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Endocrine Abstracts (2016) 44 P180 | DOI: 10.1530/endoabs.44.P180

SFEBES2016 Poster Presentations Obesity and Metabolism (26 abstracts)

Metabolic surgery with Roux-en-y Gastric bypass is an effective treatment in patients with Familial Partial Lipodystrophy and Body Mass Index Less than 35 kg/m2

Claire Adams 1 , David Savage 2 , Lisa Gaff 1 , Catherine Flanagan 1 , Charlotte Jenkins-Liu 1 , Robert Semple 2 , Elaine Withers 2 , Stephen O’Rahilly 2 & Anna Stears 1


1Wolfsen Diabetes & Endocrine Centre, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK; 2Wellcome Trust-MRC Institute of Metabolic Science, University of Cambridge, Cambridge, UK.


Introduction: Familial Partial Lipodystrophy Type 1 (FPLD1) is characterised by loss of gluteal and limb subcutaneous fat and increased abdominal fat. The genetic basis is currently unknown. FPLD1 is frequently associated with metabolic problems including diabetes, insulin resistance, dyslipidaemia and non-alcoholic fatty liver disease. Despite central adiposity and severe metabolic abnormalities, this group of patients often do not qualify for NHS funding for bariatric surgery as they often have a Body Mass Index (BMI) below 30 kg/m2.

Patients 1 & 2: Two female patients with an FPLD1 phenotype and poor glycaemic control were referred to the National Severe Insulin Resistance Service. Despite trying metformin, intensification of insulin therapy and specialist dietary input (one patient undertook a liquid diet), HbA1c in both women remained >100 mmol/mol. Exceptional funding requests were made for Roux-en-Y gastric bypass (RYGB) surgery.

Results: Post RYBG, BMI fell from 33.2 to 27.8 kg/m2 in Patient 1 and from 29.7 kg to 22.9 kg/m2 in Patient 2. HbA1c returned to the normal range in both women (114 to 55 mmol/mol and 113 to 47 mmol/mol respectively) and diabetes medication was stopped other than metformin in Patient 1. MRI-based measures of liver fat normalised in both women, with a dramatic reduction from 20% to 4.5% in patient 2. Fasting triglycerides, LDL-cholesterol and liver function tests also reduced. Patient 1 is now 3 years post RYGB and her weight and metabolic control remain stable.

Conclusion: These case studies suggest that RYGB is safe and can be highly effective in improving insulin sensitivity and diabetes control in patients with FPLD1. Access to RYGB is currently limited in accordance with NICE guidance. However RYGB should also be considered for selected patients with FPLD and severe metabolic disease despite a relatively normal BMI. This could be viewed as a key ‘metabolic’ – rather than an ‘obesity’ intervention in this setting.

Volume 44

Society for Endocrinology BES 2016

Brighton, UK
07 Nov 2016 - 09 Nov 2016

Society for Endocrinology 

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