
contracted distal segment followed by rush of liquid stool and temporary relief of symptoms
How is a Hirschsprung’s investigated? What is the GS?
AXR - contracted distal segment + dilated proximal segment
barium enema
GS = rectal suction biopsy
What complication can be caused by Hirschsprung’s?
Hirschsprung-associated enterocolitis
fever
abdo distention
diarrhoea +/- blood
features of sepsis
= life threatening, made lead to toxic megacolon and perforation fo the bowel
How is Hirschsprung’s-associated enterocolitis treated?
abx
fluid resuscitation
decompression of obstructed bowel
How is Hirschsprung’s managed?
if unwell - fluid resus, mx of BO
surgical removal of aganglionic section = swenson procedure
What are the causes of intestinal obstruction?
meconium ileus
Hirschsprung’s
oesophageal/duodenal atresia
intussusception
imperforate anus
malrotation with a volvulus
strangulated hernia
What kind of vomiting does a bowel obstruction present with?
persistent bilious vomiting - contains bright green bile
What abnormal bowel sounds may be heard with a bowel obstruction?
high pitched
tinkling early on > absent later
How does a bowel obstruction generally present on XR?
dilated loops proximal to obstruction
collapsed loops distal
absence of air in rectum
What is the general management of a bowel osbtruction?
NBM
NG tube - drain stomach and stop vomiting
IV fluids
What are the RFs for undescended testes/cryptorchidism?
FHx
low birth weight
SGA
prematurity
maternal smoking
Undescended testes in older children/after puberty leads to a higher risk of what?
testicular torsion
infertility
testicular ca
How are undescended testes managed?
W&W in newborns - most will occur spontaneously at 3-6mo
longer it takes, less likely it will happen spontaneously
6mo > refer to urologist
orchidopexy = surgical correction at 6mo-1yr
When should testicular descent occur during pregnancy?
8th month
requires testosterone
What is the difference between indirect and direct hernias?
almost all indirect - through deep then superficial inguinal ring
direct - defect in posterior inguinal wall and passes through to superficial inguinal ring, in premature babies with weak wall
What is the cause of a neonatal inguinal hernia?
patent processus vaginalis - failure to become tunica vaginalis (serous membrane that covers the testes)
What are the signs of an inguinal hernia?
bulge lateral to pubic tubercle
more prominent on crying
most can be reduced
What is an incarcerated inguinal hernia? What are the signs? What is the risk?
contents of hernia become stuck in groin and can’t be reduced back into abdomen
tender lump
irritable
vomiting
risk of strangulation
What test can differentiate between a direct and indirect inguinal hernia?
ring occlusion test
How is an inguinal hernia managed?
always surgery due to risk of incarceration
emergency surgery if incarcerated
What is hypospadias?
presence of urethral meatus on ventral aspect of penile shaft
What should be considered if a child has hypospadias + undescended testes?
causes of low testosterone
testosterone needed for meatus to move distally
How do children with hypospadias present?
meatus on ventral surface
hooded foreskin
spraying on urination
How is hypospadias managed?
surgery not necessary unless parents want it
cannot have circumcision as skin needed
What is testicular torsion?
twisting of spermatic cord cutting off blood supply to testicle
What are the RFs for testicular torsion?
high testosterone - larger testes
neonates + pubertal teenagers
undescended testes
bell clapper deformity
How does testicular torsion present?
testicle higher and more horizontal
acutely swollen and tender
vomiting due to pain
absent cremasteric reflex (stroke inner thigh, testicle doesn’t elevate)
negative prehn’s sign (pain not relieved on elevation)
How can testicular torsion and epididymitis be differentiated?
prehn’s sign
+ve = pain relieves on elevation = epididymitis
-ve = pain not relieved = torsion
How is testicular torsion managed?
surgical emergency - within 6 hours
after 24hrs very poor chance of testicular survival
What is intussusception?
a section of the bowel telescopes into another
usually ileum into caecum
What conditions is intussusception a/w?
often preceded by a viral illness
resp: CF
gastro: intestinal polyps, meckel diverticulum
lymphoma, henoch-schonlein purpura
How does intussusception present?
severe colicky abdo pain, legs draw up
pale, lethargic unwell child
signs of SBO (vomiting, constipation, distention)
sausage shaped mass in abdomen (often RUQ)
redcurrant jelly stool (due to bowel ischaemia = late sign)
How is intussusception investigated?
