Publications by authors named "Dominique Valla"

216 Publications

Multicenter study on recent portal venous system thrombosis associated with cytomegalovirus disease.

J Hepatol 2021 Sep 23. Epub 2021 Sep 23.

Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149. Paris, France. Electronic address:

Background And Aims: Recent non-malignant non-cirrhotic portal venous system thrombosis (PVT) is a rare condition. Among risk factors for PVT, cytomegalovirus (CMV) disease is usually listed based on few reported cases. The aim of this study was to determine characteristics and outcome of patients with PVT associated with CMV disease.

Methods: We conducted a French multicenter retrospective study comparing patients with recent PVT and CMV disease ("CMV positive"; n = 23) with patients with recent PVT for whom CMV testing was negative ("CMV negative"; n = 53) or unavailable ("CMV unknown"; n = 297).

Results: As compared with patients from the "CMV negative" and "CMV unknown" groups, patients from the "CMV positive" group were younger, had more frequently fever, higher heart rate, higher lymphocyte count and higher serum ALT levels (p ≤ 0.01 for all). Prevalence of immunosuppression did not differ between the 3 groups (4%, 4% and 6%, respectively). Extension of PVT was similar between the 3 groups. Thirteen out of 23 "CMV positive" patients had another risk factor for thrombosis. Besides CMV disease, number of risk factors for thrombosis was similar between the 3 groups. Heterozygous prothrombin gene mutation was more frequent in "CMV positive" patients (22%) than in the "CMV negative" (4%, p = 0.01) and "CMV unknown" (8%, p = 0.03) groups. Recanalization rate was not influenced by CMV status.

Conclusions: In patients with recent PVT, features of mononucleosis syndrome should raise suspicion of CMV disease. CMV disease does not influence thrombosis extension nor recanalization. More than half "CMV positive" patients have another risk factor for thrombosis, with a particular link with prothrombin gene mutation.

Lay Summary: Patients with CMV-associated portal venous system thrombosis have similar thrombosis extension and evolution as patients without CMV disease. They more frequently have prothrombin gene mutation, suggesting a synergy between these two entities to promote thrombosis.
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http://dx.doi.org/10.1016/j.jhep.2021.09.011DOI Listing
September 2021

Prospective external validation of a new non-invasive test for the diagnosis of non-alcoholic steatohepatitis in patients with type 2 diabetes.

Aliment Pharmacol Ther 2021 10 16;54(7):952-966. Epub 2021 Aug 16.

Paris, France.

Background: One of the unmet needs in patients with type 2 diabetes mellitus (T2DM) is the prediction of non-alcoholic liver disease by non-invasive blood tests, for each of the three main histological features, fibrosis, non-alcoholic steatohepatitis (NASH) and steatosis.

Aims: To validate externally the performances of a recent panel, Nash-FibroTest, for the assessment of the severity of fibrosis stages, NASH grades and steatosis grades.

Methods: We prospectively analysed 272 patients with T2DM. Standard definitions of stages and grades were used, and analyses were centralised and blinded. The performances of the FibroTest, NashTest-2 and SteatoTest-2 were assessed using the Obuchowski measure (OM), the main outcome recommended as a summary measure of accuracy includeing all pairwise stages and grades comparisons, which is not provided par the extensively used binary area under the ROC curve.

Results: The diagnostic performance of each component of the panel was significant. OM (SE; significance) of the FibroTest, the NashTest-2 and the SteatoTest-2 was 0.862 (0.012; P < 0.001), 0.827 (0.015; P < 0.001) and 0.794 (0.020; P < 0.01), respectively. For ballooning and lobular inflammation, OM was 0.794 (0.021; P < 0.001) and 0.821 (0.017; P < 0.001), respectively. In a post hoc analysis the FibroTest outperformed VCTE by 4.1% (2.5-6.5; P < 0.001) for reliability, with a non-significant difference for OM for fibrosis staging, 0.859 (0.012) for FibroTest vs 0.870 (0.009) for VCTE.

Conclusions: From a single blood sample, the panel provides non-invasive diagnosis of the stages of fibrosis, and the grades of NASH and steatosis in patients with T2DM.

Trial Registration Number: NCT03634098.
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http://dx.doi.org/10.1111/apt.16543DOI Listing
October 2021

Eosinophilic ascites as an uncommon presentation of eosinophilic gastroenteritis: A case report.

Arab J Gastroenterol 2021 Jun 2;22(2):184-186. Epub 2021 Jun 2.

Department of Hepatogastroenterology, Theodor Bilharz Research Institute, Giza, Egypt; Department of Pathology, Theodor Bilharz Research Institute, Giza, Egypt; Department of Tropical Medicine and Hepatogastroenterology, Faculty of Medicine, Cairo University, Cairo, Egypt; Service d'Hépatologie, Hôpital Beaujon, APHP, Clichy-la-Garenne, CRI-UMR 1149, Inserm and Université de Paris, Paris, France.

