L12: 2ry HTN & MEN Flashcards
Causes of 2ry HTN
What control Mineralocorticoids synthesis?
- Mineralocorticoids (aldosterone 100–150 micrograms/day) synthesis occurs in the zona glomerulosa, predominantly under the control of the renin– angiotensin system (outer layer of the cortex) although ACTH also contributes to its regulation.
Causes of Hyperaldosteronism
Causes of 1ry Hyperaldosteronism
Conn’s syndrome (aldosterone-producing adrenal adenoma) 35%.
Bilateral adrenal hyperplasia 60%
Causes of 2ry Hyperaldosteronism
Primary Hyperaldosteronism
The condition of excess aldosterone secretion which may result in Hypertension with Hypokalemia and Alkalosis
Causes of Primary Hyperaldosteronism
- Conn’s syndrome (aldosterone-producing adrenal adenoma) 35%.
- Bilateral adrenal hyperplasia 60%.
CP of Primary Hyperaldosteronism
INVx for Primary Hyperaldosteronism
What is the screening test for Primary Hyperaldosteronism?
Plasma aldosterone : renin ratio (ARR)
Origin of Pheochromocytoma
- Adrenal Pheochromocytoma —> Over 90%
- Extra-adrenal Pheochromocytoma –> develop in the paraganglion chromaffin tissue of the sympathetic nervous system.
Def of Pheochromocytoma
rare, catecholamine-secreting tumor derived from chromaffin cells
TTT of Primary Hyperaldosteronism
- Surgical for adenoma
- Medical for hyperplasia (Spironolactone and aldosterone receptor antagonist eplerenone)
Incidence of Pheochromocytoma
0.01 - 0.1% of hypertensive population
Rule of 10
- Pheochromocytoma
CP of Pheochromocytoma
Managment of Pheochromocytoma
Surgical if resectable:
- Medical Pre-operative treatment is mandatory with Combined α & β blockade
- Alpha blockade must precede beta blockade to avoid severe hypertension
If operation is not possible:
- long term Combined α and β blockade
Dx of Pheochromocytoma
what is tyh screening test for Pheochromocytoma?
24h urinary catecholamine and metabolites
Genetics in MEN1
MEN 1 is an AD disorder due to loss-of-function mutations of the MEN 1 gene.
Glands commonly affected in MEN1
Tumors in MEN1
Genetics in MEN2
AD condition due to activating mutations of the RET proto-oncogene
MEN2A
MTC (medullary thyroid cancer) in combination with PCC (Pheochromocytoma) and parathyroid tumors.
Glands commonly affected in MEN2
TUMORS in thyroid gland, parathyroid gland or adrenal gland
MEN2B
MTC in association with PCC and mucosal neuromas, marfanoid habitus, megacolon
Def of MEN
- Is a group of disorders that affect the endocrine system.
- There are tumors in multiple endocrine glands that may be malignant or benign and may cause the glands to become overactive and overproduce hormones.
Genettics in MEN
- Autosomal dominant inherited disorders
Types of MEN
what is another name for MEN1?
(Wermer syndrome)
what is the most affected gland in MEN1?
parathyroid gland tumors
What is th first affected gland in MEN1?
parathyroid gland tumors
what are manefestations of hypercalcemia?
- Neuropsychiatric
- Renal Dysfunction
- Musculoskeletal Symptoms
- Gastrointestinal Abnormalities
- Cardiovascular Disease
Neuropsychiatric symptoms
- Due to Hypercalcemia
Anxiety, depression, cognitive dysfunction
Lethargy, confusion → stupor, and Coma.
MSK Symptoms
- Due to Hypercalcemia
Profound muscle weakness
Bone pain due to reduction in cortical bone mass may occur in individuals with hyperparathyroidism.
Renal Dysfunction
- Due to Hypercalcemia
GI Symptoms
- Due to Hypercalcemia
- Constipation, anorexia, nausea and peptic ulcer (calcium-induces increase in gastrin secretion).
- In patients with MEN1 → Zollinger-Ellison syndrome may be present.
CVS CP of Hypercalcemia
Pituitary Gland tumors in MEN1
Pancreatic tumors in MEN1
- Site
- Nature & Incidence
- Types
What are the symptoms of zes?
(due to elevated levels of gastrin):
Peptic ulcers
Inflammation of the esophagus
Diarrhea and abdominal pain.
what is the most common functional endocrine pancreatic tumor in MEN1? and what is the second most common? and what is the TTT?
Adrenal Gland Tumors in MEN1
These tumors usually are benign.
Other Types of tumors in MEN1
what is another name of MEN2?
(Sipple syndrome)
Mutations in MEN2
MEN2A and MEN2B are caused by mutations in the RET gene.
what is the most commonly affected gland in MEN2?
People with multiple endocrine neoplasia type 2 (MEN2) have a 98 – 100 % chance of developing medullary thyroid cance
Types of MEN2
MEN2 is divided into three types:
Type 2A (MEN2A)
Type 2B (MEN2B)
Familial Medullary Thyroid Carcinoma (FMTC).
MEN2A
CP of MEN2B
Dx of MEN
TTT of MEN
Surgical TTT of MEN
Medical TTT of MEN
If surgery not possible and post-operative replacement
Recent drugs that target the mutant gen
Radiation TTT of MEN
radiation therapy for tumor ablation
