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[先天性肝血管瘤继发卡萨巴赫-梅里特综合征的普萘洛尔治疗。临床病例]

[Propranolol treatment in Kasabach-Merritt Syndrome secondary to congenital hepatic hemangioma. Clinical case].

作者信息

Selzer Soria Erika M, Malla Ivone

机构信息

Sección Hepatología Infantil y Trasplante Hepático, Hospital Universitario Austral, Pilar, provincia de Buenos Aires, Argentina.

出版信息

Arch Argent Pediatr. 2021 Feb;119(1):e65-e69. doi: 10.5546/aap.2021.e65.

Abstract

Hepatic hemangioma is the most common benign liver tumor. It can be congenital or infantile with different outcomes and complications. The clinical manifestation varies from asymptomatic to severe conditions with heart failure, Kasabach- Merritt syndrome or compartment syndrome. Diagnosis depends on medical history and imaging studies, especially ultrasound and Doppler examination in experienced hands. Differential diagnosis is essential with other hepatic lesions, mainly hepatoblastoma. In symptomatic patients, propranolol emerges as the first line treatment with good results and low frequency of adverse effects. We present the case of a newborn with a hepatic hemangioma and Kasabach-Merritt syndrome with an excellent response and tolerance to propranolol.

摘要

肝血管瘤是最常见的肝脏良性肿瘤。它可以是先天性或婴儿期的,有不同的转归和并发症。临床表现从无症状到出现心力衰竭、卡萨巴赫-梅里特综合征或骨筋膜室综合征等严重情况不等。诊断取决于病史和影像学检查,尤其是经验丰富者进行的超声和多普勒检查。与其他肝脏病变,主要是肝母细胞瘤进行鉴别诊断至关重要。对于有症状的患者,普萘洛尔成为一线治疗药物,效果良好且不良反应发生率低。我们报告一例患有肝血管瘤和卡萨巴赫-梅里特综合征的新生儿,其对普萘洛尔有极佳的反应和耐受性。

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