Background: Eosinophilic gastroenteritis (EGE) is defined by the presence of gastrointestinal symptoms, with an abnormal eosinophilic infiltrate of the intestine wall and exclusion of other causes of secondary eosinophilia. EGE has three clinical presentations, depending on the depth of eosinophilic infiltration of the bowel wall. It individualizes into three types, namely mucosal, muscular, and subserosal. Eosinophilic ascites, which is caused by edema and eosinophilic inflammation of the serosal layer of the small bowel wall, is the most uncommon presentation of EGE.

Case Summary: A 30-year-old Egyptian woman presented with pain in the epigastrium and diffuse abdominal distension. Past medical history comprised allergy to iron injections (for iron deficiency anemia). Clinical examination showed moderate abdominal distention (palpation) and shifting dullness (percussion) suggestive of moderate ascites; mild right pleural effusion was also suspected, but findings were otherwise unremarkable. Abdominal and pelvic examinations by ultrasound and contrast-enhanced computed tomography showed moderate ascites, mild right pleural effusion, and diffuse thickening of the antrum and small bowel loops. Endoscopy of the upper gastrointestinal tract revealed mild diffuse hyperemia of the esophagus, stomach and duodenum, with no relevant findings in the histopathology of biopsy specimens taken from these sites. Laboratory results showed eosinophilia in the peripheral blood and marked increase of eosinophils in the ascitic fluid. Treatment with corticosteroids resulted in normalization of the laboratory test results, and the ascites resolved within a week of initiation of therapy.

Conclusion: Eosinophilic ascites, characterized by increased eosinophils in peripheral blood and ascitic fluid, showed dramatic response to steroid therapy.
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http://dx.doi.org/10.1016/j.ajg.2021.02.002DOI Listing
June 2021

Liver Stiffness by Transient Elastography to Detect Porto-Sinusoidal Vascular Liver Disease With Portal Hypertension.

Hepatology 2021 Jul 11;74(1):364-378. Epub 2021 Jun 11.

Université de Paris, AP-HP, Hôpital Beaujon, Service d'Hépatologie, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Centre de recherche sur l'inflammation, Inserm, UMR 1149, Paris, France.

Background And Aims: Porto-sinusoidal vascular liver disease (PSVD) is a rare cause of portal hypertension. PSVD is still often misdiagnosed as cirrhosis, emphasizing the need to improve PSVD diagnosis strategies. Data on liver stiffness measurement using transient elastography (TE-LSM) in PSVD are limited. The aim of this study was to evaluate the accuracy of TE-LSM to discriminate PSVD from cirrhosis in patients with signs of portal hypertension.

Approach And Results: Retrospective multicenter study comparing TE-LSM in patients with PSVD, according to Vascular Liver Disease Interest Group criteria, with patients with compensated biopsy-proven cirrhosis associated with alcohol (n = 117), HCV infection (n = 110), or NAFLD (n = 46). All patients had at least one sign of portal hypertension among gastroesophageal varices, splenomegaly, portosystemic collaterals, history of ascites, or platelet count < 150 × 10 /L. The 77 patients with PSVD included in the test cohort had lower median TE-LSM (7.9 kPa) than the patients with alcohol-associated, HCV-related, and NAFLD-related cirrhosis (33.8, 18.2, and 33.6 kPa, respectively; P < 0.001). When compared with cirrhosis, a cutoff value of 10 kPa had a specificity of 97% for the diagnosis of PSVD with a 85% positive predictive value. A cutoff value of 20 kPa had a sensitivity of 94% for ruling out PSVD with a 97% negative predictive value. Of the patients, 94% were well-classified. Even better results were obtained in a validation cohort including 78 patients with PSVD.

Conclusions: This study including a total of 155 patients with PSVD and 273 patients with cirrhosis demonstrates that TE-LSM < 10 kPa strongly suggests PSVD in patients with signs of portal hypertension. Conversely, when TE-LSM is >20 kPa, PSVD is highly unlikely.
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http://dx.doi.org/10.1002/hep.31688DOI Listing
July 2021

Microcirculatory changes in the liver of patients with refractory ascites and their relationship with diabetes and alcohol.

Eur J Gastroenterol Hepatol 2020 Nov 17. Epub 2020 Nov 17.

DHU UNITY, Service d'Hépatologie, Hôpital Beaujon - AP-HP, Clichy France and Centre de recherché sur l'Inflammation (CRI), INSERM, Université Paris Diderot-Paris 7, CNRS, Paris, France.

Objectives: The determinants of refractory ascites have not been fully characterized. The aims of this study were to assess liver histopathological alterations associated with refractory ascites and their relationship with comorbidities.

Methods: Consecutive patients with cirrhosis who underwent liver transplantation were retrospectively included. Patients' characteristics at the time of listing were analysed. The native livers were reviewed and lesions associated with refractory ascites were examined.

