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GL is characterized by a lack of the hormone, leptin.

About GL

Generalized Lipodystrophy (GL) is a rare, complex, and clinically heterogeneous disorder characterized by the widespread lack or loss of subcutaneous adipose tissue in most or all parts of the body, and leading to a deficiency of the hormone leptin.1,2

Widespread lack/loss of tissue and/or metabolic abnormalities (diabetes mellitus, hypertriglyceridemia, insulin resistance)

Think GL

2 types of GL: Congenital and Acquired

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1

Congenital GL

  • Autosomal recessive
  • Typically presents as generalized lack of subcutaneous fat in infancy to early childhood
  • Negative genetic testing does not rule out GL (since not all mutations have been identified)
  • Also known as Berardinelli-seip syndrome
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2

Acquired GL

  • Autoimmune-related or idiopathic origin
  • More common in females
  • Can present as generalized subcutaneous fat loss in childhood to adulthood
  • Also known as Lawrence syndrome

Metabolic Consequences of GL

In GL the lack of adipose tissue can lead to leptin deficiency, which results in metabolic complications, including:

  • Insulin resistance
  • Diabetes mellitus
  • Hypertriglyceridemia

Adipose Tissue and Leptin

Adipose tissue is not a passive reservoir for energy storage—it is a complex and active organ.3

Adipose tissue plays a critical role in producing the key hormone leptin to ensure proper metabolic function.4

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1. Handelsman Y, Oral EA, Bloomgarden ZT, et al. The clinical approach to the detection of lipodystrophy—an AACE consensus statement. EndocrPract. 2013;19(1):107-116.doi:10.4158/endp.19.1.v767575m65p5mr06. 2. Brown RJ, Araujo-Vilar D, Cheung PT, et al. The diagnosis and management of lipodystrophy syndromes: a multi-society practice guideline.J Clin Endocrinol Metab. 2016;101(12):4500-4511. doi:10.1210/jc.2016-2466.