Antiphospholipid syndrome with mesenteric vein thrombosis and hepatic nodular regenerative hyperplasia in a child: A case report. Issue 48 (3rd December 2021)

• Record Type: Journal Article
• Authors: Han, Seung Hyung; Park, Kyung Duk; Kim, Soon Chul
• Editors: Saranathan., Maya
• Is Part Of: Medicine. Volume 100:Issue 48(2021)
• Page Start: e28105
• Publication Date: 2021-12-03
• Subjects: antiphospholipid syndrome -- nodular regenerative hyperplasia -- thrombosis; Medicine -- Periodicals; Medicine -- Periodicals; Médecine -- Périodiques; Geneeskunde; Medicine; Periodicals; Periodicals; 610.5
• DOI: 10.1097/MD.0000000000028105
• Languages: English
• ISSNs: 0025-7974

Abstract

Abstract: Rationale: 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. Patient concerns: 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. Diagnoses: 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. Interventions: 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. Outcomes: Her symptoms improved after 9 months of anticoagulation therapy. Lessons: 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.