Dunnigan-type familial partial lipodystrophy, also called familial partial lipodystrophy type 2 or FPLD2, is a rare inherited form of insulin resistance. People with it lose subcutaneous fat from the limbs, trunk and gluteal region. The condition reproduces the main metabolic features of the insulin resistance syndrome, including central obesity, raised insulin levels, glucose intolerance and diabetes that is usually type 2, abnormal blood lipids, hypertension and early signs of atherosclerosis, and it can also cause fatty liver. It results from mutations in the LMNA gene, which encodes the nuclear lamins A and C. The disorder is named after the Scottish doctor Matthew Dunnigan, who pioneered early study of it.
Facts
Classification
ICD-10 ChapterIV: Endocrine, Nutritional and Metabolic Diseases 1 Communicable or Non-communicable Causative Agent
Causative Agent (category) Sources
1. Dunnigan familial partial lipodystrophy (Wikipedia)
Lead section
FPLD results from mutations in LMNA gene
Lead section [condition-kind]
rare monogenic form of insulin resistance
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