Endocrine Board Review 3rd Edition Flashcards
What can you see if you image the pituitary in a patient with primary hypothyroidism?
Pituitary enlargement
What happens to the alpha subunit during pregnancy?
It is increased
Do patients with non-classical CAH typically achieve mid-parental height?
No
Which type of progestin has been shown to improve PMS symptoms when in oral contraceptive pills?
Drospirenone (anti-mineralocorticoid activity)
Which is the superior fertility treatment for women with PCOS…
Metformin or clomiphene?
Clomiphene
What is the difference between Kallman syndrome due to KAL1 gene mutations and Kallman syndrome due to FGFR1 gene mutations?
KAL1 mutations: midline abnormalities (colobomas, cleft palate, horseshoe kidney, renal agenesis) and subtle neurological changes (synkinesia).
This is X-linked.
FGFR1 mutations: increased cleft palate but nothing else.
This is autosomal (usually dominant)
What should you think of when the vas deferens is not palpable?
Cystic fibrosis (congenital absence is common)
If a patient has gynecomastia, azoospermia, very small testes in association with elevated gonadotrophins - what should you think of?
Klinefelter syndrome
What can you see if you image the pituitary during pregnancy?
Pituitary enlargement
If a male patient has:
- Tender gynecomastia
- Low libido
- Low testosterone
- High estrogen
- Suppressed gonadotropin levels
Diagnosis?
Sertoli cell tumor or Leydig cell tumor that makes estradiol.
Is regular menstrual bleeding a reliable predictor of normal ovulatory function in women with hirsutism?
No
What is the best test to check for anovulation?
Serum progesterone level at day 20 - 24 of menstrual cycle. If it is less than or equal to 3 ng/mL then that indicates anovulation.
Are testosterone levels high or low with an hCG-producing tumor?
High
Are gonadotropin levels high or low with an hCG-producing tumor?
Low
In hyperthyroidism are total testosterone levels high or low?
High
In hyperthyroidism are free testosterone levels high or low?
Low
In hyperthyroidism are free testosterone to free estradiol ratios high or low?
Low
In hyperthyroidism are estradiol levels high or low?
High
In hyperthyroidism are total SHBG levels high or low?
High
Does hyperthyroidism cause tender gynecomastia?
It can
A history of venous thrombosis is generally considered to be an absolute contraindication to all oral estrogen-progestogen contraceptive use.
True or false?
True
Do non-oral progestogen-only contraceptives increase the risk of thromboembolism?
No
Amenorrhea occurs in ____% of menstrual cycles in patients taking OCPs.
5 - 10%
Macroprolactinemia accounts for ~ ____% of biochemical hyperprolactinemia.
20
How should you treat a prolactinoma in a woman if she is not planning to get pregnant?
Oral contraceptive pills
What is the next step in treatment of erectile dysfunction if the response to a PDE-5 inhibitor is not satisfactory?
Alprostadil
What hormone can you check to screen for Turner syndrome in an amenorrheic female?
FSH (serum)
What should you suspect in a patient with symptoms of primary adrenal insufficiency and hypogonadotrophic hypogonadism?
Adrenal hypoplasia congenita
What is the best initial test with adrenal hypoplasia congenita is suspected?
A cosyntropin stimulation test
Colles fractures from trauma are not associated with a greater risk of future vertebral fractures.
True or false?
False
Increased age and previous fracture increase the risk of future fracture.
True or false?
True
What treatment results in the largest increase in lumbar spine BMD?
Teriparatide (PTH 1-34)
Which has a greater increase in bone mineral density…
Sequential use of…
- PTH (teriparatide) and then alendronate
or
- Alendronate and then PTH (teriparatide)?
Sequential use of PTH (teriparatide) and then alendronate
What is the easiest and least expensive way to check for FHH in a patient whose calcium clearance/creatinine clearance ratio is 1% (0.01)?
Check calcium levels of patient’s parents
Is the vitamin 1,25 (OH) D level high or low in tumor-induced osteomalacia?
Low
What do tumors that cause tumor-induced osteomalacia secrete high levels of?
FGF-23
What does increased vitamin 1, 25 (OH) D do?
Increases intestinal absorption of calcium
McCune Albright syndrome is caused by somatic mutations in the ____ gene leading to reduction in expression of Gsalpha by about 50%.
GNAS
McCune Albright syndrome is caused by somatic mutations in the GNAS gene leading to reduction in expression of ____ by about 50%.
