Paed's Endocrine Flashcards
Addison’s
- Paediatric S&S
Addison’s S&S
- Fatigue
- Hyperpigmentation
- Arms
- Lips - Vomiting
- Weight loss
Addison’s Paeds DDx
- Fatigue
- Hyperpigmentation
- Vomiting and weightloss
Addison’s DDx
- Vitiligo
- DM T1
- EDs
- Gastroenteritis
Short synACTHen test
- Procedure
Short synACTHen procedure
- Baseline serum cortisol
- 250mcg of IV/IM Synacthen
- 30 minute serum cortisol
Adrenal insufficiency pathophysiology
- Primary
- Secondary
- Tertiary
Adrenal insufficiency
- Primary
1. Autoimmune
2. Reduced secretion - Cortisol
- Aldosterone
- Secondary
1. Inadequate ACTH
2. Commonly - Pituitary hypoplasia
- Surgery
- Infection
- Loss of blood flow
- Radiotherapy
- Tertiary
1. Inadequate CRH
2. Hypothalamus suppression - LT oral steroids
Adrenal insuficiency
- Causes and serology
- Primary
- Secondary
Adrenal insufficiency
- Primary
1. Lesion in adrenal
2. Low cortisol/high ACTH - Hyperpigmentation
- Aldosterone affected
- Secondary/tertiary
1. Lesion in pituitary/hypothalamus
2. Low cortisol/low ACTH - No hyperpigmentation
- Aldosterone unaffected
Addison’s
- Presentation in Babies
Addison’s presentation
- Baby
- Lethargy
- Vomitting
- Poor feeding
- Hypoglycaemia
- Failure to thrive
Addison’s
- Presentation in older children
Addison’s presentation
- Older children
- N+V
- Abdominal pain
- Poor weight gain/weight loss
- Reduced appetite - Developmental delay
- Muscle weakness/cramping
- Bronze hyperpigmentation
Addison’s
- Examination signs
Addison’s examination signs
- Hyperpigmentation
- Generalised
- Palmar creases
- Scars
- Buccal mucosa - Postural hypotension
- Decreased hair
- Axillary
- Pubic
Addison’s
- Investigations
Addison’s Ix
Bedside
1 FBG
Blood
2 U&E
3 Serology
- Cortisol/Aldosterone
- ACTH
- Renin
Further tests
4 SynACTHen
- Baseline, 30 minutes, 60 minutes
- Expect doubling
Addison’s
- Mx
Addison’s Mx
- Glucocorticoid
- Hydrocortisone - Mineralcorticoid
- Fludrocortisone - Steroid card
- Emergency ID tag
- Sickness rules
- Increased to match response - Follow-up
- Paediatric endocrinologist
- Care plan
— Growth and development
— BP
— U&Es
— Glucose
— Bone profile
Addison’s
- Sick day rules
Addison’s Sick-Day Rules
- No change for minor cough/cold
- Indications
1. 38° temperature
2. Vomitting and diarrhoea - Management
- Increased steroid
- Dose and frequency - Blood sugar
- Close monitorig
- Regular carbohydrates - IM steroid as required
Addisonian Crisis
- Mx
Addisonian Crisis
- Fluids
- Hydrocortisone
- 100mg IV bolus
- IM 6 hourly until PO tolerated/24-48hrs - Fludricortisone
- Once eating and drinking - Precipitant treatment
- Usually infection - Monitor
- U&Es
Congenital Adrenal Hyperplasia
- Pathophysiology
CAH Pathophysiology
- Autosomal recessive group
- 21-hydroxylase enzyme
- 11-beta hydroxylase - Low Cortisol
- High ACTH
- Excessive androgens
- Low gluco- and minerocorticoids
Congenital adrenal hyperplasia
- Female Presentation at Birth
CAH Female Birth
- Virilised (ambiguous) genitalia
- Enlarged clitoris - Metabolic disturbance if severe
- Hyponatraemia
- Hyperkalaemia
- Hypoglycaemia
Poor feeding, vomiting, dehydration, arrhythmias
Congenital adrenal hyperplasia
- Males at birth
CAH Males at Birth
- Hyponatraemia and hyperkalaemia
- Hypoglycaemia
- Poor feeding
- Vomitting
- Dehydration
- Arrhythmias