Adrenal - Cushings, Addisons, Addisonian crisis, Congenital adrenal hyperplasia, Phaeochromocytoma Flashcards
Cushing’s syndrome
-ACTH dependent causes
-Adrenal or exogenous causes
Cushing’s disease - pituitary tumour secreting ACTH
Ectopic ACTH production - SCLC
CS use
Adrenal adenoma/carcinoma
Cushing’s syndrome
-presentation
-investigations
-diagnosis and localisation
-management
Moon face
Buffalo hump
Proximal wasting
Abdominal striae, central obesity
Low mood
Oligo/amenorrhea
Impaired immune system
Easy bruising
HTN, impaired glucose tolerance
Hypokalemic metabolic alkalosis
1st line - Overnight low dose dexmeth suppression test - LOOK AT RESPONSE OF CORTISOL
-high cortisol - cushing syndrome/disease
2x urinary free cortisol
2x bedtime salivary cortisol
Localise with high dose dexmeth
-low cortisol => cushing disease
-high cortisol + low ACTH => cushing syndrome
-high cortisol + high ACTH => ectopic ACTH
Treatment depends on source
CS - reduce dose
Tumour - surgery, RT, ketoconazole/metyrapone
CS
-relative GC and MC activity
-SE
-long term use guidance and tapering
MC - fludro
MC + GC - hydro
GC - pred
V strong GC - dex
Cushings
-Impaired glucose regulation
-Increased appetite, weight
-Osteoporosis, AVN
-Proximal myopathy
-IC, neutrophilia
-Insomnia, mania, psychosis
-Growth suppression in children
Reducing risks of Addisonian crisis
-double dose during illness
DO NOT WITHDRAW ABRUPTLY - taper if
-40mg+ for 1wk+
-3wks+
-recent repeated courses
Addison’s disease
-presentation
-investigations
-management
AI destruction of adrenals => reduced CS and ALD
Lethargy, weak, anorexia, N/V, weight loss
Low BP, hypoglycemia
Low Na, high K
AI - hyperpigmentation, vitiligo but not AI causes are not
Definitive - Synacthen
Hydro - majority given in AM
Fludro -
Patient education on
-not missing GC doses
-emergency hydro IM doses for crisis
-if unwell, 2x GC dose
Hypoadrenalism
-causes
Addisons
TB
Adrenal mets
Waterhouse-Friderichsen - meningococcal septicemia => bleed into adrenals
HIV
APS
Pituitary disorders
Addisonian crisis
-what is it
-causes
-management
Sepsis
Surgery
Adrenal hemorrhage
Steroid withdrawal
100mg IM/IV hydro - 6hrs until stable
1L 0.9% saline over 30-60mins
Dextrose if hypoglycemic
Congenital adrenal hyperplasia
-what is it
-presentation
-diagnosis
AR - impaired steroid synthesis (most often 21hydroxylase)
-overcompensated ACTH => too many androgens and aldosterone
Virilization in females at birth
Precocious puberty, height and growth abnormalities
Infertility
Salt wasting
-lowBP, dehydrated, electrolyte imbalances
ACTH testing => abnormally high 17hydroxyprosterone
GC replacement
Primary hyperaldosteronism
-causes
AI - Conn’s syndrome
MOST COMMON - Bilateral adrenal hyperplasia
Adrenal adenoma/carcinoma
Primary hyperaldosteronism
-presentation
-investigations
-management
HTN
LowK => muscle weakness
Metabolic alkalosis
1st line - Plasma aldosterone:renin ratio
Differentiate between unilateral/bilateral - high contrast CT
-2nd line => adrenal venous sampling
Unilateral - laparoscopic adrenalectomy
Bilateral - aldosterone antagonist
Phaeochromocytoma
-what is it
-associations
-presentation
-investigations
-management
Rare adrenaline secreting tumour
MEN2, neurofibromastosis
HTN
Headaches
Palpitations
Sweating
Anxiety
24hr urinary metanephrines
Stabilisation - phenoxybenzamine => propanolol
Definitive - surgery
MEN 2a and 2b
-how to differentiate between the 2
2a
Medullary thyroid cancer
Phaeochromocytoma
Parathyroid
2b
Medullary thyroid cancer
Phaeochromocytoma
Marfanoid + Neuroma
Structure and physiology of the adrenals
CRH => ACTH (derived from POMC) => cholesterol conversion to steroids
Zona glomerulosa, aldosterone
Zona fasciculata, cortisol
Zona reticulata, androgens
Medulla => chromaffin cells, NA/A
