Fatty Acid Flashcards

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1
Q

Carnitine deficiency

A

inhibits beta oxidation. important to prevent hypoketotic hypoglycemic encephalopathy. can’t use fatty acids for energy. causes: malnutrition, vegetarianism, liver disease.

high carb diet, carnitine supp. medium chain FA supp.

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2
Q

MCADD (Medium Chain Acyl-CoA Dehydrogenase Deficiency)

A

beta oxidation enzyme def: Acyl-CoA Dehydrogenase def. diagnostic: high levels of dicarboxylic acids (from w-oxidation). SIDS (sudden infant death syndrome).

low fat/high carb diet. avoid fasting. important to prevent hypoketotic hypoglycemic encephalopathy.

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3
Q

Methylmalonic acidemia

A

beta oxidation enzyme def. methylmalonyl CoA mutase def or B12 def. acidemia.

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4
Q

Zellweger

A

no peroxisome synth. no peroxisome oxidation of VERY LONG FA. elevated VLcFA

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5
Q

Adrenoleukodystrophy

A

no VERY LONG FA transport across peroxisome membrane. elevated VLcFA

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6
Q

Refsum

A

no a-oxidation of Phytanic acid. elevated Phytanic acid. severe neuro problems.

restrict foods w/ Phytanic Acid

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