Paed's Endocrine Flashcards

1
Q

Addison’s
- Paediatric S&S

A

Addison’s S&S

  1. Fatigue
  2. Hyperpigmentation
    - Arms
    - Lips
  3. Vomiting
  4. Weight loss
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2
Q

Addison’s Paeds DDx

  • Fatigue
  • Hyperpigmentation
  • Vomiting and weightloss
A

Addison’s DDx

  1. Vitiligo
  2. DM T1
  3. EDs
  4. Gastroenteritis
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3
Q

Short synACTHen test

  • Procedure
A

Short synACTHen procedure

  1. Baseline serum cortisol
  2. 250mcg of IV/IM Synacthen
  3. 30 minute serum cortisol
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4
Q

Adrenal insufficiency pathophysiology

  1. Primary
  2. Secondary
  3. Tertiary
A

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
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5
Q

Adrenal insuficiency
- Causes and serology

  1. Primary
  2. Secondary
A

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
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6
Q

Addison’s

  • Presentation in Babies
A

Addison’s presentation
- Baby

  1. Lethargy
  2. Vomitting
  3. Poor feeding
  4. Hypoglycaemia
  5. Failure to thrive
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7
Q

Addison’s
- Presentation in older children

A

Addison’s presentation
- Older children

  1. N+V
  2. Abdominal pain
  3. Poor weight gain/weight loss
    - Reduced appetite
  4. Developmental delay
  5. Muscle weakness/cramping
  6. Bronze hyperpigmentation
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8
Q

Addison’s

  • Examination signs
A

Addison’s examination signs

  1. Hyperpigmentation
    - Generalised
    - Palmar creases
    - Scars
    - Buccal mucosa
  2. Postural hypotension
  3. Decreased hair
    - Axillary
    - Pubic
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9
Q

Addison’s
- Investigations

A

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

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10
Q

Addison’s
- Mx

A

Addison’s Mx

  1. Glucocorticoid
    - Hydrocortisone
  2. Mineralcorticoid
    - Fludrocortisone
  3. Steroid card
  4. Emergency ID tag
  5. Sickness rules
    - Increased to match response
  6. Follow-up
    - Paediatric endocrinologist
    - Care plan
    — Growth and development
    — BP
    — U&Es
    — Glucose
    — Bone profile
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11
Q

Addison’s
- Sick day rules

A

Addison’s Sick-Day Rules

  1. No change for minor cough/cold
  • Indications
    1. 38° temperature
    2. Vomitting and diarrhoea
  • Management
  1. Increased steroid
    - Dose and frequency
  2. Blood sugar
    - Close monitorig
    - Regular carbohydrates
  3. IM steroid as required
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12
Q

Addisonian Crisis
- Mx

A

Addisonian Crisis

  1. Fluids
  2. Hydrocortisone
    - 100mg IV bolus
    - IM 6 hourly until PO tolerated/24-48hrs
  3. Fludricortisone
    - Once eating and drinking
  4. Precipitant treatment
    - Usually infection
  5. Monitor
    - U&Es
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13
Q

Congenital Adrenal Hyperplasia
- Pathophysiology

A

CAH Pathophysiology

  1. Autosomal recessive group
    - 21-hydroxylase enzyme
    - 11-beta hydroxylase
  2. Low Cortisol
  3. High ACTH
  4. Excessive androgens
    - Low gluco- and minerocorticoids
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14
Q

Congenital adrenal hyperplasia
- Female Presentation at Birth

A

CAH Female Birth

  1. Virilised (ambiguous) genitalia
    - Enlarged clitoris
  2. Metabolic disturbance if severe
    - Hyponatraemia
    - Hyperkalaemia
    - Hypoglycaemia

Poor feeding, vomiting, dehydration, arrhythmias

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15
Q

Congenital adrenal hyperplasia
- Males at birth

A

CAH Males at Birth

  1. Hyponatraemia and hyperkalaemia
  2. Hypoglycaemia
    - Poor feeding
    - Vomitting
    - Dehydration
    - Arrhythmias
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16
Q

Congential adrenal hyperplasia
- Adolescent presentation

A

CAH Adolescent presentation

Female
1. Tall
2. Precocious puberty, absent periods
3. Facial hair, deep voice

Male

  1. Tall for age
  2. Percocious puberty, small testicles
  3. Deep voice, large penis
17
Q

Congenital adrenal hyperplasia
— Ix

A

CAH Investigatioins

  1. New-born screening
    - Heel-prick capillary sample
    - 17-hydroxyprogesterone
    - 11-deoxycortisol
  2. Bloods
    - FBC
    - U&E
    - Calcium
  3. Genetic testing
    - Karyotyping for X&Y if ambiguous genitalia
18
Q

