10.17863/CAM.11968
Melvin, A
Flanagan, C
Gaff, L
Gratton, B
Gribble, Fiona
0000-0002-4232-2898
Roberts, Geoffrey
Semple, RK
O'Rahilly, Stephen
0000-0003-2199-4449
Rubino, F
Stears, A
Savage, David
0000-0002-7857-7032
Roux-en-Y Gastric Bypass Surgery in the management of Familial Partial 2 Lipodystrophy Type 1 (FPLD1)
DSpace at Cambridge (dev)
2017
DSpace at Cambridge (dev)
DSpace at Cambridge (dev)
2017-08-09
2017-08-09
2017-10-01
en
Article
0021-972X
https://www.repository.cam.ac.uk/handle/1810/266122
1945-7197
10.1210/jc.2017-01235
Attribution 4.0 International
Attribution 4.0 International
Attribution 4.0 International
Attribution 4.0 International
Attribution 4.0 International
http://creativecommons.org/licenses/by/4.0/
http://creativecommons.org/licenses/by/4.0/
http://creativecommons.org/licenses/by/4.0/
http://creativecommons.org/licenses/by/4.0/
http://creativecommons.org/licenses/by/4.0/
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.
This work was supported by Wellcome Trust Grants WT106262, WT098498, WT095515, and WT107064 (to F.G., G.R., R.K.S., S.O., and D.B.S.), the Medical Research Council Metabolic Disease Unit, the National Institute for Health Research Cambridge Biomedical Research Centre, and the National Institute for Health Research Rare Disease Translational Research Collaboration.
WELLCOME TRUST
107064/Z/15/Z
Evelyn Trust
MRC
MC_UU_12012/5
Wellcome Trust
095515/Z/11/Z
Wellcome Trust
098498/Z/12/Z
MRC
MC_UU_12012/3