Liviskie Caren J, Brennan Colleen C, McPherson Christopher C, Vesoulis Zachary A
J Pediatr Pharmacol Ther. 2020;25(2):155-162. doi: 10.5863/1551-6776-25.2.155.
Lymphatic malformations in neonates often manifest as a chylothorax, and although rare, morbidity and mortality can be significant. First-line treatment with medium-chain triglyceride-enriched formulas, or enteric rest with total parenteral nutrition, are not always successful. We describe the case of a premature neonate with trisomy 21 who presented with bilateral pleural effusions and a pericardial effusion that worsened with the initiation of enteral nutrition. Clinical improvement was not seen until the initiation of treatment with oral propranolol at a maximum dosage of 0.5 mg/kg/day divided every 8 hours with extubation 8 days after propranolol initiation. Two case reports have described the use of propranolol in similar patients receiving 2 mg/kg/day; however, our experience is the first to report treatment success at a much lower dose. A review of the literature for alternative medication treatments uncovered numerous case reports and series documenting variable results with incongruent definitions of treatment success in a diverse patient population. The rarity of this disease state makes accrual of patients difficult and more robust treatment data unlikely. Therefore, selection of the optimal adjunctive treatment must be based on individual patient and disease state characteristics as well as safety and efficacy profile of the medication.
新生儿淋巴管畸形常表现为乳糜胸,虽然罕见,但发病率和死亡率可能很高。使用富含中链甘油三酯的配方奶进行一线治疗,或通过全胃肠外营养进行肠道休息,并不总是成功的。我们描述了一例患有21三体综合征的早产儿病例,该患儿出现双侧胸腔积液和心包积液,在开始肠内营养后病情恶化。直到开始使用口服普萘洛尔治疗,最大剂量为0.5mg/kg/天,每8小时一次,在开始使用普萘洛尔8天后拔管,才出现临床改善。有两篇病例报告描述了在类似患者中使用普萘洛尔的剂量为2mg/kg/天;然而,我们的经验是首次报告在低得多的剂量下治疗成功。对替代药物治疗的文献综述发现了大量病例报告和系列研究,记录了不同患者群体中治疗成功定义不一致的情况下的不同结果。这种疾病状态的罕见性使得患者的积累变得困难,更可靠的治疗数据也不太可能获得。因此,选择最佳辅助治疗必须基于个体患者和疾病状态特征以及药物的安全性和有效性。