Focal nodular hyperplasia in an adolescent with glycogen storage disease type I with mesocaval shunt operation in childhood: a case report and review of the literature
Focal nodular hyperplasia in an adolescent with glycogen storage disease type I with mesocaval shunt operation in childhood: a case report and review of the literature
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儿童期I型糖原贮积病伴腔静脉分流术致局灶性结节增生一例并文献复习
DOI：
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发表时间：
1996
期刊：
影响因子：
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通讯作者：
M. Sakurai
中科院分区：
文献类型：
--
作者：
H. Sakatoku;Yoshihumi Hirokawa;M. Inoue;M. Kojima;T. Yabana;M. Sakurai
随着I型糖原累积病患者存活时间的延长，肝肿瘤的病例越来越多。一个16岁的男孩与糖原累积病I型进行了评估，为多发性肝肿瘤。他在9个月大时根据临床特征被诊断，并在5岁时接受了肠腔分流术。其中一个肿块的活检显示局灶性结节增生。与肝腺瘤或恶性肿瘤患者相比，I型糖原累积病患者中这种情况并不常见。与其他肿瘤相比，门腔静脉分流与局灶性结节增生的相关性是显著的。高雌激素浓度或性激素结合球蛋白的环境伴随分流术可引起I型糖原累积病患者肝脏局灶性结节增生。
As patients with glycogen storage disease type I survive longer, cases with hepatic tumor have been increasingly documented. A 16 year old boy with glycogen storage disease type I was evaluated for multiple liver tumors. He was diagnosed on clinical features at 9 months of age and underwent a mesocaval shunt operation at 5 years of age. The biopsy of one of the masses showed focal nodular hyperplasia. This is uncommon in patients with glycogen storage disease type I, compared to those with adenoma or malignant hepatic tumor. The association of a portacaval shunt with focal nodular hyperplasia is significant compared to other tumors. An environment of high estrogen concentration or sex hormone binding globulin accompanied by shunt operation may cause focal nodular hyperplasia to develop in the liver of patients with glycogen storage disease type I.
通过饮食疗法消退 Ia 型糖原贮积病中的肝腺瘤。
影响因子：
29.4
作者：
Parker,P;Burr,I;Slonim,A;Ghishan,FK;Greene,H
通讯作者：
Greene,H