Results: Out of the 89 patients included, 30 had refractory ascites and 59 did not (including 35 without ascites and 24 with diuretic-sensitive ascites). Patients with and without refractory ascites had a similar amount of fibrous tissue and features of fatty liver disease. By contrast, microvascular changes, namely sinusoidal dilatation (P < 0.001), diffuse perisinusoidal fibrosis (P = 0.001), hepatic venous thromboses (P = 0.004) and vascular proliferation (P = 0.01) were more frequently observed in the livers of patients with refractory ascites. Diabetes (57% vs. 31%, P = 0.02) and alcohol as a causal factor for cirrhosis (80% vs. 42%, P = 0.001) were more frequent in patients with refractory ascites than in those without. By multivariate analysis, refractory ascites was independently associated with diabetes mellitus [odds ratio (OR) (95% confidence interval, CI) 6.15 (1.47-25.71); P = 0.01], alcohol as a causal factor for cirrhosis [OR (95% CI) 4.63 (1.07-20.02); P = 0.04], higher Model For End Stage Liver Diseases [OR (95% CI) 1.21 (1.05-1.38); P = 0.008] and lower serum sodium [OR (95% CI) 0.87 (0.78-0.98); P = 0.03].

Conclusion: Liver microcirculatory changes are associated with refractory ascites. Diabetes and alcohol may explain refractory ascites by causing microangiopathy.
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http://dx.doi.org/10.1097/MEG.0000000000001990DOI Listing
November 2020

Systemic inflammation as a risk factor for portal vein thrombosis in cirrhosis: a prospective longitudinal study.

Eur J Gastroenterol Hepatol 2020 Nov 17. Epub 2020 Nov 17.

Université de Paris, Centre de recherche sur l'inflammation, Inserm, Paris.

Background And Aims: Various risk factors for portal vein thrombosis (PVT) development in patients with cirrhosis have been identified, but the role of systemic inflammatory reaction is unknown. The study aims to assess the association between markers of systemic inflammation and PVT in cirrhosis.

Methods: Between January 2014 and October 2015, 107 outpatients with cirrhosis and no PVT were recruited, and followed till February 2017. White blood cell count, serum concentrations of high-sensitive C-reactive protein, ferritin, tumor necrosis factor-alpha and interleukin-6 (IL-6) were evaluated at baseline and every 3 or 6 months till PVT diagnosis or end of follow-up.

Results: Median age, model for end-stage liver disease (MELD) score and follow-up period of the studied population was 55 years (IQR 46-62 years), 9.6 points (IQR 7.5-12 points) and 19 months (12-24 months), respectively. PVT developed in 10.3% of the patients. Lymphocyte count below 1.2 ´ 10/L [hazard ratio, 6.04; 95% confidence interval (CI), 1.29-28.2; P = 0.022], IL-6 above 5.5 pg/mL (hazard ratio, 5.64; 95% CI, 1.21-26.33; P  = 0.028) and neutrophil-to-lymphocyte ratio (hazard ratio, 1.46; 95% CI, 1.04-2.04; P = 0.028) were associated with a higher risk of PVT development. IL-6 and lymphopenia remained associated with subsequent PVT development after adjustment for nonselective beta-blockers, spleen size, portosystemic collaterals, oesophageal varices (grade ≥2) and ascites, but also with alcohol as the cause for cirrhosis and MELD ≥13.

Conclusion: In patients with cirrhosis, markers of systemic inflammation IL-6 and lymphopenia are predictive of PVT independently of markers of portal hypertension. These results draw our attention on a factor so far overlooked in the pathogenesis of PVT.
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http://dx.doi.org/10.1097/MEG.0000000000001982DOI Listing
November 2020

Benefits of molecular profiling with next-generation sequencing for the diagnosis and prognosis of myeloproliferative neoplasms in splanchnic vein thrombosis.

J Hepatol 2021 01 26;74(1):251-252. Epub 2020 Sep 26.

Hôpital Beaujon, AP-HP, DHU Unity, Pôle des Maladies de l'Appareil Digestif, Service d'Hépatologie, Centre de Référence des Maladies Vasculaires du Foie, Inserm U1149, Centre de Recherche sur l'Inflammation (CRI), Paris, Université de Paris, ERN Rare Liver Diseases, Clichy, France.

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http://dx.doi.org/10.1016/j.jhep.2020.07.043DOI Listing
January 2021

Similar performance of liver stiffness measurement and liver surface nodularity for the detection of portal hypertension in patients with hepatocellular carcinoma.

JHEP Rep 2020 Oct 17;2(5):100147. Epub 2020 Jul 17.

Department of Radiology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France.

Background & Aims: We compare the performance of liver surface nodularity (LSN) and liver stiffness measurements (LSM) using transient elastography (TE) for the detection of clinically significant portal hypertension (CSPH) in patients with cirrhosis and hepatocellular carcinoma (HCC).

Methods: All patients with cirrhosis and HCC who underwent computed tomography, LSM and hepatic venous pressure gradient (HVPG) measurements within 30 days between 2015 and 2018 were included. The estimation of CSPH by LSN and LSM, and the LSM-spleen-size-to-platelet ratio score (LSPS) were evaluated and compared.

