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Endocrine Flashcards

1
Q

17-alpha hydroxylase deficiency

  • Boys - ambiguous genitalia (phenotypically female), undescended testes
  • Girls - lacks secondary sexual development
A
  • High: MINERALOCORTICOIDS, blood pressure
  • Low: cortisol, sex hormones, [K+]
  • Labs: low androstenedione
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2
Q

21-hydroxylase deficiency

  • Boys: salt wasting in infancy, precocious puberty in childhood
  • Girls: virilization (clitoromegaly)
A

Most common CAH
Needed for MC and GC production (instead ACTH is shunted to sex hormones)
- High: sex hormones, [K+]
- Low: cortisol, mineralocorticoids, BP
- Labs: increased renin activity, increased 17-hydroxy-progesterone

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3
Q

11-beta-hydroxylase deficiency

  • Boys: ?
  • Girls: virilization
A

Low aldosterone, but high 11-deoxycorticosterone –> elevated BP

  • High: sex hormones, BP
  • Low: cortisol, [K+]
  • Labs: decreased renin activity
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4
Q

Cortisol upregulates what R?

A

Alpha1 on arterioles –> increased sensitivity to NE and Epi –> elevated BP

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5
Q

Thyroid hormones up-regulate what R?

A

Beta1 on heart –> increased CO, HR, SV, and contractility

- Also increase Na+/K+ ATPase –> increased O2 consumption, RR, body temp –> increased basal metabolic rate

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6
Q

5’-deiodinase

A

Converts T3 to T4 in peripheral tissue

- Inhibited by PTU but NOT methimazole

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7
Q

Thyroid Peroxidase

A
  • Oxidation and organification of iodide
  • Couples monoiodotyrosine to di-iodotyrosine
  • Inhibited by PTU and methimazole
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8
Q

Metyrapone Stimulation

- Adrenal insufficiency

A

Blocks 11-deoxycortisol –> cortisol (last step of cortisol synthesis)

  • Normal: decreased cortisol, compensatory increase in ACTH and 11-deoxycortisol
  • Primary adrenal insufficiency: ACTH increases, but 11-deoxycortisol remains decreased after test
  • Secondary adrenal insufficiency: both ACTH and 11-deoxycortisol remain decreased after test
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9
Q

Waterhouse-Friderichsen Syndrome

- Septicemia, DIC, endotoxic shock

A
  • Primary adrenal insufficiency due to adrenal hemorrhage

- NEISSERIA MENINGITIDIS

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10
Q

Conn Syndrome

- HTN, metabolic alkalosis, no edema

A
  • Adrenal adenoma –> primary hyperaldosteronism

- Increased aldosterone, decreased renin (negative feedback)

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11
Q

How can fibromuscular dysplasia and atherosclerosis cause hyperaldosteronism?

A
  • Activate JGA –> increased renin –> increased aldosterone
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12
Q

Neuroblastoma

  • Kids - abdominal distension, firm/irregular mass that can cross the midline (vs. Wilms tumor)
  • Opsoclonus-myoclonus syndrome (“dancing eyes-dancing feet”)
A
  • Most common tumor of adrenal medulla in children
  • Originates from neural crest cells
  • Increased HVA and VMA in urine
  • Homer Wright rosettes
  • Bombesin and NSE +
  • N-myc oncogene overexpressed
  • APUD tumor
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13
Q

Hashimoto Thyroiditis

- Hypothyroidism

A
  • Ab: antithyroid peroxidase (antimicrosomal), antithryoglobulin
  • HLA-DR5
  • Increased risk of non-hodgkin lymphoma
  • Histo: Hurthle cells, lymphoid aggregates w/ germinal centers
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14
Q 
Congenital Hypothyroidism (Cretinism)
- Neonates/infants
A

Pot-bellied, pale, puffy-faced child with protruding umbilicus, protuberant tongue, and poor-brain development
- Short stature and skeletal abnormalities due to decreased growth hormone

