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Old 07-22-2012
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Biochemistry Severe Hypoglycemia with Cardiomegaly

A 3-month-old infant boy is admitted to the hospital in a coma. He is found to have cardiomegaly and hepatomegaly and is hypoglycemic with a blood glucose of 15 mg/dL. He has no acidosis or ketosis but his blood ammonia is elevated (300 μg/dL). Further studies show a normal glucose, fructose, and galactose tolerance. Which of the following is the most likely cause of the patientís cardiomegaly?

a) Carnitine deficiency
b) Hypertension
c) Pompeís disease
d) von Gierkeís disease
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Old 07-22-2012
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Cardiomegaly......pompe's
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Old 07-22-2012
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Quote:
Originally Posted by koolkiller88 View Post
Cardiomegaly......Pompe's
No, not this time
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Old 07-22-2012
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Carnitine deficiency. Normal ketones despite hypoglycemia indicates a problem with FA metabolism.
The hyperammonemia can probably be explained on the basis of the liver not having enery to synthesize urea. Not sure though
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Haha. Ya you are right.
It's carnitine deficiency
Carnitine cycle helps transfer of long chain fatty acid to mitochondria.
Symptoms-
Cardiomegaly, Reye syndrome like increased ammonia manifestation.
Now right? ;-)
Please tell yes otherwise I will jump out of my balcony with biochem book on my head. Hehehe
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Old 07-22-2012
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Quote:
Originally Posted by Casandra View Post
A 3-month-old infant boy is admitted to the hospital in a coma. He is found to have cardiomegaly and hepatomegaly and is hypoglycemic with a blood glucose of 15 mg/dL. He has no acidosis or ketosis but his blood ammonia is elevated (300 μg/dL). Further studies show a normal glucose, fructose, and galactose tolerance. Which of the following is the most likely cause of the patientís cardiomegaly?

a) Carnitine deficiency
b) Hypertension
c) Pompeís disease
d) von Gierkeís disease
Answer is A
Thank you for a good qs!
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Quote:
Originally Posted by Casandra View Post
He has no acidosis or ketosis but his blood ammonia is elevated (300 μg/dL).


The first thing that has to come to your mind after reading that...


a) Carnitine deficiency
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Old 07-27-2012
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Default correct answer :)

yup, it's a) Carnitine deficiency
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