USS - target/donut sign
AXR - dilated proximal bowel loops
How is intussusception managed?
rectal air insufflation (pump air into colon to push it out)
surgical correction if fails/signs of ischaemia
What is pyloric stenosis?
hypertrophy of the pylorus preventing food traveling from the stomach to the duodenum
What age is intussusception likely to happen?
3mo - 2yrs
How does pyloric stenosis present?
projectile non bilious vomiting shortly after feeding
thin pale hungry baby, failing to thrive
first few weeks of life
dehydration
firm round mass in upper abdomen
hypochloric hypokalaemic metabolic alkalosis
How is pyloric stenosis investigated?
test feed - feel for olive size mass in RUQ
USS - thickened pylorus
ABG - hypochloric hypokalaemic metabolic alkalosis
What is necrotising entercolitis?
inflammatory bowel necrosis
What are RFs for NEC?
PREMATURITY - resp distress + hypoxia
indomethacin (NSAID) for PDA > bowel ischaemia (causes vasoconstriction of mesenteric arteries)
How does NEC present?
feed intolerance
vomiting +/- bile staining
PR fresh blood + mucus
abdominal distention with taught shiny skin
How is NEC diagnosised?
FBC - low platelets = poor prognosis
blood cultures
clotting screen - can lead to DIC
AXR: pneumatosis intestinalis/intramural air = pathognomonic, dilated bowel loops
How is NEC treated?
stop oral feeds
cefotaxime + vancomycin
laparotomy if rapid distention + sigs of perforation
What is GORD?
contents of stomach reflux through the LOS into the oesophagus, throat and mouth
Why are babies prone to GORD?
- immaturity of the LOS
normal for a baby to reflux feeds - most stop spontaneously after 1yr - mostly liquid diet
- mostly horizontal position
What is the most common cause of vomiting in infants?
GORD
What vomiting occurs with GORD?
persistant vomiting, NO bile
How is GORD diagnosed?
clinically usually
red flags eg bilious vomiting > referral to paeds
severe sx > 24hr pH monitoring
How is GORD treated?
- reassurance, smaller more frequent feeds, sit upright after feeds, thickening agents eg carobel for bottles, gaviscon (alginate therapy)
- PPI or H2 receptor antagonist
D2 antagonist eg domperidone (enhance gastric emptying) could also be tried - unresponsive to tx + >1yr: nissen fundoplication
How can vomiting due to GORD vs CMPA vs intestinal obstruction vs infection be differentiated?
GORD - chronic, recurrent regurg, feeding difficulty, arching of back/neck, sore throat
CMPA - chronic, abdo pain, eczema, flatulence, bloody stools, diarrhoea/constipation
obstruction - acute on chronic bilious vomiting, constipation, abdo pain
infection - acute D&V + sx of localised infection eg abdo pain in GE
What are the two types of CMPA? How can they be differentiated by the timing of their sx?
IgE mediated - type 1 hypersensitivity > immediate sx onset after only small amounts
Non-IgE mediated > sx occur about 72hrs after ingestion
What are the clinical features of IgE mediated CMPA?
urticaria, pruritus
colicky abdo pain, vomiting, bloody stool
rhinorrhea, itchy nose
What are the clinical features of non-IgE mediated CMPA?
loose frequent stool with blood + mucus
colicky abdo pain
How is CMPA diagnosed?
IgE > skin prick (4mm = +ve)
Non IgE > temporary removal from diet then reintroduction to see if sx return
What is sandifer syndrome?
brief episodes of abnormal movements a/w GORD in infants
otherwise neurologically normal
usually resolves as reflux improves
torticollis - forceful contraction of neck muscles causing twisting of neck
dystonia - abnormal muscle contractions causing twisting movements - arching back, unusual posture
How are breastfed and bottle fed babies tx for CMPA?
breastfed > remove milk from mum’s diet
bottle > extensively hydrolysed formula
What is functional constipation?
no significant underlying cause except simple lifestyle factors
number of times someone opens their bowels varies between individuals eg breastfed babies generally open them much less
What are the typical features of constipation in a child?
