Study: 'Ketogenic diet in pyruvate dehydrogenase complex deficiency'

Remy

Administrator
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

Well-Known Member
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

New Member
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

Well-Known Member
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!
 

stevenm

New Member
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.
 

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