Roux-en-Y Gastric Bypass Surgery in the Management of Familial Partial Lipodystrophy Type 1

Abstract

Context: Familial partial lipodystrophy type 1 (FPLD1) is an extreme form of central adiposity, with peripheral lipodystrophy associated with severe manifestations of the metabolic syndrome, often poorly responsive to standard therapeutic approaches. Body mass index in FPLD1 varies but, in many cases, is below the level at which metabolic surgery is usually considered as a therapeutic option.

Design: We detailed the metabolic response to gastric bypass surgery of three patients with FPLD1, refractory to medical therapy.

Results: Roux-en-Y gastric bypass (RYGB) was associated with weight loss and substantial improvements in glycemic control and insulin sensitivity. All three patients were able to stop using insulin. Glucose tolerance testing in one patient demonstrated an increase in L-cell-derived gut hormone responses postoperatively.

Conclusion: RYGB surgery substantially improved glycemic control in three patients with FPLD1, two of whom had body mass indices below 30 kg/m2. RYGB should be considered in patients with partial lipodystrophy and refractory metabolic disease.

Figure 1.

The pancreatic and enteroendocrine hormonal responses of patient A to a 50-g oral glucose tolerance test preoperatively (black line) and 3 months following RYGB surgery (dashed line). Glucose tolerance improved postoperatively. GLP-1 and PYY response to glucose load increased at 3 months, while glucose-dependent insulinotropic polypeptide was largely unchanged.

Figure 2.

The changes in fat mass between the preoperative (black bars) and 3-month postoperative (white bars) periods in two patients with FPLD1 undergoing RYGB.