Very long-chain acyl-CoA dehydrogenase deficiency (VLCAD) is a rare genetic disorder of fatty acid metabolism that is transmitted as an autosomal recessive trait. It occurs when an enzyme needed to break down certain very long-chain fatty acids is missing or not working properly. VLCAD is one of the metabolic diseases known as fatty acid oxidation (FOD) diseases. In the past, the name long-chain acyl-CoA dehydrogenase deficiency (LCAD) was applied to one such disease, but today it is clear that all cases once thought to be LCAD are actually VLCAD.

The breakdown of fatty acids takes place in the mitochondria found in each cell. The mitochondria are small, well-defined bodies that are found in the cytoplasm of cells and in which the body generates energy from the breakdown of complex substances into simpler ones (mitochondrial oxidation).

Classically, two forms of VLCAD have been described: an early-onset, severe form which, if unrecognized and undiagnosed, may lead to extreme weakness of the heart muscles (cardiomyopathy) and may be life-threatening (VLCAD-C), and a later-onset, milder form, sometimes referred to as VLCAD-H, that is characterized by repeated bouts of low blood sugar (hypoglycemia). In reality, patients may present with a combination of symptoms and the disease is best though of as being a continuum. Since the advent of expanded newborn screening programs using tandem mass spectrometry technology, most VLCAD infants in the United States are being detected neonatal period.

 Signs and symptoms can occur during infancy, childhood or adulthood depending on the form of the condition and may include low blood sugar (hypoglycemia), lack of energy, and muscle Weakness. Children affected by the most severe forms of the condition are also at risk of serious complications such as liver abnormalities and life-threatening heart problems.

VLCAD deficiency is caused by changes (mutations) in the ACADVL gene and is inherited in an autosomal recessive manner. 

Treatment is based on the signs and symptoms present in each person.

Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency is a disorder in which the body is unable to convert specific fats to energy, particularly when fasting (fasting).

• Low blood sugar (hypoglycemia), tiredness (lethargy), and muscle weakness are major symptoms of VLCAD deficiency in infancy and early childhood.
• Individuals who are affected are also at risk of major consequences, including liver problems and life-threatening heart disorders.
• When symptoms first appear in adolescence or maturity, they frequently entail muscle discomfort and muscle tissue deterioration (rhabdomyolysis).
• Muscle tissue degradation produces a protein termed myoglobin, which is processed by the kidneys and excreted in the urine (myoglobinuria). Myoglobin turns the urine crimson or brown.
• Fasting, sickness, and exercise can all cause problems with VLCAD shortage in both children and adults.
• This illness is commonly misdiagnosed in affected children as Reye syndrome, a serious disorder that can develop in children while they appear to be healing from viral infections like chicken pox or flu.
• The majority of occurrences of Reye syndrome are linked to the use of aspirin during these viral illnesses.

Conditions
Ventricular tachycardia,Ventricular fibrillation,Tachypnea,Hepatomegalyv,Jaundice,Respiratory distress,Ventricular septal defect,Lethargy
Drugs
NA
Symptoms
A hole in the heart wall dividing two upper heart chambers,Subclinical abnormal liver function tests,Rapid breathing episodes,Exercise-induced rhabdomyolysis,Impaired eating abilities due to difficulties gathering food and preparing to suck, chew, or swallow it,An abnormally large liver size,Skin discoloration,Chronic foramen ovale,Difficulties breathing,Premature for gestational age,Anus preposition

Some treatments may be recommended for some kids but not for others. When treatment is required, it is usually required for the rest of one's life. The following are common therapies for kids with VLCAD:

• Avoid fasting for an extended period of time.
• A low fat, high carbohydrate diet is sometimes advised. Carbohydrates provide the body with a variety of sugars that can be used as energy.
• In reality, for youngsters requiring this medication, the majority of their diet should consist of carbohydrates (bread, pasta, fruit, etc.) and protein (lean meat and low-fat dairy foods).
• Medium-Chain Triglyceride oil (MCT oil) is frequently utilized in the diet of persons with VLCADD. This particular oil contains medium-chain fatty acids, which can be used for energy in modest doses.
• You can get advice from your metabolic specialist or a dietitian on how to utilize this supplement.
• To obtain MCT oil, you will need to obtain medication from your physician. L-carnitine may be beneficial to some children.
• This is a natural and harmless ingredient that aids the body's energy production.
• It also aids the body in eliminating hazardous wastes.

Conditions
Ventricular tachycardia,Ventricular fibrillation,Tachypnea,Hepatomegalyv,Jaundice,Respiratory distress,Ventricular septal defect,Lethargy
Drugs
NA
Symptoms
A hole in the heart wall dividing two upper heart chambers,Subclinical abnormal liver function tests,Rapid breathing episodes,Exercise-induced rhabdomyolysis,Impaired eating abilities due to difficulties gathering food and preparing to suck, chew, or swallow it,An abnormally large liver size,Skin discoloration,Chronic foramen ovale,Difficulties breathing,Premature for gestational age,Anus preposition