Study: 'Ketogenic diet in pyruvate dehydrogenase complex deficiency'

[Remy]

A recent study showing that the ketogenic diet is a safe and effective long term treatment for patients with pyruvate dehydrogenase complex deficiency.

It is worth noting that the ketone levels required for improvement were greater than 3 mmol/L, which is difficult to achieve (but not impossible) by diet alone. The addition of exogenous ketones is likely to prove of great benefit to achieve therapeutic levels of ketones without such a severe restriction of carbohydrates as in the past.

Objectives

Our aime was to study the short- and long-term effects of ketogenic diet on the disease course and disease-related outcomes in patients with pyruvate dehydrogenase complex deficiency, the metabolic factors implicated in treatment outcomes, and potential safety and compliance issues.

Methods


Pediatric patients diagnosed with pyruvate dehydrogenase complex deficiency in Sweden and treated with ketogenic diet were evaluated. Study assessments at specific time points included developmental and neurocognitive testing, patient log books, and investigator and parental questionnaires. A systematic literature review was also performed.
Results

Nineteen patients were assessed, the majority having prenatal disease onset. Patients were treated with ketogenic diet for a median of 2.9 years. All patients alive at the time of data registration at a median age of 6 years. The treatment had a positive effect mainly in the areas of epilepsy, ataxia, sleep disturbance, speech/language development, social functioning, and frequency of hospitalizations. It was also safe—except in one patient who discontinued because of acute pancreatitis. The median plasma concentration of ketone bodies (3-hydroxybutyric acid) was 3.3 mmol/l. Poor dietary compliance was associated with relapsing ataxia and stagnation of motor and neurocognitive development. Results of neurocognitive testing are reported for 12 of 19 patients.
Conclusion

Ketogenic diet was an effective and safe treatment for the majority of patients. Treatment effect was mainly determined by disease phenotype and attainment and maintenance of ketosis.

 

[Farmgirl]

I feel good on the Ketogenic Diet! I am not super strict about it, but I have heard some families who have vaccine damaged children with continuous seizures must weigh out the fats at each meal. I checked out this book:
Fat For Fuel by Joseph Mercola
and really wanted to read it, but reading is difficult, instead, I watch his videos on Youtube about the diet.

The only issue is that my cholesterol went up a bit and my doctor was not happy. I need to eat more veggies to balance the fats out cuz I am weak and then tend to grab nuts to feed myself cuz thy are fast and easy, but not so good for the cholesterol levels. I high cholesterol is the least of my problems right now. :-D FG

 

[stevenm]

I feel good on the Ketogenic Diet! I am not super strict about it, but I have heard some families who have vaccine damaged children with continuous seizures must weigh out the fats at each meal. I checked out this book:
Fat For Fuel by Joseph Mercola
and really wanted to read it, but reading is difficult, instead, I watch his videos on Youtube about the diet.

The only issue is that my cholesterol went up a bit and my doctor was not happy. I need to eat more veggies to balance the fats out cuz I am weak and then tend to grab nuts to feed myself cuz thy are fast and easy, but not so good for the cholesterol levels. I high cholesterol is the least of my problems right now. :-D FG

do you have CFS? what level of improvement from Keto diet?

 

[Farmgirl]

do you have CFS? what level of improvement from Keto diet?

@stevenm. Hi. Yes, I do have CFS and Chronic Lyme and a lot of other issues. The diet helps me think clearer. I have a lot of weird hormonal issues and it helped me loose weight. Loosing weight is important because of my disability. I don't want to be too heavy for some one to be able to move me when I cannot move myself.

Have you tried the Ketogenic plan or thought about trying it? There are some books out there on it.

Do you also have ME? Have a good day!

 

[Farmgirl]

Does anyone eat Paleo on this site? I do good on that too.

 

[stevenm]

Yes, I have CFS. I went to a functional MD who messed up my gut, now I am too thin but better than last July. I have several books on Paleo, I am afraid to mess my gut up more, or lose weight.

 

[Ughhh]

I've tried keto and carnivore several times and haven't seen any improvements on it. I was reading last night about the theory of the kynurenine pathway trap being a possible cause of cfs (Is it a trap? – paolo maccallini this was one article that explained it fairly comprehensively) and I read other articles regarding diet and keto diet possibly influencing the kynurenine pathway, sorry I didn't save links for all the diet stuff I read about this while laying in bed with my cell. Very interesting as I've been trying to gear up to do full carnivore again. I've watched quite a lot of videos of people claiming carnivore cured their mystery illness/autoimmune illnesses and I believe that these people are telling the truth, but why and why hasn't it worked magic for me. I also saw a video this week about the gut microbiome, and they showed graphs of the microbiome after trying different diets, the only diet that significantly changed the microbiome was the low carb/keto, but they didn't explain what does that mean is it good? Still interesting, it seems like any reset change to my gut would be better than how it is now.