Pathoma Ch 7, 8, 11, 15 (Vascular, Cardiac, Liver, Endo) Flashcards
7. Vascular Path 8. Cardiac 11. Exocrine Pancreas, GB, Liver 15. Endocrine
1
Q
Explain why alk phos is elevated in primary hyperparathyroidism
A
Alkaline phosphatase = enzyme in osteoblasts that produced alkaline environment that makes it possible for Ca2+ to get deposited in bone
PTH actually activates osteoclasts (bone breakdown) indirectly by activation osteoblasts first, so elevated alk phos is a result of osteoblast activity
But basically alk phos elevated b/c of bone breakdown
2
Q
Name the JONES criteria for ARF
A
Joints: migratory polyarthritis of big joints (knees)
Cardiac: pericarditis (all 3 layers, MR then MS)
Nodules: subcutaneously
Erythema marginatum: rash is most red at the borders
Sydenham’s chorea: involuntary muscle contractions
All except the cardiac are self-limited
3
Q
Child radiated for severe acne, at increased risk for what cancer?
A
Specifically ionizing radiation increases risk for papillary carcinoma of the thyroid (MC thyroid malignancy)
4
Q
Henoch-Schloen Purpura
(a) Clinical context
(b) Physical exam finding
(c) Mechanism
(d) Associated kidney d/o
A
HSP = small vessel vasculitis
(a) Typically in a child after a upper respiratory tract infection (b/c URI stimulates IgA production)
(b) Palpable purpura
- purpura look like bruising, but they’re palpable (unlike normal bruising) b/c of the underlying vessel inflammation
(c) Mechanism = IgA immune complex deposition
(d) IgA nephropathy = IgA deposition in the mesangium
5
Q
2 main clinical features of Sheehan syndrome
A
Sheehan syndrome (hormone demand during pregnancy is so high that pituitary gland basically doubles in size and outgrows its blood supply, so very vulnerable to hypoxia/ischemia during huge volume loss such as hemorrhage during birth) presents as
- poor lactation
- **loss of pubic hair (b/c stimulated by androgens that requires stimulation of LH from anterior pituitary)
6
Q
Mechanism by which DM II increases risk of
(a) Atherosclerosis
(b) Nephrotic syndrome
(c) Peripheral neuropathy
(d) Cataracts
(e) Retinopathy
A
DM II complications
(a) Atherosclerosis 2/2 nonenzymatic glycosylation of large and medium blood vessel walls
(b) Nephrotic syndrome 2/2 nonenzymatic glycosylation of small blood vessels making up the glomeruli
(c) Peripheral neuroathy 2/2 osmotic damage: glucose converted to sorbitol damage schwann cells (that myelinate peripheral nerves)
(d) Cataracts b/c lens takes up glucose independent of insulin => damaged via sorbital like schwann cells
(e) Retinopathy b/c pericytes of retinal blood vessels undergo osmotic damage
7
Q
Dx of DM II- lab values
A
Diagnose type II diabetes w/ random glucose over 200 or fasting glucose over 126
-or serum glucose over 200 2 hrs after oral load
8
Q
MC cancer seen after pt is put on immunosuppressive therapy
A
Squamous cell carcinoma- has same but additional RF than BCC including immunosuppression and chronic inflammation
9
Q
What is dermatitis herpetiforms?
(a) Tx
A
Dermatitis herpetiforms = IgA deposition along the tip of the dermal papillae => very tiny blisters (that look like herpes, hence herptiform)
(a) High association w/ Celiac disease => dietary restriction of gluten often resolves dermatitis herpetiforms
- IgA made in Celiac disease cross react with reticulan antigen dermal papillae
10
Q
Differentiate the endocarditis caused by S. Viridans and S. Aureus
A
Strep Viridans (MC overall) is a low virulence organism => can only cause endocarditis of already damaged heart valves -also causes small vegetations => subacute endocarditis that doesn't destroy the valve
Staph aurus (MC in IVDU) is high virulence => can infect non-damaged valves, esp tricuspid (b/c right sided first to get it from IVDU) -large vegetations => acute endocarditis that destroys the valve
11
Q
2 RF for pancreatic adenocarcinoma
A
RF for pancreatic adenocarcinoma
- smoking
- chronic pancreatitis (EtOH mostly)
12
Q
Tumor marker for pancreatic andenocarcinoma
A
CA 19-9
13
Q
Classic historical cause of thoracic aneurysm
A
Tertiary syphilis
14
Q
Where do cardiac mets MC go to?
(a) MC cancers to met
A
Cardiac mets go to the pericardium and cause pericardial effusion
(a) Breast and lung, melanoma, lymphoma met to the lungs
15
Q
Significance of aldose reductase in diabetes
A
Aldose reductase catalyzes glucose to sorbitol, which causes osmotic damage in diabetes in cells that uptake glucose independent of insulin
- osmotic damage to Swann cells => neuropathy
- Pericytes of retinal blood vessels => retinopathy
- Lens => cataracts
16
Q
Explain the pathophysiology of hyperosmolar non-ketotic coma in DM II
A
Hyperosmolarity of serum due to such high serum glucose (like over 500 holey crud) causes such a life threatening (via coma) diuresis
- ketones are abscent b/c tiny bit of insulin in blood prevents production
- but hyperosmolarity of blood causes such severe diuresis => hypotension and coma
17
Q
How does T4 levels impact TSH secretion?
A
T4 feedback on TSH works by regulating TRH-receptors on anterior pitutiary
When T4 is low (hypothyroidism)- TRH-receptors on anterior pituitary are upregulated, stimulating anterior pituitary production and release of TSH
18
Q
2 main histologic features of the most common type of thyroid malignancy
A
Histologic features of papillary carcinoma (80% of thyroid malignancies)
- Orphan annie bodies (nuclei w/ central white clearing)
- Psamomma bodies (calcification)
19
Q
Polyarteritis Nodosa
(a) Spares what organ?
