Exercise Patients with lipodystrophy should be encouraged to exercise in the absence of specific contraindications. (Class IIa, Level C) Patients with subtypes of lipodystrophy predisposed to cardiomyopathy should undergo cardiac evaluation prior to initiating an exercise regimen. (Class III, Level C) Individuals with lipodystrophy engaged in intense exercise have amelioration of metabolic complications. Most patients should be encouraged to be physically active. However, strenuous exercise should be avoided in patients with cardiomyopathy. Contact sports should be avoided in patients with severe hepatosplenomegaly and CGL patients with lytic lesions in long bones. Metreleptin In generalized lipodystrophy, metreleptin (with diet) is a treatment for metabolic and endocrine abnormalities (Class I, Level B), and may be considered for prevention of these comorbidities in young children. (Class IIb, Level C) Metreleptin may be considered for hypoleptinemic (leptin < 4 ng/mL) patients with partial lipodystrophy and severe metabolic derangements > 8% and/or triglycerides > 500 (Class IIb, Level B) mg/dL). Currently, metreleptin (recombinant human methionyl leptin) is the only drug approved specifically for lipodystrophy. It is approved in the US as an adjunct to diet for treatment of metabolic complications in patients with generalized lipodystrophy. In Japan, it is approved for both generalized and partial lipodystrophy. It is available in other parts of the world (e.g. Europe) through compassionate use programs. There is no age limit for initiation of metreleptin; children as young as 15 months have been treated. A dosing algorithm is provided in Supplemental Table 2 (). Dose adjustments should be made in
Exercise Patients with lipodystrophy should be encouraged to exercise in the absence of specific contraindications. (Class IIa, Level C) Patients with subtypes of lipodystrophy predisposed to cardiomyopathy should undergo cardiac evaluation prior to initiating an exercise regimen. (Class III, Level C) Individuals with lipodystrophy engaged in intense exercise have amelioration of metabolic complications. Most patients should be encouraged to be physically active. However, strenuous exercise should be avoided in patients with cardiomyopathy. Contact sports should be avoided in patients with severe hepatosplenomegaly and CGL patients with lytic lesions in long bones. Metreleptin In generalized lipodystrophy, metreleptin (with diet) is a treatment for metabolic and endocrine abnormalities (Class I, Level B), and may be considered for prevention of these comorbidities in young children. (Class IIb, Level C) Metreleptin may be considered for hypoleptinemic (leptin < 4 ng/mL) patients with partial lipodystrophy and severe metabolic derangements > 8% and/or triglycerides > 500 mg/dL). (Class IIb, Level B) Currently, metreleptin (recombinant human methionyl leptin) is the only drug approved specifically for lipodystrophy. It is approved in the US as an adjunct to diet for treatment of metabolic complications in patients with generalized lipodystrophy. In Japan, it is approved for both generalized and partial lipodystrophy. It is available in other parts of the world (e.g. Europe) through compassionate use programs. There is no age limit for initiation of metreleptin; children as young as 15 months have been treated. A dosing algorithm is provided in Supplemental Table 2 (). Dose adjustments should be made in