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Saw our Genetic dr today

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I'm no expert but I thought from my reading that an "overabundance of mitochondrias" is produced in some mitochondrial disorders - not all of them. I thought it was because the mitochondria are not functioning well so the body has more of them in an attempt to compensate....... I could be totally wrong though, but I thought it had something to do with the ragged red fibers they sometime see when analyzing the muscle tissue.

Maybe someone else will have more clear ideas than me. You might want to check the UMDF website and read under the diagnostic testing part b/c I thought it said something like that there.

Anne R

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Well today was our yearly genetic dr appointment. Our genetic dr is still so

very stumpted as to what miss hailee has..

They found her missing chart and he pulled her muscle biopsy which was frozen

and sent to Buffalo Ny for testing, it came back negative for mitocondrial, but

he said the labs didnt make sense so now he has to call his partner who is in

Europe to discuss hailee with him again.. The lab test came back showing that

she produces an overabudance of mitocondrials... Which he says makes no sense

since she still has all the crashes. Except that we do have two other disorders

on top of the suspected mito. So this makes no sense to him, he has never seen

it, and dosnt know how to explain it.. Does anyone have any ideas?? We are still

have crashing cycles every 6-8 weeks with admits for up to 4 weeks at a time.

She still tires very easy and has many of the symptoms but now we have a

negative mito test... Help.. How conclusive is the muscle biopsy test?? Do i

look else where??

, Hailees mom

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I really do not know too much on this, sorry, but I will try to share what I know from Gracies biopsies. With her it is not too much of defficincies that make the diagnosis (even though all 4 chains are on the low end of normal or low in the muscle). Her diagnosis is due to the mutations of her mitochondria. O.K. now this is where it is strange to me so I hope I don't confuse you. Gracies liver is most affected by the disease. In the liver biopsy the mutations are even more pronounced. Complex 1,2, and 4 chains are defficiant. Only in her liver, though, her complex 3 chain actually has three times more than normal of mitochondria (even though they are mutated). Dr. Cohen was also puzzled why her body produces so many mutated mitochondria. He has consulted with practically every mito doc, and no one yet has a good reason how this can happen. The latest theory is that since the mitochondria work so poorly, her body is trying to overcompinsate for the "bad mitochondria", hence producing so many of them (even though they are bad too). Maybe in Hailey's case it is similar. Her mitochondria may not be working well, therefore her body is making a ton of them to even out due to the loss of some. This is just a theory, though. For us it has been a confusing road. We now know Grace will never have a specific diagnosis, other than non specific mitochondrial disease.

Best wishes,

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,

had a normal muscle and skin biopsy. Her liver was found

to have the bad mito. I too have signs and symptoms as do my other

two. If they wanted to do a muscle biopsy I am not sure I would

pursue it for us. I know this really doesn't help much, sorry.

Dawn

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Hi :

I felt I needed to respond to your email. Leah too has what is called a

proliferation of mitochondria cells in her body; meaning too many. And if

memory serves me right, that can be just as bad. I'll see if my husband

remembers everything they told me about that. Leah had another muscle biopsy

done just in the beginning of June and they are checking on not only that, but

it was sent to town University for a study being done on mutation. Wish I

could help more. But wanted to let you know that. Except Leah doesn't crash.

Only when she gets ill does she sleep more.

Nerenhausen

mom to LEah

Angels4hailee@... wrote:

> Well today was our yearly genetic dr appointment. Our genetic dr is still so

very stumpted as to what miss hailee has..

> They found her missing chart and he pulled her muscle biopsy which was frozen

and sent to Buffalo Ny for testing, it came back negative for mitocondrial, but

he said the labs didnt make sense so now he has to call his partner who is in

Europe to discuss hailee with him again.. The lab test came back showing that

she produces an overabudance of mitocondrials... Which he says makes no sense

since she still has all the crashes. Except that we do have two other disorders

on top of the suspected mito. So this makes no sense to him, he has never seen

it, and dosnt know how to explain it.. Does anyone have any ideas?? We are still

have crashing cycles every 6-8 weeks with admits for up to 4 weeks at a time.

She still tires very easy and has many of the symptoms but now we have a

negative mito test... Help.. How conclusive is the muscle biopsy test?? Do i

look else where??

> , Hailees mom

>

>

> Please contact mito-owner with any problems or questions.

>

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Not to sound ignorant, but what does "crash" mean when you are talking about mito?

-----Original Message-----From: Sorensen Sent: Thursday, July 01, 2004 8:26 AMTo: Mito Subject: Re: Saw our Genetic dr todayHi :I felt I needed to respond to your email. Leah too has what is called a proliferation of mitochondria cells in her body; meaning too many. And if memory serves me right, that can be just as bad. I'll see if my husband remembers everything they told me about that. Leah had another muscle biopsy done just in the beginning of June and they are checking on not only that, but it was sent to town University for a study being done on mutation. Wish I could help more. But wanted to let you know that. Except Leah doesn't crash. Only when she gets ill does she sleep more. Nerenhausenmom to LEahAngels4hailee@... wrote:> Well today was our yearly genetic dr appointment. Our genetic dr is still so very stumpted as to what miss hailee has..> They found her missing chart and he pulled her muscle biopsy which was frozen and sent to Buffalo Ny for testing, it came back negative for mitocondrial, but he said the labs didnt make sense so now he has to call his partner who is in Europe to discuss hailee with him again.. The lab test came back showing that she produces an overabudance of mitocondrials... Which he says makes no sense since she still has all the crashes. Except that we do have two other disorders on top of the suspected mito. So this makes no sense to him, he has never seen it, and dosnt know how to explain it.. Does anyone have any ideas?? We are still have crashing cycles every 6-8 weeks with admits for up to 4 weeks at a time. She still tires very easy and has many of the symptoms but now we have a negative mito test... Help.. How conclusive is the muscle biopsy test?? Do i look else where??> , Hailees mom>>> Please contact mito-owner with any problems or questions.>

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