(b) Explain appearance on imaging
A
Polyarteritis Nodosa = medium vessel vasculitis
(a) Polyarteritis meaning it affects arteries of many systems, but spares the lungs
(b) String of pearls appearance on imaging b/c the lesions are at different stages
- early lesions undergoing fibrinoid necrosis = the string
- then the beads are the older lesions that form bump/nodes (hence nodosa)
20
Q
Differentiate presentation of pancreatic adenocarcinoma at head vs. tail/body of pancreas
A
Adenocarcinoma in head of pancreas => jaundice 2/2 obstruction of biliary tree
Islets (endocrine part of pancreas) are more in the body/tail, so adenocarcinoma of tail/body may present w/ secondary diabetes mellitus
21
Q
Murmur characteristic of
(a) AS
(b) AI
(c) MS
(d) MR
(e) MVP
A
Murmur
(a) AS: systolic ejection click w/ crescendo-decrescendo murmur
(click due to opening of stenotic valve, then crescendo as high pressure forces blood out)
(b) AI: early diastolic blowing murmur
(c) MS: Opening snap followed by diastolic rumble
(d) MR: Holosystolic blowing murmur
(e) MVP: mid-systolic click as mitral valve balloons out into the LA
22
Q
Clinical features of somatostatinoma
A
Somatostatin inhibits both gastrin (=> achlorhydria = lack of H+ in gastric secretions) and CCK (=> steatorrhea and cholelithiasis)
CCK stimulates gall bladder contraction => secretion of bile salts needed for fat absorption
- w/o GB contraction, increased risk of status (cholelithiasis)
- w/o bile salt secretion, can’t absorb fats (steatorrhea)
23
Q
Congenital heart disorder MC associated w/
(a) FAS
(b) Downs
(c) Congenital rubella
(d) Maternal diabetes
A
MC congenital heart d/o associated w/
(a) Fetal alcohol syndrome = VSD
(b) Downs = ostuim primum type of ASD
(c) Congenital rubella and PDA
(d) Maternal diabetes and TGA (transposition of the great arteries)
24
Q
MC cause of death in aortic dissection
A
Cardiac tamponade
-dissection travels backwards, blood gets into the pericardial sac
25
What does hair in a mole indicate?
Indicates the melanocyte growth is respecting the hair follicle boundaries => more indicative of benign melanocyte proliferation
Most congenital nevi have hair
26
Describe Ansiptz's sign of psoriasis
Psoriasis = well-demarcated salmon colored plaques
- hyperkeratosis and elongation of dermal papillae w/ shortened epidermis on top the dermal papillae
- so when the scales are scraped off, there is very thin sheet of epidermis so you get 'pinpoint bleeding' b/c of the exposed blood vessels in the dermis
27
Hashimoto's thyroiditis
(a) Associated HLA
(b) Increased risk of what malignancy
Hashimoto's thyroiditis = AI destruction of thyroid tissue
(a) HLA-DR5
(b) Increased risk of B-cell lymphoma, specifically marginal cell, b/c in Hashimoto's there is an increased formation of thyroid germinal centers => more post-germinal center B cells (forming marginal zone)
28
Differentiate toxic vs. non-toxic multinodular goiter
Goiter becomes toxic when it no longer is responsive to TSH levels
Non-toxic: excess tissue only increases production if TSH is high
Toxic: tissue is pumping out excess thyroid hormone despite low TSH
29
Explain why kids w/ ToF squat when they get SOB
ToF: RVOT stenosis, RVH, VSD, overriding aorta
Squatting increases afterload of the LVOT, decreasing amount of R --> L shunt (high pressure due to pulmonic stenosis forces blood left). Reduced R --> L shunt lets more blood go out RVOT to the lungs to get oxygenated => improved SOB
30
Differentiate myxoma and rhabdomyoma
(a) Cell of origin
(b) Associated condition
Both are benign primary cardiac tumors
-recall heart tissue is permanent, so not likely to get cancer from the myocytes themselves
Myxoma
(a) Benign mesenchymal proliferation, so tumor is of the connective tissue (not the cardiac muscle)
(b) Location: left atrium
Rhabdomyoma
(a) Benign hamartoma (overgrowth of home tissue) of cardiac muscle- so benign tumor of striated muscle
(b) Associated w/ tuberous sclerosis
31
Occlusion of which artery causes risk for papillary muscle rupture
RCA
Right coronary artery supplies the papillary muscles => RCA occlusion can cause papillary muscle rupture
32
Differentiate Osler nodes and Janeway lesions
Both are complications of bacterial endocarditis when vegetations embolize
Osler nodes ('ouch ouch osler') = painful lesions on fingers and toes
Janeway lesions = painless/non-tender erythematous lesions on palms and soles
33
MC type of cardiomyopathy
(a) Complications
Dilated cardiomyopathy (all 4 chambers enlarged => systolic dysfunction) is the MC type
(a) Complications: MR and TR from stretching of valve annulus, then arrhythmia (think stretching of the conduction system)
34
Explain the physiology of the following signs of Cushing syndrome
(a) Abdominal striae
(b) Hyperpigmentation
Cushing syndrome = hypercortisol
(a) Abdominal striae b/c cortisol impairs proper collagen synthesis so BVs are weak, striae are popped BVs
(b) Hyperpigmentation b/c ACTH is similar (and from the same precursor) as MSH = melanocyte stimulating hormone
35
Which thyroid malignancy spreads hematogenously?
Follicular thyroid caricnoma spreads hematogenously
One of the 4 carcinomas that spreads hematogenously (instead of lymphatically like most carcinomas)
Recall 4 carcinomas that spread hematogeneously: follicular carcinoma of the thyroid, renal cell carcinoma (into renal vein), hepatocellular carcinoma (into hepatic vein), choriocarcinoma (placenta is meant to invade BV)
36
What type of arteriosclerosis accounts for incidental finding of calcification in a vessel-pattern on mammography
Monckeberg Medial Calcific Sclerosis = nonobstructive calcification of the media of a BV wall
-since it's nonobstructive, it doesn't change the lumen diameter => is clinically insignificant
Monckeberg medial calcific sclerosis is one of 3 types of arterosclerosis: others are atherosclerosis (intimal thickening of medium and large BV walls) and arteriolosclerosis (narrowing of small arterioles)
37
Inflammatory dermatosis associated w/ chronic Hep C infection
Lichen planus = purple papules 2/2 inflammation at dermal-epidermal jxn
-etiology unknown but associated w/ chronic Hep C infxn
38
What is cretinism?
(a) Etiologies
(b) Clinical features
Cretinism = congenital hypothyroidism
(a) Maternal hypothyroidism during development, iodine deficiency, thyroid agenesis, thyroid peroxidase mutation (cant product hormone)
(b) ID, short stature (thyroid hormone needed for proper brain and skeletal development)
- glossitis, umbilical hernia
39
Which is the most specific marker for pancreatitis, why?
