Adrenal - Cushings, Addisons, Addisonian crisis, Congenital adrenal hyperplasia, Phaeochromocytoma Flashcards

1
Q

Cushing’s syndrome
-ACTH dependent causes
-Adrenal or exogenous causes

A

Cushing’s disease - pituitary tumour secreting ACTH
Ectopic ACTH production - SCLC

CS use
Adrenal adenoma/carcinoma

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2
Q

Cushing’s syndrome
-presentation
-investigations
-diagnosis and localisation
-management

A

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

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3
Q

CS
-relative GC and MC activity
-SE
-long term use guidance and tapering

A

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

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4
Q

Addison’s disease
-presentation
-investigations
-management

A

AI destruction of adrenals => reduced CS and ALD

Lethargy, weak, anorexia, N/V, weight loss
Low BP, hypoglycemia
Low Na, high K
AI - hyperpigmentation of creases, 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

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5
Q

Hypoadrenalism
-causes

A

Addisons
TB
Adrenal mets
Waterhouse-Friderichsen - meningococcal septicemia => bleed into adrenals
HIV
APS

Pituitary disorders

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6
Q

Addisonian crisis
-what is it
-causes
-management

A

Sepsis
Surgery
Adrenal hemorrhage
Steroid withdrawal

100mg IM/IV hydro - 6hrs until stable
1L 0.9% saline over 30-60mins
Dextrose if hypoglycemic

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7
Q

Congenital adrenal hyperplasia
-what is it
-presentation
-diagnosis

A

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

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8
Q

Primary hyperaldosteronism
-causes

A

AI - Conn’s syndrome
MOST COMMON - Bilateral adrenal hyperplasia
Adrenal adenoma/carcinoma

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9
Q

Primary hyperaldosteronism
-presentation
-investigations
-management

A

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

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10
Q

Phaeochromocytoma
-what is it
-associations
-presentation
-investigations
-management

A

Rare adrenaline secreting tumour

MEN2, neurofibromastosis

HTN
Headaches
Palpitations
Sweating
Anxiety

24hr urinary metanephrines

Stabilisation - phenoxybenzamine => propanolol
Definitive - surgery

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11
Q

MEN 2a and 2b
-how to differentiate between the 2

A

2a
Medullary thyroid cancer
Phaeochromocytoma
Parathyroid

2b
Medullary thyroid cancer
Phaeochromocytoma
Marfanoid + Neuroma

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12
Q

Structure and physiology of the adrenals

A

CRH => ACTH (derived from POMC) => cholesterol conversion to steroids

Zona glomerulosa, aldosterone
Zona fasciculata, cortisol
Zona reticulata, androgens
Medulla => chromaffin cells, NA/A

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