Severe Fredrickson Type V Hypertriglyceridemia
Severe Fredrickson Type V Hypertriglyceridemia
Severe Fredrickson Type V hypertriglyceridemia (SFT-V) is a rare genetic disorder characterized by extremely high levels of triglycerides in the blood. This condition is caused by defects in the lipoprotein lipase enzyme and presents with a wide range of symptoms and complications..
People with SFT-V may have elevated levels of triglycerides up to 10,000 mg/dL or even higher. This is far in excess of what is typically seen in healthy individuals, which is usually less than 150 mg/dL. Though the exact cause of SFT-V is still unknown, it is believed to be an inherited disorder.
Due to the extremely high levels of triglycerides in the blood, people with SFT-V can experience several health complications. These include:
- Abdominal pain
- High cholesterol levels
- High blood pressure
- Enlarged liver or spleen
- Pancreatitis
- Increased risk of cardiovascular disease
In order to diagnose and manage SFT-V, it is important to perform a comprehensive family history review as well as clinical and laboratory assessment. In some cases, a tissue biopsy may be needed to confirm the diagnosis. Treatment typically involves lifestyle changes such as a low-fat diet and exercise to help reduce the triglyceride levels.
Medication may also be prescribed to help reduce the levels of triglycerides. These medications include omega-3 fatty acid supplements, fibrates, niacin, and statins. In severe cases, a form of triglyceride-lowering apheresis—a procedure in which high levels of triglycerides in the blood are removed—may be recommended.
Early diagnosis and prompt treatment of SFT-V is important in order to decrease the possibility of experiencing serious health complications. It is important that people with SFT-V regularly monitor their blood triglyceride levels in order to ensure that the levels do not increase too high.
