Ray Gautam, Das Kausik, Sarkar Avik, Bose Debarshi, Halder Prasenjit
Division of Pediatric Gastroenterology, School of Digestive and Liver Disease (SDLD), Institute of Post Graduate Medical Education & Research (IPGMER), 244, AJC Bose Road, Kolkata, PIN-700020, West Bengal, India.
Department of Hepatology, SDLD, IPGMER, 244, AJC Bose Road, Kolkata, PIN-700020, West Bengal, India.
J Clin Exp Hepatol. 2023 Jul-Aug;13(4):707-712. doi: 10.1016/j.jceh.2023.02.005. Epub 2023 Feb 15.
Infantile hepatic hemangioma (IHH) is the most common benign liver tumor in children, and multifocal and diffuse tumors often become life-threatening, necessitating therapy. Propranolol is now considered the first choice of therapy with ample data in Caucasian children. We present a series of nine Indian children with multifocal (n = 5) and diffuse (n = 4) IHH treated with propranolol monotherapy.
This was a retrospective clinical data-based single-center study. Propranolol was used at a median dose of 3.2 mg/kg/day (range 3-3.3 mg/kg/day) for a median duration of 12 months (range 6-32 months).
The presentations of IHH (either in isolation or combination) were hypothyroidism in six patients (diagnosed by elevated serum TSH levels), heart failure in three (diagnosed based on clinical and echocardiographic features), and imaging evidence of macrovascular shunting in two patients. A good response to propranolol monotherapy (with a median dose of 3.2 mg/kg/day for a median duration of 12 months) was observed in eight patients, with a poor response in one. One patient experienced recurrence but responded adequately to propranolol retreatment.
Our data reiterate the excellent response (88.9% responded) and safety profile with propranolol monotherapy in complicated IHH and strengthen the data in Asian (Indian) children. It includes the maximum proportion of complicated IHH treated with propranolol in East and South Asia, and the largest series from India.
婴儿肝血管瘤（IHH）是儿童最常见的肝脏良性肿瘤，多灶性和弥漫性肿瘤常危及生命，需要进行治疗。普萘洛尔目前被认为是首选治疗药物，在白种儿童中有大量数据支持。我们报告了一组9例接受普萘洛尔单药治疗的印度儿童，其中多灶性（n = 5）和弥漫性（n = 4）IHH。
这是一项基于回顾性临床数据的单中心研究。普萘洛尔的中位剂量为3.2 mg/kg/天（范围3 - 3.3 mg/kg/天），中位治疗时间为12个月（范围6 - 32个月）。
IHH的表现（单独或合并出现）为6例甲状腺功能减退（通过血清促甲状腺激素水平升高诊断）、3例心力衰竭（根据临床和超声心动图特征诊断）以及2例有大血管分流的影像学证据。8例患者对普萘洛尔单药治疗（中位剂量3.2 mg/kg/天，中位治疗时间12个月）反应良好，1例反应不佳。1例患者出现复发，但再次使用普萘洛尔治疗反应良好。
我们的数据再次证实普萘洛尔单药治疗复杂IHH时具有良好的反应（88.9%有反应）和安全性，并加强了亚洲（印度）儿童的数据。该研究纳入了东亚和南亚接受普萘洛尔治疗的复杂IHH的最大比例病例，也是来自印度的最大系列研究。