Lipase is more specific than amylase b/c amylase can also be elevated in damage to the salivary gland
40
How long after MI do you get see following complications:
(a) Dressler syndrome
(b) Arrythmia
(c) Fibrinoid pericarditis
(d) Aneurysm
(e) Rupture- of free wall, papillary muscle, IV septum
Post-MI
(a) Autoimmune pericarditis 6-8 wks post-MI
- assumes pericardium was exposed to immune cells, so then 6-8 weeks we've made antibodies against self pericardial antigens
(b) Arrhythmia w/in the first 24 hrs- b/c if conducting system is damaged it'll show early
(c) Fibrinoid pericarditis due to neutrophil inflammation (so 1-3 days) after transmural infarction
(d) Aneurysm once the wall is weakened by scaring- so weeks to months after
(e) Rupture risk highest at days 4-7 when macrophages come in to eat the necrosed tissue
41
Which of the congenital heart defects requires a shunt
TGA requires a shunt
| -b/c two separate circuits are created (R heart w/ systemic circulation and L heart w/ lungs) that need to be connected
42
Pt presents w/ sudden onset of multiple seborrheic dermatoses
Next step?
Sudden onset of multiple seborrheic dermatoses = Lesser-Trelat sign
(a) Suggestive of underlying carcinoma, esp of GI tract
43
10% rule for pheo
10% bilateral
10% familial
10% malignant (so 90% are benign!)
10% extra-adrenal (organ of Zuckerkandle, bladder wall
44
Explain the physiology of the following signs of Cushing syndrome
(a) Muscle weakness with frail extremities
(b) Moon facies, buffalo hump
Cushing syndrome = hypercortisol
(a) Cortisol stimulates gluconeogenesis which uses amino acids as substrate so breaks down muscle
(b) Cortisol stimulates increase in serum glucose (thru gluconeogenesis and glycogenolysis) which stimulates release of insulin, insulin stimulates fat storage => buffalo hump, moon facies, truncal obesity
45
Name some clinical features of bacterial endocarditis
Bacterial endocarditis: fever w/ new murmur
-septic emboli => Janeway lesions (painless erythematous lesions of palms and soles), Osler nodes (painful lesions on finger tips and toes), splinter hemorrhages
46
Explain the mechanism of the two visible clinical features of Graves disease
Graves disease clinical features:
- excess stimulation of TSH-receptors (by same IgG that work on TSH-receptors in thyroid) on fibroblasts behind the orbit => exophthalmos
- excess stimulation of TSH-receptors on fibroblasts of shins => pretibial myxedema
47
Clinical feature differentiating giant cell arteritis and Takayasu's arteritis
The two large vessel vasculitides:
1. Temporal giant cell arteritis is of the more distal vessels, the internal carotid and its branches => ocular symptoms (opthalmic artery), headache (temporal artery), jaw claudication
2. Takayasu's is of more proximal vessels: the aorta and its major branches => pulselessness of the upper extremities
- also visual and neurologic defects
48
Clinical features of VIPoma
Watery diarrhea, hypokalemia, achlorhydria (lack of H+ in gastric secretions)
- VIP increases water and K+ secretion into intestinal lumen
- VIP inhibits gastrin
49
Bacteria responsible for endocarditis w/
(a) MC cause overall
(b) MC cause in IVDU
(c) MC on prosthetic valves
(d) Associated w/ underlying CRC
(e) Negative blood cultures
Bacterial endocarditis
(a) Strep viridans = MC overall
(b) MC in IVDU = Staph aureus
(c) MC on prosthetic valves = Staph epidermidis
(d) Strep bovis associated w/ underlying colorectal carcinoma
(e) Negative blood cultures associated w/ HACEK organisms: Hemophilis, actinobacillus, cardiobacterium, eikenella, kingella
50
Explain where the tear is in an aortic dissection
(a) What layer of the BV wall
(b) What part of the aorta
Aortic dissection
(a) Tear in the intima, blood gets into the media
- normal anatomy: intima --> media --> adventitia
(b) Proximal 10cm of the aorta- so the first 10cm right off the heart, b/c this is where the highest pressure is
51
Size of adrenal glands in the following cause of Cushing's syndrome
(a) Exogenous steroids
(b) Primary adrenal adenoma
(c) ACTH-secreting pituitary adenoma
(d) Ectopic ACTH
Causes of Cushing's syndrome (hypercortisolism)
(a) Exogenous steroids: will suppress ACTH secretion => b/l adrenal atrophy (both adrenals will be small)
(b) Primary adrenal adenoma- one will be large, while the other will atrophy due to negative suppression on ACTH
(c,d) B/l enlargement due to ACTH stimulation
52
What indicates prophylactic thyroidectomy?
Prophylactic thyroidectomy indicated if RET protooncogene mutated is discovered
ex: FHx suggests MEN2A or MEN2B so do genetic testing and pt tests positive for RET mutation => prophylactically remove thyroid b/c almost 100% chance of medullary thyroid cancer
53
MC cardiac chamber damaged by MI
LV
B/c the MC major arteries involved (LAD, then RCA, then LCx) all supply walls of the left ventricle
54
MC cause of Cushing's syndrome
MC cause of Cushing's syndrome = exogenous steroid use
55
Hyperplastic arteriolosclerosis
(a) Mechanism
(b) Histologic hallmark
(c) Etiology
Hyperplastic arteriolosclerosis
(a) Smooth muscle hyplerplasia in small BV wall
(b) Histo: onion skin appearance due to concentric layers of smooth mucsle
(c) Etiology = malignant HTN
- smooth muscle becomes hyperplastic to accommodate stress of malignant HTN on BV wall
56
3 activities of PTH
(a) Main mechanism for free Ca2+ increase
PTH works to increase free (ionized, not bound to albumin or phosphorus) calcium
- increases bone resorption
- increases intestinal absorption of both Ca and PO4 (by increasing active vitamin D)
- increases renal resorption of Ca2+ and excretion of PO4
(a) Only one of the above that doesn't also increase phosphate (which will bind and therefore decrease free ionzied Ca2+) is activity at the kidney
- PTH as 'phosphate trashing hormone'
57
Different Dubin-Johnson and Rotor's syndrome
Both are congenital deficiency in bilirubin canilucli transport protein => bilirubin can be conjugated but gets trapped in the liver
Both are clinically insignificant, but Dubin-Johnson has a grossly black appearance of the liver, while in Rotor's the liver lacks discoloration
58
Basal cell carcinoma- upper or lower lip?
