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β受体阻滞剂和类固醇疗法治疗婴儿肝血管内皮瘤

Beta blocker and steroid therapy in the treatment of infantile hepatic hemangioendothelioma.

作者信息

Özdemir Zeynep Canan, Düzenli Kar Yeter, Şöhret Nurullah Cihan, Kebapçı Mahmut, Bör Özcan

机构信息

Division of Pediatric Hematology/Oncology, Department of Pediatrics, Eskişehir Osmangazi University Faculty of Medicine.

Department of Pediatrics, Eskişehir Osmangazi University Faculty of Medicine.

出版信息

Drug Discov Ther. 2017 Jul 31;11(3):161-164. doi: 10.5582/ddt.2017.01025. Epub 2017 Jul 25.

Abstract

Infantile hepatic hemangioendothelioma (IHHE) is the most common benign vascular liver tumor and typically occurs during the first 6 months of life. A 4-month-old male patient presented with abdominal distention. A physical examination revealed massive hepatomegaly. Liver enzyme levels were normal. The alpha fetoprotein level was 1,323 mg/dL (6-1,000). Abdominal magnetic resonance imaging (MRI) showed multiple, well-defined and hyperintense nodular lesions in the liver. MRI findings suggested IHHE. The thyroid stimulating hormone (TSH) level was high (177.2 µU/mL). He was started on sodium levothyroxine 50 μg daily. The patient has hypoxemia due to abdominal distention during the follow-up period. Oral methylprednisolone therapy was started at a dose of 2.5 mg/kg/dose, and propranolol at a dose of 1 mg/kg/dose, bid. Fifteen days later his TSH level remained elevated at 212.3 μU/mL despite repeatedly increasing the dose of levothyroxine up to 200 μg/daily. One month after the initial presentation, his TSH level was reduced to 11.28 µU/mL. We observed a marked improvement in abdominal distention and respiratory distress within 15 days and an average reduction of 50% in the lesion diameters after a month. Despite its benign nature, IHHE may lead to development of complications. Steroid and propranolol treatment may be useful in in the management of emergency complications.

摘要

婴儿肝血管内皮瘤(IHHE)是最常见的肝脏良性血管肿瘤,通常发生在出生后的前6个月。一名4个月大的男性患者出现腹胀。体格检查发现肝脏肿大明显。肝酶水平正常。甲胎蛋白水平为1323mg/dL(6 - 1000)。腹部磁共振成像(MRI)显示肝脏内有多个边界清晰的高信号结节性病变。MRI表现提示为IHHE。促甲状腺激素(TSH)水平较高(177.2µU/mL)。开始给他每天服用50μg左甲状腺素钠。在随访期间,患者因腹胀出现低氧血症。开始口服甲泼尼龙治疗,剂量为2.5mg/kg/次,普萘洛尔剂量为1mg/kg/次,每日两次。15天后,尽管多次将左甲状腺素钠剂量增加至每日200μg,但其TSH水平仍升高至212.3µU/mL。首次就诊1个月后,其TSH水平降至11.28µU/mL。我们观察到15天内腹胀和呼吸窘迫有明显改善,1个月后病变直径平均缩小50%。尽管IHHE本质上是良性的,但可能会导致并发症的发生。类固醇和普萘洛尔治疗可能有助于处理紧急并发症。

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