Gsalpha
McCune Albright syndrome is caused by somatic mutations in the GNAS gene leading to reduction in expression of Gsalpha by about __%.
50%
What do activating mutations in the LRP5 gene do to bone mass?
Increase it
Long term glucocorticoid therapy causes direct suppression of osteoblast function leading to decreased bone formation.
True or false?
True
Long term glucocorticoid therapy causes direct suppression of gonadotropin secretion by the pituitary, leading to decreased gonadal sex steroid secretion and subsequent decreased BMD.
True or false?
True
Long term glucocorticoid therapy causes decreased intestinal calcium absorption.
True or false?
True
Long term glucocorticoid therapy causes increased urinary calcium loss.
True or false?
True
Germline mutation in the _____ gene causes hyperparathyroidism-jaw tumor syndrome.
HRPT2
Patients with humoral hypercalcemia of malignancy describes a clinical syndrome that arises most commonly from over-production of PTH-related peptide by tumor cells.
True or false?
True
Patients with humoral hypercalcemia of malignancy have decreased intestinal calcium absorption due to decreased 1,25-dihydroxyvitamin D levels.
True or false?
True
Ectopic PTH production has not been reported with humoral hypercalcemia of malignancy?
True or false?
False
PTH-related peptide (PTH-rP) leads to increased expression of RANKL (receptor activator of nuclear factor-kappa B ligand) and decreased osteoprotegerin (OPG), with resultant increased bone turnover.
True or false?
False
Patients with humoral hypercalcemia of malignancy have an increase in their renal tubular phosphorus reabsorption threshold and an increase in their serum phosphate levels.
True or false?
False
What is the difference between pseudohypoparathyroidism and pseudopseudohypoparathyroidism?
Patients with pseudohypoparathyroidism are resistant to PTH (thus high levels of PTH, with low calcium) and patients with pseudopseudohypoparathyroidism are responsive to PTH.
What happens to the urinary cyclic AMP levels in pseudohypoparathyroidism?
They are decreased.
X-linked hypophosphatemic rickets is caused by genetic mutations in the ___ gene.
PHEX
What is the treatment of Prader-Willi Syndrome?
Growth hormone replacement
Is sitosterolemia associated with tendon xanthomas?
Yes
Is there evidence that cholestyramine reduces cardiovascular risk?
Yes
Are omega-3 fatty acids effective in treating hypertriglyceridemia during pregnancy?
Not particularly
Can fibrates be used in pregnancy?
Yes
Does elevated C-reactive protein indicate increased risk of cardiovascular disease?
Yes
Which are the only drugs that causes paradoxical increase in cholesterol (because of increased VLDL synthesis)?
Bile acid binding resins
Which is more important - LDL particle size or number?
LDL particular number
Gastric bypass operations typically produce weight loss of about ____ % of excess weight.
65%
What do anabolic steroids do to HDL levels?
Reduce them
What needs to be tested to diagnose anabolic steroid use?
Testosterone and LH
What endocrine abnormality caused by hereditary Hemochromatosis tends to improve with treatment of this underlying disorder?
Diabetes improves
What is the likely diagnosis if a woman has signs and symptoms of diabetes insipidus during pregnancy?
Excessive placental vasopressin made
Somatostatin inhibits pituitary hormones including ACTH, GH, TSH and prolactin.
True or false?
True
Somatostatin inhibits insulin secretion and stimulates glucagon secretion.
True or false?
False
Hypercalcemia is usually the first sign, followed by lesions of the pancreatic islet and pituitary.
Which syndrome is this?
MEN 1
How is MEN 1 genetically transmitted?
Affected patients inherit one normal and one mutant allele. A second hit knocking out the normal allele results in attenuation of the constraint on cell growth by menin.
The concordance of type 1 diabetes mellitus in genetically identical twins is _____ %.
30 - 60%
The concordance of type 2 diabetes mellitus in genetically identical twins is _____ %.
> 90%
About _____ % of first degree relatives of individuals with type 1 diabetes will have islet-cell antibodies or GAD antibodies.
3 - 4%
Does insulin administration prevent type 1 diabetes in first-degree relatives who are islet-cell antibody positive?
No (Diabetes Prevention Trial)
Should the diagnosis of diabetes be made on the basis of home glucose values?
No - blood glucose values should be confirmed by a laboratory.
Lifestyle modification, merformin, rosiglitazone, pioglitazone, and acarbose have been shown to delay the progression to diabetes.
True or false?