BCC- upper lip
SCC- lower lip
'BS'
59
Key clinical features of hypocalcemia
(a) Early sign
(b) Later sign
Hypocalcemia
(a) Perioral numbness and tingling
(b) Tetany/muscle spasms- Chvosket's sign (facial twitching when tap facial nerve) and Trousseau (BP cuff)
60
Mechanism by which obesity increases risk of DM II
Obesity downregulates insulin receptors on skeletal muscle and fat tissue
= mechanism of resistance to insulin seen in obesity
61
Tx of Kaposi Sarcoma in 80 yo HIV- E. European male vs. 30 yo HIV+ male
Kaposi sarcoma (low grade malignant proliferation of endothelial cells 2/2 HHV-8 infection) tx:
HIV- elderly white male: tx w/ surgical removal
HIV+ pt: tx the HIV w/ antiretrovirals
62
Name the thyroid cancers in descending order of prevalence
MC (80%) Papillary carcinoma --> Follicular carcinoma --> Medullary carcinoma (C-cells) --> Anaplastic carcinoma
63
Explain the K+ status of the body and blood in DKA
DKA: serum hyperkalemia w/ total body low K+
-no insulin (K+ pushed into cells by insulin)
-elevated H+ (b/c of ketones): H+ pushed into cells in exchange fro K+ out
Then hyperkalemia in blood causes high urinary excretion of K+
-so body down of total K+ despite high serum K+
64
Percent of atherosclerotic stenosis before complications arise
70%
65
Size of adrenal glands in 21-hydroxylase
W/o 21-hydroxylase pt can't synthesize cortisol => elevated ACTH which causes hyperplasia of both adrenal glands
(hence congenital adrenal hyperplasia, talking about b/l hyperplasia)
66
Differentiate location of the two types of large vessel vasculitides
Large vessel vasculitides:
1. Temporal giant cell arteritis = major branches of the carotid artery => ocular symptoms, HA
2. Takayasu's is more distal in the aorta or aorta's major branches => pulselessness of upper extremities
67
MC complication of thoracic aneurysm
Aortic insufficiency
Thoracic aneurysm causes aortic root dilation => AI
68
Locate mass if thyroid fails to descend properly during development
Thyroid starts at the base of the tongue, so if fails to descend child may have mass on base of tongue
69
Explain the physiology of the following signs of Cushing syndrome
(a) HTN
(b) Immunosuppression
Cushing Syndrome = hypercortisol
(a) Cortisol upregulates alpha1 receptors on arterioles => cells more sensitive to norepi stimulation
- cortisol upregulates alpha1 receptors to increased arteriole susceptibility to norepi
(b) Immunosuppression b/c cortisol inhibits phospholipase A, release of IL-2, and release of histamine from mast cells needed for vascular permeability seen in inflammation
70
2 states associated w/ nonbacterial thrombotic endocarditis
Nonbacterial thrombotic endocarditis = sterile vegetations associated w/
- hypercoagulable state
- underlying adenocarcinoma
71
Conjugated or unconjugated hyperbilirubinemia
(a) Extravascular hemolysis
(b) Gilbert
(c) Dubin-Johnson
(d) Rotors
(e) Crigler-Najjer
CB or UCB
(a) Extravascular hemolysis: UCB produced overwhelms liver's conjugating abilities => UCB elevated
(b) Gilbert = congenital mildly low UGT activity => UCB
(c,d) Both Dubin-Johnson and Rotors are deficiencies in the bilirubin caniliculi transport protein => conjugated hyperbilirubin
(e) Crigler-Najjar = congenital absence of UGT activity => fatal UCB 2/2 kernicterus
72
Why is Waterson-Friedrichson syndrome fatal?
B/c cortisol is necessary for life, cortisol needed to maintain vascular tone to maintain BP
73
Differentiate presentation of growth hormone adenoma in children vs. adults
Growth hormone adenoma (excess GH) =>
- gigantism in children: growth plates aren't fused so you get excess linear growth
- acromegaly: growth plates fused, so get enlargement of facial features of visceral organs (actually cardiomegaly is MC cause of death)
74
Palpating neck mass as "hard as wood"
Dx
Buzzword for "hard as wood neck" mass = Reidel Fibrosing thyroiditis = nontender fibrosis of thyroid gland that can extend to local structures (ex: airway) in young females
Other thyroid mass that can extend to local structures is anaplastic carcinoma, but this is seen in elderly
75
Classic EKG finding of restrictive cardiomyopathy
Classically low-voltage, QRS w/ low-amplitude
76
Classic example of molecular mimicry causing heart disease
In ARF: M-protein of strep pyogenes (GAS) looks similar to some self-antigen
So the body makes antibodies against group A strep's M-protein that cross-react w/ self antigens
77
Kawasaki disease
(a) Typical pt
(b) Features
(c) MC vessel involved
Kawasaki disease (think of kid on Kawasaki motorcycle)
(a) 4 yo Asian child
(b) Features: 5+ days of fever, limbic-sparing conjunctivitis, rash on hands and feet (hence motorcycle), cervical lymphadenopathy
(c) MC vessel involved = coronary vessels
- hence why this is so scary, these 4 yo kids can have heart attacks and coronary aneurysms
78
Differentiate the type of LVH seen as a complication from AS vs. AI
LVH can be concentric (sarcomeres added in parallel: wall thickens, lumen narrows) or eccentric (sarcomeres added in series, ventricle/lumen dilates w/ relative wall thinning)
-concentric dilation 2/2 pressure overload, eccentric dilation 2/2 volume
So AS increases pressure => concentric hypertrophy
AI increases volume => ieccentric hypertrophy
79
Common endocrine complication of growth hormone adenoma
Secondary diabetes mellitus
B/c growth hormone inhibits glucose uptake by cells
80
How to differentiate follicular adenoma vs. carcinoma
Need frozen section or at least to visualize entire capsule (so can't differentiate from FNA alone)
Follicular adenoma: follicular hyperplasia is contained within a capsule clearly demarcating tumor from rest of the gland
Follicular carcinoma: fibrous capsule present but cancer spreads beyond the capsule
81
How to differentiate physiologic neonatal jaundice from jaundice 2/2 biliary atresia
Physiologic neonatal jaundice is unconjungated hyperbilirubinemia (b/c the UGT enzyme in the liver to conjugate bilirubin is immature)
While biliary atresia is a failure of the extrahepatic biliary tree to canulize => conjugated bilirubin can't get out of liver
82
Tx of SIADH
Tx of SIADH (so much ADH that you hold onto all free water)
- free water restriction
- Demeclocycline = abx that also blocks collecting duct response to ADH
83
In addition to directly measuring growth hormone levels, name 2 ways to diagnose a growth hormone adenoma
Diagnose excess GH by:
- oral glucose load fails to suppress GH
- elevated IGF-1 (insulin-like growth factor 1) whose production by the liver is stimulated by GH
84
Name the two actions of angiotensin II
Angiotensin II (activated by renin) does 2 things to increase BP
1. Stimulates distal convoluted tubule to reabsorb more Na+ (then water follows to increase intravascular volume)
2. Directly causes arteriolar vasoconstriction to increase peripheral vascular resistance
85
Explain how chronic HTN increases risk of aortic dissection
Chronic HTN => hyaline arteriolosclerosis of the vaso vasorum (small BVs that supply the outer wall of large BVs)
So the adventitia of the aorta gets decreased blood flow => weakening of aorta wall => increased risk of dissection
86
MC primary cardiac tumor in
(a) children
(b) adults
MC primary cardiac tumor in
-both are still benign, MC overall is mets
(a) Children = rhabdomyoma
(b) Adults = myoxoma
87
What would the main histologic feature of myocytes be during unstable angina?
