Aberrant von Willebrand factor expression of sinusoidal endothelial cells and quiescence of hepatic stellate cells in nodular regenerative hyperplasia and obliterative portal venopathy

Zhang, Xuchen; Thomas, Clayton L.; Schiano, Thomas D.; Thung, Swan N.; Ward, Stephen; Fiel, M. Isabel

doi:10.1111/his.14083

Cited by 8 publications

(5 citation statements)

References 42 publications

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“…These include coagulation factor abnormalities, 18,19,29 antiphospholipid antibody syndrome (SAAF), and myeloproliferative diseases, 2,8 as well as endothelial alterations. 30 The liver biochemical abnormalities identified in our patients follow patterns described in previous series, with a predominance of patients presenting elevated liver enzymes, especially GGT, which was elevated in 76% of reported cases. 6,16 The presence of autoantibodies reached 39% in Asian and Western series, with 12% and 17% being associated autoimmune diseases, respectively.…”

Section: Discussionsupporting

confidence: 87%

“…Other studies reported procoagulant disorders occurring in 5–33% of the studied patients, which is consistent with the 20% rate found in our population. These include coagulation factor abnormalities, 18,19,29 antiphospholipid antibody syndrome (SAAF), and myeloproliferative diseases, 2,8 as well as endothelial alterations 30 …”

Section: Discussionmentioning

confidence: 99%

“…These include coagulation factor abnormalities, 18 , 19 , 29 antiphospholipid antibody syndrome (SAAF), and myeloproliferative diseases, 2 , 8 as well as endothelial alterations. 30 …”

Section: Discussionmentioning

confidence: 99%

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Obliterative portal venopathy: A neglected and probably misdiagnosed disease with peculiar etiology in South America

Nunes

Freitas

et al. 2022

JGH Open

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Background and Aim Obliterative portal venopathy (OPV) is one of the causes of non‐cirrhotic portal hypertension. However, many aspects of OPV remain unclear, including the etiology, pathogenesis, and natural history. The aim of this study was to describe the clinical features of OPV in a series of patients in Brazil in whom OPV was diagnosed through liver biopsy. Methods Forty‐three consecutive adult patients with OPV were retrospectively selected as a case series based on histologic criteria, defined by the presence of at least portal fibrosis, phlebosclerosis, disappearance and/or reduction of the caliber of portal vein branches, and exclusion of cirrhosis. Clinical and laboratory data were analyzed. Clinically significant portal hypertension was considered in the presence of esophageal varices and/or ascites. Results The mean age of patients at diagnosis was 44.5 ± 11 years, who were predominantly female (81%). Clinically significant portal hypertension was found in 28% of cases. The most frequent indication for liver biopsy was the elevation of liver enzymes, mostly γ‐glutamyl transferase (GGT) in 76% of patients, averaging 222 IU/L (upper limit of normality up to 40 IU/L) and alanine aminotransferase (ALT) in 64%, mean 84 IU/L (38 IU/L). One‐third of our patients had exposure to medications, especially herbal medicines, at the time of enzymatic changes. Other risk factors highlighted were features of autoimmunity in 25% of patients or thrombophilia in 20%. Conclusion OPV can be diagnosed even before the onset of portal hypertension, ALT elevation, and especially GGT elevation in most cases. Its etiology is not defined, but autoimmune diseases, thrombophilia, and the use of medications or herbal medicines may play a role.

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Section: Discussionsupporting

confidence: 87%

Section: Discussionmentioning

confidence: 99%

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Obliterative portal venopathy: A neglected and probably misdiagnosed disease with peculiar etiology in South America

Nunes

Freitas

et al. 2022

JGH Open

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“…7,10 Related studies have proved that varicose veins can be evaluated by imaging examination, and it has high sensitivity and specificity, which can be used in the diagnosis of PH. [22][23][24][25][26][27] By means of imaging examination, the method of defining PH is more comprehensive according to the diagnostic criteria of specificity and non-specificity. This is a retrospective study analyzed a large number of patients with PBC and is the first study to focus on combined PH in patients with simple precirrhotic stage PBC from multiple perspectives.…”

Section: Discussionmentioning

confidence: 99%

Precirrhotic Primary Biliary Cholangitis with Portal Hypertension: Bile Duct Injury Correlate

