Dietary Intake during 56 Weeks of a Low-Fat Diet for Lomitapide Treatment in Japanese Patients with Homozygous Familial Hypercholesterolemia

Abstract

Aim:Lomitapide is an oral inhibitor of the microsomal triglyceride transfer protein used to treat homozygous familial hypercholesterolemia (HoFH); patients require a low-fat diet to minimize gastrointestinal adverse effects and dietary supplements to prevent nutrient deficiencies. We investigated the diet and nutritional status during lomitapide treatment. Methods: Japanese patients with HoFH, who were in a phase 3 trial of lomitapide, were instructed to start low-fat diets with supplements of vitamin E and essential fatty acids 6 weeks before starting lomitapide treatment. Dietary education was conducted by registered dietitians 16 times during the study period, which included a pre-treatment run-in phase (Weeks 6-0), a lomitapide treatment efficacy phase (Weeks 0-26) and a safety phase (Weeks 26-56). Two-day dietary records were collected at each dietary counseling session. Anthropometric and biochemical parameters were measured at Weeks 0, 26 and 56. Results: Eight patients completed the 56 weeks of lomitapide treatment. Their median energy intakes derived from lipids were 19.2% and 17.9% during the efficacy and safety phases, respectively. "Fats and oils" intakes, and "Fatty meat and poultry" intakes in two patients, were successfully reduced to achieve low-fat diets. Although intakes of energy, fatty acids and fat-soluble vitamins did not differ significantly among phases, body weight, serum fatty acid levels and vitamin E concentrations were decreased at Week 26 as compared with Week 0. Conclusion: HoFH patients can adhere to low-fat diets with ongoing dietary counseling. Instructions about intakes of energy, fatty acids and fat-soluble vitamins, as well as periodic evaluations of nutritional status, are necessary.

The microsomal triglyceride transfer protein inhibitor lomitapide improves vascular function in mice with obesity

“…Interestingly, treatment of HFD mice with lomitapide significantly decreased body weight and the percentage of fat mass and increased the percentage of lean mass in comparison to mice on HFD alone. Our findings are in accordance with a previous clinical study conducted with Japanese patients showing that treatment with lomitapide significantly induced a decrease in body weight (26). A phase 3 study of lomitapide treatment in patients with HoFH showed that decreased body weight was not associated with energy expenditure or food intake changes (27).…”

Guidelines for the Diagnosis and Treatment of Adult Familial Hypercholesterolemia 2022

“…A study was conducted in HoFH patients in Japan, and it is now on the market 99) . However, since the frequencies of adverse events such as fatty liver and diarrhea are high with MTP inhibitor, it is essential to strictly control fat and alcohol intake 100) . Probucol reportedly exerts a certain Blood access in lipoprotein apheresis is common via the cubital or brachial vein.…”

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“…Our data are in agreement with a clinical study conducted on Japanese patients with HoFH. In this study, the treatment with lomitapide significantly decreased the body weight of the patients [ 49 ]. Additionally, in Zucker fatty rats, lomitapide treatment decreased their body weight and food intake, this was accompanied by a significant improvement in glucose tolerance [ 36 ].…”