Adrenal Pathology + Fluid Balance Flashcards
What is the pathology behind Congenital adrenal hyperplasia ?
Autosomal recessive disorder
Reduced corticoids due to deficiency in 21-hydroxylase. Causes increase in ACTH to increase in androgens. Salt losing crisis causes hyperkalaemia + hyponatraemia
Why do you get hypovolaemic hyponatraemia?
mild dehydration
S+S Addisons
Fatigue Weakness N+V Weight loss Abdo pain Diarrhoea/ constipation Muscle cramps Hyperpigmentation Cravings for salt Hypotension Crisis brought on by infection of stress
Primary + secondary Addisons
Primary = inability to produce enough steroids in adrenal glands Secondary = inadequate stimulation by pituitary glands
Blood results for Addisons
Hyponatraemia Hyperkalaemia Hypercalcaemia Raised LFTs Reduced cortisol ACTH = high in primary, low in secondary
Management of addisons
Glucocorticoids (hydrocortisone) + mineralcorticoids (fludracortisone)
Steroids + chickenpox
At risk of severe chicken pox when on steroids
Cause pneumonia, hepatitis + shingles
When should addisons be suspected in hypothyroidism + diabetes?
Hypothyroidism = when symptoms worsen with thyroxine T1DM = unexplained hypos
How is Addisons diagnosed?
Adrenocorticotrophic hormone stimulation test
Management of adrenal crisis
IM or IV hydrocortisone
Mechanism of familial hypercholesterolaemia
Inherited defected gene
What blood results indicate FH may be present?
<30 y/o with total cholesterol >7.5
>30 y/o with total cholesterol >9
What is the primary care management of FH?
Lifestyle advice
High intensity statin or ezetimibe
Anti HTN drugs
Aspirin as primary prevention of CVD
What is the pathology of Cushings?
Prolonged exposure to high levels of steroids
What are the causes of Cushings?
ACTH dependant:
Cushings disease, ACTH producing tumours or excess ACTH meds
Non-ACTH dependant:
excess steroids, adrenal adenoma or carcinoma
What is Cushings disease?
Excessive ACTH from pituitary (pituitary adenoma)
RF for Cushings
Females
25-40
S+S of Cushings
Truncal obesity, buffalo hump, supraclavicular fat pads Facial fullness/ moon face Proximal muscle wasting + weakness Diabetes Gonadal dysfunction HTN Skin atrophy, easy bruising, hirsuitism, acne Depression Osteoporosis Thirst, polyuria/dipsia
Investigations for ?Cushings
24hr urine cortisol Overnight dexamethasone suppression test Late night salivary cortisol RBC - raised WCC Hypokalaemia, metabolic alkalosis Plasma ACTH (high + elevated cortisol = ACTH dependant Cushings)
What is inferior petrosal sinus sampling?
Performed with CRH stimulation to aid in diagnosing source of excess ACTH
Management of Cushings
Surgery or RT
Metyrapone, ketoconazole, and mitotane can all be used to lower cortisol
Complications of Cushings
Metabolic syndrome HTN Diabetes Obesity Hyperlipidemia Osteoporosis Thrombophilia Nelson's syndrome Primary pituitary tumour
Presentation of congenital adrenal hyperplasia
Females = ambiguous genitalia
Boys with classic form = no signs at birth, possible penis enlargement. Early virilisation at 2-4 y/o
Boys with salt-losing form = present at 7-14 days with vomiting, weight loss, lethargy, dehydration
Non classic = hyperandrogenism in later childhood (early puberty, infertility, hirsutism, acne, PCOS)
Investigations for ?congenital adrenal hyperplasia
Hyponatraemia Hyperkalaemia Hypoglycaemia High serum 17-hydroxyprogesterone Corticotropin stimulation test Pelvic US for ambiguous genitalia