Results: In total, 140 patients were included (109 men [78%], mean age 63 ± 9 years old), including 39 (28%) with CSPH. LSN measurements were valid in 130 patients (93%) and significantly correlated with HVPG (r = 0.68; <0.001). Patients with CSPH had higher LSN measurements compared with those without [3.1 ± 0.4 . 2.5 ± 0.3, <0.001; area under the receiver operating characteristic (AUROC): 0.87 ± 0.31]. LSM and LSPS were valid in 132 patients (94%) and significantly correlated with HVPG (r = 0.75, <0.001; AUROC 0.87 ± 0.04 and r = 0.68, <0.001; AUROC 0.851 ± 0.04, respectively). There was no significant difference in the diagnostic performance between LSN and LSM-LSPS (DeLong,  = 0.28, 0.37, and 0.65, respectively) in patients with both valid tests (n = 122). LSN <2.50 had a 100% negative predictive value for CSPH. A 2-step algorithm combining LSN and LSPS for the diagnosis of CSPH classified 108/140 patients (77%) with an 8% error.

Conclusions: The diagnostic performance and feasibility of LSN measurements were similar to those of LSM for the detection of CSPH in patients with compensated cirrhosis and HCC. Combining LSN and LSPS accurately detected CSPH in >75% of patients. Such a combination could be useful in centres where the HVPG measurement is unavailable.

Lay Summary: The diagnostic performance and feasibility of liver surface nodularity was similar to that of liver stiffness measurement (LSM) for the detection of clinically significant portal hypertension in patients with compensated cirrhosis. Thus, liver surface nodularity could be an option for the preoperative detection of clinically significant portal hypertension in patients with hepatocellular carcinoma. Combining liver surface nodularity with LSM-spleen-size-to-platelet ratio score resulted in the accurate detection of clinically significant portal hypertension in >75% of patients, thus limiting the need for HVPG measurements.
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http://dx.doi.org/10.1016/j.jhepr.2020.100147DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7452899PMC
October 2020

Portal cavernoma or chronic non cirrhotic extrahepatic portal vein obstruction.

Clin Res Hepatol Gastroenterol 2020 09 17;44(4):491-496. Epub 2020 Aug 17.

French Network for Rare Liver Diseases (FILFOIE), Saint-Antoine Hospital, APHP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France; Department of Gastroenterology and Hepatology, Rangueil Hospital, University Hospital of Toulouse, 1, avenue du Professeur Jean Poulhès, 31400 Toulouse, France.

Chronic non cirrhotic extrahepatic portal vein obstruction (EHPVO) refers to the cavernomatous transformation of the portal vein (the so-called "portal cavernoma") which occurs following acute thrombosis of the portal vein in the absence of recanalization. In adults, EHPVO mainly occurs following thrombosis, while in children it may be related to congenital malformations and/or neonatal umbilical venous catheterization. However, 50% of the cases of EHPVO remain idiopathic [1]. Risk factors and associated diseases should be investigated (chapter 1). Indeed, the presence of a thrombophilic alteration, in particular myeloproliferative neoplasm impacts prognosis and determine a causal treatment.
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http://dx.doi.org/10.1016/j.clinre.2020.03.016DOI Listing
September 2020

Molecular profiling and risk classification of patients with myeloproliferative neoplasms and splanchnic vein thromboses.

Blood Adv 2020 08;4(15):3708-3715

Centre d'Investigations Cliniques (CIC) 1427, INSERM, Hôpital Saint-Louis, Université de Paris, Paris, France.

Myeloproliferative neoplasms (MPNs) are the most frequent underlying causes of splanchnic vein thromboses (SVTs). MPN patients with SVTs (MPN-SVT) often have a unique presentation including younger age, female predominance, and low Janus kinase 2 (JAK2) mutation allele burden. This study aimed at identifying risk factors for adverse hematologic outcomes in MPN-SVT patients. We performed a retrospective study of a fully characterized cohort of MPN-SVT patients. The primary outcome was the incidence of evolution to myelofibrosis, acute leukemia, or death. Eighty patients were included in the testing cohort. Median follow-up was 11 years. Most of the patients were women with a mean age of 42 years and a diagnosis of polycythemia vera. The primary outcome was met in 13% of the patients and was associated with a JAK2V617F allele burden ≥50% (odds ratio [OR], 14.7) and presence of additional mutations in genes affecting chromatin/spliceosome (OR, 9). We identified high-risk patients (29% of the cohort) as those harboring at least 1 molecular risk factor: JAK2-mutant allele burden ≥50%, presence of chromatin/spliceosome/TP53 mutation. High-risk patients had worse event-free survival (81% vs 100%; P = .001) and overall survival at 10 years (89% vs 100%; P = .01) than low-risk patients. These results were confirmed in an independent validation cohort of 30 MPN-SVT patients. In conclusion, molecular profiling identified MPN-SVT patients with dismal outcome. In this high-risk population, a disease-modifying therapy should be taken into consideration to minimize the probability of transformation.
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http://dx.doi.org/10.1182/bloodadvances.2020002414DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7422133PMC
August 2020

Elastography improves accuracy of early hepato-biliary complications diagnosis after allogeneic stem cell transplantation.