Unstable angina = reversible damage (ischemia not infarction) which is characterized by edema or swelling
88
Differentiate the blisters seen in pemphigus vulgaris and bullous pemphigoid
Both are autoimmune diseases
Pemphigus vulgaris = AI where IgG are against desmosomes (hold together keratinocytes of stratum spinosum)
-get separation of stratum spinosum and basal layer => very thin top layer => blisters easily pop
Bullous pemphigoid = AI where IgG are against hemidesmosomes (hold basal cells down to the basement membrane)
89
Explain the following findings in DM I
(a) Low muscle mass
(b) Increased lipolysis that can lead to DKA
DM type I
(a) Low muscle mass b/c unopposed glucagon stimulates gluconeogenesis that uses up amino acid precursors
(b) DKA 2/2 stress (ex: infection) that increases epinephrine, epinephrine stimulates lipolysis that increases free fatty acids that are converted to ketones in the liver
90
Why is dark urine a sign of extravascular hemolysis
Dark urine is due to excess urobilinogen (NOT CB or UCB) in the urine
-CB gets excreted into intestinal lumen, converted into urobilinogen by intestinal flora, reabsorbed into blood stream then filtered by kidneys
91
Lab values in pseudohypoparathyroidism (PTH, Ca2+)
(a) Mutation in what protein?
Pseudohyperparathyroidism = elevated PTH but hypocalcemia due to pierpheral resistant to PTH
(a) Defect in Gs protein that is coupled to PTH receptor => PTH receptors don't activate downstream effect in end-organ (renal tubular cells, intestinal mucosa, osteclasts/blasts)
92
Conjugated or unconjugated hyperbilirubinemia
(a) Defective RBC formation
(b) Physiologic jaundice
CB or UCB?
(a) Defective RBC formation: too much UCB overwhelms the conjugating ability of the liver => unconjugated hyperbilirubinemia
(b) Physiologic jaundice: normal supply of UCB but UGT enzyme in liver is immature/underactive => unconjugated hyperbilirubinemia
93
Where is melanin made?
Melanin is made in melanosomes (vesicles), by conversion of tyrosine --> melanin
-then these melanosomes are moved upwards throughout keratinocytes
94
Clinical features of aortic regurgitation
AR causes hyperdynamic circulation => bounding pulses, pulsating nail beds, head bobbing
95
If UV light is so dangerous, why would UV light be used in tx of psoriasis
Psoriasis- exact mechanism unknown but thought to be autoimmune and is 2/2 excessive keratinocyte proliferation
Use UVA (not as carcinogenic as UVB) light to damage the keratinocytes to stop proliferation
96
Mechanism by which excess thyroid hormone
(a) Increases basal metabolic rate
(b) Stimulates sympathetic nervous system
Thyroid hormone
(a) Increases BMR by upregulating synthesis of NaK ATPase
(b) Upregulating beta-1 receptors to increase sympathetic nervous system activity
97
Location of cells in the islets of Langerhans that secrete insulin
Beta cells in the center of the islets secrete insulin
98
Differentiate prototypical murmur of acute vs. chronic rheumatic heart disease
MR first b/c of vegetations
then MS w/ scarring as repetitive attacks of acute disease cause chronic disease to scar the mitral valve
99
Buerger Disease
(a) Explain the autoamputation of digits
(b) Association w/ Raynaud's phenomenon
(c) Tx
Buerger disease = medium vessel vasculitis seen in smokers
(a) Basically vasculitis of small vessels leading to fingers and toes => autoamputation
(b) Raynaud's = vasospasms of peripheral arterioles, so associated w/ cutoff supply to fingers and toes
(c) Tx = smoking cessation
- can't grow a finger back...but can prevent from losing more
100
What are 'heart failure cells'?
HF cells = hemosiderin-laiden macrophages in the alveoli
High pressure in the pulmonic circulation (from backup from L heart) causes overdistended pulmonary capillaries to rupture => macrophages eat up the iron from dead cells
101
32 yo F, BMI 22 p/w severe HTN 180/120
First thought for dx?
Young F w/o risk factors w/ severe HTN- think fibromuscular dysplasia!!
