What is Lipodystrophy?
Lipodystrophy is a general term for a collection of disorders involving the complete (generalized) or partial loss of fat tissue. This disorder can be inherited as a generic disorder (congenital) or acquired, at any age, due to an autoimmune disorder. Patients with severe, generalized lipodystrophies share an inability to produce enough of the hormone leptin, which is made by fat tissue. Leptin regulates appetite in the body. Leptin deficiency can lead to excessive hunger, difficult to control diabetes, and extremely high triglycerides, as well as other metabolic problems.
How common is Lipodystrophy?
Generalized forms of lipodystrophy are extremely rare. Acquired generalized lipodystrophy has been reported in approximately 100 - 250 individuals, and is more common in women than men. There have been approximately 300 cases of congenital generalized lipodystrophy reported with an estimated worldwide prevalence at roughly 1 in 10 million individuals. 1
Questions regarding your Lipodystrophy therapy?
You can reach the lipodystrophy care team, anytime, day or night, seven days a week by phone or online.
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1. Garg, A. Acquired and Inherited Lipodystrophies. NEJM 2004; 350:1220-34
What are the symptoms of Lipodystrophy?
Signs and symptoms related to generalized lipodystrophy include, but are not limited to:
- Near total absence of body fat
- Excessive appetite in infants and children
- Acanthosis nigricans: abnormally increased coloration and thickening of the skin
- Fat accumulation in the liver (hepatic steatosis)
- High triglycerides, leading to risk of pancreatitis
- Imbalance of sex hormones in women (polycystic ovary syndrome)
A variety of tests can be used to aid in the diagnosis of lipodystrophy including assessment of the amount of leptin in the blood. For individuals with congenital lipodystrophy, genetic testing can detect mutations specific to the disorder to confirm the diagnosis in most cases.
The following specialty medications are available at Accredo, a specialty pharmacy for Lipodystrophy.
|Myalept™ (metreleptin for injection)||Aegerion Pharmaceuticals, Inc.|
急救性质的特殊药品很昂贵。通过此视频，了解 Accredo 团队如何帮助个人找到可以负担他们救命药品费用的方式。
Accredo’s Lipodystrophy care team is dedicated to serving you and understands the difficulty of this complex condition. Our expert clinicians are always available, 24 hours a day, seven days a week, to answer any questions.