<3 stools/week
hard rabbit dropping stools that are difficult to pass + a/w straining, pain and bleeding
retentive posturing
abdo pain that waxes and wanes
avoidance of eating due to fear of pain
What can happen to bowel movements in chronic constipation?
overflow soiling - rectum distends leading to loss of sensation > incontinence with loose smelly stools
When is faecal incontinence/encopresis considered pathological?
4 yrs
usually due to chronic constipation where the rectum has stretched and lost sensation > loose stools bypass blockage and leak out
What are some pathological causes of encopresis?
spina bifida
hirschprung’s disease
cerebral palsy, LD, psychosocial stress/abuse
What lifestyle factors can contribute to constipation?
habitually not opening bowels
low fibre diet
poor fluid intake and dehydration
sedentary lifestyle
psychosocial problems
What are some secondary causes of constipation?
hirschsprung’s disease
CF - meconium ileus
IO
anal stenosis
hypothyroid
spinal cord lesons
cows milk intolerance
sexual abuse
How should constipation be investigated?
examination
look for red flags
FBC, TFTs, anti TTG
long term/suspicion of hirschsprung’s > paeds referral
How should constipation be tx?
reassurance, hydration, toilet habits (scheduling visits, bowel diary, rewards, high fibre diet
osmotic laxative eg movicol = 1st line
+/- stimulant laxative eg senna
may require disimpaction regimen initially
tx for 6m - slowly wean off
What is bronchiolotis?
viral LFTI leading to inflammation and infection of the bronchioles
What is the most common cause of bronchiolitis?
RSV - respiratory syncytial virus
Which children is bronchiolitis more likely to happen to?
very common in children under 1yr (mostly under 6mo) in winter
Why does bronchiolitis affect children differently to adults?
smaller airways > small amount of inflammation/mucus in airway has significant effect on infant’s ability to circulate air to the alveoli > harsh breath sounds
What are the S&S of bronchiolitis?
coryzal sx: runny/snotty nose, sneezing, mucus in throat, watery eyes
precede a dry cough
signs of resp distress
dyspnoea, tachypnoea
poor feeding
mild fever
apnoeas
wheeze + crackles on auscultation
What are the signs of resp distress?
raised resp rate
use of accessory muscles to breath - sternocleidomastoid, abdo, intercostal
inter/subcostal recessions
nasal flaring
head bobbing
tracheal tugging
cyanosis
abnormal airway noises
What is a wheeze? When is it heard? Where from?
continuous whistling musical sound
polyphonic - caused by air being forced through multiple lower airways
affects lower airways, comes from lungs
heard more on expiration
What is stridor? When is it heard? Where from?
high pitched turbulent sound, monophonic - through singular upper airway
affects upper airway outside of chest cavity
heard more in inspiration
= emergency - sign of impending airway obstruction
What are the causes of a wheeze?
asthma, COPD
bronchiolitis, bronchitis, bronchiectasis
viral induced wheeze
pneumonia
pulmonary oedema
PE
HF
What are the causes of stridor?
croup
epiglottitis
bacterial tracheitis
diphtheria
laryngomalacia
inhaled foreign body
angioedema/anaphylaxis
Why does grunting occur?
exhaling with glottis partially closed to increased +ve end-exp pressure
How long does bronchiolitis tend to last?
starts as an URTI with coryzal sx - usually half get better
other half develop chest sx
sx worst on day 3/4
last 7-10 days total and recover within 2/3 weeks
Infants who have had bronchiolitis are at higher risk of what in childhood?
viral induced wheeze
How is bronchiolitis managed?
usually supportive mx - adequate intake, saline nasal drops
humidified o2
admission if <3mo/pre-existing condition/clinically unwell
supplementary o2 if sats below 92%
ventilatory support if required
What are the 3 stages of ventilatory support?
