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

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 t...

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Autores principales: Kameyama, Noriko, Maruyama, Chizuko, Kitagawa, Fuyuha, Nishii, Kazunobu, Uenomachi, Kaori, Katayama, Yayoi, Koga, Hiromi, Chikamoto, Naoko, Kuwata, Yuko, Torigoe, Junko, Arimoto, Masako, Tokumaru, Toshiaki, Ikewaki, Katsunori, Nohara, Atsushi, Otsubo, Yoshihiko, Yanagi, Koji, Yoshida, Masayuki, Harada-Shiba, Mariko
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Japan Atherosclerosis Society 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6308262/
https://www.ncbi.nlm.nih.gov/pubmed/29899183
http://dx.doi.org/10.5551/jat.44107
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author Kameyama, Noriko
Maruyama, Chizuko
Kitagawa, Fuyuha
Nishii, Kazunobu
Uenomachi, Kaori
Katayama, Yayoi
Koga, Hiromi
Chikamoto, Naoko
Kuwata, Yuko
Torigoe, Junko
Arimoto, Masako
Tokumaru, Toshiaki
Ikewaki, Katsunori
Nohara, Atsushi
Otsubo, Yoshihiko
Yanagi, Koji
Yoshida, Masayuki
Harada-Shiba, Mariko
author_facet Kameyama, Noriko
Maruyama, Chizuko
Kitagawa, Fuyuha
Nishii, Kazunobu
Uenomachi, Kaori
Katayama, Yayoi
Koga, Hiromi
Chikamoto, Naoko
Kuwata, Yuko
Torigoe, Junko
Arimoto, Masako
Tokumaru, Toshiaki
Ikewaki, Katsunori
Nohara, Atsushi
Otsubo, Yoshihiko
Yanagi, Koji
Yoshida, Masayuki
Harada-Shiba, Mariko
author_sort Kameyama, Noriko
collection PubMed
description 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 pretreatment 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.
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spelling pubmed-63082622019-01-05 Dietary Intake during 56 Weeks of a Low-Fat Diet for Lomitapide Treatment in Japanese Patients with Homozygous Familial Hypercholesterolemia Kameyama, Noriko Maruyama, Chizuko Kitagawa, Fuyuha Nishii, Kazunobu Uenomachi, Kaori Katayama, Yayoi Koga, Hiromi Chikamoto, Naoko Kuwata, Yuko Torigoe, Junko Arimoto, Masako Tokumaru, Toshiaki Ikewaki, Katsunori Nohara, Atsushi Otsubo, Yoshihiko Yanagi, Koji Yoshida, Masayuki Harada-Shiba, Mariko J Atheroscler Thromb Original Article 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 pretreatment 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. Japan Atherosclerosis Society 2019-01-01 /pmc/articles/PMC6308262/ /pubmed/29899183 http://dx.doi.org/10.5551/jat.44107 Text en 2019 Japan Atherosclerosis Society This article is distributed under the terms of the latest version of CC BY-NC-SA defined by the Creative Commons Attribution License.http://creativecommons.org/licenses/by-nc-sa/3.0/
spellingShingle Original Article
Kameyama, Noriko
Maruyama, Chizuko
Kitagawa, Fuyuha
Nishii, Kazunobu
Uenomachi, Kaori
Katayama, Yayoi
Koga, Hiromi
Chikamoto, Naoko
Kuwata, Yuko
Torigoe, Junko
Arimoto, Masako
Tokumaru, Toshiaki
Ikewaki, Katsunori
Nohara, Atsushi
Otsubo, Yoshihiko
Yanagi, Koji
Yoshida, Masayuki
Harada-Shiba, Mariko
Dietary Intake during 56 Weeks of a Low-Fat Diet for Lomitapide Treatment in Japanese Patients with Homozygous Familial Hypercholesterolemia
title Dietary Intake during 56 Weeks of a Low-Fat Diet for Lomitapide Treatment in Japanese Patients with Homozygous Familial Hypercholesterolemia
title_full Dietary Intake during 56 Weeks of a Low-Fat Diet for Lomitapide Treatment in Japanese Patients with Homozygous Familial Hypercholesterolemia
title_fullStr Dietary Intake during 56 Weeks of a Low-Fat Diet for Lomitapide Treatment in Japanese Patients with Homozygous Familial Hypercholesterolemia
title_full_unstemmed Dietary Intake during 56 Weeks of a Low-Fat Diet for Lomitapide Treatment in Japanese Patients with Homozygous Familial Hypercholesterolemia
title_short Dietary Intake during 56 Weeks of a Low-Fat Diet for Lomitapide Treatment in Japanese Patients with Homozygous Familial Hypercholesterolemia
title_sort dietary intake during 56 weeks of a low-fat diet for lomitapide treatment in japanese patients with homozygous familial hypercholesterolemia
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6308262/
https://www.ncbi.nlm.nih.gov/pubmed/29899183
http://dx.doi.org/10.5551/jat.44107
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