Department of Pediatrics, Jeonbuk National University Medical School and Hospital, Research Institute of Clinical Medicine of Jeonbuk National University - Biomedical research Institute of Jeonbuk National University Hospital, Jeonju, Korea.
Medicine (Baltimore). 2021 Dec 3;100(48):e28105. doi: 10.1097/MD.0000000000028105.
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.
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.
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.
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.
Her symptoms improved after 9 months of anticoagulation therapy.
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.
肝结节性再生性增生（NRH）是一种肝脏实质的非特异性改变，在儿童中非常罕见。肝微血管变化可能是其病因，因为这些血管变化在儿童中并不常见。抗磷脂综合征（APS）是一种以血管血栓栓塞为特征的自身免疫性疾病，在儿童中极为罕见。
一名 13 岁女孩因腹痛和肝酶升高被转至我院。腹部计算机断层扫描和磁共振成像显示广泛肠系膜静脉血栓形成和肝转移的恶性肿块。
她的免疫检查结果显示抗核抗体（ANA）滴度为 1/160（核仁型）、抗心磷脂抗体（aCL）IgG 和抗组蛋白抗体阳性。肝脏活检显示肝 NRH。
患者住院后最初接受肝素治疗，随后转为华法林和维生素 K 拮抗剂，并继续使用国际标准化比值监测进行治疗。
抗凝治疗 9 个月后，她的症状改善。
在儿童存在肝 NRH 或血管血栓形成时，建议使用狼疮抗凝物和 aCL 进行 APS 的鉴别诊断，并进行相应的管理。
