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COMLEX STEP 1 2017 > Endocrine > Flashcards

Endocrine Flashcards

1
Q

What is a common presentation of Neuroblastoma in children?

A

palpable asymptomatic abdominal mass that Crosses Midline

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2
Q

What are histological features of a Neuroblastoma?

A
  1. ganglion cells
  2. primitive, small, round blue cells organised in Rosettes
  3. embedded in a fibrillary pink matrix
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3
Q

What gene disorders are a/w Neuroblastoma?

A

amplification of MYCN oncogene

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4
Q

Flushing
Diarrhea
Bronchospasm (cough, wheezing)

A

Carcinoid Syndrome

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5
Q

What gene and tumor marker is associated with Adrenal Neuroblastoma?

A

N-myc oncogene

Bombesin Tumor marker

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6
Q

How do PTH and serum calcium levels compare in primary Osteoporosis to normal levels?

A

PTH and Ca2+ levels are typically normal in primary osteoporosis

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7
Q

identify:
female infant
subsequent recurrent vomiting, volume depletion, hypotension, failure to thrive
Increased Skin pigmentation

A

Congenital adrenal hyperplasia (21-hydroxylase deficiency)

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8
Q

During sex determination what factor causes testes development? what is the role of sertoli cells and leydig cells?

A

TDF (from SRY gene on Y chromosome) act on undifferentiated gonad to form testes

Sertoli cells - Antimullerian hormone (AMH) - involution of paramesonephric ducts

Leydig cell - Testosterone - male internal genitalia
–DHT - male external genitalia

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9
Q

What are Symptoms of Von-Hippel-Lindau disease?

A

CNS tumors (ie decreasing vision in an eye)
dermatologic hemangiomas
renal cell carcinoma
pheochromocytoma

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10
Q

What can hypothyroidism develop into if left untreated?

A

myxedema coma (social withdrawal, lethargy, drowsiness, confusion

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11
Q

Which DM drug can cause acute pancreatitis?

A

Exanatide (GLP-1 analog)

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12
Q

Which DM drug can cause weight gain and edema and progress to congestive heart failure in the most severe cases?

A

Pioglitazone (TZD’s)

-fluid retention from increased sodium reabsorption

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13
Q

Which DM drug has a dangerous adverse effect of lactic acidosis? (Presents with nausea, vomiting, fatigue, altered levels of consciousness, low serum bicarb)

A

Metformin

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14
Q

Triad of Pituitary, Pancreas, Parathyroid dysfunction

A

MEN 1

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15
Q

Triad of Parathyroid, pheochromocytoma, medullary thyroid carcinoma?

A

MEN 2a

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16
Q

Pheochromocytoma, medullary thryoid carcinoma, oral/intesetinal ganglioneuromatosis (wide spread benign neuromas most often in the mucosal surface of the eyes and mouth)

Marfan-type appearance

A

MEN 2b

mucosal neuromas - could possibly be tumor of the tongue

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17
Q

what neurotransmitter stimulates the adrenal medulla?

A

ACh from sympathetice PREganglionic neurons

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18
Q

Where are the following located and what is their fxn:

  1. GLUT-1
  2. GLUT-2
  3. GLUT-3
  4. GLUT-4
  5. GLUT-5
A
  1. RBC, brain, cornea, placenta (insulin independent)
  2. bidirectional: B islet cells, liver, kidney, small intestine (insulin independent)
  3. brain, placenta (independent)
  4. Adipose, striated muscle (INSULIN DEPENDENT)
  5. fructose: spermatocyte, GI tract
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19
Q

What hypothalamic secretion increases prolactin production?

A

TRH

increase in prolactin production in primary or secondary hypothyroidism

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20
Q

what 2 hormones inhibit GH secretion?

A

glucose
somatostatin

via negative feedback by somatomedin

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21
Q

How do magnesium levels influence PTH secretion?

A

slightly decreased Mg = increased PTH secretion

greatly decreased Mg = decreased PTH secretion

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22
Q

what are the 4 functions of T3 hormone?

A

4B’s

  1. Brain maturation
  2. Bone growth
  3. B-adrenergic fx (increase B1 in heart)
  4. increased Basal metabolic rate
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23
Q

What is the mechanism of action of Propylthiouracil (PTU)

A

inhibits both:
1. thyroid peroxidase = responsible for oxidation and organification of iodide, couples MIT, DIT to T4 and T3

  1. 5’-deiodinase: changes T4 to T3
24
Q

What is the mechanism of action of Methimazole?

