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Endocrine System IV

Endocrine System IV

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Published by api-26938624

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Published by: api-26938624 on Oct 19, 2008
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Multiple Endocrine Neoplasia (MEN)
- Usually
family history of multiple endocrine tumours
- Patients are younger than those with single sporadic tumours
Type 1 = (Wermer Syndrome)
\ue000Combination of adenoma of the pituitary, parathyroid hyperplasia or adenoma, & islet
cell tumor of the pancreas
Type 2A = (Sipple Syndrome)
\ue000Combo of pheochromocytoma, and medullary carcinoma of the thyroid & parathyroid
Type 2B
\ue000Combo of pheochromocytoma, and medullary carcinoma of the thyroid and mucosal
neruoma syndrome
Other Catacolamine Producing Tumors
chromaffin tumours that originate in extra-adrenal sites - more often malignant
- Rare tumor of the
chromaffin cells that secretes catecholamines (adrenaline & and noradrenaline/ EPI
and norEPI)\ue001 produces high levels of both hormones and their breakdown products (
VMA) which are

excreted in the urine in excess and used as diagnostic test.
- Majority are sporadic and cause is unknown
- In sporadic cases\ue001 80% tumors = unilateral, 10% bilateral and 10% extra-adrenal
- Metastases most common in regional lymph nodes. bone, LU, LV
- Occur at any age, high incidence in infancy
- In MEN\ue001 2/3rds are bilateral, in 5-10% of cases\ue002 tumours = malignant
- Sustained(2/3) or episodic(1/3) hypertension, & it\u2019s complications
- May present with MI, aortic dissection, convulsions, anxiety & hyperventilation
- Crisis often precipitated by pressure on abdominal contents (lifting, bending etc\u2026)
- \u2191 BMR, sweating, heat intolerance & weight loss may mimic hyperthyroidism (send for thyroid panel)

Cathecholamine Cardiomyopathy.
\ue000Myocardial necrosis causing angina & MI in the absence of coronary artery disease
- Untreated\ue001 patients die of hypertensive complications

- Malignant tumour of the adrenal medulla or sympathetic ganglia
- Readily infiltrate surrounding tissue, and metastasize to regional lymph nodes, LV, LU & bones
- Peak incidence in the first 3 years of life
- One of the most important malignant tumours of childhood
- Often = enlarging abdomen in a young child
- Firm, irregular, non-tender mass on abdominal palpation
- Highly variable depending on where it is, and where it metastasizes too
- Increased urinary levels of catecholamine metabolites (VMA)

Only moderate catecholamine levels, vs. high in pheochromocytoma
- May differentiate into a more mature benign tumour = ganglioneuroma
\ue000Generally found in older children & young adults
Parathyroid Glands and Parathyroid Hormone

90% of parathyroid masses in lower poles &/or posterior surface of thyroid = impalpable
- Parathyroid hormone breaks down bone, secreted by chief cells
- Regulates calcium concentration in extracellular fluid

- Magnesium inhibits PTH secretion (b/c when you breakdown bone, you get Ca+ and Mg+)\ue001 so be
careful with long term Mg+ intake (
Increase in Calcium = decrease in PTH, Decrease in Calcium =
Increase in PTH)
- Normal relationship lost in hyperparathyroidsm\ue001 PTH will be inappropriately high in relation to Ca+
(so, Ca+ high, but PTH still pumped out)
- Test Creatinine with all PTH assays for KI function (KI = target organ, so is it a problem with PTH or
with KI)
Different Parathyroid Tests

\ue000Increased PTH = Primary and pseudo hyperparathyroidism (renal insensitivity)
\ue000Increased PTH-COOH = neoplasms, get renal failure
\ue000Decreased PTH = most likely hypoparathyroidism
\ue000Decreased PTH-NH2 = acute secretory changes best reflected by this variant

Increased levels of PTH seen with milk ingestion, Vit A and D overdoses
Decreased levels of PTH seen with Phosphate intake
Decreased secretion of PTH, or end organ insensitivity to the hormone (

- Most common cause is surgical resection of the parathyroids as a complication of thyroidectomy
- Symptoms are related to hypocalcemia (increased neuromuscular excitability and get mild hand/feet

tingling\ue001 severe muscle cramps, laryngeal stridor, & convulsions)
- Depression, paranoia, psychosis
- Elevated cerebrospinal fluid pressure & papilledema may mimic brain tumour
- PTH antibodies reported in idiopathic hypoPTH (IP)\ue001 suggests auto-immune basis
- All forms are successfully treated with Vitamin D & calcium supplementation

Pseudohypoparathyroidism (Secondary)
- Group of hereditary conditions in which

hypocalcemia is caused by target organ insensitivity to PTH
- Inadequate resporption of Ca+ from the glomerular filtrate
- Resistant to other hormones : TSH, glucagons, FHS & LH

-Albright- hereditary osteodystrophy
Short stature, abnormally short metacarpals/tarsals
Obesity, mental retardation
Resistance to PTH
Primary Hyperparathyroidism
Excessive secretion of PTH by paraTh adenoma (80%), Primary hyperplasia of all the paraTh(15%) and

carcinoma (rare) (1%)
- With a functioning adenoma, the other paraTh glands tend to be atrophic
- See Hypercalcemia and hypophosphatemia
- Nervous system: see depression, Kidney: 10% renal colic and kidney stones

- Skeletal:
Osteitis, fibrosa cystica:
\ue000Minority of patients with a serious form of the disease, get bone pain, fractures, cysts,
bone swellings, chondrocalcinosis
Secondary Hyperparathyroidism
- Chronic
hypocalcemia due to renal retention of phosphate, inadequate production of activated vitamin
D, & some degree of skeletal resistance to PTH\ue001 all lead to compensatory hyper secretion of PTH
- Chronic renal insufficiency associated with decreased phosphate excretion\ue002 hyperphosphatemia. High
serum phosphate directly depresses serum calcium\ue002 stimulates parathyroid gland activity
- Can get Renal osteodystrophy:
Osseous manifestations of hyperparathyroidism (primary symptoms are those related to chronic
renal failure), Cause: chronic renal failure = m/c, vitamin D deficiency

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