Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Endocrinology > Lipids/Obesity > Flashcards

Lipids/Obesity Flashcards

1
Q

Familial Chylomicronemia:

  1. due to pathogenic variants in?
  2. clinical features
  3. laboratory findings?
  4. main treatment?
A
  1. LIPOPROTEIN LIPASE (LPL), apolipoprotein CII, apolipoprotein AV, lipase maturation factor 1, and glycosylphosphatidylinositol anchored HDL binding protein
  2. eruptive xanthomas, hepatosplenomegaly, lipemia retinalis. Increased lifetime risk of recurrent pancreatitis.
  3. TG > 1000 mg/dl and milky appearance of plasma
  4. restrict dietary fat to < 10-30 g daily. Fibrates, omega 3- FAs, or statins do not work that well
eruptive xanthomas
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Familial Hypercholesterolemia

  1. due to pathogenic variants in?
  2. clinical features
  3. laboratory findings?
  4. main treatment?
A
  1. LDL receptor (most common), ApoB100 (defective), PCSK9
  2. premature CAD, tendon xanthomas (on extensor tendons of the hands or achilles). Also arcus cornealis.
  3. high LDL >190
  4. high intensity statins/ezetimibe/PCSK9i’s
tendon xanthoma
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

(Familial) Dysbetalipoproteinemia

  1. due to pathogenic variants in?
  2. clinical features
  3. laboratory findings?
  4. main treatment?
A
  1. Apo E (E2/E2)
  2. palmar xanthomas, orange discoloration of the skin creases, tuberoeruptive xanthomas on elbows and knees; premature CAD
  3. high VLDL and IDL –> high total cholesterol and TG in a 2:1 ratio
  4. high intensity statin and fibrates
palmar xanthomas
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

% category for ASCVD risks:

  1. low
  2. borderline
  3. intermediate
  4. high
A
  1. < 5%
  2. 5-7.5%
  3. 7.5-20%
  4. > 20%
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Woman who desires pregnancy and is considering bariatric surgery, but has a h/o GERD/Barrett esophagus. What procedures are contraindicated?

A

sleeve gastrectomy and gastric banding

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is an adverse effect of bile-acid sequestrant, colesevelam?

A

Hypertriglyceridemia

Therefore, avoid using in patients with at least moderate hyperTG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q
  1. Indications for starting a PCSK9 inhibitor (-rocumab)?
  2. MOA?
  3. % LDL reduction?
A
  1. very high risk (h/o multiple major ASCVD events) OR 1 major event + multiple high-risk conditions, whose LDL is still 70 mg/dl or higher on maximally tolerated statin and ezetimibe therapy.
  2. MOA: bind to liver PCSK9 serine protease and prevent LDL-Receptor degradation thus lowering LDL levels in the blood
  3. up to 60% from baseline w/ or w/o statin
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what is the most common lipid abnormality among patients with coronary artery disease?

A

Familial combined hyperlipidemia

  • 1 of 3 lipid abnormalities: hypercholesterolemia, hypertriglyceridemia, or both
  • due to overproduction of ApoB
  • also associated with insulin resistance and metabolic syndrome
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Hypobetalipoproteinemia:

  1. Etiology
  2. complications?
  3. how is abetalipoproteinemia different?
A
  1. defect in liver production of apo-B containing lipoproteins due to defective apoB production or microsomal triglyceride transfer protein. Decreased LDL (cholesterol) and VLDL (triglyceride).
  2. inability to efficiently secrete lipoproteins from the liver can lead to NAFLD
  3. LDL is much lower and patients develop deficiencies of fat-soluble vitamins. FTT, steatorrhea, neurologic sx’s; weakness and balance problems as adults.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Eruptive xanthomas, characterized by yellow papules with surrounding erythema, are pathognomonic of?

A

hypertriglyceridemia, with a number of these patients also having a diagnosis of diabetes mellitus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

When is IV iron administration indicated after bariatric surgery?

A

When response to oral iron supplementation is poor or if a patient is symptomatic

(decreased iron absorption due to missing proximal duodenum)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is a common complication in a patient who has had sleeve gastrectomy?