Secondary HTN due to irregular thickening of the renal artery
102
Diagnostic test for central DI
(a) Drug of choice for tx of central DI
Dx central DI w/ water deprivation test- in central DI watre deprivation doesn't increase urine osmoles (b/c pt can't concentrate urine)
(a) Desmopressin = ADH analogue
103
Name some causes of restrictive cardiomyopathy
Restrictive cardiomyopathy: filling (diastolic) defect that progresses to CHF
- amyloidosis: light chain deposition
- sarcoidosis: granuloma deposition
- hemochromatosis: Fe deposits
104
Cause of AAA
AAA is due to atherosclerosis
Atherosclerotic plaques block blood supply to the aorta wall => wall weakens
105
(a) Cause of SCD in HOCM
| (b) Explain why pts w/ HOCM syncopize w/ exercise
(a) Ventricular arrhythmia
- athlete just suddenly drops dead
(b) B/c LVOT is obstructed due to such hypertrophy of the LV, so can't increase supply during high-demand states (exercise)
106
Ddx for hardened thyroid gland due to fibrosis of thyroid and local structures
(a) How to distinguish the two
Extensive fibrosis of thyroid gland that spreads to local structures (ex: airway, esophagus) includes anaplastic carcinoma (poorly differentiated carcioma) vs. Reidel fibrosing thyroiditis
(a) Reidel fibrosing thyroiditis seen in young women, anaplastic carcinoma in elderly
107
Hyaline arteriolosclerosis
(a) Mechanism
(b) Histologic hallmark
(c) Etiology
Hyaline arteriolosclerosis
(a) Hyaline (protein) deposition in wall of arterioles
(b) Pink on histo
(c) 2 etiologies:
1. chronic benign HTN: constantly high BP forces proteins out of BV wall
2. diabetes: nonenzymatic glycosylation weakens the basement membranes so proteins leak out
108
Name the 2 major mechanisms by which type II diabetes causes complications
DM II complications 2 major mechanisms = nonenzymatic glycosylation and osmotic damage
1. Non-enzyme mediated adding of glucose to things: such as BV walls (aterhoscerlosis, hylaine arteriolosclerosis) and Hb
2. Osmotic damage: glucose --> sorbitol by aldose reductase
- cells that can take up glucose independent of insulin
109
Name the MC organs/systems involved in the three small vessel vasculitides
(a) Wegener's granulomatosis
(b) Microscopic polyangiitis
(c) Churg-Strauss
Systems involved
(a) 'Wec-eners': nasopharynx, lungs, kidneys
(b) MIcroscopic polyangiitis: lungs and kidneys
- no nasopharynx and no granulomas
(c) Churg-Strauss: lungs and heart
- peripheral eosinophilia
110
Cholesterol and serum glucose levels seen in hyper vs. hypothyroid
Hyperthyroidism => hypocholesterolemia and hyperglycemia
-thyroid hormone stimulates both gluconeogenesis and glycogenolysis
Hypothyroidism => hypercholesterolemia and hypoglycemia
111
What causes hyperpigmentation in a freckle?
Hyperpigmentaiton in a freckle is due to increased number of melanosomes (NOT melanocytes)
112
MC cause of chronic pancreatitis in
(a) Adults
(b) Children
Chronic pancreatitis MC etiology in
(a) Adults = EtOH
(b) Children = CF
- excretions are so thick they clog up ducts
113
Sodium status and serum osmolality seen in
(a) Central DI
(b) SIADH
(a) Central diabetes insipidus = problem in either hypothalamus or posterior pituitary causing low ADH production/secretion
Low ADH = low water reabsorption => hypernatremia and high serum osmoles b/c just losing tons of free water
(b) SIADH = tons of ADH around => reabsorbing all free water so hyponatremia and low serum osmolarity
114
First line medical treatment of growth hormone adenoma
Octreotide = somatostatin analogue
| blocks response of anterior pituitary to GHRH
115
What type of hypersensitivity is DM I?
(a) Histologic finding of DM I
DM I is a type IV hypersensitivity (delayed cell response, not antibody mediated)
-there can be anti-insulin antibodies present way before clinical symptoms but mainly cell mediated
(a) B/c cell mediated inflammation => histologically classically see islet inflammation
116
Differentiate angiosarcoma from Kaposi sarcoma
(a) Aggressiveness
(b) Associated exposure/condition
Both are cancer of blood vessels
Angiosarcoma
(a) Malignant, aggressive proliferation of endothelial cells
(b) Associated w/ PVC or arsenic exposure
Kaposi sarcoma
(a) Low-grade malignant proliferation of endothelial cells
(b) HHV-8 infection
117
Does type I or type II diabetes have a higher genetic component?
Type II diabetes has a higher genetic component!!! (not type I...)
118
Limitation of thyroid FNA
Thyroid FNA gives a sampling of cells, not a cross section (like from a frozen section). Do FNA of the thyroid b/c it's highly vascularized and don't want bleeding in the neck...
Limitation of FNA is that it can't differentiate follicular adenoma from follicular carcinoma b/c you're not visualizing the capsule (therefore if you get follicular cells you dk if they are confined by fibrous capsule or spread out of capsule)
119
Name the 3 categories of arteriosclerosis
Arteriosclerosis = hardening of arterial walls
1. Atherosclerosis = thickening of intima of medium and large vessels w/ cholesterol plaques (necrotic lipid core w/ fibromuscular cap)
2. Arteriolosclerosis = narrowing of the lumen in small arterioles
- divided into hyaline (protein deposition) and hyperplastic (smooth muscle proliferation)
3. Monckeberg medial calcific stenosis- clinically insignificant nonobstructive calcification of the media
120
Lab values to differentiate primary and secondary hyperparathryoidism
Primary hyperparathyroidism: elevated PTH, elevated Ca2+
Secondary hyperparathyroidism (ex: chronic renal failure): low serum Ca2+ causing high PTH
121
RF for adult development of coarctation of the aorta
(a) Clinical presentation
Bicuspid aortic valve increases risk of adult-type coarctation of the aorta
(a) HTN in upper extremities (proximal to narrowing) w/ hypotension and diminished/weak pulses in lower extremities (distal to narrowing)
122
Key difference to distinguish congenital adrenal hyperplasia due to 21-hydroxylase vs. 11-hydroxylase deficiency
Salt wasting
11-hydroxylase deficiency a weak mineralocorticoid can still be produced so these ppl aren't salt wasters
123
2 ways to differentiate aortic stenosis 2/2 age/wear and tear from acute rheumatic fever
Aortic stenosis
If from ARF: will always be w/ concomitant mitral stenosis, also will have fusion of the commisures
If no concomitant MS and commisures of the valves are not fused = wear and tear calcific age-related AS
124
MC cancer that metastasizes to b/l adrenal glands
Lung cancer!!!
Weird but lung cancer loves the adrenal glands (no idea why???)
