Sarıalioğlu Faik, Yazıcı Nalan, Erbay Ayşe, Boyvat Fatih, Demir Şenay, Özçay Figen, Uslu Nihal
Department of Pediatric Oncology-Hematology, Baskent University Faculty of Medicine, Ankara, Turkey.
Exp Clin Transplant. 2017 Mar;15(Suppl 2):74-78. doi: 10.6002/ect.TOND16.L19.
Propranolol was first used in 2008 to treat hemangioma; its efficacy and safety have since changed the classical treatment indications. Infantile hepatic hemangioma presents as a spectrum of clinical conditions varying from simple asymptomatic lesions to lethal complications. Tufted hemangioma and Kaposiform hemangioendothelioma are congenital vascular tumors that lead to Kasabach-Merritt syndrome. Hemangiomas, like pure arteriovenous malformations, can cause hyperdynamic heart failure, and diffuse nodular-type hemangiomas can present with hypothyroidism. Respiratory problems and hepatic failure can be associated with diffuse nodular-type liver hemangiomas. There is a spectrum of approaches to management, varying from "watchful waiting" to liver transplant. In the age of propranolol, there has been a prominent change in the infantile hepatic hemangioma treatment algorithm. Our suggestion is early treatment with 3 mg/kg/day propranolol plus 1.0 to1.5 mg/kg/day prednisolone in all patients. This protocol is the most effective strategy for type 3 infantile hepatic hemangioma. Approximately one-third of patients with abdominal compartment syndrome in the era before propranolol treatment required liver transplant; this new treatment obviates transplant for many of these patients.
普萘洛尔于2008年首次用于治疗血管瘤；自那时起，其疗效和安全性改变了经典的治疗指征。婴儿肝血管瘤表现为一系列临床情况，从简单的无症状病变到致命并发症不等。丛状血管瘤和卡波西样血管内皮瘤是导致卡萨巴赫-梅里特综合征的先天性血管肿瘤。血管瘤，如同单纯动静脉畸形一样，可引起高动力性心力衰竭，弥漫性结节型血管瘤可伴有甲状腺功能减退。呼吸问题和肝衰竭可能与弥漫性结节型肝血管瘤有关。治疗方法多种多样，从“观察等待”到肝移植。在普萘洛尔时代，婴儿肝血管瘤的治疗方案有了显著变化。我们的建议是，所有患者均采用3毫克/千克/天的普萘洛尔加1.0至1.5毫克/千克/天的泼尼松龙进行早期治疗。该方案是治疗3型婴儿肝血管瘤最有效的策略。在普萘洛尔治疗时代之前，约三分之一患有腹腔间隔室综合征的患者需要进行肝移植；这种新的治疗方法使许多此类患者无需进行移植。
