Özdemir Zeynep Canan, Düzenli Kar Yeter, Şöhret Nurullah Cihan, Kebapçı Mahmut, Bör Özcan
Division of Pediatric Hematology/Oncology, Department of Pediatrics, Eskişehir Osmangazi University Faculty of Medicine.
Department of Pediatrics, Eskişehir Osmangazi University Faculty of Medicine.
Drug Discov Ther. 2017 Jul 31;11(3):161-164. doi: 10.5582/ddt.2017.01025. Epub 2017 Jul 25.
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.
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。