Very long chain acyl-CoA dehydrogenase deficiency (VLCAD) is an inherited disorder that affects the body's ability to break down certain fats. It is caused by a deficiency of the enzyme very long chain acyl-CoA dehydrogenase (VLCAD). This enzyme is responsible for breaking down long-chain fatty acids, which are found in foods such as meat, dairy, and certain vegetable oils. Without this enzyme, the body is unable to properly break down these fats, leading to a buildup of toxic byproducts in the body. Symptoms of VLCAD can include low blood sugar, muscle weakness, and heart problems. Treatment typically involves dietary changes and medications to help the body break down fats.

The symptoms of Very long chain acyl-CoA dehydrogenase deficiency (VLCAD) can vary from person to person, but may include:

-Low blood sugar (hypoglycemia)
-Lethargy
-Weakness
-Poor feeding
-Vomiting
-Liver dysfunction
-Enlarged liver (hepatomegaly)
-Enlarged heart (cardiomegaly)
-Heart arrhythmias
-Muscle pain
-Muscle weakness
-Exercise intolerance
-Rhabdomyolysis (breakdown of muscle tissue)
-Lactic acidosis (buildup of lactic acid in the blood)
-Developmental delay
-Seizures
-Loss of consciousness

Very long chain acyl-CoA dehydrogenase deficiency (VLCAD) is caused by mutations in the ACADVL gene. These mutations can be inherited from a parent or can occur spontaneously.

The primary treatment for Very long chain acyl-CoA dehydrogenase deficiency (VLCAD) is dietary management. This includes a low-fat diet and supplementation with medium-chain triglycerides (MCTs). Other treatments may include carnitine supplementation, enzyme replacement therapy, and gene therapy. Additionally, supportive care such as monitoring of blood glucose levels, electrolytes, and other metabolic parameters may be necessary.

1. Family history of VLCAD deficiency
2. Consanguinity
3. Exposure to certain drugs or toxins
4. Premature birth
5. Low birth weight
6. Low levels of carnitine in the blood
7. Low levels of VLCAD enzyme activity in the blood
8. Abnormal liver function tests

Yes, there are medications and treatments available for Very long chain acyl-CoA dehydrogenase deficiency. Treatment typically involves dietary modifications, enzyme replacement therapy, and medications to reduce the risk of complications. Dietary modifications may include avoiding foods high in long-chain fatty acids, such as red meat, dairy products, and certain vegetable oils. Enzyme replacement therapy involves taking a medication that contains the missing enzyme, which helps the body break down fatty acids. Medications may also be prescribed to reduce the risk of complications, such as seizures and heart problems.