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Focal nodular hyperplasia of the liver: an emerging complication of hematopoietic SCT in children

机译:肝脏局灶性结节性增生:儿童造血SCT的新兴并发症

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Hepatic focal nodular hyperplasia (FNH) is a nonmalignant condition rarely affecting children previously treated for cancer, especially those who received hematopoietic SCT (HSCT). Some aspects of its pathogenesis still remain unclear and a strong association with specific risk factors has not yet been identified. We report here a single institution's case series of 17 patients who underwent HSCT and were diagnosed with FNH, analyzing retrospectively their clinical features and the radiological appearance of their hepatic lesions. We aimed to compare the diagnostic accuracy of ultrasound (US) and magnetic resonance imaging (MRI) and to explore the role of transient elastography (FibroScan) to evaluate the degree of hepatic fibrosis in FNH patients. Our analysis showed an association of FNH with age at transplant <= 12 years (hazard ratio (HR) 9.10); chronic GVHD (HR 2.99); hormone-replacement therapy (HR 4.02) and abdominal radiotherapy (HR 4.37). MRI proved to be a more accurate diagnostic tool compared with US. Nine out of 12 patients who underwent FibroScan showed hepatic fibrosis. Our study points out that FNH is an emerging complication of HSCT, which requires a lifelong surveillance to follow its course in cancer patients.
机译:肝局灶性结节性增生(FNH)是一种非恶性疾病,很少影响以前接受过癌症治疗的儿童,特别是那些接受造血SCT(HSCT)治疗的儿童。其发病机制的某些方面仍不清楚,并且尚未确定与特定危险因素的强相关性。我们在这里报告一家机构的17例行HSCT并被诊断为FNH的患者的病例系列,回顾性分析其临床特征和肝病变的放射学表现。我们旨在比较超声(US)和磁共振成像(MRI)的诊断准确性,并探讨瞬时弹性成像(FibroScan)在评估FNH患者肝纤维化程度中的作用。我们的分析表明,FNH与移植年龄<= 12岁(危险比(HR)9.10)有关。慢性GVHD(HR 2.99);激素替代疗法(HR 4.02)和腹部放疗(HR 4.37)。与美国相比,MRI被证明是更准确的诊断工具。在接受FibroScan扫描的12例患者中,有9例显示肝纤维化。我们的研究指出,FNH是HSCT的一种新兴并发症,需要对癌症患者进行终生监测以遵循其病程。

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