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  #1  
Old 09-07-2012
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Arrow Belindalimm Daily Questions: Pathology #6

A 12-year-old Caucasian male is found to have turbid plasma that forms a creamy-appearing supernatant on standing. His plasma lipoprotein lipase activity measured after heparin injection is substantially lower than normal. In which of the following ways would this patient be most likely to present for medical attention?

A. Chest pain
B. Abdominal pain
C. Tendon xanthomas
D. Xanthelasmas
F. Mental retardation
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  #2  
Old 09-07-2012
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D. Xanthelasmas ????????????
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Old 09-07-2012
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Will go with D
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  #4  
Old 09-07-2012
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Default artherosclerosis

I say AAAAAA
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Old 09-07-2012
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Default my answer :)

A. Chest pain
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  #6  
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Quote:
Originally Posted by Casandra View Post
A. Chest pain
When is your exam? I always forget to ask you this.
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Old 09-07-2012
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Quote:
Originally Posted by Doc4Step1 View Post
When is your exam? I always forget to ask you this.
Why? ....
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Quote:
Originally Posted by Casandra View Post
Why? ....
Just curious.

looks like you like the word ''why''?
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  #9  
Old 09-07-2012
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Smile :)

Quote:
Originally Posted by belindalimm View Post
A 12-year-old Caucasian male is found to have turbid plasma that forms a creamy-appearing supernatant on standing. His plasma lipoprotein lipase activity measured after heparin injection is substantially lower than normal. In which of the following ways would this patient be most likely to present for medical attention?

A. Chest pain
B. Abdominal pain
C. Tendon xanthomas
D. Xanthelasmas
F. Mental retardation
Acute pancreatitis ( Increase TG due to lipoprotien lipase deficiency)
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  #10  
Old 09-07-2012
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Quote:
Originally Posted by step1success View Post
Acute pancreatitis ( Increase TG due to lipoprotien lipase deficiency)
Good point
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  #11  
Old 09-07-2012
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i'll go with B) abdominal pain
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Old 09-07-2012
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this q was posted in dis forum b4

Explanation:
Dietary lipids are transported to the peripheral tissues as chylomicrons, at which point they are hydrolyzed by lipoprotein lipase, releasing triglycerides. Lipoprotein lipase deficiency is a rare disorder that results in increased concentrations of serum chylomicrons. The body is unable to clear dietary lipid loads due to the defective hydrolysis of triglycerides in chylomicrons.
Patients present in childhood with marked hyperlipidemia, pancreatitis (abdominal pain) lipemia retinalis, eruptive skin xanthomas (small yellowish papules surrounded by erythema that occur mainly on extensor surfaces of extremities) and hepatosplenomegaly. The risk of pancreatitis is significantly increased with serum triglyceride concentrations above 1000 mg/dL.
In normal individuals heparin releases endothelium-bound lipases, encouraging the clearance of triglycerides from the circulation. In patients with lipoprotein lipase deficiency, heparin-induced lipoprotein lipase activity is significantly reduced.
(Choices A, C, D) Low-density lipoprotein (LDL) is composed predominantly of cholesterol. The main mechanism by which LDL is cleared from the circulation is by “receptor-mediated uptake” by the liver. Defects in the LDL receptor lead to hypercholesterolemia. Individuals heterozygous for the defective LDL receptor gene have an LDL receptor density of about 50% normal. Serum LDL in these individuals ranges from 200-400 mg/dL. Individuals homozygous for the defect have LDL receptor activity that is less than 2% of normal, with dramatically elevated of LDL concentrations.
Patients with hypercholesterolemia are at high risk for accelerated coronary artery disease. Those with the homozygous defect may present at a very young age. (In contrast, patients with lipoprotein lipase deficiency are not usually at increased risk for premature coronary artery disease making chest pain an unlikely presentation for the patient in the vignette.) Clinically patients with defective LDL receptors have tubular xanthomas, which are nodular lipid deposits in the tendons (most often seen in the Achilles and elbow tendons). Tubular xanthomas are the hallmark of familial hypercholesterolemia. Some patients also have xanthelasma and arcus cornea.

