Endocrinology Flashcards
(227 cards)
What are the classifications of Acromegaly?
Growth hormone releasing hormone (GHRH) independent:
- Pituitary adenoma (most common)
- Primary pituitary hyperplasia
GHRH dependent (rarer):
- Hypothalamic source = excess GHRH from he hypothalamus causes secondary pituitary hyperplasia
- Ectopic release = excess GHRH from ectopic tissue
Define Acromegaly
A disorder caused by an excessive amount of growth hormone w/ characteristic clinical features
What’s the difference between acromegaly and gigantism?
Age of onset - gigantism occurs before the epiphyseal plates close leading to excess linear growth, whilst acromegaly occurs after the plates close leading to enlargement of bones and soft tissue
Acromegaly pathophysiology
- Excess GH results in the excess production of insulin-like growth factor 1 (IGF-1)
- IGF-1 receptor is widely distributed across a wide range of tissues, and excess stimulation results in abnormal growth of these tissues
- Excess GH also results in increased gluconeogenesis, lipolysis and insulin resistance
Acromegaly features
Hands:
- spade-like, large, doughy hands
- Sweaty
- Thick skin w/ large pores
- Carpal tunnel syndrome
Face:
- Interdental separation
- Prognathism
- Large ears, nose, lips
- Prominent supraorbital ride and facial creases
- Macroglossia
- Acne
Visual field defects = bitemporal hemianopia/upper quadrantanopia
Axilla:
- Acanthosis nigricans
- Skin tags
Neck:
- Acanthosis nigricans
- Goitre
Chest:
- Cardiomegaly
- Pulmonary oedema
- Gynaecomastia
Legs:
- Peripheral pitting oedema
- Proximal muscle weakness
- OA
- Large feat
Back:
- Kyphoscoliosis
Systemic:
- Secondary HTN
- Organomegaly
- Increased risk of colonic polyps and thus colorectal cancer
Acromegaly investigations
Bedside:
- Fundoscopy -=optic atrophy
- Visual fields testing
- Urine dip = glycosuria
- ECG = LVH due to HTN
Bloods:
- IGF-1 levels
- If raised, do confirmatory test = oral glucose tolerance test - fails to supress GH
Imaging:
- MRI pituitary
Acromegaly management
Medical = if surgery is contraindicated, mechanism not due to pituitary adenoma, or is refractory to surgery:
- Somatostatin analogues (1st line) = octreotide (supress GH release)
- GH antagonists = pegvisomant
- Dopamine agonist = cabergoline
Surgery (1st line):
- Transphenoidal surgery to remove pituitary adenoma (1st line)
- Transfrontalr resection of the pituitary
- +/- radiotherapy
Define Adrenal insufficiency
A clinical syndrome that arises due to the insufficient production of glucocorticoids and mineralocorticoids from the adrenal cortex
What are the classifications of Adrenal insufficiency?
Primary = Addison’s disease:
- AI destruction
- Surgical removal
- Trauma
- TB
- Haemorrhage
- Infarction
- Neoplasm
- Steroid overuse
Secondary:
- Congenital disorders
- Basal skull fractures
- Pituitary surgery
- Pituitary neoplasm
- Infiltration or infection of the brain
- Corticotrophin-releasing hormone deficiency
Adrenal insufficiency features
- Hypotension
- Fatigue and weakness
- GI symptoms
- Syncope
- Skin pigmentation due to increased ACTH production and thus increased melanocyte stimulating hormone
Adrenal insufficiency investigations
- U&Es and serum cortisol = hyponatraemia, hyperkalaemia and low serum cortisol
- Hypoglycaemia
- ABG = hyperkalaemia, hyponatraemic metabolic acidosis
- Ab testing
- CT adrenals
- MRI pituitary
Short synacthen test is confirmatory:
- Measure cortisol before
- Give syacthen (syhnethtic ACTH)
- Measure cortisol 30 min later
- Cortisol should be >420, else probably Addison’s
Adrenal insufficiency management
Chronic:
- Replace glucocorticoids (w/ hydrocortisone - 10mg in the morning, 5mg at noon, 5mg at night- or prednisolone)
- Replace mineralocorticoids (w/ fludrocortisone)
- Education on sick day riles (doubling steroid dose when ill)
Addisonian crisis:
- Aggressive fluid resuscitation
- IV/IM hydrocortisone STAT
- Glucose if hypoglycaemia
Define Addisonian crisis
A life-threatening condition due to an acute deficiency in cortisol and aldosterone
Presents w/:
- Hypotension
- Hyperkalaemia
- Hyponatraemia
- Metabolic acidosis
What are the side effects of amiodarone?
- Hypothyroidism
- Hyperthyroidism (rarer - amiodarone thyrotoxicosis)
- Corneal deposits
- SJS
- Grey discoloration of the skin
- Liver failure
- Pneumonitis and pulmonary fibrosis
Define Amiodarone induced thyrotoxicosis
A side effect of amiodarone which is rich in iodine, presents w/ symptoms of thyrotoxicosis
Manifests in 2 types:
- AIT 1 = pt has underlying thyroid nodules, amiodarone increases thyroid hormone production due to high iodine content
- AIT 2 = pt has normal function gland, amiodarone triggers destructive thyroiditis
Amiodarone induced thyrotoxicosis investigations
- TFTs
- Thyroid uptake scan - AIT 1 = normal uptake, AIT 2 = decreased uptake
- Doppler US - AIT 1 = increased vascularity, AIT 2 = reduced vascularity
Amiodarone induced thyrotoxicosis management
- AIT 1 = carbimazole
- AIT 2 = corticosteroids
- Consult w/ cardio about stopping amiodarone
What are the causes of vasopressin deficiency?
- Head trauma
- Inflammatory conditions e.g. sarcoidosis
- Cranial infections e.g. meningitis
- Vascular conditions e.g. sickle cell
- Rare genetic causes
What are the causes of vasopressin resistance?
- Drugs e.g. lithium
- Metabolic disturbances e.g. hypercalcaemia, hypokalaemia and hyperglycaemia
- Chronic renal disease
- Rare genetic causes
Vasopressin disorders (diabetes insipidus) features
- Large volumes of dilute urine (> 3L in 24 hrs and urine osmolality of <300 mOsm/kg)
- Nocturia
- Excessive thirst
Vasopressin disorders (diabetes insipidus) investigations
- U&Es = sodium normal/raised
- Urine dip
- Paired serum and urine osmolality (raised serum osmolality w/ inappropriately dilute urine w/ a low osmolality)
Water deprivation test is diagnostic and can distinguish from primary polydipsia:
- Deprive pt of fluids and monitor urine osmolality and weight changes (stop if pt loses >5% body weight)
- In vasopressin disorders, urine osmolality wont decrease
- In vasopressin deficiency, urine osmolality will decrease on administration of ADH
- In vasopressin resistance, urine osmolality wont change w/ administration of ADH
Vasopressin deficiency management
- Desmopressin
- Monitor sodium levels
Vasopressin resistance management
- Correct underlying metabolic abnormalities
- Stop any offending drugs
- High dose desmopressin has varying results
- Thiazide diuretics and NSAIDs can reduce urine volume
Define Carcinoid tumour
A slow-growing, neuroendocrine tumour which secretes hormones (usually serotonin) - most often occur in the appendix, lungs and small intestine
Can become malignant