A

GERD. Treat with high dose PPI.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Bempedoic acid
1. what is it?
2. MOA?
3. adverse effects?

A
  1. A non-statin LDL-lowering medication (new). It has been approved for use as an adjunct to diet in patients with atherosclerotic CVD or heterozygous familial hypercholesterolemia who, despite taking maximally tolerated statin dosages, do not achieve the desired LDL-cholesterol target concentration.
    -Also approved as monotherapy and as a fixed-dose combination with ezetimibe
  2. MOA: inhibits ATP citrate lyase (upstream of HMGCoA reductase).
  3. hyperuricemia, gout, thrombocytopenia, leukopenia, upper respiratory infections
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Are fenofibrate and icosapent ethyl LDL-cholesterol lowering agents?

A

NO.
They are TG-lowering agents.

In patients who had elevated TGs with CVD or diabetes with other risk factors, in addition to statin, high doses of icosapent ethyl reduced the primary endpoint (CV death, nonfatal MI, nonfatal stroke, coronary revascularization, unstable angina).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

According to the 2018 AHA/ACC guideline on the management of cholesterol, what are the 4 benefit groups in deciding whether a patient is a candidate for cholesterol-lowering medication?

A
  1. patients with clinical atherosclerotic cardiovascular disease
  2. patients with familial hypercholesterolemia
  3. adults with T1 or T2DM age 40-75 yo
  4. adults 40-75 yo without diabetes and LDL-cholesterol >/= 70 mg/dl to < 190 mg/dl depending on their 10-yr ASCVD risk –> risk stratification for this group is divided into: low (< 5%), borderline (5-7.5%), intermediate (7.5-20%), high (> 20%). LDL goal < 100 mg/dl.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Evolocumab
1. MOA?
2. approved for what use?

A
  1. a fully human monoclonal Ab against PCSK9. Blocking PCSK9 increases LDL receptor recycling leading to lowering of LDL cholesterol
  2. approved for use with diet + maximally tolerated statin in adults with familial hypercholesterolemia or with clinical ASCVD requiring additional cholesterol lowering
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Ezetimibe
1. MOA?
2. recommended dose is?
3. % LDL reduction

A
  1. a cholesterol absorption inhibitor. Inhibits the Niemann-Pick C1-like 1 protein. Used to lower LDL cholesterol.
  2. 10 mg daily
  3. up to 25% with statin
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Icosapent ethyl
1. MOA?
2. Approved for?

A
  1. unknown
  2. TG lowering in patients with hyperTG; AND for cardiovascular risk reduction in the setting of mild hyperTG (> 150 mg/dl) who are taking maximally tolerated statin + established CVD or T2DM with 2 or more risk factors for CVD.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Colesevelam
1. MOA?
2. approved for?
3. adverse effects?

A
  1. A bile acid sequestrant. Lowers LDL by decreasing the hepatic bile acid pool, which leads to an increase in hepatic bile acid synthesis from cholesterol
  2. decreasing LDL in conjunction with statins (lowers LDL up to 16%)
  3. constipation, dyspepsia, nausea, increasing TGs
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What are the contraindications for using statins?

A

decompensated cirrhosis and acute liver failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

renal dosing for
1. fenofibrate
2. gemfibrozil
3. MOA?

A
  1. fenofibrate: decrease dose when eGFR < 60
  2. gemfibrozil: decrease dose at CDK 4 or above
  3. lower TG by activating peroxisome proliferator-activated receptor alpha –> increase LPL activity –> TG catabolism
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Immunosuppressants and effect on cholesterol:
1. calcineurin inhibitors (cyclosporine > tacrolimus)
2. antimetabolites (azathioprine, mycophenolate sodium)
3. mTOR inhibitor (sirolimus)
4. corticosteroids

A
  1. increase LDL only (bind LDL receptor and increase activity of hepatic lipase, and decrease bile acid synthesis)
  2. no change
  3. TG (impairs LPL and increases VLDL secretion)
  4. LDL + TG
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

In consideration of bariatric surgery, in patients with GERD, what procedures should be avoided?

