Paediatric Endocrinology

Question 1

What is the first line management of DKA in a child?

Answer 1

Resus fluid of 10ml/kg over 10 mins

Question 2

How can adrenal insufficiency present in a child?

Answer 2

Lethargy
Vomiting
Poor feeding
Hypoglycaemia
Jaundice
Failure to thrive

Question 3

What are lab results in primary adrenal insufficiency (Addison’s)?

Answer 3

Low cortisol
Low Aldosterone
High ACTH
High Renin

Question 4

What are lab results in secondary adrenal insufficiency (due to pituitary)?

Answer 4

Low cortisol
Low ACTH
Normal aldosterone + Normal renin (Aldosterone is produced independently of the pituitary/hypothalamus)

Question 5

What is congenital adrenal hyperplasia?

Answer 5

Congenital deficiency of 21-hydroxylase

Question 6

What is the hereditary pattern of congenital adrenal hyperplasia?

Answer 6

Autosomal recessive

Question 7

How does congenital adrenal hyperplasia affect hormone levels?

Answer 7

Low cortisol
Low aldosterone

Raised androgens (testosterone)

Question 8

How does a severe case of congenital adrenal hyperplasia present?

Answer 8

Presents in neonatal period
Hyponatraemia
Hyperkalaemia
Hypoglycaemia
Poor feeding, dehydration, vomiting

In females - ambiguous genitalia

Question 9

How does mild congenital adrenal hyperplasia present?

Answer 9

Presents during childhood/after puberty

Females - tall for age, facial hair, best periods, deep voice

Males - tall for age, deep voice, large penis, small testicles

Early puberty in both girls and boys

Question 10

How is congenital adrenal hyperplasia treated?

Answer 10

Lifelong hydrocortisone + fludrocortisone

Question 11

How does growth hormone deficiency present?

Answer 11

Presents at birth

Hypoglycaemia
Severe jaundice
Micropenis (in boys)

If presents later in life - poor growth, short stature, slow development of movement, delayed puberty

Question 12

How is growth hormone deficiency investigated?

Answer 12

Growth hormone stimulation test (with glucagon/insulin)

MRI Brain

Xray of wrist to determine bone age

Question 13

How is growth hormone deficiency managed?

Answer 13

Daily SC growth hormone (Somatropin)

Question 14

How does puberty progress in girls?

Answer 14

Breast buds –> Pubic hair –> menstruation

Question 15

How does puberty progress in boys?

Answer 15

Testicles enlargement –> Penis enlargement –> Scrotum darkening –> Pubic hair –> Deepening of voice

Question 16

What age should delayed puberty be investigated in girls?

Answer 16

13

Question 17

What age should delayed puberty be investigated in boys?

Answer 17

14

Question 18

An 8 year old girl presents to urgent care with a one day history of vomiting and abdominal pain, and a 2 week history of drinking a lot and getting up 9 times a night to urinate. Her mother is also concerned that she has lost weight recently. She is otherwise a healthy child. Her sister has a thyroid condition.

On examination, the girl is alert and chatty. Her abdomen is soft and nontender without organomegaly. A urine dip shows glucose +++ and ketones ++. A random blood sugar measurement is 33.2. The diagnosis is explained and the child is admitted overnight for an insulin infusion. She is seen by a specialist team and discharged a few days later with an insulin pen. For the next 2 weeks, her blood glucose level is well controlled on just one unit of insulin a day.

What best explains this patient’s current insulin requirements?

A. Residual function of remaining beta cells

B. Rapid regeneration of beta cells

C. Insufficient dose of exogenous insulin

D. Excessive exogenous insulin dose

E. Perfect lifelong insulin regimen identified

Answer 18

A. Residual function of remaining beta cells

This patient with very low insulin requirements after a recent diagnosis of type 1 diabetes mellitus (polydipsia, polyuria, weight loss, raised random blood glucose) presenting in moderate DKA (vomiting, ketonuria) is experiencing a honeymoon period. The ‘honeymoon period’ in T1DM describes a time where the disease has been identified and is being treated with exogenous insulin, but there is still some residual insulin production as not all beta pancreatic cells have been destroyed. As a result, the patient’s insulin requirements may be very low. Children should be monitored closely during this period, as once the last few pancreatic cells are destroyed and endogenous insulin production completely stops, the child’s exogenous insulin requirements will suddenly jump

Question 19

A 15 year old girl is brought to A&E unconscious after a seizure. Her friends said she had been acting strangely and said her stomach hurt that morning. She was sweaty in class, and then fainted and started jerking for a few minutes. She has a background of type 1 diabetes mellitus.