- high-flow humidified O2: via tight nasal cannula, delivers air and O2 continuously with some added pressure, adds +ve end-exp pressure (PEEP) to maintain airway at end of exp
- continuous positive airway pressure (CPAP): sealed nasal cannula that can deliver much higher and more controlled pressures
- intubation + ventilation: inserting endotracheal tube into trachea to full control ventilation
What is the best test to monitor resp distress/children on ventilatory support? What results indicate poor ventilation?
capillary blood gas
rising pCO2 - airways have collapsed and can’t clear CO2
falling pH - CO2 building up and not able to buffer acidosis this creates = resp acidosis, if + hypoxic = T2RF
What preventative monthly injection can be given for bronchiolitis caused by RSV? Which babies are given it?
IM palivizumab (mab) - provides passive protection
for high risk babies eg premature, congenital heart disease, CF, bronchopulmonary dysplasia
How is bronchiolitis diagnosed? What is a DDx that must be ruled out?
clinically
CXR to rule out pneumonia - suspect if high fever + localised signs suggesting consolidation
What is croup?
URTI causing oedema in the larynx, trachea and bronchi (acute laryngotracheobronchitis)
What is croup caused by?
parainfluenza virus
sometimes influenza, adenovirus, RSV
When/which children get croup?
children between 6mo and 6yrs, in autumn
What are the S&S of croup?
initially low grade fever + coryzal sx
stridor
hoarse voice
BARKING/SEAL-LIKE COUGH in cluster episodes
breathing difficulty
nocturnal sx
How is croup treated?
supportive tx at home usually - reassurance, fluids, rest, measures taken to avoid spread, if <12mo - low threshold for admission
1st line = oral dexamethasone (2nd = prednisolone)
usually improves in 48hrs, repeat after 12hrs if not better
then stepwise:
O2
nebulised budesonide
nebulised adrenaline if severe
intubation + ventilation
Croup caused by diptheria causes what?
epiglottitis - high mortality
What is epiglottitis caused by?
haemophilus influenza B
others - strep pyogenes, strep pneumoniae
Why has the incidence of influenza reduced?
HiB vaccine - be suspicious in children without the vaccine
What are the S&S of epiglottitis?
very acute onset
4Ds: dysphagia, dysphonia (muffled voice), distress, DROOLING
extremely sore throat
high fever, look septic/unwell
no/minimal cough
tripod position - sx improve on sitting upright and learning forward (often child will be immobile sitting upright with open mouth)
increasing resp difficulty
inspiratory SOFT STRIDOR
How is epiglottitis investigated?
no time!!
in theory:
laryngoscopy - beefy-red-stuff oedamatous epiglottitis = diagnostic
lateral neck XR - thumbprint sign
exclude foreign body
How is epiglottitis managed?
life threatening emergency - need senior paediatrician + anaesthetist
DO NOT EXAMINE THROAT + don’t panic pt
protect airway - nasotracheal intubation if needed, most don’t need
emergency tracheostomy if throat closes - admit to ITU if intubated
BCs once airway secured
IV cefotriaxone + dexamethasone
rifampicin for close contact prophylaxis
What are the complications of epiglottitis?
death
epiglottic abscess - collection of pus around epiglottitis, also a life-threatening emergency, tx similar
How is croup and epiglottitis differentiated?
epiglottitis can present similar but with a more rapid onset
look for: unvaccinated children, fever, sore throat, difficulty swallowing, sitting forward and drooling
How is croup differentiated from bacterial tracheitis?
BT has more toxic appearance
How is croup differentiated from laryngomalacia?
laryngomalacia has no chest signs
What conditions are asthma a/w?
atopic conditions eg eczema, hay fever, food allergies
fhx of atopic conditions
What triggers are asthma a/w?
infection
exercise
cold air
dust
animals
smoke/pollution
food allergens
NSAIDs/beta blockers
What are the 3 components in the pathophysiology of asthma?
reversible increase in airway resistance in response to irritant stimuli
caused by:
1. bronchial smooth muscle contraction - hypersensitive
2. mucosal inflammation (t-helper cell activation + cytokine production)
3. increased mucus production
What is bronchoconstriction caused by asthma reversible with?
bronchodilators
How does the sympathetic and parasympathetic nervous system affect bronchial calibre?
sympathetic: bronchodilation + decreases mucous secretion via B2-adrenoceptors
parasympathetic: bronchoconstriction + increases mucous secretion via M3 receptors
What features of a history make a resp presentation less likely to be asthma?
wheeze only related to coughs/colds > viral induced wheeze
isolated/productive cough
normal ix
no response to treatment
unilateral wheeze > focal lesion/foreign body/infection
What are the sx of asthma?
dry cough with bilateral widespread polyphonic wheeze, SoB and a tight chest
diurnal variability - worse at night and early morning
episodic sx with intermittent exacerbations
sx improve with bronchodilator
typical triggers
low exercise tolerance
hx/fhx of other atopic conditions
sx better on days off work
What are the signs of an acute asthma attack?
progressively worsening SoB
signs of resp distress
tachypnoea
What obs and signs indicate a severe/life-threatening asthma attack?