A

Sleeve gastrectomy and gastric banding

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

FDA approved weight loss medications:

Phentermine-Topiramate ER (Qsymia)

  1. MOA?
  2. Cautions and contraindications?
A
  1. Sympathomimetic/activates GABA receptors. Appetite suppressant.
  2. Phentermine = CVD, tachyarrhythmias, HTN
  3. Topiramate = glaucoma, kidney stones, acid-base disturbances. Risk of birth defects.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

List weight-promoting medications (9)

A
  1. Beta blockers
  2. insulin/insulin analogs
  3. insulin secretagogues (sulfonylurea, meglitinides)
  4. Thiazolidinediones
  5. Antihistamines (especially cetirizine)
  6. glucocorticoids
  7. antipsychotics (olanzapine, clozapine, risperidone)
  8. anticonvulsants (gabapentin, pregabalin, carbamazepine)
  9. SSRI paroxetine
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

List weight-neutral medications (10)

A
  1. ACEIs
  2. ARBs
  3. Calcium channel blockers
  4. metformin
  5. DPP4-inhibitors
  6. alpha-glucosidase inhibitors
  7. statins
  8. fibrates
  9. antidepressants
  10. Tricyclic antidepressants (amitriptyline, imipramine, clomipramine, doxepin) -wt neutral to wt loss
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

List-weight loss promoting medications (3)

A
  1. GLP1-RA
  2. SGLT2 inhibitors
  3. Anti-seizure, migraine meds (topiramate, zonisamide)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

FDA approved weight loss medications:

Orlistat (Xenical, Alli)

  1. MOA?
  2. Cautions and contraindications?
A
  1. Blocks intestinal fat absorption
  2. can impair absorption of fat soluble vitamins such as vitamin K which impacts warfarin dosing for anticoagulation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

FDA approved weight loss medications:

Liraglutide (Saxenda)

  1. MOA?
  2. Cautions and contraindications?
A
  1. GLP1 RA, appetite suppressant
  2. History of pancreatitis, medullary thyroid cancer. Gastroparesis. Gallstones.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

FDA approved weight loss medications:

Naltrexone-Bupropion ER (Contrave)

  1. MOA?
  2. Cautions and contraindications?
A
  1. NE/Dopamine re-uptake inhibitor, opioid receptor antagonist. Appetite suppressant.
  2. Concomitant opiate treatment. Seizure disorders = bupropion lowers seizure threshold. Contraindicated in HTN.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

FDA approved weight loss medications:

Phentermine monotherapy

  1. MOA?
  2. Cautions and contraindications?
A
  1. a sympathomimetic agent; suppresses appetite through its effects on the hypothalamus
  2. uncontrolled HTN, hyperthyroidism, CAD
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Sitosterolemia

  1. due to pathogenic variants in?
  2. clinical features
  3. laboratory findings?
  4. main treatment?
A
  1. A rare A.R. disorder due to increased absorption and decreased excretion of plant sterols, leading to high levels of sitosterol and campesterol. Pathogenic variant in a gene encoding either ATP-binding cassette subfamily G member 5 or 8.
  2. hematologic abnormalities: macrothrombocytopenia and stomatocytosis (erythrocytes with a central slit instead of a circular area of pallor). Premature atherosclerosis/CAD
  3. normal or high normal cholesterol
  4. avoid plant sterols, and give ezetimibe (blocks sterol absorption)
33
Q

Familial Combined Hyperlipidemia:

  1. due to pathogenic variants in?
  2. clinical features
  3. laboratory findings?
  4. main treatment?
A
  1. multifactorial
  2. the presence of multiple hyperlipidemias within a family, premature heart disease. Often associated with another comorbidity (insulin resistance and metabolic syndrome).
  3. hypercholesterolemia, hypertriglyceridemia, or mixed hyperlipidemia; also high ApoB (but not defective leading to LDL > 200 as in familial hypercholesterolemia)
  4. depends on the type of lipid disorder
34
Q

cerebrotendinous xanthomatosis

  1. pathogenesis?
  2. clinical features
  3. laboratory findings?
  4. main treatment?
A
  1. cholestanol deposition
  2. adult-onset progressive neurologic dysfunction (ataxia, dystonia, dementia, epilepsy, psychiatric disorders, peripheral neuropathy, myopathy), premature nonneurologic manifestations (tendon xanthomas, childhood-onset cataracts, diarrhea, premature atheroclerosis, osteoporosis, respiratory insufficiency)
35
Q

