RF24 | PSUN126 A Very-Low Calorie Diet Can Reduce Insulin Resistance and Cause Remission of Diabetes Mellitus and Hypertriglyceridemia in a Patient With Familial Partial Lipodystrophy Type 2
Authors: De Freitas Maria Foss , Neidert Adam , Nay Catherine , Chenevert Thomas , Oral Elif , Rothberg Amy 
Published: November 2022

Background

Lipodystrophy syndromes are associated with inadequate adipocyte capacity that results in an earlier threshold for ectopic deposition and metabolic dysfunction. We have previously reported that patients with familial partial lipodystrophy (FPLD) present with metabolic severity equivalent to what is observed at 8-12 BMI points higher in a population with common obesity. Currently, there is no strong evidence that any specific diet is the preferred treatment, but bariatric surgery has been reported to improve metabolic outcomes in a few patients. Here, we report the effect of an aggressive calorie-restricted diet in a patient with FPLD who showed remarkable benefit after 16 weeks of the intervention.

Clinical Case

This is a 38-year-old female diagnosed with FPLD type 2, with LMNA p. R482Q mutation who had a history of recurrent pancreatitis, NAFLD, impaired ovulation, and diabetes mellitus. She had a short-term goal of improving her metabolic state rapidly to achieve pregnancy by IVF. Upon presentation, body weight was 81.6kg, BMI 23.2kg/m2, HbA1c 7.7% and triglycerides 215mg/dL. She was started on very-low calorie diet (VLCD) (800 kcal/day) employing a formula total liquid diet meal replacement: 160 kcal per shake (18g total carbohydrates, 15g net carbohydrates, 3.5g fat, and 16g protein) with the goal to reduce body weight by 15% as has been described for patients in the University of Michigan Weight Management Program. After 1 week on diet, she discontinued glucose and lipid lowering therapies. After 12-weeks of VLCD followed by 4 weeks of transition to low-calorie diet (incorporating 3 meal replacements, one meal, and ad libitum non-starchy vegetables), we observed 12.3kg of body weight and a 1.4% decrease in HbA1c. Insulin resistance was reduced by 66.7 and 61.5% (HOMA-IR and ADIPO-IR, respectively). Fasting insulinemia was reduced by 64.2%. We also performed an OGTT with 75g of glucose for 180 min and observed a decrease in the area under the curve of insulin (-33.2%), triglycerides (-40.7%), and free fatty acids (-34%). DEXA scan showed a reduction in total body fat of 16%. Liver fat via the chemical shift MRI method was reduced from 10±2% to 2±1%. She did not experience any adverse events during the intervention. More importantly, her egg retrieval rate and egg quality during IVF were far superior to past attempts.

Conclusion

This case illustrates the strong effect and safety of a VLCD intervention in a patient with lipodystrophy. We observed rapid reduction in body weight, and in insulin resistance leading to the remission of diabetes mellitus, hypertriglyceridemia and fatty liver disease. Moreover, there was improved response to ovulation induction. Our data encourage the use of this medical approach for other patients with similar metabolic and reproductive abnormalities due to adipose tissue distribution abnormalities.

Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m., Sunday, June 12, 2022 12:36 p.m. - 12:41 p.m.