Very long-chain acyl-coenzyme A (CoA) dehydrogenase (VLCAD) deficiency is an autosomal recessive fatty acid oxidation disorder characterized by rhabdomyolysis, hypoglycemia and cardiomyopathy. The general treatment approach in adult patients is based on the prevention of catabolism. High carbohydrate, low fat diet and supplementation of medium-chain triglycerides are essential in the treatment. There is little experience with pregnancy follow-up in this patient group. We present a complicated peripartum course and successful management in a patient with VLCAD deficiency. Although high-dose glucose infusion was initiated, creatine kinase levels significantly increased in the immediate postpartum period, but the patient remained asymptomatic and rhabdomyolysis resolved rapidly after increasing the glucose infusion rate.

极长链酰基辅酶 A (CoA) 脱氢酶 (VLCAD) 缺乏症是一种常染色体隐性脂肪酸氧化障碍，其特征是横纹肌溶解症、低血糖症和心肌病。成人患者的一般治疗方法是预防分解代谢。高碳水化合物、低脂肪饮食和补充中链甘油三酯在治疗中是必不可少的。该患者组的妊娠随访经验很少。我们在 VLCAD 缺陷患者中介绍了复杂的围产期过程和成功的管理。虽然开始大剂量葡萄糖输注，但产后即刻肌酸激酶水平显着升高，但患者仍无症状，增加葡萄糖输注速度后横纹肌溶解迅速消退。