Conditions associated with tendinous xanthomas

A
  1. Familial hypercholesterolemia
  2. Cerebrotendinous xanthomatosis
  3. Familial defective Apo B (but NOT familial combined heterozygous hyperlipidemia)
  4. Sitosterolemia
36
Q
  1. HYPOalphalipoproteinemia is also known as?
  2. Findings?
A
  1. Apolipoproteinemia A1 deficiency
  2. very low HDL
37
Q

Current guidelines recommend screening for (what) in persons at very high cardiovascular risk and those with a history of premature cardiovascular disease, a first-degree relative with premature CVD or known (what) elevation, progressive cardiovascular disease despite maximal LDL-cholesterol lowering, or borderline 10-year CV risk with need for additional information to inform treatment decisions?

A

Lipoprotein A -

has been associated with very high CVD risk, as well as aortic stenosis.

Need aggressive lipid lowering

38
Q

How does hypothyroidism effect lipid levels?

A

Subclinical and overt hypothyroidism can increase LDL and total cholesterol by up to 30% (in overt).

39
Q

What did data reveal from RCTs regarding the effect of weight-loss interventions on mortality?

A

There is a mortality benefit resulting from weight loss through lifestyle intervention in people with PREdiabetes
(study is Diabetes Prevention Program and a study in China)

40
Q

what are the changes in energy expenditure that occur with weight loss?
1. ghrelin (hunger hormone)
2. GLP-1, CCK, peptide YY (satiety hormones)
3. total energy expenditure (resting metabolic rate, the thermic effect of feeding, energy expended in physical activity)

A
  1. increased
  2. decreased
  3. decreased (since it is linearly related to lean body mass). Decrease in resting energy expenditure has the greatest effect on weight regain if nutrient/energy intake is stable

increased appetite and weight regain are favored

41
Q

As adiposity increases, what happens to the following:
1. adiponectin
2. leptin
3. TNF alpha
4. resistin
5. plasminogen activator inhibitor-1

A
  1. decreases (adiponectin is suppressed by proinflammatory cytokine released by adipocytes)
  2. increases (it correlates with the amount of adipose tissue)
  3. increases (induces insulin resistance)
  4. increases (also causes insulin resistance)
  5. increases (a major risk factor for CVD)

adiponectin is insulin-sensitizing, anti-inflammatory, antiapoptotic.

42
Q

Patient with previously normal HDL now presenting with very low HDL should prompt evaluation for?

A

a malignancy (can be from assay interference)

In primary genetic HDL disorder (such as ApoA1 deficiency, Tangier disease, LCAT deficiency), HDL would not be previously normal

43
Q

Binge eating disorder
1. initial treatment?
2. tx for moderate-severe cases

A
  1. CBT + SSRIs (fluoxetine, sertraline)
  2. lisdexamfetamine
44
Q

Uncontrolled HTN is contraindicated in what weight loss medications?

A
  1. phentermine
  2. phentermine/topiramate
  3. naltrexone/bupropion
45
Q

Uncontrolled HTN is contraindicated in what weight loss medications?

A
  1. phentermine
  2. phentermine/topiramate
  3. naltrexone/bupropion
46
Q

Anticonvulsants associated with:
1. weight gain
2. weight loss
3. weight neutral

A
  1. valproic acid, the “gaba’s”
  2. topiramate, zonisamide
  3. levetiracetam, phenytoin, lamotrigine
47
Q

After sleeve gastrectomy, symptoms of:
1. dysphagia, nausea, emesis can be symptoms of?
Diagnostic test to order?
2. abdominal pain and fever

A
  1. gastric stenosis or severe GERD
    Order an upper GI series (will show stenosis if present). If negative, then order esophageal pH testing.
  2. concern for an abscess/leak (can occur days to weeks after surgery)
    Order a CT or US
48
Q

How do oral estrogens lead to TG elevations?

