Unidad de Lípidos, Servicio de Medicina Interna, Hospital Virgen de la Victoria, Málaga, España.
Unidad de Lípidos, Servicio de Medicina Interna, Hospital Virgen de la Victoria, Málaga, España; Departamento de Medicina y Dermatología, Universidad de Málaga, Málaga, España; Instituto de Investigación Biomédica de Málaga, Málaga, España.
Clin Investig Arterioscler. 2021 May;33 Suppl 2:75-79. doi: 10.1016/j.arteri.2021.01.004.
Fasting chylomicronaemia appears in type V (multifactorial chylomicronaemia syndrome, MCS), and in type I (familial chylomicronaemia syndrome, FCS). MCS needs to be treated as in any general hypertriglyceridaemia: low-calorie diet, avoid sugar and alcohol, reduce body weight, control of diabetes and, in some cases, common lipid lowering-drugs, such as fibrates or omega-3 fatty acids. For type I HLP, FCS, patients should adhere to a strict very low fat diet, usually less than 15-20 g per day. In spite of this, many patients with FCS suffer from recurrent abdominal pain and/or acute pancreatitis. Volanesorsen, an antisense oligonucleotide against apolipoprotein C-III, is the only drug approved to control the disease. As shown in the APPROACH study, the administration of volanesorsen at a weekly dose of 285 mg induced at three month a reduction of triglycerides of 77% (primary end-point) and a reduction of 1712 mg/dL from the baseline. Among patient receiving volanesorsen, 77% reached a fasting triglyceride value below 750 mg/dL. The most frequent side effects were a skin reaction at injection site and low platelet levels, which should be monitored.
空腹乳糜微粒血症见于 V 型（多因素乳糜微粒血症综合征，MCS）和 I 型（家族性乳糜微粒血症综合征，FCS）。MCS 需要像治疗任何一般高甘油三酯血症一样进行治疗：低热量饮食，避免糖和酒精，减轻体重，控制糖尿病，在某些情况下，使用常见的降脂药物，如贝特类或ω-3 脂肪酸。对于 I 型高脂蛋白血症，FCS 患者应坚持严格的低脂饮食，通常每天少于 15-20 克。尽管如此，许多 FCS 患者仍会反复发作腹痛和/或急性胰腺炎。Volanesorsen 是一种针对载脂蛋白 C-III 的反义寡核苷酸，是唯一获准控制该疾病的药物。如 APPROACH 研究所示，每周 285 毫克的 volanesorsen 给药在三个月时可使甘油三酯降低 77%（主要终点），并使基线时的甘油三酯降低 1712 毫克/分升。在接受 volanesorsen 治疗的患者中，77%的患者空腹甘油三酯值低于 750 毫克/分升。最常见的副作用是注射部位皮肤反应和血小板计数低，应进行监测。