True
Which is more effective at delaying progression of impaired glucose tolerance to diabetes…
Intensive lifestyle modification or metformin?
Intensive lifestyle modification
The ADA recommends metformin (rather than thiazolidinediones or acarbose) as the initial therapy for individuals with impaired glucose tolerance.
True or false?
False
Lifestyle modification is preferred to medications, as it has been demonstrated not only to delay the progression to diabetes but also to reduce microvascular and cardiovascular complications of diabetes.
True or false?
False
Which drug class has been shown to delay the progression of nephropathy for a hypertensive individual with type 1 diabetes mellitus and microalbuminuria or macroalbuminuria…
ACEIs or ARBs?
ACEIs
Which drug class reduces progression to macroalbuminemia in type 2 diabetes mellitus patients with hypertension and microalbuminuria…
ACEIs or ARBs?
Both ACEIs and ARBs
Which drug class is usually sufficient as monotherapy to control hypertension in patients with type 2 diabetes mellitus…
ACEIs or ARBs?
Neither
Which drug class is accompanied by improvements in renal outcomes greater than what would be predicted by BP lowering alone…
ACEIs or ARBs?
Both ACEIs and ARBs
What pregnancy class are sulfonylureas?
Category C
Which sulfonylurea is preferred in the second and third trimester because it does not cross the placenta?
Glyburide
What pregnancy category are ACEIs and ARBs?
Category C in the first trimester and Category D in the second and third trimester.
Is there greater evidence for fetal anomalies in the first trimester with ACEIs or ARBs?
ACEIs
Is the HbA1c accurate in a patient with anemia secondary to renal disease?
No
Fluid retention, but not left ventricular dysfunction, is a side effect of both rosiglitazone and pioglitazone.
True or false?
True
Rosiglitazone is likely less safe than pioglitazone and currently is restricted to a company-regulated Access Program.
True or false?
True
Newer liquid chromatography-tandem mass spectrometry assays do detect all sulfonylureas available in the US and repaglinide.
True or false?
True
In fasting hypoglycemia caused by a nonpancreatic tumor, the insulin and c-peptide levels are suppressed during hypoglycemia.
True or false?
True
Intensive glycemic control in non-pregnant patients may worsen retinopathy initially (over months) but reduces the progression of retinopathy with longer-term follow-up (years).
True or false?
True (DCCT)
What race/ethnicity typically has ketosis-prone type 2 diabetes?
African-American or Hispanic
Can insulin glargine be mixed with other insulin formulations?
No (formulated at acidic pH)
What is the accelerated pharmacokinetic action of insulins Aspart, Lispro, and Glulisine related to?
Their molecular structure
Bedtime insulin glargine and NPH insulin are similar in HbA1c reduction and risk of severe hypoglycemia when added to oral agents for the treatment of type 2 diabetes mellitus.
True or false?
True
Which lipid disorder causes markedly elevated HDL levels?
CETP deficiency
MODY is inherited in an autosomal dominant fashion with a 30% penetrance rate.
True or false?
False
MODY is most often caused by mutations in the glucokinase or a hepatocyte nuclear factor (HNF) gene.
True or false?
True
When are GLP-1 agonists prone to cause hypoglycemia?
When they are used with other drugs that cause hypoglycemia like sulfonylureas.
Whole pancreas transplantation is superior to islet-cell transplantation at nearly normalizing glucose homeostasis 2 - 5 years after transplantation (without the need for supplemental insulin).
True or false?
True
Post-transplant diabetes is associated with reduced graft survival and patient survival after transplantation.
True or false?
True
What is the HbA1c goal for children younger than 6 years old?
8.5%
What is the HbA1c goal for children 6 - 12 years old?
8%
What is the HbA1c goal for children 13 - 19 years old?
7.5%
Does diabetic retinopathy always get worse during pregnancy?
No
What does diffuse uptake in the thyroid on a PET scan most likely indicate?
General thyroid abnormalities like Hashimoto’s thyroiditis,
Does transdermal estrogen affect levothyroxine dose?
No
What should you check when C-cell hyperplasia is discovered on pathology?
TPO antibodies
What is the adult dose of potassium iodide required after a nuclear accident?
130 mg daily for as long as the exposure is on-going.
What should you do if a patient is still hypothyroid after large doses of levothyroxine because of malabsorption?
Increase the dose - even if it’s crazy high.
How is resistance to thyroid hormone inherited?
Autosomal dominant inheritance
A fetus with a TR beta is more likely to survive pregnancy.