Hu,

Li,

Liu

et al. 2024

Gut and Liver

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Background/Aims: The histological characteristics and natural history of precirrhotic primary biliary cholangitis (PBC) with portal hypertension (PH) are unclear. Our aim was to clarify the prevalence, risk factors, and histological characteristics of precirrhotic PBC patients with PH.Methods: This retrospective study compared the clinical features, histological characteristics, and response to ursodeoxycholic acid (UDCA) between the PH and non-PH groups of precirrhotic PBC patients.Results: Out of 165 precirrhotic PBC patients, 40 (24.2%) also had PH. According to histological stage 1, 2 and 3 disease, 5.3% (1/19), 17.3% (17/98), and 45.8% (22/48) of patients also had PH, respectively. Precirrhotic PBC with PH was significantly positively correlated with bile duct loss, degree of cytokeratin 7 positivity, and degree of fibrosis in the portal area, but significantly negatively correlated with lymphoid follicular aggregation. Compared to the non-PH group, patients in the PH group showed a higher prevalence of obliterative portal venopathy, incomplete septal fibrosis, portal tract abnormalities and non-zonal sinusoidal dilatation (p<0.05). In addition, patients with PH were more likely to present with symptoms of jaundice, ascites, epigastric discomfort, a poorer response to UDCA, and more decompensation events (p<0.05). High alkaline phosphatase levels, low white blood cell counts, high Mayo scores, and high FIB-4 index values were risk factors for precirrhotic PBC with PH.Conclusions: Approximately 24.2% of precirrhotic PBC patients have PH, which is histologically related to the injury of bile ducts. High alkaline phosphatase levels, low white blood cell counts, high Mayo scores, and high FIB-4 index values are associated with increased risk of precirrhotic PBC with PH.

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“…There is limited knowledge about the endothelial CD34 expression pattern in INCPH cases. Periportal sinusoidal CD34 immunoexpression has been mentioned in a few studies in patients with NRH and OPV [ 24 , 25 ].…”

Section: Introductionmentioning

confidence: 99%

Evaluation of the histologic and immunohistochemical (CD34, glutamine synthetase) findings in idiopathic non-cirrhotic portal hypertension (INCPH)

Büyük,

Berker,

Bakkaloğlu

et al. 2024

Hepatol Int

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Aim Idiopathic non-cirrhotic portal hypertension (INCPH) is a vascular disorder of uncertain origin. Diagnosis can be challenging on liver biopsy. Despite diverse histomorphologic findings documented in literature, studies on the frequency of these findings are lacking. This study aims to assess both the histomorphologic features and the immunoexpression patterns of CD34 and glutamine synthetase (GS) in liver biopsies and searched for their contribution to the pathologic diagnosis of INCPH. Materials and methods Hematoxylin–eosin, CD34, and GS-stained liver needle biopsy sections of 16 patients clinically diagnosed with INCPH were retrospectively analyzed. Histologic findings such as portal vein narrowing, obliteration, or loss were grouped as major findings, while portal vein herniation, hypervascularized portal tracts, and periportal abnormal vessels were grouped as minor findings, and their frequency were evaluated. Periportal endothelial CD34 stained areas were measured via ocular micrometer. The distribution of GS immunoexpression was evaluated. Eighteen healthy liver donor biopsies were evaluated as controls. Results In INCPH cases, 58% of portal tracts showed major findings, compared to 15% in the control group (p < 0.001). Minor findings were observed in 16% of INCPH cases and 7% of controls (p = 0.014). The number of portal tracts with histologic findings is significantly higher in INCPH than in control liver biopsies. Abnormal portal tract distribution, like being close to each other, was seen in 75% of INCPH cases but not in controls (p < 0.001). Nodular regenerative hyperplasia (NRH) was present in 31% of cases. Periportal CD34 expression was higher in INCPH, and affected areas were larger than in controls (p < 0.001). Irregular GS staining, i.e. GS staining with patchy distribution in zone 3, and/or periportal and zone 2 hepatocytes, was found in 62% of INCPH cases, while controls showed the usual pattern (p < 0.001). Conclusion In the biopsy diagnosis of INCPH, in addition to the presence of major histologic findings and the amount of portal tracts displaying these features, the expression of endothelial CD34 in periportal areas, and irregular hepatocellular GS expression can also be considered as supporting feature.

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Aberrant von Willebrand factor expression of sinusoidal endothelial cells and quiescence of hepatic stellate cells in nodular regenerative hyperplasia and obliterative portal venopathy

Cited by 8 publications

References 42 publications

Obliterative portal venopathy: A neglected and probably misdiagnosed disease with peculiar etiology in South America

Obliterative portal venopathy: A neglected and probably misdiagnosed disease with peculiar etiology in South America

Precirrhotic Primary Biliary Cholangitis with Portal Hypertension: Bile Duct Injury Correlate

Evaluation of the histologic and immunohistochemical (CD34, glutamine synthetase) findings in idiopathic non-cirrhotic portal hypertension (INCPH)

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