Keywords: 40 y.o woman Has diabetes & hypertension for 6 months On oral hypoglycemia & antihypertensive medication Height: 165cm Weight: 85kg BMI: overweight/obese Moon shaped facies Truncal obesity Abdominal striae Diminished motor strength of proximal limb muscles Also on prolonged oral corticosteroid treatment for rheumatoid arthritis for 5 years Wanted to stop the cortocosteroid immediately,…
Recap: Cushings excess cortisol Congenital Adrenal Hyperplasia decrease aldosterone & cortisol excess testosterone & DHEA-S Addison’s Disease failure to produce all adrenal cortex hormone Conn’s syndrome/ Aldosteronism Excess aldosterone Pheochromocytoma Excess epinephrine & norepinephrine. _____________________________________________________________________ General concept for investigation: 1) Establish whether it’s hyper/hyposecretion Use screening tests : Short synacthen (synthetic analogue of ACTH) dexamethasone suppression…
Pheochromocytoma Also known as: paraganglioma, chromaffinoma Intra-adrenal sympathetic paraganglioma Sporadic lesions: tend to be unilateral Familial lesions: tend to be bilateral 1.4-18% are incidentalomas Co-exist with extra-adrenal paragangliomas (can also occur outside adrenal medulla) Rule of 10 Pheochromocytoma associated genetic syndromes: Multiple Endocrine Neoplasia (MEN) type 2 Von-Hippel-Lindau (VHL) syndrome Neurofibromatosis (NF) type 1 Hereditary…
OSPE: Refer histoloy of adrenal gland to identify the zones. TUMOURS OF THE ADRENAL CORTEX Adenoma Adenocarcinoma Adenoma Adenocarcinoma (malignant) Rare Hormonal symptoms (depending on the hormone hypersecretion) Virilization Hyperaldosteronism, Cushing’s Association with Li-Fraumeni syndrome (rare autosomal dominant hereditary disorder) Carney complex (autosomal dominant) CT scan. White: bone MRI. Bone is not white. _____________________________________________________________ Adrenal…
It is an autosomal recessive syndrome: Insufficient cortisol syntesis Increased ACTH production –> increases cortisol to maintain circulating levels Stress cortisol – inadequate Mineralocorticoid synthesis affected 21 beta-hydroxylase deficiency & 17 alpha-hydroxylase deficiency for steroid hormone production 21 beta-hydroxylase deficiency: (refer diagram) Coding gene at chromosome 8 Mineralocorticoid defect results in hyperkalemia, hyponatremia & low…
Pheochromocytoma: A catecholamine-producing tumour derived from the chromaffin cells (of the adrenal medulla/ outside the adrenal medulla) Dusky coloured tumor 10% tumour bilateral disease (10%) malignant (10%) located in chromaffin tissue OUTSIDE the adrenal gland (10%) arise in childhood (10%) familial (10%) recur after being resected (10%) Can be inherited as an autosomal dominant trait…
Deficiency of any of the adrenocorticoid steroid hormones Hypoadrenalism Reduced adrenocortical function Deficient synthesis of steroid hormones ACTH deficiency – Can’t stimulate the adrenal cortex Primary Adrenocortical insufficiency Disease of the adrenal gland Acute: Adrenal crisis Sudden loss of adrenocortical function caused by: Bilateral adrenalectomy (as a treatment of cortical hyperfunction & hypertension) Septicaemia (from…
Adrenal virilism: The development or premature development of male secondary sexual characteristics caused by male sex hormones (androgens) excessively produced by the adrenal gland. This disorder can occur before birth and can lead to sexual abnormalities in newborns. It can also occur in girls and women later in life. Boys will gain sexual maturation faster…
Frequent in adult females Primary Hyperaldosteronism Also known as Conn’s Syndrome Overproduction of aldosterone Adrenal cortex lesion: -Adrenocortical adenoma -Adrenal carcinoma -Bilateral adrenal hyperplasia (in children) Associated with low plasma renin levels (positive feedback? due to increased blood volume from the action of aldosterone, initiating the renin-angiotensin-aldosterone system) Low K+ level (in exchange of Na+…
Cushing’s Syndrome Chronic hypercortisolism due to excessive production of cortisol 20-40 years of age 3 times higher frequency in women Pituitary Cushing’s syndrome Adrenal Cushing’s syndrome Ectopic Cushing’s syndrome Iatrogenic Cushing’s syndrome Pituitary Cushing’s syndrome (60-70%) Cushing’s disease Excessive ACTH, due to Pituitary lesion: corticotroph adenoma, multiple corticotroph microadenoma hypothalamic origin: excessive CRH secretion –>…