Haematologica 2021 Sep 1;106(9):2374-2383. Epub 2021 Sep 1.

Hematology and transplantation unit, Saint Louis Hospital, APHP, Paris, France.

Significant morbidity and mortality have been associated with liver complications after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Causes and consequences of these hepato-biliary complications are various and might be life-threatening. A high misdiagnosis rate has been reported because of a weak correlation between clinical, laboratory and imaging data. Liver elastography, a liver stiffness measure, is able to assess liver fibrosis and portal hypertension in most liver diseases, but data after allo-HSCT are scarce. Our aim was to determine the interest of sequential liver stiffness measurements for the diagnosis of early hepatic complications after allo-HSCT. Over a two years period of time, 161 consecutive adult patients were included and 146 were analyzed. Ultrasonography and elastography measurements were performed before transplantation, at day+7 and day+14 by three different experienced radiologists unaware of patients'clinical status. Eighty-one (55%) patients had liver involvements within the first 100 days after allo-HSCT. Baseline elastography was not predictive for the occurrence of overall liver abnormalities. A significant increase in 2D real-time shearwave elastography (2D-SWE) was found in patients with sinusoidal obstruction syndrome (SOS). Fifteen patients (10%) fulfilled EBMT score criteria and twelve (8%) reached Baltimore criteria for SOS diagnosis, but only six (4%) had a confirmed SOS. 2D-SWE at day+14 allowed early detection of SOS (AUROC=0.84, p=0.004) and improved sensibility (75%), specificity (99%) and positive predictive value (60%) over the Seattle, Baltimore or EBMT scores. A 2D-SWE measurement above 8.1kPa at day+14 after allo-HSCT seems a promising, non-invasive, and reproducible tool for early and accurate diagnosis of SOS.
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http://dx.doi.org/10.3324/haematol.2019.245407DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8409044PMC
September 2021

Risk factors for vascular liver diseases: Vascular liver diseases: position papers from the francophone network for vascular liver diseases, the French Association for the Study of the Liver (AFEF), and ERN-rare liver.

Clin Res Hepatol Gastroenterol 2020 09 7;44(4):410-419. Epub 2020 Jul 7.

Department of Laboratory Hematology, Beaujon Hospital AP-HP, 100, boulevard du Général Leclerc, 92118 Clichy, France; French Network for Rare Liver Diseases FILFOIE, Saint-Antoine Hospital AP-HP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France; Reference center of vascular liver diseases, European Reference Network (ERN) Rare-Liver.

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http://dx.doi.org/10.1016/j.clinre.2020.03.010DOI Listing
September 2020

Ischemic cholangiopathy: An update.

Clin Res Hepatol Gastroenterol 2020 09 24;44(4):486-490. Epub 2020 May 24.

French Network for Rare Liver Diseases FILFOIE, Saint-Antoine hospital, AP-HP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France; Department of hepatology, Beaujon hospital AP-HP, 100, boulevard du Général-Leclerc, 92118 Clichy, France; Reference center of vascular liver diseases, European Reference Network (ERN) "Rare-Liver", Hamburg, Germany.

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http://dx.doi.org/10.1016/j.clinre.2020.03.018DOI Listing
September 2020

Recent (non-cirrhotic) extrahepatic portal vein obstruction.

Clin Res Hepatol Gastroenterol 2020 09 21;44(4):460-465. Epub 2020 May 21.

Department of Hepatology and reference center of vascular liver diseases, Beaujon Hospital, AP-HP, 100, boulevard du Général-Leclerc, 92118 Clichy, France; French Network for Rare Liver Diseases FILFOIE, Saint-Antoine Hospital, AP-HP, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France.

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http://dx.doi.org/10.1016/j.clinre.2020.03.003DOI Listing
September 2020

Hepatobiliary MR contrast agents are useful to diagnose hepatocellular carcinoma in patients with Budd-Chiari syndrome.

JHEP Rep 2020 Jun 9;2(3):100097. Epub 2020 Mar 9.

Department of Radiology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France.

Background & Aims: Hepatobiliary phase (HBP) images can discriminate between benign and malignant liver lesions, but it is unclear if this approach can be used in patients with Budd-Chiari syndrome (BCS). Thus, we aimed to assess the diagnostic utility of HBP images in patients with BCS.

Methods: This retrospective study included all patients admitted to our institution with a diagnosis of BCS and focal liver lesions on hepatobiliary contrast agent-enhanced MR imaging (HBCA-MRI) from 2000 to 2019. MR images were reviewed by 2 radiologists blinded to the diagnosis of the lesions. Patient and lesion characteristics were recorded, focusing on HBP imaging features.

