Nitric Oxide Production in MELAS Syndrome
| Status: | Completed |
|---|---|
| Conditions: | Other Indications |
| Therapuetic Areas: | Other |
| Healthy: | No |
| Age Range: | 3 - 65 |
| Updated: | 4/21/2016 |
| Start Date: | July 2009 |
| End Date: | April 2016 |
Arginine Flux and Nitric Oxide Production in Patients With MELAS Syndrome and the Effect of Arginine and Citrulline Supplementation
Introduction
Baylor College of Medicine and Texas Children's Hospital are recruiting individuals with
MELAS syndrome for a clinical study. MELAS syndrome is a mitochondrial disease; patients
with this disease have muscle weakness and often develop brain strokes, where blood does not
flow normally to different parts of the brain. It is believed that these strokes could be
due to decreased production of nitric oxide, a naturally occurring compound important for
normal blood vessel function. Nitric oxide is made from arginine and citrulline that are
normally found in our bodies.
What is the purpose of this study? The purpose of this study is to measure nitric oxide in
individuals with MELAS and see if giving arginine or citrulline will increase the formation
of nitric oxide. Nitric oxide is thought to be helpful in preventing strokes. Therefore, if
arginine and/or citrulline are shown to increase the formation of nitric oxide, they could
be used to prevent or treat the strokes in patients with MELAS syndrome.
Baylor College of Medicine and Texas Children's Hospital are recruiting individuals with
MELAS syndrome for a clinical study. MELAS syndrome is a mitochondrial disease; patients
with this disease have muscle weakness and often develop brain strokes, where blood does not
flow normally to different parts of the brain. It is believed that these strokes could be
due to decreased production of nitric oxide, a naturally occurring compound important for
normal blood vessel function. Nitric oxide is made from arginine and citrulline that are
normally found in our bodies.
What is the purpose of this study? The purpose of this study is to measure nitric oxide in
individuals with MELAS and see if giving arginine or citrulline will increase the formation
of nitric oxide. Nitric oxide is thought to be helpful in preventing strokes. Therefore, if
arginine and/or citrulline are shown to increase the formation of nitric oxide, they could
be used to prevent or treat the strokes in patients with MELAS syndrome.
What does the study involve? Individuals with MELAS syndrome will be admitted twice to the
General Clinical Research Center (GCRC) at Texas Children's Hospital. Each time they will
stay for five days, during which a special diet will be provided. Nitric oxide production
will be measured by stable isotopes infusion technique that involves placing small tubes in
veins (IV catheter), blood sampling, and injecting a harmless stable isotope. Stable
isotopes are forms of normal compounds that can be measured accurately. They are not
radioactive and there are no known risks to giving them; they are already part of your body
in small amounts.
During the first admission nitric oxide levels will be measured, then arginine
supplementation will be provided for 48 hours, after which nitric oxide levels will be
re-measured to determine the effect of arginine supplementation. During the second
admission, the effect of citrulline supplementation will be measured.
Who can participate in the study? Adults or children affected with MELAS syndrome and
carrying the DNA change that causes the condition (3243 A>G mutation) can participate.
Adults without MELAS disease will be recruited to participate as control subjects.
How to get more information? Subjects interested in participation or getting more
information can contact Dr. Ayman El-Hattab at email: elhattab@bcm.edu, office phone:
832-822-4289, cell phone: 646-660-5666, or pager: 832-824-7243 (5523).
General Clinical Research Center (GCRC) at Texas Children's Hospital. Each time they will
stay for five days, during which a special diet will be provided. Nitric oxide production
will be measured by stable isotopes infusion technique that involves placing small tubes in
veins (IV catheter), blood sampling, and injecting a harmless stable isotope. Stable
isotopes are forms of normal compounds that can be measured accurately. They are not
radioactive and there are no known risks to giving them; they are already part of your body
in small amounts.
During the first admission nitric oxide levels will be measured, then arginine
supplementation will be provided for 48 hours, after which nitric oxide levels will be
re-measured to determine the effect of arginine supplementation. During the second
admission, the effect of citrulline supplementation will be measured.
Who can participate in the study? Adults or children affected with MELAS syndrome and
carrying the DNA change that causes the condition (3243 A>G mutation) can participate.
Adults without MELAS disease will be recruited to participate as control subjects.
How to get more information? Subjects interested in participation or getting more
information can contact Dr. Ayman El-Hattab at email: elhattab@bcm.edu, office phone:
832-822-4289, cell phone: 646-660-5666, or pager: 832-824-7243 (5523).
Inclusion Criteria:
1. Age from 3 - 65 years
2. Clinical diagnosis of MELAS syndrome
3. Carrying the m.3243A>G mutation
Exclusion Criteria:
1. Having acute or chronic disease or physical disability that will interfere with the
ability to undergo the study procedures
2. Being pregnant
We found this trial at
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Texas Children's Hospital Texas Children's Hospital, located in Houston, Texas, is a not-for-profit organization whose...
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