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http://refsumdisease.org/patients/dietwhichfoods.shtml
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Where does the body get phytanic acid?
Phytanic acid is not produced by the human body but is obtained from meat, dairy, and fish products. Phytanic acid is a branched chain fatty acid. can also be produced through the breakdown of a substance that is found in green leafy vegetables
What is the cause of Refsum's disease?
Refsum's disease is caused by a deficiency of phytanic acid hydroxylase, an enzyme that breaks down a fatty acid called phytanic acid.
What type of seafood high in uric acid?
Mackerel and shrimps are the seafood in which there is high content of uric acid present. If you are from India, then you can get them easily from the markets.
How is Refsum disease diagnosed?
The diagnosis for Refsum disease is made based on the development of clinical manifestations and biochemical analysis detecting elevated phytanic acid in the blood.
What causes Refsum disease?
It is currently felt to be caused by mutations in a gene (PAHX) that encodes a protein called phyanoly-CoA hydroxylase and is important for metabolizing phytanic acid.
Why does citric acid cook seafood?
through a process called denaturation, when marinated the citric acid changes effects the protein molecules in the seafood(fish) which allows it to "cook"
What happens in Refsum disease?
Refsum disease is a rare genetic disorder that affects the ability of the body to breakdown fats, a process called fatty acid oxidation. As a result, a metabolite called phytanic acid accumulates in the blood as well as other tissues.
What are recipes for foods with high uric acids?
Meats, particularly organ meats, are high in uric acid. Other choices might include seafood, yeast breads, and vegetables like cauliflower and mushrooms.
What is are some common names for Refsum disease?
ataxia hereditaria hemeralopia polyneuritiformis, hemeralopia heredotaxia polyneuritiformis, hereditary motor sensory neuropathy type IV, heredopathia atactica poluneuritiformis, and phytanic acid storage syndrome.
Are fish with scales are high in uric acid?
Yes as a mater of fact all seafood is particularly high in "Purines" which is metabolized into uric acid. There are purines in the cells of all living things and when they are consumed , this includes the dead cels of your own body The purines t make up a part of them are metabolized int uric acid to be disposed of by the kidney and large intestine and in perspiration.
Why does seafood raise the uric acid level in blood?
sea foods are high in purines and these turn into uric acid. if the sufferer has a genetic predisposition to producing more uric acid or the system is faulty and is not able to cope with and eliminate it a point of saturation may be reached where it turns into crystals around joints or form stones in the kidneys.
What diet restrictions can benefit those with Refsum's disease?
A diet free of phytanic acid (found in dairy products, tuna, cod, haddock, lamb, stewed beef, white bread, white rice, boiled potatoes, and egg yolk) can reduce some of the symptoms.