Results: Twenty-six patients (mean 35 ± 11 years old [13-65]; 21 women [81%] 35 ± 12 years old [13-65]; 5 men [19%] 36 ± 10 years old [19-44]) with 99 benign liver lesions and 12 hepatocellular carcinomas (HCCs) were analyzed. Patients with HCC were significantly older than those with benign lesions (mean 50 ± 10 33 ± 9 years old,  = 0.003), with higher alpha-fetoprotein (AFP) levels (3/4 [75%] 1/22 [5%] with AFP >15 ng/ml, <0.001). Homogeneous hypointense signals were identified on HBP in 14 lesions, including 12/12 (100%) HCCs, and 2/99 (2%) benign lesions ( <0.001). Most benign liver lesions showed either peripheral (n = 52/99 [53%]) or homogeneous hyperintensity (n = 23/99 [23%]) on HBP. Lesions with signal hypointensity on HBP in patients with AFP serum levels >15 ng/ml were all HCCs.

Conclusion: Most benign lesions showed homogeneous or peripheral hyperintensity on HBP images while all HCCs were homogeneously hypointense. HBP images are helpful to differentiate between benign lesions and HCCs and outperform other sequences. They should be systematically acquired for the characterization of focal lesions in patients with BCS.

Lay Summary: Hepatobiliary phase imaging is an approach that has recently been shown to discriminate between benign and malignant lesions in the liver. However, it was not known whether this imaging approach could be used effectively in patients with Budd-Chiari syndrome. Herein, we have shown that hepatobiliary phase imaging appears to be useful for differentiating between benign and malignant liver lesions in patients with Budd-Chiari syndrome.
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http://dx.doi.org/10.1016/j.jhepr.2020.100097DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7232085PMC
June 2020

Drug induced liver injury and vascular liver disease.

Clin Res Hepatol Gastroenterol 2020 09 1;44(4):471-479. Epub 2020 May 1.

French Network for Rare Liver Diseases FILFOIE, Saint-Antoine Hospital, AP-HP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France; Department of Gastroenterology and Hepatology, Rangueil Hospital, University Hospital of Toulouse, 1, avenue du Professeur Jean-Poulhès, 31400 Toulouse, France.

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http://dx.doi.org/10.1016/j.clinre.2020.03.020DOI Listing
September 2020

No evidence for an increased liver uptake of SARS-CoV-2 in metabolic-associated fatty liver disease.

J Hepatol 2020 09 30;73(3):717-718. Epub 2020 Apr 30.

Université de Paris, Centre de recherche sur l'inflammation, Inserm, U1149, CNRS, ERL8252, Paris, France; Service d'Hépatologie, DMU Digest, Hôpital Beaujon, AP-HP, Clichy, France; Centre de Référence des Maladies Vasculaires du Foie, French Network for Rare Liver Diseases (FILFOIE), European Reference Network on Hepatological Diseases (ERN RARE-LIVER), Hôpital Beaujon, AP-HP, Clichy, France. Electronic address:

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http://dx.doi.org/10.1016/j.jhep.2020.04.035DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7191298PMC
September 2020

Porto-sinusoidal vascular disease. Vascular liver diseases: Position papers from the francophone network for vascular liver diseases, the French Association for the Study of the Liver (AFEF), and ERN-rare liver.

Clin Res Hepatol Gastroenterol 2020 09 22;44(4):447-451. Epub 2020 Apr 22.

Department of hepatology, DHU Unity, Beaujon hospital, AP-HP, 100, boulevard du Général-Leclerc, 92118 Clichy, France; Inserm, center for research in inflammation, university of Paris, 75018 Paris, France; Reference center of vascular liver diseases European Reference Network (ERN) "Rare-Liver", France; French Network for Rare Liver Diseases (FILFOIE), Saint-Antoine hospital, AP-HP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France.

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http://dx.doi.org/10.1016/j.clinre.2020.03.005DOI Listing
September 2020

Extrahepatic portal vein obstruction (EHPVO) in cirrhosis.

Clin Res Hepatol Gastroenterol 2020 09 17;44(4):497-502. Epub 2020 Apr 17.

Department of Hepatology, DHU Unity, Beaujon Hospital, AP-HP, 100, boulevard du Général-Leclerc, 92118 Clichy, France.

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http://dx.doi.org/10.1016/j.clinre.2020.03.014DOI Listing
September 2020

Congenital portosystemic shunts: Vascular liver diseases: Position papers from the francophone network for vascular liver diseases, the French Association for the Study of the Liver (AFEF), and ERN-rare liver.

Clin Res Hepatol Gastroenterol 2020 09 9;44(4):452-459. Epub 2020 Apr 9.

French Network for Rare Liver Diseases (FILFOIE), European Reference Network (ERN) "Rare-Liver" Saint-Antoine Hospital, AP-HP, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France; Department of Hepatology, DHU Unity, Beaujon Hospital, AP-HP, 100, boulevard du Général-Leclerc, 92118 Clichy, France.