125
Explain how chronic mitral stenosis can result in AFib
Chronic mitral stenosis = increased pressure on the left atrium against the stenotic valve => LA dilation
LA dilation, dilates conduction system => increased risk AFib = increased risk clot
126
Explain the following findings of DKA
(a) Kussmaul respirations
(b) Dehydration
(c) Fruity smelling breath
DKA
(a) Kussmaul respirations in attempt of respiratory compensation for the metabolic compensation
(b) Dehydration b/c of osmotic diuresis from such high serum glucose
- glucose excreted in urine and pulls out water with it
(c) Fruity smelling breath 2/2 ketones (specifically acetone)
127
Key for tx of hemangioma
Hemangioma = benign tumor of blood vessels found usually at birth, and often self-resolve
So key here is to not surgically remove and give kid a scar when it should resolve on its own
128
Explain how conjugated bilirubin gives color to both stool and urine
Unconjugated bilirubin bound to albumin to travel to the liver, then conjugated in the liver
In the liver gets put into bile, excreted into intestinal lumen where intestinal flora converts CB into urobilinogen
Urobilinogen then gives stool its color, also some gets reabsorbed by intestines into blood then filtered by kidneys, making urine yellow
129
Melanocytes
(a) What layer of epidermis?
(b) Derived from where embryologically?
Melanocytes
(a) In the basal layer of the epidermis- so sit down on the bottom w/ the stem cells
(b) Derived from neural crest
130
Differentiate EKG findings of unstable angina and Prinzmetal angina
Unstable angina- endocardium is the farthest from the blood supply so the most susceptible to ischemia. Subendocardial ischemia => ST depressions
Prinzmetal angina completely blocks of blood flow => transmural ischemia => ST elevations
131
Conjugated or unconjugated hyperbilirubinemia
(a) Biliary tract obstruction
(b) Viral hepatitis
CB or UCB
(a) Biliary tract obstruction = conjugated hyperbilirubinemia- b/c nothing wrong w/ the hepatocyte's ability to conjugate, but CB can't leave
(b) Viral hepatitis causes elevation in both CB and UCB
- b/c inflammation affects the hepatocytes (impaired conjugating ability) and the small bile ductules (impairs leaving liver)
132
Pt who develops episodic hypertension and palpitations w/ urination
Think extra-adrenal pheochromocytoma in the bladder wall (LOLZ I'd never get that)
133
Etiology when endocarditis has vegetations on both the surface and undersurface of the valve
Vegetations on both sides- think SLE-induced endocarditis = Libman-Sacks Endocarditis
-sterile vegetations on both sides of the mitral valve (surface and undersurface) causing mitral regurg
134
Differentiate vitiligo and albinism
Both are pigmentation d/o
Vitiligo = AI destruction of melanocytes
Albinism = congenital enzymatic defect (usually in tyrosinase) that prevents melanin production
135
MC viral cause of dilated cardiomyopathy
(a) Other causes of dilated cardiomyopathy
Coxsackie virus => dilated cardiomyopathy, this can be lethal!
(a) Dilated cardiomyopathy can also be caused by: pregnancy, cocaine, Doxyrubicin (chemo agent), EtOH abuse
136
Kawasaki disease treatment- 2 tx
Kawasaki = medium vessel vasculitis in 4yo Asian kids, tx
1. IVIG
2. ASA
- ironic b/c you usually don't give aspirin to kids w/ viral illness (but this isn't a viral illness, it just looks like one!) to prevent Reye syndrome (encephalopathy and liver stuff)
- but here give ASA to inhibit plts and prevent thrombosis => MI
137
How to differentiate etiology of Cushings syndrome when b/l adrenal glands are enlarged
B/l adrenal gland enlargement occurs due to ACTH-secreting pituitary adenoma or ectopic ACTH production
-while NOT from exogenous corticosteroids or adrenal adenoma
Differentiate top 2 thru dexamethasone (cortisol analog) suppression test: give dexa
- dexa suppresses ACTH release from pituitary (measured cortisol will decreased)
- dexa will not suppress ACTH released from ectopic source (ex: small cell lung carcinoma) => measured cortisol will not be suppressed
138
Why type of hypersensitivity rxn is
(a) Atopic dermatitis
(b) Contact dermatitis
Hypersensitivity rxn type
(a) Type I = atopic dermatitis = eczema
- associated w/ allergy and asthma
- preformed antibodies
(b) Contact dermatitis = type IV HSR
- delayed cellular
139
Consequences of hyperparathyroidism
(a) Kidney
(b) Urine
(c) Bowel activity
(d) Bones
Consequences of hyper-PTH = Hypercalcemia
(a) Nephrocalcinosis = metastatic calcification, deposition of excess Ca2+ in renal tubules
(b) Nephrolithliasis- excess Ca2+ in urine increases risk of supersaturation and calcium oxalate kidney stones
(c) Constipation
(d) Osteitis fibrosa cystica- bone fibrosis from so much breakdown
140
Which of the three small-vessel vasculitides
(a) C-ANCA
(b) not granulomatous
(c) pts have h/o asthma and peripheral eosinophilia
Small-vessel vasculitides
(a) C-ANCA = Wegeners (think Wec-eners)
- cANCA meaning the antinuclear cytoplasmic antibodies are in the periphery of the cytoplasm, while pANCA have ANCA peri-nuclear
(b) No granulomas = Microscopic Polyangiitis
- p-ANCA
(c) H/o asthma and peripheral eosinophilia = Churg Strauss syndrome
- p-ANCA
- has granulomas
141
MC cause of primary and secondary hyperparathyroidism
Primary hyperparathyroidism: 80% due to parathyoid adenoma (benign)
-other causes: hyperplasia and carcinoma (less common)
MC cause of secondary hyperparathyroidism is chronic renal disease
-kidney can't trash phosphate => elevated serum phosp binds free Ca2+ so you get low Ca2+ w/ high PTH
142
Genetic cause of HOCM
HOCM is due to a genetic mutation in sarcomere proteins, often autosomal dominant
- basically dilation of the LVOT
- wall is so noncompliant, diastolic dysfunciton
143
Explain CRF vs. ARF caused by the two types of arteriolosclerosis
Arteriolosclerosis (thickening of arteriolar wall => narrowing of arteriolar lumen) types:
1. hyaline arteriolosclerosis (protein deposition): protein deposition in afferent arterioles gradually reduces GFR => glomerular scarring => CRF
2. hyperplastic arteriolosclerosis (smooth muscle hypertrophy): in response to malignant HTN causes ARF w/ flea-bitten appearance of kidney
- flea bitten appearance = pinpoint hemorrhages on kidney surface from blown out BV from the malignant HTN
144
BMP abnormalities seen in hyperaldosteronism
Aldo stimulates alpha-intercalated and parietal cell activity in the collecting duct
- alpha-intercalated cells => increased H+ excretion => metabolic alkalosis
- parietal cells => increased Na+ resorption w/ increased K+ excretion => hypernatremia (w/ concomitant hypertension b/c of high volume) and hypokalemia
145
What kind of pt do you want to suspect gallbladder adenocarcinoma in?
