Effectiveness of Gastric Bypass Surgery in a Patient With Familial Partial Lipodystrophy
- Kristina M. Utzschneider, MD12 and
- Dace L. Trence, MD1
- 1Department of Medicine, Division of Metabolism, Endocrinology and Nutrition, University of Washington, Seattle, Washington
- 2Department of Medicine, VA Puget Sound Health Care System, Seattle, Washington
- Address correspondence and reprint requests to Kristina M. Utzschneider, MD, VA Puget Sound Health Care System, 1660 S. Columbian Way, Seattle, WA 98108. E-mail: kutzschn{at}u.washington.edu
Familial partial lipodystrophy (FPL) is associated with loss of subcutaneous fat in the extremities but preservation, or increase, of fat in the face, neck, and trunk. Patients with FPL manifest marked insulin resistance and develop diabetes and hypertriglyceridemia that are often very difficult to manage with pharmacologic agents, the current standard of therapy for FPL. Roux-en-Y gastric bypass (RYGB), a surgical procedure that leads to marked weight loss in morbidly obese subjects, improves insulin sensitivity (1,2) and reverses (3) or prevents diabetes (4). This is the first report of RYGB in a patient with FPL that resulted in unanticipated dramatic improvement in the metabolic control of her lipodystrophy.
HISTORY AND EXAMINATION
The patient is a 55-year-old Caucasian woman with FPL who underwent a laparoscopic RYGB for treatment of severe gastroesophageal reflux disease and gastroparesis, failing standard medical therapy. She reported a history of truncal obesity and thin extremities throughout her life. She was diagnosed with FPL type 1 based on physical exam findings of distal loss of subcutaneous fat in her extremities with excess fat in her chin and face and marked abdominal obesity. Her medical problems included poorly controlled type 2 diabetes, hypertriglyceridemia with a history of recurrent pancreatitis, coronary artery disease, hypertension, depression, and asthma. Her family history was significant for early coronary artery disease in her mother. …
