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Pathology final #2 misc.
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Terms in this set (35)
Most common cause of Cushing's syndrome
Iatrogenic steroid administration. The second most common cause is Cushing's disease
Adrenal cortex hyperplasia can result in a syndrome called?
McCune Albright Syndrome
Mutation in McCune Albright syndrome
GNAS
ACTH is High, suppressed by high dose of dexamethasone, urinary steroid secretion decreased
Cushing Dz
ACTH is High, not suppressed by high dose of dexamethasone, urinary steroid secretion not suppressed
Ectopic ACTH
ACTH is low, urinary steroid secretion not affected by dexamethasone
Exogenous cs tx
Or
Adrenal tumour
Secondary hyperaldosteronism
kidney perception of low intravascular volume results in an overactive renin angiotensin system, will have HIGH plasma renin
Most common cause of primary hyperaldosteronism
Bilateral idiopathic adrenal nodular hyperplasia
HTN in bilateral idiopathic hyperplasia is less/more severe than in adrenal tumour
Less
Syndromeof Unilateral adrenal adenoma causing primary hyperaldosteronism
Conn syndrome
The most common unilateral cause of primary hyperaldosteronism
Adenoma
Age group: SCLC
Middle aged men more
Age group: cushing disease
Young adult females more
Age group: bilateral idiopathic hyperaldosteronism
Older age
Age group: conn syndrome
Middle-aged woman more
Elevated plasma aldosterone to renin ratio is seen in
Primary hyperaldosteronism
Confirmatory test for primary hyperaldosteronism
Aldosterone suppression test by salt or captopril
The most common cause of secondary hypertension
Primary hyperaldosteronism
Congenital adrenal hyperplasia is inherited as
Autosomal recessive
The most common enzymatic defect in congenital adrenal hyperplasia
21 alpha hydroxylase deficiency
In a neonate with ambiguous genitalia always rule out________
CAH
Acute adrenocortical insufficiency is termed
Adrenal crisis
Chronic adrenocortical insufficiency is termed
Addison disease
Hyperpigmentation can be seen in which type of adrenocortical insufficiency
Primary
Normal aldosterone is seen in which type of adrenocortical insufficiency
Secondary
Abnormalities in 1° adrenocortical insufficiency: blood glc, K, Na, BP
Hypoglycaemia
Hyperkalaemia
Hyponatraemia
HYpotension
Meningococcaemia & resulting haemorrhage can lead to?
Water-friderichsen syndrome & acute 1° adrenal insufficiency/adrenal crisis
Most common cause of Addison disease in developed countries
Autoimmune adrenalitis
Infections that can lead to addison
HIV
TB
fungal
The most common adrenal malignancy is
Metastases esp from lung or breast
Serious complications of pheochromocytoma
Catecholamine cardiomyopathy
Myocardial instability
Ventricular arrhythmia
CVA
rule of 10's for pheochromocytoma
10% malignant
10% bilateral
10% extra-adrenal = paraganglioma
10% kids
10% not associated with HTN
25% familial
Familial genes associated with pheochromocytoma
RET
NF1
VHL
SDHB C D
Pheochromocytoma features
Nests ->
zellballen pattern
Capillary network
Sustentacular sells
Most common extracranial solid tumour of childhood
Neuroblastoma
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