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15
Q

Subacute Granulomatous Thyroiditis (de Quervain)

- Hypothyroid

A
  • Follows viral infection
  • Histo: granulomatous inflammation (macs and giant cell)
    TENDER THYROID
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16
Q

Riedel Thyroiditis

- Hypothyroid

A
  • Thryoid replaced w/ fibrous tissue w/ inflammatory infiltrate
  • Mimics anaplastic carcinoma
  • IgG4-related systemic disease
  • Hard, rock-like painless goiter
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17
Q

Graves Disease

- Hyperthyroid

A

Ab: thyroid-stimulating Ig (IgG, type II HSN)

  • Dermal fibroblasts –> pretibial myxedema
  • T cells –> increase CKs (TNF-alpha, IFN-gamma) –> increased fibroblast secretion of hydrophilic GAGs –> exophthalmos
  • Histo: tall, crowded follicular epi cells, SCALLOPED COLLOID
  • Rx: beta blockers, thiomide (blocks peroxidase), radioiodine ablation, prednisone for severe opthalmopathy
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18
Q

Treatment of thyroid storm

A

Beta blockers (propanolol), PTU, corticosteroids (prednisolone), potassium iodide

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19
Q

Wolff-Chaikoff Effect

A

Excess iodine temporarily inhibits thyroid peroxidase –> decreased iodine organification –> decreased thyroid hormone

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20
Q

Jod-Basedow Phenomenon

A

Patient w/ iodine deficiency and partially autonomous thyroid tissue is given iodine –> thyrotoxicosis
- Opposite of Wolff-Chaikoff effect

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21
Q

What nerve runs with inferior thyroid artery?

A

Recurrent laryngeal nerve

- Risk of injury during thyroidectomy –> hoarseness

22
Q

What nerve runs with superior artery?

A

Superior laryngeal nerve –> cricothyroid muscle

23
Q 
Papillary Carcinoma (Thyroid)
- Most common, excellent prognosis
A
  • Orphan Annie nuclei, psammoma bodies, nuclear grooves

- Increased risk with RET and BRAF mutations and childhood irradiation

24
Q 
Follicular Carcinoma (Thryoid)
- Good prognosis
A
  • Invades thyroid capsule and vasculature (vs. follicular adenoma)
  • Uniform follicles
  • Hematogenous spread
  • RAS mutation
25
Q

Medullary Carcinoma (Thyroid)

A
  • Parafollicular “C” cells
  • Neuroendocrine
  • Produces calcitonin –> diarrhea, flushing
  • Sheets of cells in AMYLOID STROMA (Congo red)
  • Associated with MEN2A and MEN2B (RET)
26
Q 
Anaplastic Carcinoma (Thyroid)
- Very poor prognosis, older patients
A
  • invades local structures –> dysphagia, respiratory compromise
27
Q

Thyroid Lymphoma

A

Associated with Hash thyroiditis

28
Q

Chvostek and Trousseau sign?

A
  • HYPOPARATHYROIDISM
  • Hypocalcemia
  • Chvostek –> facial nerve
  • Trousseau –> BP cuff
29
Q

Pseudohypoparathyroidism Type 1A (Albright hereditary osteodystrophy)
- Shortened 4th/5th digit, short stature

A
  • Unresponsiveness of kidney to PTH –> hypocalcemia despite increased PTH levels
  • AD –> defective Gs protein alpha subunit –> end-organ resistance to PTH
  • Must be inherited from mother (imprinting)
30
Q

Pseudopseduohypoparathyroidism

A
  • Findings of Albright hereditary osteodystrophy but without end-organ PTH resistance
  • Defective Gs protein alpha subunit is inherited from father
31
Q

Familial Hypocalciuric Hypercalcemia

A

Defective Ca2+ sensing receptor in multiple tissues

- Excessive renal Ca2+ reuptake –> mild hypercalcemia with hypocalciuria with normal/elevated PTH levels