A

inhibits thyroid peroxidase (responsible for oxidation and organification of iodide, couples MIT, DIT to T4 and T3)

25
Q

What is the mechanism/classification of Glipizide, Glyburide, Glimepiride what is a side effect to be careful of?

A

Sulfonylureas - close the K+ channel in pancreatic B-cells = insulin release

Hypoglycemia

26
Q

How is congenital adrenal hyperplasia managed?

A

exogenous corticosteroids to suppress excessive ACTH secretion

27
Q

What structures are derived from the Neural Crest Cells?

A

MOTEL PASS

Melanocytes
Odontoblasts
Tracheal cartilage
Entercromaffin Cells
Laryngeal Cartilage

Parafollicular Cells of thyroid
Adrenal Medulla + all ganglia
Schwann Cells
Spiral Membrane (Aorticopulmonary septum)

28
Q

What is the mechanism of action of Leuprolide? what malignancy can it be used in?

A

GnRH analog - initial use can cause increase in LH (increase testosterone) but prolonged use decreases LH (decreases testosterone)

Prostate Cancer (test reduction can slow progression)

29
Q

How do you distinguish Central Diabetes Insipidus caused by damage to Hypothalamic Nuclei (Supraoptic) or the Pituitary Gland?

A

Damage to pituitary = transient DI because axonal regeneration can occur

damage to hypothalamic nuclei = Permanent DI

30
Q

moodswings, difficulty concentrating, hand tremor
pain in front of neck radiates to ears, worse with swallowing
history of flu-like illness few weeks ago
thyroid is tender
diffuse DECREASE in radioactive iodine uptake
elevated ESR

identify dz and histological appearance of biopsy

A

subacute granulomatous thyroiditis (de Quervains)

  • follows viral illness
  • mixed cellular infiltrate with multinucleated giant cells
31
Q

How does a Prolactinoma affect GnRH, LH, Testosterone levels?

A

decrease in all

increased serum levels of prolactin suppress GnRH secretion

32
Q

47 year old man present with weightloss + swollen lips for past 2 months
3 weeks of painful migrating rash (begins as blisters and crusts over)
–erythematous papules and plaques with scaling/crusting at borders involving face, extremities
–Some papules coalesced, other have central clearing with bronze pigmentation replacing the erythema
Fingerstick glucose = 274mg/dL

Identify dz and decrease serum element?

A

Glucagonoma

  • -weightloss, Necrolytic migratory erythema
  • Normacytic Anemia
33
Q

Which HLA type is a/w Hashimotos Thyroiditis?

A

HLA-DR5

34
Q

Pts with Hashitmotos Thyroiditis are at an increased risk for what malignancy?

A

non-Hodgkin lymphoma

35
Q

5 month boy presents w poor feeding
difficulty holding up head, low body weight, head size, length
hepatomegaly, hypotonia
cardiac gallop rhythm, cardiomegaly
Muscle biopsy = enlarged lysosomes containing periodic acid-Schiff (PAS)-positive material

identify dz and deficient enzyme

A 
Pompe disease (glycogen storage dz type II)
--deficiency of acid a-glucosidase
36
Q

What is the mechanism of action of Nateglinide, Repaglinide?

A

Stimulate postprandial insulin release by binding to K+ channels on B-cell membranes (site differs from sulfonylureas)

37
Q

What is the mechanism of action of Exenatide, Liraglutide?

A

GLP-1 analogs (increased glucose dependent insulin release, decrease glucagon release, decrease gastric emptying, increase satiety)

38
Q

What is the mechanism of action of Linagliptin, Saxagliptin, Sitagliptin?

A

DPP-4 inhibitors

–DPP-4 deactives GLP-1

39
Q

What is the mechanism of action of Pramlintide? What is its use?

A 
Type 1 DM
Amylin analog (decreased gastric emptying, decreased glucagon)
40
Q

3 year old present with neck swelling, painful, started 1 week ago
brief hx of URI
no PHM, met all milestones

Tender, midline mass just below the hyoid bone

identify

A

Thyroglossal duct cyst

41
Q

What electrolyte activity does insulin cause?

A

activates the Na,K ATPase pump

increased cellular uptake of potassium, release of sodium

42
Q

12 hour old infant with respiratory distress and difficulty feeding. Tachypneic
generalized hypotonia and hoarse cry. Significant macroglossia
mother from south america

identify cause

A

Congenital hypothyroidism - most common cause worldwide is Iodine deficiency

43
Q

What antibody is associated with Hashimoto thyroiditis?

