Paediatric Endocrinology

Question 1

Investigations for T1DM

Answer 1

CBG
Urinary ketones
ABG - metabolic acidosis

Question 2

Fasting blood glucose level

Answer 2

Positive = > 7mmol/l

Question 3

Randome blood glucose level

Answer 3

Positive = > 11mmol/L

Question 4

Ketoacidosis blood pH

Answer 4

pH < 7.3

Question 5

Presentation of T1DM in children

Answer 5

May present with DKA

• polydipsia
• polyuria
• fatigue and lethargy
• weight loss

May have:

• secondary enuresis
• recurrent UTIs

Question 6

Management of T1DM

Answer 6

Patient and family education

• how ro take insulin subcut
• how to monitor blood sugar
• how to measure carb intake
• how to monitor for complications

Insulin - long and short acting for a basal bolus regime

Question 7

Insulin pump

Answer 7

Devices that continuously infuse insulin at different rates to control blood sugar levels

Cannula must be replaced every 2 - 3 days and the insertion site should be rotated

Question 8

Who qualifies for an insulin pump

Answer 8

Over 12 yo

Difficulty controlling their HbA1c

Question 9

Advantages and disadvantages of an insulin pump

Answer 9

Advantages:

• less injections
• less restrictions on diet
• better blood control

Disadvantages:

• difficult to use
• needs to be worn at all times
• may get blocked
• small risk of infections

Question 10

Types of insulin pump

Answer 10

Tethered pump - attached to belt that connects from the pump to the insertion site, controlled by the pump

Patch pump - sits directly on skin without tubes, when insulin runs out, the whole thing is replaced. Controlled by a remote

Question 11

Causes of hypoglycaemia in diabetics

Answer 11

Diarrhoea 
Vomiting 
Poor carbohydrate, insulin balance 
Infection - sepsis 
Malabsorption - coeliacs

Question 12

Presentation of hypoglycaemia

Answer 12

Hunger 
Fatigue 
Tremor 
Sweating 
Irritability 
Dizziness 
Pallor

Question 13

Management of hypoglycaemia

Answer 13

Rapid acting glucose - lucozade
Slower acting carbs - biscuit

Severe:

• IV fluids
• IV dextrose 10%
• IM glucagon

Question 14

Pottasium during DKA

Answer 14

Can be high as lack of insulin to drive it into cells but less store

When treated with insulin, may get hypokalaemia which can lead to arrythmias

Therefore give pottasium via fluids

Question 15

Complications of DKA

Answer 15

Cerebral oedema - dehydration and high blood sugar can move water into the extracellular space by osmosis, rapid correction of blood glucose can cause rapid water intake into brain cells causing oedema

Hypokalaemia/hyperkalaemia - arrythmias

Coma

Question 16

Management of cerebral oedema

Answer 16

Slowing IV infusion of fluids
IV mannitol
IV hypertonic saline

Question 17

Presentation of DKA

Answer 17

Polyuria 
Polydipsia 
Fatigue
Nausea and vomiting 
Altered consciousness 
Acetone smell on breath 
Weight loss 
Dehydration

Question 18

Diagnostic criteria for DKA

Answer 18

Random CBG > 11mmol/l
Blood ketones > 3 mmol/l
Acidosis pH < 7.3

Question 19

Management of DKA

Answer 19

1. IV fluids over 48 hours ( not too fast to prevent cerebral oedema)
2. Fixed rate insulin infusion
3. Once blood glucose falls < 14mmol/l give IV dextrose

Question 20

Addisons disease pathophysiology

Answer 20

Deficiency in cortisol or aldosterone due to a primary adrenal insufficiency

Question 21

Primary, secondary and tertiary adrenal insufficiency

Answer 21

Primary - adrenal glands do not produce enough cortisol or aldosterone

Secondary - lack of adrenal gland stimulation by ACTH commonly due to pituitary hypoplasia, surgery or infection

Tertiary - inadequate CRH release by the hypothalamus, often due to long term steroids ( 3+ weeks)

Question 22

Presentation of adrenal insufficiency

Answer 22

Babies:

• vomiting and poor feeding
• hypoglycaemia
• jaundice
• failure to thrive
• lethargy

Children:

