21 - 126 - XANTHOMAS AND LIPOPROTEIN DISORDERS Flashcards

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1
Q

plaques or nodules consisting of an abnormal excess of lipid primarily in the skin or tendons.

A

Xanthomas

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2
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A

Eruptive xanthomas are multiple, small, red-yellow papules, usually less than 5 mm in size, that may appear suddenly and are arranged in crops on the extensor surface of the extremities and the buttocks, often forming a confluent rash (Fig. 126-1).

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3
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They are associated with severe hypertriglyceridemia, usually resulting from accumulation of chylomicrons as in Types I and V dyslipidemia, and may be aggravated by underlying diabetes, obesity, excessive ethanol intake, and drugs such as retinoids, estrogen therapy, and protease inhibitors (Fig. 126-2).

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4
Q
A

Tuberous xanthomas are nodules that are localized to pressure areas including the extensor surfaces of the elbows, knees, knuckles, and buttocks (Figs. 126-3 and 126-4).

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5
Q

firm, subcutaneous nodules found in fascia, ligaments, Achilles tendons, or extensor tendons of the hands, knees, and elbows

A

Tendinous xanthomas

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13
Q

Marie came into your clinic due to multiple red-yellow papules in crops on the extensor extremities and buttocks. You suspected Eruptive Xanthoma and requested for lab tests. Her serum triglyceride is 1500mg/dL, there is also an increase in serum chylomicrons and on genetic testing, there was a functional defect in LPL.

  1. With the diagnosis in mind, what other noncutaneous symptoms should we watch out for?
    a. acute pancreatitis
    b. hypertension
    c. myocardial infarction
    d. xanthoma
  2. What is the mode of inheritance of her disease?
    a. autosomal dominant
    b. autosomal recessive
    c. X-linked dominant
    d. X-linked recessive
A

a. acute pancreatitis
b. autosomal recessive

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14
Q

Faye, a 22 year old female came into your clinic complaining of multiple yellow papules on her extremities. What other pertinent data should you elicit in your history taking?

a. history of coronary heart disease
b. recent lipid profile results
c. both
d. neither

A

b. recent lipid profile results

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15
Q

Queenie, a 30 year old Filipino female came into your clinic complaining of a sudden eruption of multiple red-yellow papules on her extensor extremities.

  1. Which of these is LEAST associated with her condition?
    a. intake of estrogen
    b. use of illicit drugs
    c. alcohol intake
    d. diabetes
  2. She asked you if this is commonly seen in her age and gender. What will you tell her?
    a. this condition is more commonly seen in females
    b. there is no age and gender predilection for this disease
    c. this disease unusual to be seen at her age group
    d. this disease is commonly seen in Asians and Hispanics
  3. She also asked about the noncutaneous manifestations of her condition. Which of these is/are a noncutaneous manifestations of this condition?
    a. intracranial lesions
    b. carotid stenosis
    c. peripheral vascular disease
    d. all of the above
A

b. use of illicit drugs
b. there is no age and gender predilection for this disease
d. all of the above

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16
Q

What is the first line pharmacotherapy for lowering LDL-C levels in patients with xanthoma?

a. HMG-CoA reductase inhibitor
b. HMG-CoA oxidase inhibitor
c. fibrates
d. Omega-3 fatty acids

A

a. HMG-CoA reductase inhibitor

17
Q

Which of these is a common side effect of HMG-CoA reductase inhibitor

a. headache and diarrhea
b. risk for Diabetes
c. Pruritus
d. flu-like symptoms

A

b. risk for Diabetes

18
Q

Overweight adults with xanthoma should aim for a _______ kcal/day energy deficit?

a. 250-500
b. 500-750
c. 100-250
d. 75-100

A

b. 500-750

19
Q

Which is TRUE regarding Alagille syndrome?

a. Cirrhosis develops in 80% of patients
b. They present with characteristic facies such as a prominent forehead, hypotelorism and nasal dystrophy
c. It is a sporadic syndrome of biliary hypoplasia and elevated serum cholesterol
d. Serum cholesterol is elevated when patients are young but decreases over time

A

d. Serum cholesterol is elevated when patients are young but decreases over time