A

ApoB production is increased.
Estrogen stimulates hepatic TG synthesis and decreases hepatic lipase and LPL activity.
These effects are not seen with transdermal estrogen

49
Q

different types of dietary fats: best to worst for lipid profiles

A

polyunsaturated (soybean, safflower, sunflower, and corn oil) > monounsaturated (canola, olive, peanut, safflower, sunflower oil) > saturated (coconut oil, butter, lard palm oil, palm kernel oil, beef tallow)

poly and monounsaturated fats lower CVD risk

50
Q
  1. Apolipoprotein A1 is an important protein for?
  2. APO A1 deficiency leads to?
  3. Patients with apoA1 deficiency is also known as?
A
  1. HDL cholesterol
  2. very LOW HDL cholesterol (< 20 mg/dl) but NOT low LDL cholesterol or low TGs
  3. HYPOalphalipoproteinemia
51
Q

Describe the conditions that cause very low HDL cholesterol levels (3)

(Imagine a cat with cloudy fish eyes, reciting the ABC’s with orange tonsils after eating a tangerine, and holding an A because she’s number 1!)

A
  1. LCAT deficiency - Fish Eye Disease (clouded corneas)
  2. ABCA-1 deficiency - Tangier’s Disease (orange tonsils)
  3. Apolipoprotein A1 Deficiency - Structural lipoprotein on HDL particles –> INCREASED CVD RISK! SO, START STATIN FOR PRIMARY PREVENTION
52
Q

What are the three deficiencies/disorders that lead to increased HDL?

A
  1. cholesterol ester transfer protein (CETP) deficiency is most commonly seen in patients of Japanese ancestry.
  2. hepatic lipase deficiency = cholesterol and TGs are also increased. Patients also have premature CAD
  3. pathogenic variants in SR-B1 = higher HDL cholesterol (70s) vs noncarriers (50s).

“Sarb’s liver lipase has a high CET point”

53
Q

Brain regions that regulate food intake and their functions:
1. prefrontal cortex
2. ventral tegmentum and the nucleus accumbens
3. Brodmann area 17 in the occipital lobe
4. Arcuate nucleus
5. Nucleus of the tractus solitarius and the dorsal vagal nucleus

A
  1. impulse control (“fight urge to overeat”)
  2. reward center (dopa)
  3. processing visual food cues
  4. regulation of hunger and satiety (leptin, insulin, ghrelin, etc)
  5. GI to brain; regulates mechanics of food intake and digestion
54
Q

Is laparoscopic gastric banding associated with vitamin or mineral deficiencies?

A

NO, since it does not bypass any intestinal segment and does not cause malabsorption.

55
Q

vitamin B12 deficiency is associated with what bariatric surgical procedure?

A

gastric bypass > sleeve gastrectomy

56
Q

vitamin deficiencies that occur after Roux-en-Y gastric bypass

A

thiamine, B12, zinc (rash and diarrhea)

57
Q

cholesterol ester storage disease
1. mode of inheritance and gene affected?
2. pathogenesis
3. clinical presentation
4. treatment