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http://dx.doi.org/10.1016/j.clinre.2020.03.004DOI Listing
September 2020

Management of anticoagulation in adult patients with chronic parenchymal or vascular liver disease: Vascular liver diseases: Position papers from the francophone network for vascular liver diseases, the French Association for the Study of the Liver (AFEF), and ERN-rare liver.

Clin Res Hepatol Gastroenterol 2020 09 8;44(4):438-446. Epub 2020 Apr 8.

French Network for Rare Liver Diseases (FILFOIE), Saint-Antoine hospital, AP-HP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France; Department of hepatology, Beaujon hospital AP-HP, 100, boulevard du Général Leclerc, 92118 Clichy, France; Reference center of vascular liver diseases, European Reference Network (ERN) "Rare-Liver", Clichy, France.

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http://dx.doi.org/10.1016/j.clinre.2020.03.006DOI Listing
September 2020

Pregnancy and vascular liver diseases: Vascular liver diseases: position papers from the francophone network for vascular liver diseases, the French Association for the Study of the Liver (AFEF), and ERN-rare liver.

Clin Res Hepatol Gastroenterol 2020 09 8;44(4):433-437. Epub 2020 Apr 8.

DHU Unity, Department of Hepatology, Beaujon Hospital, AP-HP, 100, boulevard du Général Leclerc, 92118 Clichy, France; Reference center of vascular liver diseases, European Reference Network (ERN) "Rare-Liver", Hambourg Germany; French Network for Rare Liver Diseases FILFOIE, Saint-Antoine Hospital, AP-HP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France.

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http://dx.doi.org/10.1016/j.clinre.2020.03.007DOI Listing
September 2020

Hereditary hemorrhagic telangiectasia and liver involvement: Vascular liver diseases: position papers from the francophone network for vascular liver diseases, the French Association for the Study of the Liver (AFEF), and ERN-rare liver.

Clin Res Hepatol Gastroenterol 2020 09 7;44(4):426-432. Epub 2020 Apr 7.

Department of Hepatology and reference center of vascular liver diseases, Beaujon Hospital, AP-HP, 100, boulevard du Général Leclerc, 92118 Clichy, France; French Network for Rare Liver Diseases (FILFOIE), Saint-Antoine Hospital, APHP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France; Reference center of vascular liver diseases, European Reference Network (ERN) Rare-Liver.

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http://dx.doi.org/10.1016/j.clinre.2020.03.008DOI Listing
September 2020

Sinusoidal obstruction syndrome.

Clin Res Hepatol Gastroenterol 2020 09 3;44(4):480-485. Epub 2020 Apr 3.

Department of Hepatology, DHU Unity, Beaujon Hospital, AP-HP, 100, boulevard du Général-Leclerc, 92118 Clichy, France; French Network for Rare Liver Diseases FILFOIE, Saint-Antoine Hospital, AP-HP, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France; Reference center of vascular liver diseases, European Reference Network (ERN) 'Rare-Liver', France.

Sinusoidal obstruction syndrome (SOS), previously known as veno-occlusive disease, is characterized by concentric and non-thrombotic obstruction of the sinusoid and central vein lumen with no identified primitive or thrombotic hepatic vein lesions. The initial lesion is a result of endothelial denudation, corresponding to the migration of damaged sinusoidal cells to the central veins of the hepatic lobules, leading to sinusoidal and veno-occlusive congestive obstruction. SOS may be associated with other lesions such as centrilobular perisinusoidal fibrosis, peliosis, or nodular regenerative hyperplasia. The first cases of SOS were documented in 1920 in South Africa, after ingestion of food sources contaminated by pyrrolizidine alkaloids. SOS is a well-known complication of hematopoietic stem cell transplantation (HSCT). Numerous toxins and drugs have been associated with SOS, mainly chemotherapies and immunosuppressive therapies, as well as total body or liver irradiation and ABO mismatch platelet transfusion. The pathogenesis of this entity remains unknown.
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http://dx.doi.org/10.1016/j.clinre.2020.03.019DOI Listing
September 2020

Budd-Chiari syndrome.

Clin Res Hepatol Gastroenterol 2020 09 2;44(4):420-425. Epub 2020 Apr 2.

French Network for Rare Liver Diseases FILFOIE, Saint-Antoine Hospital, APHP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France; Department of Gastroenterology and Hepatology, Rangueil Hospital, University Hospital of Toulouse, 1, avenue du Professeur Jean-Poulhès, 31400 Toulouse, France.

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http://dx.doi.org/10.1016/j.clinre.2020.03.015DOI Listing
September 2020

Long-term Evolution of Hepatocellular Adenomas at MRI Follow-up.

Radiology 2020 05 17;295(2):361-372. Epub 2020 Mar 17.