A

anti-peroxidase antibodies

44
Q

elderly pt with T2DM present with progressively decreasing eyesight over the past two years. has a glare when looking directly at lights. what is happening and what is mechanism of damage?

A

Cataracts - converion of glucose to sorbitol in the lens (toxic)

45
Q

pt w worsening dysphagia “feels like food gets stuck her throat”
Prominent, nontender thyroid gland
normal TSH, normal T4
Histopathology of thyroid: mix of fibrous tissue and infiltrative lymphocytes, neutrophils, plasma cells, eosinophils.

What is the dz? what can be a complication?

A

Riedel (Infiltrative) thyroiditis -

painless thyroid enlargement, normal thyroid hormone production

complication: Obstructive pneumonia

46
Q

What is the initial step in management of a pheochromocytoma

A

alpha blockers (a1 and a2) FIRST
then beta blockers
then surgery

47
Q

12 year old has notably short stature and rounded face. fourth metacarpals are shortened. Labs show hypocalcemia, hyperphosphatemia, increased PTH
what is dz?

A

Psuedohypoparathyroidism (end-organ resistance to PTH)

-hypocalcemia, hyperphosphatemia, increased PTH.

pt has short stature, rounded face, shortened fourth/fifth metacarpals.

48
Q

40 yr old pt finds a slowly enlarging nodule in the thyroid gland. TSH is low. Radioiodine studies show increase radioiodine uptake in the solitary nodule. what is the most likely dx?

A

Follicular adenoma - suspected when a nodule discovered is hyperfunctioning

–does not require therapy unless they produced signs of hyperthyroidism

49
Q

What should diabetic pts be aware of when prescribed B-blockers?

A

B-blockers can mask the symptoms of hypoglycemia:

–palpitations, paresthesias, behavioral changes when plasma blood sugar is low

50
Q

What vitamin deficiency can be seen with carcinoid syndrome? what are symptoms?

what is an additional finding of carcinoid syndrome (20% initial presentation)

A

Niacin deficiency
—tryptophan is the precursor for both serotonin and niacin, all the tryptophan is used up to make serotonin

Sx: Dermatitis, Diarrhea, Dementia, Death

–Tricuspid regurgitation

51
Q

A 5 year old pt is treated for T1DM. four hours later, pt presents with muscle weakeness, fatigue, chest palpitations

what is the cause of the new sx?

A

Excess insulin causes HYPOKALEMIA
–enhances the Na/K ATPase pump in skeletal muscle

–weakness, cramps, fatigue, palpitations, arrhythmias

52
Q

What serum level should be measured when a pt presents with Sx of acromegaly

A

IGF-1

GH fluctuates too much to be accurate

53
Q

What is the most appropriate med to relieve gastroparesis from poor glycemic control?

A

Metoclopramide - prokinetic agent that sensitive GI tract to acetylcholine

–also used as an antiemetic agent

54
Q

What other lab should be checked if oral potassium fails to correct hypokalemia?

A

Check magnesium levels

  • -Mg is a cofactor in potassium channel function in the kidney
  • -Mg closes channels in absence of aldosterone (low Mg = channels stay open and K is lost)
55
Q

63 yr old pt w weight loss, diarrhea, chest pain. Hx of recent flu-like illness. Initial exam shows sinus tach, painful and enlarged thyroid. what is the dx?

A

Subacute granulomatous thyroiditis

  • -leakage of thyroid hormone from inflamed thyroid gland
  • -commonly follows viral URI
  • -the only form of thyroiditis that results in a PAINFUL thyroid gland.
56
Q

pt w tachycardia, frequent sweating, tremor, and reports “feeling hot”. labs show decreased TSH. what is the drug of choice to initially control her cardiac symptoms? why?

A

Propranolol

  • -nonselective beta blocker that decreases sympathetic Sx
  • -also INHIBITS 5’deiodinase
57
Q

38 yr old African American female presents complaining of chronic fatigue and lower back pain for 11 months. she is taking phenytoin for epilepsy. Exam shows wide-based “waddling” gait, accentuated lumbar lordosis, proximal muscle weakness in the lower extremities. Palpation of hip = symmetrical nonradiating pain.

  1. What is dz.
  2. What serum lab findings?
A
  1. Osteomalacia from vit D deficiency
  2. Increased PTH
  • blacks - higher prevalence of vit D deficiency
  • phenytoin activates p450 to degrade Vit D faster

COMLEX STEP 1 2017 (19 decks)

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