• nausea and vomiting
• poor weight gain or weight loss
• reduced appetite
• abdominal pain
• muscle weakness or cramps
• developmental delay
• bronze hyperpigmentation

Question 23

Investigations for adrenal insufficiency

Answer 23

Bloods - FBC, U+Es (hyperkalaemia) 
Blood glucose 
Synacthen test - ACTH 
Morning cortisol 
Aldosterone: renin ratio

Question 24

Addisons disease cortisol, renin, aldosterone and ACTH levels

Answer 24

Cortisol - low
Aldosterone - low
ACTH - high
Renin - high

Question 25

Secondary adrenal insufficiency cortisol, renin, aldosterone and ACTH levels

Answer 25

Cortisol - low Aldosterone - normal ACTH - low Renin - normal

Question 26

Short synacthen test

Answer 26

ACTH stimulation test performed in the morning - synacthen is synthetic ACTH Synacthen is given which should increase cortisol levels - cortisol measured at baseline and then 30 and 60mins after administration Helps differentiate between primary and secondary adrenal insufficiency

Question 27

Management of adrenal insufficiency

Answer 27

IV Hydrocortisone - glucocorticoid which replaces cortisol Fludrocortisone - mineralocorticoid which replaces aldosterone Steroid card Doses of ateroid increased during illness Regular monitoring

Question 28

Monitoring for adrenal insufficiency

Answer 28

``` Growth and devlopment Blood pressure U+Es Blood glucose Bone profile Vitamin D ```

Question 29

Addisonian crisis presentation

Answer 29

``` Reduced consciousness Hypotension - dizziness Hypoglycaemia - loss of consciousness and fatigue Hyponatraemia - seizures Hyperkalaemia - arrythmias ```

Question 30

Cause of an Addisonian crisis

Answer 30

Abrupt cessation of steroids First presentation of Addisons disease

Question 31

Management of a Addisonian crisis

Answer 31

IV fluids IV hydrocortisone IV fludrocortisone Correct hypoglycaemia

Question 32

Pathophysiology of congenital adrenal hyperplasia

Answer 32

Congenital deficiency of the 21 hydroxylase enzyme which converts progesterone into cortisol and aldosterone causing an underproduction of cortisol and aldosterone and an overproduction of androgens Autosomal recessive

Question 33

Presentation of congenital adrenal hyperplasia

Answer 33

Females - at birth can present with ambiguous genitalia and enlarged clitoris due to high testosterone - tall for age - facial hair - absent periods - deep voice - early puberty - Hyperpigmentation - Males - large penis, small testicles Severe: hyponatraemia, hyperkalaemia and hypoglycaemia - poor feeding - vomiting - dehydration - arrythmias

Question 34

Management of congenital adrenal hyperplasia

Answer 34

Hydrocortisone - cortisol replacement Fludrocortisone - aldosterone replacement Females - may undergo surgery for ambiguous genitals

Question 35

Presentation of growth hormone deficiency

Answer 35

Neonates: - Micropenis - hypoglycaemia - severe jaundice Infants: - poor growth - short stature - slow development of movement - delayed puberty

Question 36

Investigations for growth hormone deficiency

Answer 36

Growth hormone stimulation test - given glucagon or insulin (normally stimulates GH) and monitor levels of GH 2 - 4 hours after Bloods - TFTs, cortisol, aldosterone, renin and ACTH MRI brain - pituitary and hypothalamus Genetic testing - Prader - Willi syndrome and Turners syndrome Xray DEXA scan - bone age

Question 37

Treatment for growth hormone deficiency

Answer 37

Daily subcut injections of growth hormone - somatropin Treatment of other hormone deficiencies Close monitoring of height and development

Question 38

Presentation of congenital hypothyroidism

Answer 38

``` Prolonged neonatal jaundice Poor feeding Constipation Increased sleeping Reduced activity Slow growth and development ```

Question 39

Conditions associated with hypothyroidism

Answer 39

Coeliacs disease | T1DM

Question 40

Common cause of aquired hypothyroidism

Answer 40

Hashimotos disease - due to anti- TPO antibodies (antithyroid peroxidase)

Question 41

Management of hypothyroidism

Answer 41

Levothyroxine orally ods

Question 42

Investigations for hypothyroidism

Answer 42

TFTs Thyroid USS Thyroid antibody tests