Suspect GB adenocarcinoma when an elderly women presents w/ cholecystitis
-b/c the typical demographic is women in 40s not elderly
146
Complication of neonatal jaundice (why do we treat it if it's physiologic...?)
Kernicterus = CNS complications when unconjugated bilirubin (fat soluble) deposits in the basal ganglia
=> treat w/ phototherapy which doesn't conjugate the bilirubin, but makes unconjugated bilirubin water soluble (so can be excreted in urine)
147
Seborrheic keratosis vs. actinic keratosis
Seborrheic keratosis = benign, 'stuck on' dark plaque of pseudocysts
Actinic keratosis = dysplasia of squamous cells, precursor to SCC
-hyperkeratotic scaly plaque
148
25 yo F s/p viral URI presents w/ tender neck mass and palpitations
(a) Dx?
(b) Tx
(a) Subacute (deQuervain) granulomatous thyroiditis = granulomatous inflammation of the thyroid gland following viral infection that causes transient hyperthyroidism w/ tender thyroid
- key here is that the thyroid is tender (while nontender in Reidel's thyroiditis)
(b) No tx needed, self-limited, doesn't progress to hypothyroidism
149
2 key places where myxedema occurs in hypothyroidism
Myxedema (swelling 2/2 increased CT deposition) classically occurs in the tongue and larynx
Tongue => glossitis
Larynx => deepening of voice
150
Name some etiologies of SIADH
SIADH
- ectopic ADH production from small cell lung carcinoma
- lung disease: COPD, lung infection
- drugs: cyclophosphamide
- CNS trauma
151
Top cause of mortality from acute rheumatic fever
In acute rheumatic fever, if the pt dies it will be from myocarditis
152
Another name for hypothyroidism in
(a) Neonates
(b) Children/Adults
Hypothyroidism
(a) In neonates (congenitally) = Cretinism
(b) Children/Adults = Myxedema
153
Explain how acne vulgaris is
(a) Hormonally reactive
(b) Sensitive to anti-bacterials
Acne vulgaris
(a) Hormones stimulate sebacous gland oversecretion, increase in sebum blocks the hair follicle creating an itus for infection
(b) This infxn is by P. acnes bacteria => susceptible to benzoyl peroxide
154
Histo report of FNA shows malignant cells in an amyloid stroma
Dx
Dx = medullary carcinoma (3rd MC thyroid malignancy)- only thyroid malignancy of the parafollicular (C) cells that produce calcitonin
-excess calcitonin deposits in the gland as amyloid
155
Cutoff measurement for aortic stenosis
Normal diameter of the aortic valve is greater than 4cm2
Aortic stenosis defined when diameter is under 1cm2
156
How to differentiate primary vs. secondary hyperaldosteronism
Renin level
Primary hyperaldo: high aldo w/ low renin
Secondary hyperaldo: high aldo 2/2 high renin (ex: renal artery stenosis)
157
Differentiate the two subtypes of arteriolosclerosis by
(a) Main histologic feature
(b) Etiology
(c) Effect on kidney
Arteriolosclerosis (narrowing of lumen of small arterioles) divided into
1. Hyaline arteriolosclerosis
(a) Hyaline (pink) protein deposition in BV wall
(b) Chronic benign HTN and diabetes
(c) Responsible for glomerular scarring/CRF in HTN and DM
2. Hyperplastic arteriolosclerosis
(a) Onion skinning appearance due to hyperplasia of smooth muscle
(b) Malignant HTN
(c) ARF
158
Classic clinical implication of hyaline arteriolosclerosis
Hyaline arteriolosclerosis: classically you get protein deposition in afferent arteriole, narrowing the inflow and reducing GFR. Reduced GFR over time => glomerular scarring = arteriolonephrosclerosis
This mechanism explains CRF in chronic HTN and diabetes
159
Why is Crohn's disease a RF for gallstones
Crohns disease most commonly involves the terminal ileum, the ileum is where bile salts are reabsorbed
Bile salts help solublize cholesterol in bile => w/ less bile salts it's easier for cholesterol to precipitate out into stones
160
Differentiate etiology of medial vs. lateral neck mass in child 2/2 embryologic remnant
Child w/
- medial neck mass = thyroglossal duct cyst from remnant of the thyroglossal duct formed as thyroid descends from tongue down into neck
- lateral neck mass = brachial cleft cyst from failure of obliteration of the 2nd brachial pouch
161
Name some other causes of acute pancreatitis besides EtOH and gallstones
Additional (less common) causes of acute pancreatitis (account for overall 20% of cases)
- trauma in children, like MVA where seatbelt crushes pancreas
- hypercalcemia: calcium activates enzymes
- hyperlipidemia (dk why who cares)
162
Virus responsible for
(a) Verruca
(b) Molluscum contagiosum
(a) Verruca = wart = HPV virus
| (b) Molluscum contagiosum = round, firm painless bumbs from infxn w/ pox virus
163
MC cause of adrenal insufficiency in developed vs. underdeveloped countries
Adrenal insufficiency
Autoimmune (Addison's disease) MC in developed
Underdeveloped- Tb is the MC cause
164
Role of phenoxybenzamine before endocrine surgery
Phenoxybenzamine = irreversible alpha blocker
Give before removing pheochromocytoma to avoid lethal HTN intra-op if tons of catecholamines get into circulation when tumor is removed
By blocking alpha receptors, impede Norepi ability to skyrocket BP
165
2 types of gallstones
(a) MC?
(b) Which is easier to see on Xray?
Gallstones = precipitation of either cholesterol or bilirubin
-can be due to supersaturation, obstruction of flow, or reduced things to solubilize components (ex: need bile acids to solubilize cholesterol)
(a) MC = cholesterol gallstones
(b) Cholesterol stones are radiolucent (can't see on Xray) while bilirubin stones (also called pigmented stones) are radiopaque (visible on Xray)