On examination, the girl is not responsive to pain. Her respiratory rate is 16, heart rate is 100 and blood pressure is 98/54. Her pupils are 3 mm diameter, equal and reactive to light.

What is the most appropriate definitive management at this stage in the emergency department?

A. Encourage to eat a biscuit

B. IV Naloxone

C. Intramuscular glucagon

D. 2ml/kg IV 10% dextrose

E. Pour lucozade into her mouth

Answer 19

D. 2ml/kg IV 10% dextrose

This teenager with known Type 1 diabetes who missed a meal and experienced abdominal pain, strange behaviour, sweating, loss of consciousness and seizure is presenting with acute hypoglycaemia. As she has arrived in A&E, the most appropriate definitive management is an intravenous glucose solution

Question 20

shocked vs not shocked IV fluid bolus:

Answer 20

shocked: 10ml/kg bolus over 15 mins
not shocked: 10ml/kg bolus over 30 mins

Question 21

A 5-year-old boy presents to Accident & Emergency with a one-day history of abdominal pain and vomiting. He weighs 40 kg and has no past medical history of note.

Observations on triage: Temperature 36.5°C, pulse 90, blood pressure 100/70, SpO2 98% on room air.

On examination, his abdomen is soft with mild generalised tenderness, and bowel sounds are present.

Initial investigations are as follows:

Blood glucose 30.5 mmol/L
Blood ketones 4 mmol/L
Na 124 mmol/L
K 5.5 mmol/L
Cl 85 mmol/L
Urea 8.0 mmol/L
Creatinine 100 μmol/L
Arterial blood gas:

pH 7.15
pO2 12 kPa
pCO2 4.0 kPa
HCO3 14 mmol/L
Which is the next most appropriate prescription to administer?

A. 400 ml bolus of 0.9% normal saline over 1 hour

B. 100 ml of 2.7% hypertonic sodium chloride over 15 minutes

C. Intravenous sodium bicarbonate

D. Oral calcium resonium

E. 1900 ml of 0.9% normal saline over 24 hours

Answer 21

A. 400 ml bolus of 0.9% normal saline over 1 hour

This patient has presented with diabetic ketoacidosis, meeting the criteria of hyperglycaemia, acidosis and ketonaemia. A resuscitation fluid bolus of maximum 10-20ml/kg 0.9% normal saline should be given initially. It should not exceed this amount due to the increased risk of cerebral oedema. Subsequently, the maintenance fluid volumes should be calculated, and the volume deficit gradually replaced over the next 48 hours. The initial resuscitation fluid bolus should be subtracted from the maintenance fluid volume if the patient is not in shock

Not: B; 100 ml of 2.7% hypertonic sodium chloride over 15 minutes

This is reserved for symptomatic hyponatraemia with a depressed level of consciousness and a high risk of cerebral oedema, which may occur if fluid administration is given too rapidly. This should not be given without discussing with a senior first! In diabetic ketoacidosis, the sodium levels should always be corrected for hyperglycaemia

Not E: 1900 ml of 0.9% normal saline over 24 hours : This would be a suitable maintenance fluid regimen. Maintenance fluid volumes for children in DKA can be calculated as recommended by the BPSED DKA guideline as: 100ml/kg/day for the first 10kg of bodyweight, 50ml/kg/day for weight between 10-20kg and then 20ml/kg/day for each additional kg above 20kg. For a 40kg boy, it would be (100ml x 10kg) + (50ml x 10kg) + (20ml x 20kg) = 1000ml + 500ml + 400ml = 1900ml over 24 hours