sepsis/HSP/VSDs/biliary atresia

Question 1

concerning symptoms

Answer 1

if a child is not rousable or does not stay awake when roused

bradycardia / tachycardia

bradypnoea / tachypnoea

mottled skin

peripheral or central cyanosis

non-blanching rash

Question 2

symptoms in children with sepsis

Answer 2

Generally feeling unwell
Lethargy
Poor social interaction
Altered conscious level
Continuous crying in babies
Pale or mottled skin
Rash
Decreased urine output
Poor feeding or oral intake
Cool peripheries

Question 3

diagnosis of sepsis

Answer 3

FBC
CRP
U&Es
blood culture
creatine
clotting screen
lactate
blood gas
urinalysis
CXR
imaging of abdomen and pelvis
lumbar puncture
swabs

Question 4

mx of sepsis

Answer 4

empirically broad-spectrum antibiotics early
high flow oxygen and fluid blouse
escalate to senior paediatrician for review within 1 hour
close monitoring

Question 5

A 3 year old boy is brought to A&E by his mother who is very worried about him. He has been lethargic for the last 24 hours, not wanting to eat or drink much and his mother says he looks pale compared to normal. Prior to that he was well, with no cough, coryzal symptoms or sign of infection. He has a history of type 1 diabetes mellitus, which is managed with a basal-bolus insulin regime, administered in three daily doses. His blood sugars are well controlled.

On examination the boy is laid down on the bed, rousable to voice but closes his eyes again soon after. There is no visible rash, heart sounds are normal and chest clear on auscultation. He has some mild suprapubic tenderness but no guarding or rigidity of the abdomen. Bowel sounds are normal. Vital observations: temperature 38.4 degrees Celsius, heart rate 120bpm, respiratory rate 24/min, oxygen saturation 98% on room air, blood pressure 100/70.

Which is the following is the most appropriate first-line investigation?

Answer 5

urinalysis - suprapubic tenderness

to rule out urinary tract infection

Question 5

A 3 year old boy is brought to A&E by his mother who is very worried about him. He has been lethargic for the last 24 hours, not wanting to eat or drink much and his mother says he looks pale compared to normal. Prior to that he was well, with no cough, coryzal symptoms or sign of infection. He has a history of type 1 diabetes mellitus, which is managed with a basal-bolus insulin regime, administered in three daily doses. His blood sugars are well controlled.

On examination the boy is laid down on the bed, rousable to voice but closes his eyes again soon after. There is no visible rash, heart sounds are normal and chest clear on auscultation. He has some mild suprapubic tenderness but no guarding or rigidity of the abdomen. Bowel sounds are normal. Vital observations: temperature 38.4 degrees Celsius, heart rate 120bpm, respiratory rate 24/min, oxygen saturation 98% on room air, blood pressure 100/70.

Which is the following is the most appropriate first-line investigation?

Answer 5

urinalysis - suprapubic tenderness

to rule out urinary tract infection

Question 6

what is biliary atresia characterised by

Answer 6

progressive fibrosis and destruction of the biliary tree

Question 7

what is henoch schonlein purpura

Answer 7

(HSP) is the most common small vessel vasculitis in children
commonly affects 3-5 year olds

Question 8

how does HSP present

Answer 8

purpura or petechia on the buttocks or lower limbs
abdominal pain
arthralgia
nephritis
pyrexia

and is commonly preceded by a viral URTI

Question 9

mx of HSP

Answer 9

NSAIDs for analgesia and their anti-inflammatory effect

antihypertensives may be needed to control BP

after an episode of HSP children should have regular urine dips for 12 months to check for renal impairment

Question 10

what is biliary atresia

Answer 10

a rare condition where the bile ducts of an infant are progressively fibrosed and destroyed leading to conjugated hyperbilirubinaemia, liver failure and death if not treated

Question 11

how does biliary atresia present

Answer 11

prolonged jaundice
signs of biliary obstruction

Question 12

diagnosed of biliary atresia

Answer 12

bloods = raised conjugated bilirubin and deranged LFTs
hepatic scintigraphy = show poor excretion into the bowel (as the bile ducts connecting the liver and the gut have been destroyed)
abdominal US = echogenic fibrosis
definitive diagnosis is with CHOLANGIOGRAPHY

Question 13

mx of biliary atresia

Answer 13

surgery - Kasai procedure (hepatoportoenterostomy)

Question 14

pansystolic murmur harsh, heard loudest at the left lower sternal edge

Answer 14

ventricular septal defect

Question 15

what syndrome is most commonly associated with ventral septal defect

Answer 15

Down syndrome ie epicanthic folds, single palmar crease, up-slanting palpebral fissures

Question 16

how are VSDs definitively diagnosed

Answer 16

echocardiogram

Question 17

continuous murmur heard loudest under left clavicle

Answer 17

PDA ‘acyanotic’

Question 18

Diagnosis of HSP

Answer 18

Urine dip

Need to establish if there is any renal involvement > as IgA immune complexes can deposit in the kidneys and cause haematuria and proteinuria

also do BP

Question 19

What is the classic triad for Henoch Schonlein purpura

Answer 19

Abdominal pain, arthralgia and purpura