32
Q

Sheehan Syndrome

- Postpartum failure to lactate, absent menstruation, cold intolerance, loss of pubic hair

A
  • Ischemic infarct of pituitary following postpartum bleeding (pituitary is much larger during pregnancy but there is no increase in blood flow –> increased susceptibility to hypoperfusion)
33
Q

Empty Sella Syndrome

- Idiopathic, obese women

A

Atrophy or compression of pituitary

34
Q

Pituitary Apoplexy
- Sudden onset severe HA, visual impairment (bitemporal hemianopia, diplopia due to CN III palsy), features of hypopituitarism

A
  • Sudden hemorrhage of pituitary gland –> compresses normal tissue
  • Often in presence of existing pituitary adenoma
35
Q

Type 1 DM Antibodies

A

Glutamic acid decarboxylase antibodies

36
Q

Most common cause of death in DM

A

MI

37
Q

Nodules seen in diabetic nephron

A

Kimmelstiel-Wilson Nodules

38
Q

Type 1 DM HLA

A

HLA-DR3 and DR4

39
Q

Histology of T1DM vs. T2DM

A

T1DM: islet leukocytic infiltrate
T2DM: islet amyloid polypeptide deposits

40
Q

Deadly infection in DKA?

A

Mucormycosis (Thizopus)

41
Q

Treatment for DKA?

A
  • IV fluids –> sugar in urine is diuretic (dehydrated)
  • IV insulin
  • K+ –> lost lots of K+ in urine (even though labs show hyper K+, plus insulin drives K+ into cells)
  • Glucose if necessary
42
Q

Hyperosmolar Hyperglycemia Nonketotic Syndrome

  • Thirst, polyuria, lethargy, focal neuro defects (seizures), can progress to coma and death
  • elderly T2 diabetic w/ limited ability to drink
A
  • Profound hyperglycemia-induced dehydration (osmotic diuresis)
  • Labs: hyperglycemia, increased serum osmolarity, no acidosis, no ketones
  • Rx: aggressive IV fluids, insulin therapy
43
Q

Presentation of glucagonoma?

A
  • Dermatitis (necrolytic migratory erythema)
  • Diabetes (hyperglycemia)
  • DVT
  • Declining weight
  • Depression
    Rx: octreotide, surgery
44
Q

Presentation of insulinoma?

A
  • Whipple triad: hypoglycemia, sx of hypoglycemia (lethargy, syncope, diplopia), and resolution of sx after normalization of glucose levels
  • Labs: decreased blood glucose, increased C-peptide
  • MEN1 syndrome
45
Q

Presentation of somatostatinoma?

A
  • Diabetes/glucose intolerance, steatorrhea, gallstones due to decreased secretion of secretin, CCK, glucagon, insulin, gastrin
46
Q

Presentation of carcinoid syndrome?

A
  • Recurrent diarrhea, cutaneous flushing, asthmatic wheezing, R-sided valvular heart DZ, pellagra (niacin deficiency), 5-HIAA in urine
  • Only seen if tumor has spread out of GI tract
  • ROSETTES
  • Serotonin
47
Q

Presentation of gastrinoma?

A

Abdominal pain (PUD), diarrhea (malabsorption)

  • Gastrin levels remain elevated after administration of secretin
  • MEN1
48
Q

Presentation of VIPoma?

A

Watery diarrhea, hypokalemia, achlorhydria

49
Q

MEN1

A

Pituitary adenoma, pancreatic endocrine tumor, parathyroid adenoma
- MEN1 (menin) - tumor suppressor, Chr 11

50
Q

MEN2A

A
  • MEDULLARY THYROID CARCINOMA (amyloid on bx, prophylactic thyroidectomy), pheochromocytoma, parathyroid hyperplasia (hypercalcemia)
  • RET (RTK in cells of neural crest origin)
51
Q

MEN2B

A

Medullary thyroid cancer, pheochromocytoma, MUCOSAL NEUROMAS, MARFINOID HABITUS
- RET (oncogene)

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