True or false?
True
What happens to the free T4 level in familial dysalbuminemic hyperthyroxinemia?
Usually normal
total T4 and free thyroxine index is elevated
What is the ‘donut’ sign (thyroid gland encircling the trachea) indicative of?
Thyroid lymphoma
What medication is helpful in Riedel thyroiditis?
Tamoxifen
What is the most likely adverse effect of exposure to radioactive iodine during pregnancy?
Miscarriage
If a patient has a goiter that appears to be because of Hashimoto’s disease but they have negative TPO antibodies… what is the likely cause of the goiter?
Thyroid peroxidase gene mutation (disorder of organification)
What is the classic cause of ‘black thyroid’?
Minocycline
If a patient has a lobectomy with medullary thyroid cancer, but then has a negative RET mutation, no family history of MTC, normal level of calcitonin… does the patient need a completion thyroidectomy?
No
Do patients with follicular thyroid cancer and capsular invasion alone have increased mortality due to disease?
No
Treatment of toxic multinodular goiter with radioactive iodine may lead to Graves disease.
True or false?
True
Can acute symptomatic hyponatremia (hypervolemic or euvolemic) be treated with conivaptan?
Yes
Which axis is most important to be assessed after head trauma?
Hypothalmic-pituitary-adrenal axis
Which treatment of acromegaly has a good chance of efficacy (both IGF-1 levels and tumor size) within a year?
Somatostatin anologs i.e. octreotide
When should follow-up imaging be done for a macroadenoma of the pituitary?
6 months
When should formal visual testing be done for a pituitary adenoma?
When the tumor abuts the optic chiasm
Does transspenoidal surgery treat the supra-sellar component of a pituitary adenoma?
No
Does transcranial surgery treat the sellar component of a pituitary adenoma?
No
In panhypopituitarism secondary to a macroadenoma causing visual symptoms how should the hormone replacement be handled?
Treatment with hydrocortisone should be initiated immediately, and then evaluate adrenal, thyroid and testicular function in 4 - 6 weeks.
What should be done when dopamine agonists are suppressing the prolactin but not decreasing the size of the pituitary macroadenoma?
Transsphenoidal surgery to remove the ‘non-secretory’ part of the pituitary macroadenoma.
Do somatostatin anologs cause gallstones?
Yes
If a patient gets gallstones with cholecystitis on somatostatin anologs then what is the next step?
Cholecystectomy.
No point stopping the therapy if it is effective.
Is high dose bromocriptine effective for Cushing’s disease?
No
Does phenoxybenzamine give false positive results when testing for pheochromocytomas?
No
Does amitriptyline give false positive results when testing for pheochromocytomas?
Yes
What should you suspect in a young patient with hypertension, spontaneous hypokalemia and a renin level that is not suppressed?
Renovascular disease caused by fibromuscular dysplasia
“Eversion of the upper and lower eyelids”
Diagnosis?
MEN 2B
Is MEN 2B associated with primary hyperparathyrodism?
No
Is MEN 2B associated with medullary thyroid cancer?
Yes
Is MEN 2A associated with medullary thyroid cancer?
Yes
Adrenocortical adenomas are associated with pure glucocorticoid excess and low adrenal androgens.
True or false?
True
Are the aldosterone levels high or low in Liddle syndrome?
Low
Is the renin plasma activity high or low in Liddle syndrome?
Low
Is hypertension a feature of Liddle syndrome?
Yes
What is the inheritance pattern of congenital adrenal hyperplasia?
Autosomal recessive
What is multi-nodular bilateral adrenal enlargement in the setting of mild Cushing syndrome diagnostic of?
ACTH-independent macronodular adrenal hyperplasia (AIMAH)
When there is maternal imprinting then there is higher penetrance when the mutation is inherited from…?
the father
Is SDHC inherited via maternal imprinting?
No
Is SDHD inherited via maternal imprinting?
Yes
Can you continue phenoxybenzamine for a 123 I-MIBG scan?
Yes
Can you continue labetalol for a 123 I-MIBG scan?
No
Can you continue nifedipine for a 123 I-MIBG scan?
No
Can you continue amitriptyline for a 123 I-MIBG scan?
No
Can you continue prochlorperazine for a 123 I-MIBG scan?
No
IPSS data are not valid if the patient does not have active hypercortisolism on the day of sampling.
True or false?
True
What is the best test to detect subclinical Cushing’s syndrome?
Over night dexamethasone test