Educational Objective:
Abdominal pain due to acute pancreatitis is the most likely presentation for hyperchylomicronemia (hypertriglyceridemia). Patients with this disorder are not usually at increased risk for premature coronary artery disease. Skin xanthomas may be present in hypertriglyceridemia, but tubular/tendon xanthomas and xanthelasmas are present with hypercholesterolemia (high LDL).
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Old 09-07-2012
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Quote:
Originally Posted by belindalimm View Post
A 12-year-old Caucasian male is found to have turbid plasma that forms a creamy-appearing supernatant on standing. His plasma lipoprotein lipase activity measured after heparin injection is substantially lower than normal. In which of the following ways would this patient be most likely to present for medical attention?

A. Chest pain
B. Abdominal pain
C. Tendon xanthomas
D. Xanthelasmas
F. Mental retardation
high Tg.. hereditary Familial HyperChylomichronemia
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Quote:
Originally Posted by Doc4Step1 View Post
Just curious.

looks like you like the word ''why''?

Yes, that's right I also have another one 'what's the mechanism?'

@ belindalimm - thanks for posting this question

@ricko335 - thanks for the exaplnation!
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  #15  
Old 09-08-2012
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Hyperlipidemia is very high-yield for the exam, I've heard! Thank you for posting such an important question belinda! And amazing explanation, combines everything well!
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  #16  
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Quote:
Originally Posted by Casandra View Post
Yes, that's right I also have another one 'what's the mechanism?'
lolz...So when is your exam?
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  #17  
Old 09-10-2012
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Default Yes, the answer is abdominal pain.

Quote:
Originally Posted by ricko335 View Post
this q was posted in dis forum b4

Explanation:
Dietary lipids are transported to the peripheral tissues as chylomicrons, at which point they are hydrolyzed by lipoprotein lipase, releasing triglycerides. Lipoprotein lipase deficiency is a rare disorder that results in increased concentrations of serum chylomicrons. The body is unable to clear dietary lipid loads due to the defective hydrolysis of triglycerides in chylomicrons.
Patients present in childhood with marked hyperlipidemia, pancreatitis (abdominal pain) lipemia retinalis, eruptive skin xanthomas (small yellowish papules surrounded by erythema that occur mainly on extensor surfaces of extremities) and hepatosplenomegaly. The risk of pancreatitis is significantly increased with serum triglyceride concentrations above 1000 mg/dL.
In normal individuals heparin releases endothelium-bound lipases, encouraging the clearance of triglycerides from the circulation. In patients with lipoprotein lipase deficiency, heparin-induced lipoprotein lipase activity is significantly reduced.
(Choices A, C, D) Low-density lipoprotein (LDL) is composed predominantly of cholesterol. The main mechanism by which LDL is cleared from the circulation is by “receptor-mediated uptake” by the liver. Defects in the LDL receptor lead to hypercholesterolemia. Individuals heterozygous for the defective LDL receptor gene have an LDL receptor density of about 50% normal. Serum LDL in these individuals ranges from 200-400 mg/dL. Individuals homozygous for the defect have LDL receptor activity that is less than 2% of normal, with dramatically elevated of LDL concentrations.
Patients with hypercholesterolemia are at high risk for accelerated coronary artery disease. Those with the homozygous defect may present at a very young age. (In contrast, patients with lipoprotein lipase deficiency are not usually at increased risk for premature coronary artery disease making chest pain an unlikely presentation for the patient in the vignette.) Clinically patients with defective LDL receptors have tubular xanthomas, which are nodular lipid deposits in the tendons (most often seen in the Achilles and elbow tendons). Tubular xanthomas are the hallmark of familial hypercholesterolemia. Some patients also have xanthelasma and arcus cornea.

Educational Objective:
Abdominal pain due to acute pancreatitis is the most likely presentation for hyperchylomicronemia (hypertriglyceridemia). Patients with this disorder are not usually at increased risk for premature coronary artery disease. Skin xanthomas may be present in hypertriglyceridemia, but tubular/tendon xanthomas and xanthelasmas are present with hypercholesterolemia (high LDL).
Nice detailed explanation. Thank you ricko335!
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