How important are these mt dz for Step 1

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DarlynVMD

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So I'm currently reviewing Biochemistry and I came across an interesting set of mitochondrial dz's and mitochondrial myopathies, which include:

Leber's hereditary optic neuropathy,
Kearns-Sayre Syndrome,
MELAS Syndrome, &
MERRF Syndrome.

How important are these dz for the Step 1. I remember getting a q on MERRF in USMLESteps123 Q-bank, but I don't know if I should even bother knowing them. For those who took it, what do you guys think.

Thanks in advance.

D

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I know quite a few people who got such questions. All you need to know is the mode of inheritance and how to recognize that on a pedigree. I wouldn't stress to memorize the details.
 
know the concepts behind mitoch diseases...so, the inheritance pattern, the physical diagnosis, reason for inheritance pattern, and the such is probably enough. i never found any need to know the ridiculous detail and it definitely worked for me. be a bit comfortable knowing what the symptoms would be like and you should be ok. good luck!
 
just an add, on top of knowing the inheritance pattern, think about which areas of the body are likely to be most dependent upon on mitochondria/energy (brain, muscle, eye, etc). these are the areas most likely to be affected. don't bother memorizing the different acronyms and syndromes
 
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