Congential adrenal hyperplasia
- Management

A

CAH Management

  1. Paediatric endocrinologist
  2. Steroid replacement
    - Hydrocortisone
    - Fludrocortisone
  3. Corrective surgery
    - Vaginoplasty
    - Clitoral recession
19
Q

Paed’s DM
- Diagnosis

A

Paed’s DM Dx

  1. Symptoms
  2. Random CBG >11.0
20
Q

Paeds DM
- Ix

A

Paeds DM Ix

  1. HBA1c
  2. FBG
  3. OGTT
  4. Auto antibodies
21
Q

DMT1
- Natural History

A

DMT1 Natural Hx

  1. Genetic predisposition
  2. Beta Cells
    - Normal Mass at Birth
  3. Trigger
    - eg. Viral infection
  4. Beta Cell Loss
    - Immunologic abnormality
    - Stage 1 (normal CBG)
    - Stage 4 (long-standing T1DM)
22
Q

DKA
- Parameters

A

DKA Parameters

D iabetic - >11.0 CBG

Ketonaemic - 3mmol/l

Acidosis - pH < 7.3
(bicarb<15)

23
Q

DKA Complications

A

DKA

  • Cerebral Oedema
    1. <1% of all DKAs
    2. 20% Mortality
  • Hypokalaemia
    1. Monitor for cardiac change
    2. Consider changing insulin infusion
24
Q

Cerebral Oedema

  • S&S
  • Triad
A

Cerebral Oedema S&S

  1. Headache
  2. Confusion
  3. Irritability
  4. Drowsiness
  5. HTN
  6. Bradicardia
  7. Focal neuropathy
    - Palsies
    - Papilloedema
25
Q

Paeds DKA
- Mx

A

Paeds DKA

  1. ABC
    - Resus fluids
    (15 min bolus in shock)
  2. Deficit fluids
    Based on pH at presentation
  3. Maintenance fluids (Paeds rules)
  4. IV Insulin
    - 1-2hrs AFTER fluids
  5. Potassium
    - Monitor and replace
    - Do not replace in anuric patients
    - ECG monitoring
26
Q

Paeds DKA
- Insulin Regimin

A

Paeds DKA Insulin

  1. 1-2hrs AFTER insulin
  2. 0.05-0.1 units/kg/hour
27
Q

Paeds Hypothyroid

  1. Epidemiology
  2. Presentation
A

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
  1. Jaundiced
  2. Hypothermic
  3. Open sutures/fontanelles
28
Q

Paeds Hypothyroid

  1. Ix
  2. Mx
A

Paeds Hypothyroid

  • Ix
  1. TSH, T4
  2. TPO
  3. Tc-99m (pertechnetate scintigraphy)
  4. USS
29
Q

Puberty

  • Progression/consonance
A

Puberty

  1. Female Onset
    - 8-13
    - Thelarche, growth, hair, menarche
  2. Male Onset
    - 9-14
    - Testicular size, growth, hair, voice
30
Q

Precocious puberty

  • Ix
  • Tx
A

Precocious puberty

  • Ix
    1. TFT
    2. LH/FSH
  • Mx
    1. GnRH analogue
  • Non-pulsatile
    2. Cease at 10/11 yo
31
Q

Delayed puberty

  1. Epidemiology
  2. Management
A

Delayed puberty

  • Common in boys
    1. Constitutional
    2. Treat with T
  • Uncommon in girls
32
Q

Child growth
- Determinants

A

Child growth determinants

  1. 90% parental height
  2. Hormones
    - GH
    - Sex H
    - THs
  3. Nutrition
  4. Chronic illness
    - Celiac
  5. Home environment
    - Care and affection
33
Q

Low Growth Hormone
- Causes

A

GH deficiencies

  1. Primary GHD
    - Pituitary dwarfism
    - Congenital/acquired
  2. 45, X0 (Turner)
  3. Prader-Willi
    - Ch 15
  4. Renal disease
34
Q

57, XXY (Klinefelter’s)

  • S&S
A

57, XXY (Klinefelter’s)

  1. Tall
  2. Gynaecomastia
  3. Delayed puberty