SpO2 < 92%
severe = too breathless to talk/feed, HR >140, RR >40, use of accessory muscles
life-threatening = silent chest, poor resp effort, agitation, altered consciousness, cyanosis, trachea tug, subcostal recessions
What kind of wheeze is a/w asthma?
Bilateral widespread expiratory polyphonic wheeze
What chest sounds are a red flag in an asthmatic child?
silent chest > chest so tight that child can’t move air through airways to create a wheeze
a/w reduced resp effort due to fatigue
no wheeze doesn’t always = no resp distress!
How is asthma diagnosed?
diagnosis = ix demonstrate variable airway obstruction/inflammation + clinical picture
if ix inconclusive > try tx and monitor sx - improvement = diagnostic
5-16yrs:
1. spirometry
2. if +ve > BDR test
3. still uncertain > fractional exhaled NO
4. still uncertain > monitor PEF variability for 2-4 weeks
under 5yrs: based on S&S, review regularly, do ix if still symptomatic at 5yrs
What are the 2 mainstays of asthma treatment?
bronchodilator relievers
anti-inflammatory preventors
How is asthma treated in <5yrs?
- SABA inhaler PRN
- add low dose corticosteroid inhaler OR leukotriene antagonist
- add other option from step 2
- refer
How is asthma treated in >5yrs?
- SABA PRN
- add low dose corticosteroid inhaler
- add LABA, continue only if good response
- titrate up corticosteroid to medium dose, consider adding an oral leukotriene receptor antagonist/oral theophylline/inhaled LAMA
- titrate up corticosteroid to high dose, combine additional tx from step 4/add oral beta-2 agonist
- refer
- consider oral daily steroids at lowest possible dose
What are the basic steps of treating an acute asthma attack?
O2 (if sats <94%/working hard)
bronchodilators - beta agonist +++
steroids - PO prednisolone/IV hydrocortisone (reduce airway inflammation)
abx if bacterial cause suspected - amoxicillin/erythromycin
How should a salbutamol inhaler be used for a mild asthma attack?
regular salbutamol inhalers via a spacer
4-6 puffs every 4hrs
How should a mod-severe asthma attack be managed?
stepwise until control achieved:
1. salbutamol inhalers via spacer - start with 10 puffs every 2hrs
2. nebulisers with salbutamol/ipratropium bromide
3. oral prednisolone - 1mg/kg 1/day for 3 days
4. IV hydrocortisone
5. IV MgSO4
6. IV salbutamol
7. IV aminophylline
peak flow before and after tx, must be >75% after tx before discharge
control achieved > gradually work back down
still no control > need anaesthetist + ICU, consider intubation + ventilation
A child can be discharged after an acute asthma attack when they are needing how much salbutamol?
well on 6 puffs 4hrly
prescribe reducing regime for home to get down to 2-4 puffs PRN
What is a possible dangerous SE of salbutamol?
causes K+ to be absorbed into cells > hypokalaemia
consider monitoring serum K+
also tachycardia, tremor
What is the most common reason for uncontrolled asthma?
poor technique
What medications are CI in asthma?
NSAIDS - inhibit COX pathway > promote arachidonic acid conversion to leukotrienes > can provoke asthma
Beta-blockers > CI!!! - reduce effectiveness of B2-agonists
What is the fractional exhaled nitric oxide test for asthma?
measured exhaled NO = marker of eosinophilic inflammation
What is spirometry? What is a poor result?
measures volume of air expelled from lungs after maximal inspiration
FEV1:FVC < 70%
How can spirometry be used to check reversibility?
bronchodilator reversibility: check spirometry change before and 15 mins after SABA inhalation
improvement in FEV1 of 12%+
What is peak expiratory flow? What is a result indicative of asthma?
measures max speed of expiration
monitor twice daily for 2-4wks
variability 20%+
What is a direct bronchial challenge test?
measures change in spirometry after methacholine/histamine inhalation
How does a SABA work? Give an example? What are the SEs?
