Nodular regenerative hyperplasia – An under-recognized vascular disorder of liver

Bakshi, Neha; Gulati, Nitya; Rastogi, Archana; Chougule, Abhijit; Bihari, Chhagan; Jindal, Ankur

doi:10.1016/j.prp.2020.152833

Cited by 19 publications

(10 citation statements)

References 28 publications

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“…However, the histological diagnosis of porto-sinusoidal vascular disease can be complex because the characteristic lesions may be unevenly distributed and not always concurrent. Reticulin staining and a systematic analysis of the microcirculation on liver biopsy, including vascular markers of sinusoids, portal venules and arteries, are essential for a diagnosis of porto-sinusoidal vascular disease [109]. Besides the pathological features of portosinusoidal vascular disease, bile duct dilation and mucosal biliary plugs are usually absent, except in the presence co-existent biliary fibrosis.…”

Section: Porto-sinusoidal Vascular Diseasementioning

confidence: 99%

Cystic fibrosis-related liver disease: Clinical presentations, diagnostic and monitoring approaches in the era of CFTR modulator therapies

Dana

Debray

Beaufrère

et al. 2022

Journal of Hepatology

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Section: Porto-sinusoidal Vascular Diseasementioning

confidence: 99%

Cystic fibrosis-related liver disease: Clinical presentations, diagnostic and monitoring approaches in the era of CFTR modulator therapies

Dana

Debray

Beaufrère

et al. 2022

Journal of Hepatology

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“…Furthermore, it has recently been reported that changes in CK7 and CK34 expression in hepatocytes and sinusoidal endothelial cells can cause portal hypertension, which is an important finding to aid in diagnosis. [ 8 , 9 ] Imaging modalities can help diagnose the disease. Sonographic findings of NRH include small isoechoic nodules scattered in any lobe.…”

Section: Discussionmentioning

confidence: 99%

Antiphospholipid syndrome with mesenteric vein thrombosis and hepatic nodular regenerative hyperplasia in a child

Han¹,

Park²,

Kim³

2021

Medicine

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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.

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“…Nodular regenerative hyperplasia (NRH) is often associated with a variety of systemic diseases [75], characterised by diffuse transformation of liver parenchyma into small regenerative nodules in the absence of significant fibrosis [76]. A total of 6 cases about the relationship between NRH and APS have been published [22,[77][78][79][80][81].…”

Section: Non-thrombotic Liver Diseasesmentioning

confidence: 99%

The digestive system involvement of antiphospholipid syndrome: pathophysiology, clinical characteristics, and treatment strategies

Zhang

Han

et al. 2021

Annals of Medicine

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Antiphospholipid syndrome (APS) is an autoimmune disease mainly characterised by vascular thrombosis and pregnancy morbidity. APS has broad spectrum of clinical manifestations. The digestive system involvement of antiphospholipid syndrome is a critical but under-recognised condition. Digestive system involvement may be the result of direct (autoimmune-mediated) or indirect (thrombotic) mechanisms. Liver is the most commonly involved organ, followed by intestines, oesophagus, stomach, pancreas and spleen. This review describes possible digestive system manifestations in APS patients, and illustrates the epidemiology and possible pathophysiology of APS. The role of different treatment strategies in the management of digestive system manifestations of APS were also discussed. Key messages Antiphospholipid syndrome is a multi-organ, multi-system disease and its clinical manifestation spectrum is gradually expanding. Since the first diagnosis of APS, the clinical manifestations of digestive system have been reported successively. This narrative review describes the major digestive system manifestations of APS and illustrates the epidemiology, pathophysiology and the role of therapeutic strategies of these patients.

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Nodular regenerative hyperplasia – An under-recognized vascular disorder of liver

Cited by 19 publications

References 28 publications

Cystic fibrosis-related liver disease: Clinical presentations, diagnostic and monitoring approaches in the era of CFTR modulator therapies

Cystic fibrosis-related liver disease: Clinical presentations, diagnostic and monitoring approaches in the era of CFTR modulator therapies

Antiphospholipid syndrome with mesenteric vein thrombosis and hepatic nodular regenerative hyperplasia in a child

The digestive system involvement of antiphospholipid syndrome: pathophysiology, clinical characteristics, and treatment strategies

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