Espat J, Chamberlain R S, Sklar C, Blumgart L H
Hepatobiliary Service, Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, N.Y., USA.
Dig Surg. 2000;17(6):640-643. doi: 10.1159/000051977.
Turner's syndrome is a genetic disorder of females with well-described karyotypic abnormalities and phenotypic features. Recombinant human growth hormone (HGH) therapy is one component of a hormonal treatment strategy for these patients and is used to promote sexual maturity and to increase height.
Literature review of hepatic complications following the initiation of growth hormone therapy for patients with Turner's syndrome, and case report presentation of a 13-year-old female with Turner's syndrome developing a hepatic adenoma following 3 years of HGH treatment.
The association between Turner's syndrome and HGH treatment-associated hepatic adenoma has not been described previous to this report. In this patient, surgical resection was contraindicated and the patient was successfully treated by hepatic artery embolization. The unique management issues relating to this case, and a possible association between HGH therapy and the development of hepatic adenoma are discussed.
This work represents the first documentation of a hepatic adenoma developing in a patient with Turner's syndrome following HGH treatment, and suggests a novel and causal association between HGH treatment and the development of hepatic adenoma in patients with Turner's syndrome.
特纳综合征是一种女性遗传性疾病,具有明确的核型异常和表型特征。重组人生长激素(HGH)治疗是这些患者激素治疗策略的一个组成部分,用于促进性成熟和增加身高。
对特纳综合征患者开始生长激素治疗后肝脏并发症的文献进行综述,并报告一名13岁特纳综合征女性在接受3年HGH治疗后发生肝腺瘤的病例。
本报告之前尚未描述特纳综合征与HGH治疗相关肝腺瘤之间的关联。在该患者中,手术切除禁忌,患者通过肝动脉栓塞成功治疗。讨论了与该病例相关的独特管理问题,以及HGH治疗与肝腺瘤发生之间可能的关联。
这项工作首次记录了特纳综合征患者在接受HGH治疗后发生肝腺瘤,并提示HGH治疗与特纳综合征患者肝腺瘤发生之间存在新的因果关联。