B2-agonism > bronchial smooth muscle relaxation > dilates bronchi > improves airflow in obstructed airway
eg salbutamol
SEs: TREMOR, tachycardia, palpitations
How do ICS work? Give an example? What are the SEs?
reduce leukocyte proliferation + downregulate pro-inflammatory cytokine, leukotriene and chemokine production > reduces mucosal inflammation, dilates airways + reduces mucous secretion
eg beclometasone
SEs: ORAL CANDIDIASIS, hoarse voice, regular high dose > adrenal suppression (monitor growth)
How do leukotriene receptor antagonists work? Give an example.
down-regulate inflammatory leukotrienes
eg montelukast
Give an example of a LABA. What must they always be given alongise?
salmeterol
given alongside an ICS
Give an example of a LAMA. How do they work?
tiotropium
bind to M3 muscarinic receptors and block bronchoconstriction effect of acetylcholine at these receptors in the airway smooth muscle
What class of drug is theophylline?
xanthine - bronchodilator
What is viral induced wheeze?
acute wheezy illness caused by viral infection
What viruses generally cause a viral induced wheeze?
RSV, rhinovirus
Why do viral illnesses lead to a viral induced wheeze?
children have small airways > inflammation + oedema occurs in virus > restrict air flow + inflammation triggers bronchoconstriction = proportionally large air flow restriction
How do distinguish between a viral induced wheeze and asthma?
VIW: present before 3yrs, no atopic hx, only occurs during viral infections
asthma: can be triggered by a viral infection but has lots of other triggers, variable and reversible airflow obstruciont
What is the classic presentation of a viral induced wheeze?
viral illness (fever, cough, coryzal sx) for 1-2 days before:
SoB
signs of resp distress
expiratory wheeze throughout the chest
What should a focal wheeze make you concerned about?
inhaled foreign body
tumour
How is viral induced wheeze managed?
same as acute asthma attack
What is the genetic mutation that causes CF? How is it passed on?
AR
mutation of the CF transmembrane conductance regulatory gene on chromosome 7 > affects mucous glands
most common variation affects a particular type of Cl- channel
What are the key consequences of the CF mutation?
- thick pancreatic + biliary secretions > blockage of the ducts > lack of digestive enzymes in digestive tract eg pancreatic lipase
- low volume thick airway secretion > reduce airway clearance > bacterial colonisation > susceptibility to airway infections
- congenital bilateral absence of vas deferens in males > healthy sperm but no route from testes to ejaculate > male infertility
How is CF screened for?
newborn bloodspot test
What is usually the first sign of CF?
meconium ileus > meconium thick and sticky > gets stuck and obstructs the bowel
> no meconium passed within 24hrs, abdominal distention, vomiting
= pathognomonic for CF
What are the sx of CF?
chronic cough
thick sputum production
recurrent RTIs
steatorrhoea (lack of lipase)
abdo pain + bloating
salty sweat
FtT - poor weight and height gain
What are the signs of CF?
low weight/heigh on growth chart
nasal polyps
finger clubbing
crackles/wheezes on auscultation
abdo distention
What are the causes of clubbing in children?
hereditary
cardiac: cyanotic heart disease, IE
resp: CF, TB
gastro: IBD, liver cirrhosis
What are the three key diagnostic tests for CF?
newborn blood spot testing - picks up most cases
GS = sweat test
genetic testing for CFTR gene (on amniocentesis, chorionic villous sampling or as blood test after birth)
What is the CF sweat test?
electrodes placed either side of patch of skin > small current passed causes skin to sweat > sweat absorbed and tested for Cl- conc
What are the most common colonisers in the lungs in children with CF?
struggle to clear secretions > lots of moisture and O2 for colonies
staph aureus
pseudomonas
What can CF pts take long-term to prevent staph aureus chest infection?
prophylactic flucloxacillin
Why are children with CF advised to avoid contact with each other?
