Endocrine/Puberty

Question 1

Familial hypocalciuric hypercalcemia

Answer 1

Defect Ca sensing receptor of PT cells

PTH not suppressed by high Ca levels

Question 2

Jod-Basedow phenomenon

Answer 2

Thyrotoxicosis if patient that was formerly iodine def is give some iodine

Question 3

Thyroid storm symptoms

Answer 3

Agitation, delirium, fever, diarrhea, coma, tachyarrthymia (cause of death)

Increased ALP due to increased bone turnover

Treat with 3 Ps: Propranolol, propothiouracil, prednisolone (prevents T4 to T3)
Give propranolol first

Question 4

What are the signs of cretininism (congenital hypothyroidism?

Answer 4

6 Ps

Pot-bellied, Pale, Puffy faced, Protruding umbilicus, Protuberant tongue, Poor brain development

Question 5

What is the Wolff-Chaikoff effect?

Answer 5

Thyroid gland downregulation due to excess iodine

Question 6

What is the inflammation of subacute thyroiditis (de Quervain)

Answer 6

Granulomatous inflammation

Increased ESR, jaw pain, VERY TENDER THYROID

Question 7

What is the difference in hashimotos thyroiditis and de Quervain?

Answer 7

Hashimoto there is not pain
De Quervain there IS PAIN

Hashimoto has lymphocyte infiltrate with Germinal Centers
Associated with HLA-DR5

Question 8

How do you diagnose cushing’s disease from syndrome?

Answer 8

Screen: Increase free cortisol on 24 hr urine

• Increase midnight salivary cortisol
• No suppression low-dose dexamethasone test

If ACTH low SUSPECT ADRENAL TUMOR

IF ACTH HIGH distinguish between adenoma and ectopic production

• Adenoma: Secretion suppressed by dexamethasone
• Ectopic: Secretion not suppressed by dexa

CRH will NOT increase ECTOPIC secretion

Question 9

How to diagnose adrenal insufficiency?

Answer 9

ACTH stimulation test (no increase is related to primary)

METYRAPONE TEST: Blocks 11-deoxycortisol-cortisol

• Should produce increase in ACTH
• If no increase in ACTH you know you have adrenal insufficiency (secondary)

Question 10

What is the most common cause of adrenal insufficiency in the modern world?

Answer 10

Autoimmune

Question 11

What diseases are associated with pheochromocytoma?

Answer 11

NF type 1, VHL disease, MEN2s
- Symptoms occur in spells (relapse remit

Treatment is alpha antagonism then B antagnoism

Derived from Chromaffin cells (neural crest)

Question 12

What are neural crest cell derivatives?

Answer 12

Melanocytes, chromaffin cells,

Question 13

What are the lab findings in hypothyroidism?

Answer 13

Incresae TSH
Decresae T3/T4
HYPERCHOLESTEROL (due to decrease in LDL receptor)

Question 14

What are the lab findings in hyperthryoidism?

Answer 14

Decreased TSH
Increased T3,T4
Hypocholesteroleia (due to upregulaiton of LDL receptors)

Question 15

What causes severe fetal hypothyroidism?

Answer 15

Maternal hypothyroidism
Thyroid agenesis (DiGeorge)
Thyroid Dysgenesis (most common in US)
Iodine def
Dyshormonogenetic goiter

Question 16

How do you diagnose Acromegaly?

Answer 16

Incresaed serium IGF-1

Cannot suppress GH after oral glucose test

Pituitary mass on MRI

Question 17

How to treat excess GH?

Answer 17

Resect adenoma

Treat with OCtreotide or PEGVISOMANT (GH receptor antagonist)

Question 18

How to distinguish Central DI vs. Nephrogenic DI?

Answer 18

Water deprivation test

- Give ADH: If increase >50% osmolality then have Central if no change then Nephrogenic

Question 19

What are some causes of neprhogenic DI?

Answer 19

Hereditary, 2 to hypercalcemia,
Lithium
Demeclocycline (ADH antagonist)

Treat: HCTZ, Indomethacin, Amiloride
Hydration

Question 20

What are some causes of central DI?

Answer 20

Pituitary tumor
Autoimmune
Trauma
Surgery
Ischemic
Idiopathic

Treatment: Desmopressin acetate
Hydration

Question 21

How does the body respond in SIADH?

Answer 21

Will decrease aldosterone (hyponatremia) to maintain euvolemia

Must correct slowly or get osmotic central pontine myelinolysis

Causes: Ectopic ADH (Small Cell Lung cancer)
CNS disorder
Pulmonary disease
Drugs (Cylcophosphamide)

Treat: Fluid restrict: IV hypertonic saline, conivaptan, tolvaptan, demeclocycline

Question 22

What are symptoms of glucagonoma?

Answer 22

Dermatitis (necrolytic with central induration)

Diabetes
DVT
Depression

Question 23

What is the most common cause of death in Diabetes?

Answer 23

MI is the most common

Question 24

What causes cataracts in diabetics?

Answer 24

Sorbitol accumulation in organs (osmotic damage)

- Due to aldose reductase and decrease in sorbitol dehydrogenase (eyes and neurons)

Question 25

What type of mutation is seen in MEN1?

Answer 25

MEN1 gene
menin, a tumor suppressor
3 Ps, parathyroid, pancreas, and Pituitary (prolactin/GH)
Pancreatic: ZES, insulinoma, glucagonoma, VIPomas

Question 26

What type of mutation is seen MEN2A?

Answer 26

Parathyroid
Pheochromocytoma
RET gene (tyrosine kinase)

Medullary thyroid carcinoma

Question 27

What type of mutation is seen in MEN2B?

Answer 27

Pheochromocytoma

Medullary thyroid carcinoma

ORAL/INTESTINAL ganglioneuromatosis

Question 28

What are the presentations of ZES?

Answer 28

Abd pain
Diarrhea (malabsorption)
Positive secretin stim test: gastrin remains the same after secretin is administered (secretin blocks gastrin usually)

Question 29

What is the whipple triad?

Answer 29

Tumor of B cells

Low blood glucose
symptoms of hypoglycemia (lethary, syncope, diplopia)
Resolution after glucose

Patients with insulinomas have this
Insulinomas: Increase insulin with C peptide

Treatment of Insulinoma: Surgical resection

Question 30

Which hormones use cAMP (Gs)?

Answer 30

FLAT ChAMP
FSH
LH
ACTH
TSH
CRH
hCG
ADH
MSH
PTH

calcitonin
Glucagon
GHRH

Question 31

Which hormones use IP3 (Gq)?

Answer 31

GOAT HAG

GnRH
Oxytocin
ADH
TRH
Histamine
Angiotensin II
Gastrin

Question 32

What hormones are intracellular?

Answer 32

Steroids

Vit A
Estrogen
Testosterone
T3, T4
Cortisol
Aldosterone
Progesterone

Question 33

What hormones use Intrinsic Tyrosine Kinase?

Answer 33

MAP Kinase pathway (inhibit by serine phos)
Think GFs

Insulin
IGF-1
FGF
PDGF
EGF

Question 34

What hormones use Receptor associated Tyrosine kinase?

Answer 34

PIGGlET

Prolactin
Immunomodulators (cytokines)
GH
G-CSF
Erythropoietin
Thrombopoietin

JAK/STAT pathway