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
Congential adrenal hyperplasia
- Adolescent presentation
CAH Adolescent presentation
Female
1. Tall
2. Precocious puberty, absent periods
3. Facial hair, deep voice
Male
- Tall for age
- Percocious puberty, small testicles
- Deep voice, large penis
Congenital adrenal hyperplasia
— Ix
CAH Investigatioins
- New-born screening
- Heel-prick capillary sample
- 17-hydroxyprogesterone
- 11-deoxycortisol - Bloods
- FBC
- U&E
- Calcium - Genetic testing
- Karyotyping for X&Y if ambiguous genitalia
Congential adrenal hyperplasia
- Management
CAH Management
- Paediatric endocrinologist
- Steroid replacement
- Hydrocortisone
- Fludrocortisone - Corrective surgery
- Vaginoplasty
- Clitoral recession
Paed’s DM
- Diagnosis
Paed’s DM Dx
- Symptoms
- Random CBG >11.0
Paeds DM
- Ix
Paeds DM Ix
- HBA1c
- FBG
- OGTT
- Auto antibodies
DMT1
- Natural History
DMT1 Natural Hx
- Genetic predisposition
- Beta Cells
- Normal Mass at Birth - Trigger
- eg. Viral infection - Beta Cell Loss
- Immunologic abnormality
- Stage 1 (normal CBG)
- Stage 4 (long-standing T1DM)
DKA
- Parameters
DKA Parameters
D iabetic - >11.0 CBG
Ketonaemic - 3mmol/l
Acidosis - pH < 7.3
(bicarb<15)
DKA Complications
DKA
- Cerebral Oedema
1. <1% of all DKAs
2. 20% Mortality - Hypokalaemia
1. Monitor for cardiac change
2. Consider changing insulin infusion
Cerebral Oedema
- S&S
- Triad
Cerebral Oedema S&S
- Headache
- Confusion
- Irritability
- Drowsiness
- HTN
- Bradicardia
- Focal neuropathy
- Palsies
- Papilloedema
Paeds DKA
- Mx
Paeds DKA
- ABC
- Resus fluids
(15 min bolus in shock) - Deficit fluids
Based on pH at presentation - Maintenance fluids (Paeds rules)
- IV Insulin
- 1-2hrs AFTER fluids - Potassium
- Monitor and replace
- Do not replace in anuric patients
- ECG monitoring
Paeds DKA
- Insulin Regimin
Paeds DKA Insulin
- 1-2hrs AFTER insulin
- 0.05-0.1 units/kg/hour
Paeds Hypothyroid
- Epidemiology
- Presentation
Paeds Hypothyroid
- Epidemiology
1. 1/2000 congenital
2. Primary agenesis/dystrophy
3. HPT axis
4. Transient (1-3yo) - Presentation
1. Sleepy
2. Poor growth
3. LD
- Jaundiced
- Hypothermic
- Open sutures/fontanelles
Paeds Hypothyroid
- Ix
- Mx
Paeds Hypothyroid
- Ix
- TSH, T4
- TPO
- Tc-99m (pertechnetate scintigraphy)
- USS
Puberty
- Progression/consonance
Puberty
- Female Onset
- 8-13
- Thelarche, growth, hair, menarche - Male Onset
- 9-14
- Testicular size, growth, hair, voice
Precocious puberty
- Ix
- Tx
Precocious puberty
- Ix
1. TFT
2. LH/FSH - Mx
1. GnRH analogue - Non-pulsatile
2. Cease at 10/11 yo
Delayed puberty
- Epidemiology
- Management
Delayed puberty
- Common in boys
1. Constitutional
2. Treat with T - Uncommon in girls
Child growth
- Determinants
Child growth determinants
- 90% parental height
- Hormones
- GH
- Sex H
- THs - Nutrition
- Chronic illness
- Celiac - Home environment
- Care and affection
Low Growth Hormone
- Causes
GH deficiencies
- Primary GHD
- Pituitary dwarfism
- Congenital/acquired - 45, X0 (Turner)
- Prader-Willi
- Ch 15 - Renal disease
57, XXY (Klinefelter’s)
- S&S
57, XXY (Klinefelter’s)
- Tall
- Gynaecomastia
- Delayed puberty