Specialist Weight Management and Adjustable Gastric Banding for Refractory Obesity in Laurence-Moon-Bardet-Biedl Syndrome

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: SAT 660-676-Clinical Obesity Treatment
Clinical
Saturday, June 15, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board SAT-673
Maria Pallayova*1, Adrian Brown2, Paul Super3 and Shahrad Taheri2
1University of Birmingham, 2Specialist Weight Management Services, Heart of England NHS Foundation Trust, Birmingham, United Kingdom, 3Heart of England NHS Foundation Trust
Background: Laurence-Moon-Bardet-Biedl Syndrome (LMBBS) comprises rare hereditary disorders with a similar, yet highly variable phenotype including obesity, retinal dystrophy, polydactyly, and hypogenitalism. Progressive neurological, ophthalmologic and endocrine manifestations can each contribute to development of refractory obesity, presenting a significant treatment challenge.

Clinical Case: A 26-year-old Caucasian woman with LMBSS and end-stage renal failure requiring renal dialysis was referred to the specialist weight management service (SWMS). The patient had previous unsuccessful weight loss attempts to qualify for a renal transplant, as local criteria required her to be a maximum BMI of 35kg/m2 before consideration for transplantation. The patient’s referral weight was 136kg (BMI 51.1kg/m2). In addition to obesity and end-stage renal failure, she had mild sleep apnoea. She was registered blind and attending a specialist college. Her medications included furosemide, losartan, warfarin, aspirin, amitriptyline, tramadol, alfacalcidol, calcium acetate, and ferrous sulphate.

The patient attended the SWMS, having appointments with both a specialist physician and dietitian. She followed a programme focusing on lifestyle and behaviour change. Barriers to change included: restrictions due to dialysis, the patient’s understanding of satiety, portion perception, and unconscious family sabotage. In the initial 6 months, her weight reduced slightly to a low of 132.8kg (2.4% weight loss). Over the next 6 months, focus was placed on portion control, behaviour change, and changing parental perceptions of reward. During this period her weight reduced to 126.4kg (BMI 48.2kg/m2), a clinically significant weight loss of 7.1%. She was provided with funding for surgery and followed a pre-operative diet and lost a further 5.4kg, making a total medical weight loss of 15.4kg, a total pre surgery weight loss of 11.3%.

The patient successfully underwent laparoscopic insertion of a gastric band. She followed postoperative dietary changes to aid fullness and satiety, and increased her physical activity. Since surgery, her weight dropped to 106kg, BMI 40.4kg/m2, a loss of 14.6kg, another 10.7% reduction in total weight (33.6% excess weight loss) within the first 7 months. The patient continues with on-going weight loss and lifestyle changes towards the goal of a renal transplant.

Conclusion: Specialist weight management focusing on behaviour change and social support, combined with bariatric surgery enabled a patient with a genetic predisposition to obesity to lose clinically significant weight. Early effective treatment of obesity in LMBBS is important to limit further weight gain, and reduce complications of obesity that are likely to develop over time.

Disclosure: MP: Recipient Award, Lilly USA, LLC. ST: Clinical Researcher, Lilly USA, LLC, Clinical Researcher, Novo Nordisk. Nothing to Disclose: AB, PS

*Please take note of The Endocrine Society's News Embargo Policy at http://www.endo-society.org/endo2013/media.cfm

Sources of Research Support: M. Pallayova is supported by the Slovakian Diabetes Association/Lilly Diabetes Clinical Research Initiative. S. Taheri is supported by the UK National Institute of Health Research Collaborations for Leadership in Applied Health Research and Care.