pseudomonas difficult to get rid of - becomes resistant to multiple abx
significant increase in morbidity and mortality
easily passed on between CF pts
How is a pseudomonas colonisation treated in a child with CF?
long-term nebulised abx eg tobramycin
or oral ciprofloxacin
How is CF managed?
chest physio + exercise
prophylactic fluclox
bronchodilators
nebulised DNase - breaks down DNA material in resp secretions > less viscous
nebulised hypertonic saline
high calorie diet - due to malabsorption, increased resp effort, coughing, infections
CREON (pancrelipase)
vaccines - pneumococcal, influenza, varicella
What conditions do CF pts need to be regularly monitored and screened for?
pseudomonas - sputum
diabetes
OP, vit D deficiency
liver failure
What is the prognosis of CF? What affects the prognosis?
depends on severity of sx, type of mutation, adherence to tx, frequency of infection/lifestyle
median LE = 47yrs
90% have pancreatic insufficiency
50% have CF related diabetes
30% have liver disease
most males infertile
What is laryngomalacia?
the supraglottic larynx is structured in a way that causes partial airway obstruction
short aryepiglottic folds > pulls on epiglottis = omega shape > tissue surrounding is softer/less tone > can flop across the airway, esp during inspiration = partial occlusion
How does laryngomalacia present?
intermittent inspiratory stridor, prominent on feeding, upset, lying on back, in URTI
no resp distress
generally resolves with age
How does pneumonia present?
cough - wet, productive
high fever
tachypnoea, tachycardia
increased work of breathing
lethargy
delirium
What are the characteristic chest sounds heard in pneumonia?
bronchial breath sounds - harsh sounds equally loud in insp + exp due to consolidation of lung tissue around airway
focal coarse crackles - due to air passing through sputum
dullness to percussion - due to lung collapse or consolidation
What is the most common bacterial and viral cause of pneumonia?
bact: strep pneumonia
viral: RSV
What is the 1st line mx for pneumonia?
amoxicillin
+ macrolide eg erythromycin to cover atypical pneumonia/pencillin allergy
IV if sepsis/malabsorption
What virus is mumps caused by?
RNA paramyxovirus - mumps
How is mumps transmitted?
resp droplets
How does mumps present?
prodrome of coryzal sx - fever, muscle aches, lethargy, reduced appetite, headache
PAROTID GLAND SWELLING - uni/bilateral, a/w pain
trismus - spasm of muscles of mastication when chewing
possibly ear ache
What are the possible complications of mumps?
pancreatitis (abdo pain)
orchitis (testicular pain + swelling)
meningitis/encephalitis (confusion, neck stiffness, headache)
sensorineural hearing loss
How is mumps managed?
notifiable disease
supportive - rest, fluids, analgesia
school exclusion for 7 days
What virus causes measles?
RNA paramyxovirus - morbillivirus
How does measles present?
catarrhal stage 10-12 days after exposure: 4Cs - cough, cranky, coryza, conjunctivitis
koplik spots 2 days after fever - grey/white spots on buccal mucosa = pathognomonic
macular erythematous rash 3-5 days after fever starts - starts on face, classically behind ears, spreads down
What are the possible complications of measles?
1/3rd have a complication
pneumonia
diarrhoea, dehydration
encephalitis, meningitis
hearing/vision loss
What is the most common complication of measles?
otitis media
How is measles managed?
rest
isolation for 4 days from rash onset
notifiable disease
highly contagious
What virus causes rubella?
RNA paramyxovirus - rubivirus
What are the clinical features of rubella?
coryzal prodrome - mild fever, sore throat
milder pink macular erythematous rash than measles, starts on face and spreads to rest of body, lasts about 3 days
joint pain
lymphadenopathy - sub occipital/posterior auricular
How are the MMR diseases diagnosed?
clinically
saliva swab for igM as all are notifiable
How is rubella managed?
isolation for 5 days after rash appears
rest
Which group of people is rubella most dangerous in?
pregnant women
<13 wks transmission to fetus = 80% > TOP offered
>16 wks transmission = 25%, unlikely to cause defects
What is the congenital rubella triad?
sensorineural deafness
cardiac abnormalities
eye abnormalities eg cataracts
