HPAA clinical aspects Flashcards Preview

203: The endocrine system > HPAA clinical aspects > Flashcards

Flashcards in HPAA clinical aspects Deck (18)
Loading flashcards...
1

Presentation of Cushing's syndrome

Central obesity

Puffy, moon face

Buffalo hump- fat deposit in the back of the neck.

Hypertension, tachycardia, arrhythmia.

Hirsutism

Diabetes

Osteoporosis

Depression

Thin skin

2

Pathogenesis of Cushings

Excess cortisol production.

Causes
- Pituitary adenoma secreting excess ACTH
- Adrenal adenoma
- Adrenal carcinoma
- Paraneoplastic ectopic ACTH
- Iatrogenic treatment with steroids
- CRH-secreting tumour

3

Diagnostic tests for too much cortisol

24 hr free cortisol in urine levels

Midnight saliva/ blood cortisol

9 AM ACTH levels compared with cortisol
- Determines if it is pituitary, adrenal or ectopic.

Dexamethasone suppression
- Inhibits ACTH production
- Should see a drop in cortisol if normal.

Imaging after biochemical tests
- CXR
- MRI pituitary
- CT adrenal

4

Diagnostic tests for too little cortisol

Hypoglycaemic stress
- Insulin resistance test
- Injection of insulin will not see rise in cortisol levels.

SynACTHen test
- ACTH injection will not see rise in cortisol

9.am cortisol
- Lower than normal

Urine and electrolytes
- Hyponatraemia
- Hypokalcaemia
- Low aldosterone and renin

Blood glucose test
- Hypoglycaemic

5

Cushing's syndrome
- Treatment

Surgical
- Transphenoidal adenectomy
- Adrenalectomy

Pituitary radiotherapy

If iatrogenic: slowly ween off steroid therapy then stop.

6

Addison's disease
- Treatment

Steroid replacement therapy
- Hydrocortisone
- Dose increased to cover stress [esp after surgeries or if nil my mouth]

If primary adrenal insufficiency
- Mineralocorticoid and glucocorticoid replacement therapy

Secondary adrenal insufficiency
- Mineralocorticoid not needed
- Other hormone replacement therapy

7

11-beta-hydroxysteroid dehydrogenase enzyme

Enzyme that is able to convert cortisol into the inactive cortisone
- and vice versa

Does so in specific tissue, depending on the demand for mineralocorticoid or glucocorticoid.
- If in kidneys, colon, salivary glands--> Mineralocorticoid is preferred= inactivation of cortisol into cortisone.
- In the liver---> Cortisol is activated.

Enzyme allows tissue specificity
- Gating of GC access to receptors via pre-receptors

8

Circulating adrenal androgens

Androstenedione

DHEAS [dehydroepiandrosterone]

9

Addison's disease
- Presentation

Malaise
Weakness

Anorexia
Weight loss

Increased skin pigmentation

Hypotension

Hypoglycaemia

10

Primary adrenal insufficiency
- Causes/ pathogenesis

Includes Addison's disease
- Mainly caused by autoimmune destruction of adrenal glands.

Other causes:
- Metastasis
- TB

11

Secondary adrenal insufficiency
- Causes

Hypoadrenalism caused by impairment of the hypothalamus or pituitary gland.

Causes:
- Pituitary adenoma
- Iatrogenic [ sudden halt of long term steroidal treatment ]

12

Autoimmune polyendocrine syndromes Type 1

Genetic condition that gives rise to many endocrine and autoimmune conditions.

Caused by mutation in AIRE gene
- Autosomal recessive inheritance

Presentation
- Rare
- Onset infancy

Common phenotype
- Addison's disease
- Hypoparathyroidism
- Candidiasis

13

Autoimmune polyendocrine syndromes Type 2

Condition caused by polygenetic factors
- Can give rise to many autoimmune/ endocrine diseases
- More common than Type 1

Onset occurs between infancy and adulthood.

Phenotypes
- Addison's disease
- T1 diabetes
- Autoimmune thyroid disease

14

Examples of autoimmune polyendocrine syndromes that can occur together.

T1 DM [endocrine]

Autoimmune thyroid disease [endocrine]

Coeliac disease [gut]

Addison's disease [endocrine]

Pernicious anaemia [blood]

Alopecia [skin]

Vitiligo [skin]

Hepatitis [liver]

Premature ovarian failure [GYN]

Myasthenia gravis [Neuro]

15

Patient with:
- T1 DM
- Fatigue
- Weight loss
- Hypoendocrine disorders
Is highly suspicious of having...

Addison's disease

16

Patient with:
- T1 DM
- Non-specific GI symptoms
- Diarrhoea

Is highly suspicious of having...

Coeliac disease

17

Screenings to consider in patients with T1 DM and/or Addison's disease

Coeliac disease

Thyroid function test
- Esp in pregnancy/ post-partum

18

Method of overcoming sudden stop of long term steroid treatment

Steroid treatment card
- Reminds doctors and patient