A
  1. A.R.; pathogenic variant in the gene encoding lysosomal acid lipase (LIPA) –> decreased activity
  2. progressive accumulation of cholesteryl ester and TG in hepatocytes and macrophages.
  3. Presentation is highly variable. FTT in infancy. In older pop, increased risk for CVD; multiorgan dysfunction; cellular cholesteryl ester accumulation in the liver; elevated transminases; microvesicular steatosis; marked dyslipidemia (high LDL and apoB, low HDL).
  4. high-intensity statin, enzyme replacement with sebelipase alfa in severe cases
58
Q
  1. Lipoprotein lipase (LPL) - function?
  2. LPL deficiency results in?
  3. other names for this?
  4. specific physical features include?
  5. Treatment?
A
  1. breaks down TGs
  2. severely elevated TGs >1000 mg/dl increasing risk for pancreatitis AND total cholesterol (chylomicronemia)
  3. Familial hypercholesterolemia, familial defective apolipoprotein B, and familial chylomicronemia syndrome
  4. tendinous xanthomas
  5. Dietary and lifestyle interventions. Fibrates are ineffective. Plasmapheresis rarely has a role.
59
Q
  1. Apolipoprotein CII is a cofactor of?
  2. APO CII deficiency (and LPL deficiency) leads to?
A
  1. Lipoprotein lipase (LPL)
  2. severely elevated TGs >1000 mg/dl
60
Q
  1. Cholesterol ester transferase protein (CETP) function?
  2. CETP deficiency leads to?
  3. More common in?
A
  1. transfers cholesterol esters from HDL to VLDL, IDL, and remnant particles in exchange for TG, essentially clearing HDL cholesterol
  2. HIGH HDL usually >100 mg/dl (“HIGH HDL CETPoint)
  3. more common in individuals of Asian descent
61
Q

Identify monogenic forms of early-onset childhood obesity (prevalence, pathophysiology, characteristics)

Melanocortin 4 Receptor (MCR4)

A

prevalence 8%

  • pathophysiology - loss of satiety, hyperphagia
  • characteristics - increased linear growth, tall for age, increased lean mass
62
Q

Identify monogenic forms of early-onset childhood obesity (prevalence, pathophysiology, characteristics)

Leptin (LEP)

A

prevalence < 1%

  • pathophysiology - leptin from adipocytes acts centrally to promote satiety
  • characteristics - hyperphagia, hypothyroidism, hypogonadism, impaired immune function
63
Q

Identify monogenic forms of early-onset childhood obesity (prevalence, pathophysiology, characteristics)

Leptin Receptor (LEPR)

A

prevalence < 1%

  • pathophysiology - receptor to mediate effects of leptin
  • characteristics - same as for leptin (hyperphagia, hypothyroidism, hypogonadism, impaired immune function)
64
Q

Identify monogenic forms of early-onset childhood obesity (prevalence, pathophysiology, characteristics)

Pro-opiomelanocortin (POMC)

A

prevalence 1%

  • pathophysiology - regulates energy expenditure and appetite. POMC is cleaved into melanocyte stimulating hormone and ACTH. Important for adrenal function.
  • characteristics - hyperphagia (like LEP/LEPR), ACTH deficiency, adrenal insufficiency, hypopigmentation
65
Q

Identify monogenic forms of early-onset childhood obesity (prevalence, pathophysiology, characteristics)

Fat mass and obesity gene (E. FTO)

A

prevalence: most common, associated with generalized obesity, not early childhood-onset obesity
- mechanism - largely unknown, increased food intake

66
Q

common medications used in combination with statins that can increase the risk of myositis (5)

A

“Eric and Gemma Cycled then ate Ketos and Proteins (and got myositis stat)” Erythromycin Gemfibrozil (combined hyperlipidemias) Cyclosporin (transplant) Ketoconazole Protease inhibitors

67
Q

Identify the physical findings of hyperlipidemias

A. Lipemia retinalis

B. Corneal arcus

C. Eruptive xanthomas

D. Palmar xanthomas

E. Arthropathy

A

A. severe hypertriglyceridemia >3000 mg/dl

B. Familial hypercholesterolemia: increased TC/LDL ratio (ApoB) + premature CAD Homozygous - atherosclerosis before 20-30 yo. Treatment is liver translplant. Treatment for both hetero- and homozygous is high dose statin

C. severe hypertriglyceridemia >2000 mg/dl

D. Dysbetalipoproteinemia: high TC and TGs

E. NOT a sign of hyperlipidemia

68
Q

Case: A patient who was diagnosed with low cholesterol as an infant. Total cholesterol is low, LDL is low, Apo B is low.

