TABLE 1.

Treatment Considerations in Lipodystrophy

• Diagnosis can lead to major anxiety for patients and their families.
• Cosmetic surgery to address fat loss can be considered with caution.
• Dietary considerations: high-carbohydrate, low-fat diet
 ○ Improves marked chylomicronemia but may increase VLDL cholesterol
  ■ Fibric acid agents and peroxisome proliferator–activated receptor agents may be helpful but are untested
  ■ Fish oil supplementation should be considered
• There are special cardiovascular concerns with CGL4 because of arrhythmia risk:
 ○ Avoid strenguous exercise
 ○ Possible role for β-blockers
 ○ Electrophysiology consultation should be given strong consideration
• Newest consideration: metreleptin
 ○ Leads to improved metabolic parameters
 ○ Glucose, triglyercides, fatty organ disease