how do y'all differentiate between glucose 6 phosphatase deficiency and MCAD deficiency? - USMLE Forums
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Old 05-24-2014
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Default how do y'all differentiate between glucose 6 phosphatase deficiency and MCAD deficiency?

Please differentiate between Glucose 6 phosphatase deficiency and MCAD deficiency. What signs and symptoms and labs do you guys look in a vignette before deciding whether it's MCAD or glucose 6 phis deficiency
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DIFFERENTIAL:
BOTH have profound hypoglicemia, and both have early onset, so heres the differential in the vignette:

VONGIERKES= they will tell you cirrhosis/hepatomegaly, probably show or say that a liver biopsy has fat accumulation (hallmark) + hyperuricemia, hyperlipidemia which are characteristic of vongierkes.

MCAD= NONE of the above will be present here, on the contrary you will have a normal liver, and hallmark here is HYPOKETONEMIA with the profound hypoglicemia, and they can mention that they found increase of medium chain fatty acids in the blood.

thats how i tell them apart, if im wrong please correct me. hope it helps.
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Quote:
Originally Posted by drpisho View Post
DIFFERENTIAL:
BOTH have profound hypoglicemia, and both have early onset, so heres the differential in the vignette:

VONGIERKES= they will tell you cirrhosis/hepatomegaly, probably show or say that a liver biopsy has fat accumulation (hallmark) + hyperuricemia, hyperlipidemia which are characteristic of vongierkes.

MCAD= NONE of the above will be present here, on the contrary you will have a normal liver, and hallmark here is HYPOKETONEMIA with the profound hypoglicemia, and they can mention that they found increase of medium chain fatty acids in the blood.

thats how i tell them apart, if im wrong please correct me. hope it helps.
This and also they can mention the baby was breast-feeding exclusively or can even mention it suddenly died and asked the most likely reason, which would be MCAD (considering the rest of the vignette of course).
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Thank you guys for your excellent explanations! Very helpful! I usually get lucky with these kinds of questions but wanted to make sure my understanding was correct
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