  1. What does this patient have? Caused by?
  2. function of this protein?
  3. absence of this results in?
A
  1. Abetalipoproteinemia, a rare autosomal recessive disorder caused by MICROSOMAL TRIGLYCERIDE TRANSFER PROTEIN (MTTP) deficiency
  2. crucial in making apolipoprotein B-containing lipoproteins in the endoplasmic reticulum through transfer of TGs or phospholipids
  3. NO apolipoprotein B protein
69
Q

What are the rare genetic disorders in the differential diagnosis of severe hypolipidemias? 3 total

Matty without Betty, low Betty with fatty liver, both wearing a Sari.

A
  1. Abetalipoproteinemia - MTTP deficiency. Patients present with failure to thrive, steatorrhea, NEUROLOGIC SYMPTOMS (weakness/balance problems as adults)
  2. Familial hypobetalipoproteinemia - defective Apolipoprotein B. Cannot efficiently secrete lipoproteins from the liver and hence NAFLD. Homozygous form - cholesterol between 50-100 mg/dL Heterozygous form - APOB gene defect
  3. Chylomicron retention syndrome - SAR1B gene. Very low circulating cholesterol and TGs
70
Q

What are the commonly performed bariatric surgical procedures? 5 total

A
  1. biliopancreatic diversion - most weight loss (32-35%) and largest effect on glucose levels, but more frequent complications and adverse effects (severe and potentially difficult-to-treat vitamin deficiencies)
  2. Roux-en-Y gastric bypass - criterion standard operation. Second to the most weight loss (25-28%) and often dramatically improves glucose levels in patients with T2DM. Also results in lifelong need for vitamin supplementation and at risk for metabolic bone disease
  3. laparoscopic banding - less weight loss (18-22%) and more mechanical problems
  4. sleeve gastrectomy - good weight loss (22-25%), easy surgery to perform. Not as effective as gastric bypass in either producing weight loss or improving glucose control in T2DM.
  5. endoscopically placed dual balloon device - weight loss 7% 6 months after placement). Temporary weight loss.
71
Q
  1. HYPObetalipoproteinemia results in a reduction of?
  2. How is hypobetalipoproteinemia distinguished from Abetalipoproteinemia?
A
  1. both LDL (cholesterol) and VLDL (TG) The inability to inefficiently secrete lipoproteins from the liver can lead to NAFLD, due to a defect in the production of Apo B in the liver
  2. Abetalipoproteinemia is a rare A.R. disorder. MTTP deficiency. Patients present with failure to thrive, steatorrhea, neurologic symptoms (weakness/balance problems as adults)
72
Q
  1. Abetalipoproteinemia results in?
  2. symptoms?
A
  1. low LDL and TG (much lower than hypobetalipoproteinemia)
  2. neurologic symptoms (weakness) and balance problems as adults
73
Q
  1. What is the mode of inheritance of DYSbetalipoproteinemia?
  2. This results in what biochemical abnormalities?
  3. Clinical characteristics?
A
  1. A.R., associated with an APOE*E2/APOE*E2 GENOTYPE
  2. HIGH total cholesterol and TGs
  3. palmar xanthomas
74
Q

In a patient with primary biliary cirrhosis, elevated total cholesterol levels in these patients are most often due to the presence of?

A

an abnormal LDL-like particle named LIPOPROTEIN X

75
Q

Patients with collagen-vascular disease can develop severe hyperlipidemias. What medication can exacerbate hypertriglyceridemia in these patients?

A

beta blockers

76
Q

Have fenofibrate, niacin, or ezetimibe been shown to produce reductions in CV end points when used as a single agent?

A

NO. Therefore, need to put patient on highest tolerated statin dose + the aforementioned.

77
Q

What group of individuals do not benefit from statin?

A

ESRD (on HD)

78
Q

Patient with h/o bariatric surgery presents with normal calcium and vitamin 25OHD, elevated PTH.
1. suspicious for?
2. what lab to obtain?

A
  1. calcium deficiency
  2. 24-hour urinary calcium excretion
79
Q

partial lipodystrophy
1. typical age of onset
2. gene affected

A
  1. childhood, puberty, or adulthood
  2. LMNA; A.D. inheritance

Endocrinology (8 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions