Myoglobinuria in Carnitine deficiency

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MudPhud20XX

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Alright so anything that results in muscle damage can lead to myoglobinuria, so one of the hallmarks for McArdle dz is myoglobinuria since accumulation of glycogen in muscle can damage the muscles.

Then how do you explain the myoglobinuria in carnitine deficiency? First Aid doesn't mention about myoglobinuria, but Kaplan biochemistry does.

I am assuming the accumulation of fatty acids in muscle may lead to myoglobinuira I guess?

If so, can anyone explain how the accumulation of glycogen or fatty acids can damage muscle?

Many thanks in advance.

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Correlate it with NASH: What happens to AST & ALT? Once you make that correlation, it's not hard to take it a step further: If a muscle component can escape into the systemic circulation (rhabdomyolysis --> myoglobinemia), then you would expect the body to excrete it through the kidneys (myoglobinuria).
 
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Alright so anything that results in muscle damage can lead to myoglobinuria, so one of the hallmarks for McArdle dz is myoglobinuria since accumulation of glycogen in muscle can damage the muscles.

Then how do you explain the myoglobinuria in carnitine deficiency? First Aid doesn't mention about myoglobinuria, but Kaplan biochemistry does.

I am assuming the accumulation of fatty acids in muscle may lead to myoglobinuira I guess?

If so, can anyone explain how the accumulation of glycogen or fatty acids can damage muscle?

Many thanks in advance.

fatty acids can be toxic when the accumulate, i believe
 
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We learned that the reduced ability to produce ATP is just as pathologic as the accumulation of glycogen/FFA in muscle. So in Carnitine deficiency long chain FFA accumulate, leading to less ATP production, leading to cell damage/death. I don't know if the distinction is necessary for boards, but it is a different answer than things being toxic when they accumulate.
 
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