Abstract：
BACKGROUND: Hepatic nodular regenerative hyperplasia (NRH), a nonspecific change in the liver parenchyma, is very rare in children. Hepatic microvascular changes may be the cause, as these vascular changes are uncommon in children. Antiphospholipid syndrome (APS), an autoimmune disease characterized by vascular thromboembolism, is extremely unusual in children.
METHODS: A 13-year-old girl who presented with abdominal pain and elevated liver enzymes was transferred to our hospital. Abdominal computed tomography and magnetic resonance imaging showed a massive mesenteric venous thrombus and a malignant mass with liver metastasis.
METHODS: Her immunological profile was positive for antinuclear antibodies (ANA) at a titer of 1/160 (nucleolar pattern), anticardiolipin antibodies (aCL) immunoglobulin G, and anti-histone antibody. A liver biopsy revealed hepatic NRH.
METHODS: The patient was initially started on heparin upon hospitalization and switched to warfarin and a vitamin K antagonist and continued treatment with international normalized ratio monitoring.
RESULTS: Her symptoms improved after 9 months of anticoagulation therapy.
CONCLUSIONS: In the presence of hepatic NRH or vascular thrombosis in children, we recommend that APS be differentially diagnosed using lupus anticoagulant and aCL and appropriate management be implemented.
METHODS: A 13-year-old girl who presented with abdominal pain and elevated liver enzymes was transferred to our hospital. Abdominal computed tomography and magnetic resonance imaging showed a massive mesenteric venous thrombus and a malignant mass with liver metastasis.
METHODS: Her immunological profile was positive for antinuclear antibodies (ANA) at a titer of 1/160 (nucleolar pattern), anticardiolipin antibodies (aCL) immunoglobulin G, and anti-histone antibody. A liver biopsy revealed hepatic NRH.
METHODS: The patient was initially started on heparin upon hospitalization and switched to warfarin and a vitamin K antagonist and continued treatment with international normalized ratio monitoring.
RESULTS: Her symptoms improved after 9 months of anticoagulation therapy.
CONCLUSIONS: In the presence of hepatic NRH or vascular thrombosis in children, we recommend that APS be differentially diagnosed using lupus anticoagulant and aCL and appropriate management be implemented.
摘要：
背景：肝结节性再生增生（NRH），肝实质的非特异性改变,在儿童中非常罕见。肝脏微血管改变可能是原因，因为这些血管变化在儿童中并不常见。抗磷脂综合征（APS），一种以血管血栓栓塞为特征的自身免疫性疾病,在儿童中非常不寻常。
方法：一名13岁女孩出现腹痛和肝酶升高，被转到我院。腹部计算机断层扫描和磁共振成像显示大量肠系膜静脉血栓和恶性肿块伴肝脏转移。
方法：她的免疫谱对抗核抗体（ANA）呈阳性，滴度为1/160（核仁模式），抗心磷脂抗体（aCL）免疫球蛋白G，和抗组蛋白抗体。肝活检显示肝NRH。
方法：患者最初在住院后开始接受肝素治疗，转用华法林和维生素K拮抗剂，并在国际标准化比率监测下继续治疗。
结果：抗凝治疗9个月后，患者症状有所改善。
结论：在儿童存在肝NRH或血管血栓的情况下，我们建议使用狼疮抗凝药和aCL对APS进行鉴别诊断,并实施适当的管理.
方法：一名13岁女孩出现腹痛和肝酶升高，被转到我院。腹部计算机断层扫描和磁共振成像显示大量肠系膜静脉血栓和恶性肿块伴肝脏转移。
方法：她的免疫谱对抗核抗体（ANA）呈阳性，滴度为1/160（核仁模式），抗心磷脂抗体（aCL）免疫球蛋白G，和抗组蛋白抗体。肝活检显示肝NRH。
方法：患者最初在住院后开始接受肝素治疗，转用华法林和维生素K拮抗剂，并在国际标准化比率监测下继续治疗。
结果：抗凝治疗9个月后，患者症状有所改善。
结论：在儿童存在肝NRH或血管血栓的情况下，我们建议使用狼疮抗凝药和aCL对APS进行鉴别诊断,并实施适当的管理.