From the University of Paris, Paris, France (F.V., M.R., F.C., O.S., D.V., V.P., V.V.); Departments of Radiology (M.R., M.D.B., V.V.), Liver Transplantation and Hepatobiliary Surgery (F.C., S.D., O.S.), and Hepatology (D.V.), and Pathology Department (V.P.), University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, Hauts-de-Seine, France; INSERM, UMR 1149, Paris, France (M.R., F.C., O.S., D.V., M.D.B., V.P., V.V.); Department of Biostatistics and Bioinformatics, Duke University School of Medicine, Durham, NC (K.R.C.); INSERM, UMR 1162, Génomique Fonctionnelle des Tumeurs Solides, Université Paris Descartes, Université Paris Diderot, Université Paris 13, Labex Immuno-Oncology, Paris, France (J.Z.), Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties, University of Palermo, Palermo, Italy (F.V.).

Background Hepatocellular adenomas (HCAs) are rare benign liver tumors. Guidelines recommend continued surveillance of patients diagnosed with HCAs, but these guidelines are mainly based on small studies or expert opinion. Purpose To analyze the long-term evolution of HCAs, including solitary and multiple lesions, and to identify predictive features of progression with MRI. Materials and Methods In a retrospective study, patients diagnosed with pathologically proven solitary or multiple HCAs between January 2004 and December 2015 were included; β-catenin-mutated HCAs and HCAs with foci of malignancy were considered to be at risk for progression. MRI examinations were analyzed, and tumor evolution was evaluated by using Response Evaluation Criteria in Solid Tumors, version 1.1. Student Mann-Whitney, χ, Fisher exact, and McNemar tests were used, as appropriate. Results In total, 118 patients (mean age, 40 years ± 10 [standard deviation]; 108 women) were evaluated, including 41 with a solitary HCA (mean age, 40 years ± 14; 36 women) and 77 with multiple HCAs (mean age, 40 years ± 10; 72 women). At a median follow-up of 5 years, 37 of 41 (90%) patients with a solitary HCA and 55 of 77 (71%) patients with multiple HCAs showed stable or regressive disease. After resection of solitary HCAs, new lesions appeared in only two of 29 (7%) patients, both of whom had HCAs at risk of progression. In patients with multiple HCAs, hepatocyte nuclear factor 1α-inactivated HCAs showed a higher rate of progression compared with inflammatory HCAs (11 of 26 [42%] vs seven of 37 [19%], = .04) despite lower use (28 of 32 patients [88%] vs 45 of 45 patients [100%]; = .03) and shorter duration (mean, 12.0 years ± 7.5 vs 19.2 years ± 9.2; = .001) of oral contraceptive intake. Conclusion Long-term MRI follow-up showed that 78% of hepatocellular adenomas had long-term stability or regression. After resection of solitary hepatocellular adenomas, new lesions occurred only in hepatocellular adenomas at risk of progression. Patients with multiple hepatocellular adenomas were more likely to show progressive disease, with hepatic nuclear factor 1α-inactivated hepatocellular adenomas being the most common subtype showing progression. © RSNA, 2020
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http://dx.doi.org/10.1148/radiol.2020191790DOI Listing
May 2020

Recent developments in the field of vascular liver diseases.

Liver Int 2020 02;40 Suppl 1:142-148

Service d'hépatologie, Hôpital Beaujon, Assistance Publique-Hôpitaux de Paris, Université de Paris and Inserm, Clichy la Garenne, France.

Knowledge in the field of vascular liver disease is continuously expanding. The present update will discuss recent data on i) the Abernethy malformation in adults; ii) portal vein thrombosis in cirrhosis; iii) advancing expertise in recanalization of the portal vein and iv) experience in using direct oral anticoagulants in the field of vascular liver disease.
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http://dx.doi.org/10.1111/liv.14348DOI Listing
February 2020

Nonselective β-Blockers May Progress the Thrombosis of Portal Venous System in Cirrhotic Patients: A Retrospective Observational Study.

Adv Ther 2020 04 19;37(4):1452-1463. Epub 2020 Feb 19.

Department of Gastroenterology, General Hospital of Northern Theater Command (Formerly General Hospital of Shenyang Military Area), Shenyang, China.

Introduction: Occlusive portal venous system thrombosis (PVT) is significantly associated with poor outcomes in cirrhotic patients. Nonselective β-blockers (NSBBs) may be associated with the development of PVT. However, the role of NSBBs in progressing thrombosis remains unclear.

Methods: Forty-three patients on whom contrast-enhanced computed tomography or magnetic resonance imaging was performed twice, and for whom there was detailed information regarding NSBBs, were eligible in this study, including 16 in the NSBBs group and 27 in the no NSBBs group. A composite endpoint of progressing thrombosis included the development of PVT in patients without PVT and aggravation of PVT in patients with PVT. Logistic regression analysis was employed to identify the effect of NSBBs on the progression of PVT.

Results: At the last admission, 13 patients had progressing thrombosis. The incidence of progressing thrombosis was significantly higher in the NSBBs group than in the no NSBBs group [50.0% (8/16) vs. 18.5% (5/27), P = 0.030]. The use of NSBBs (odds ratio 4.400, 95% confidence interval 1.107-17.482, P = 0.035) was significantly associated with progressing thrombosis in univariate logistic regression analyses, but not significant (odds ratio 4.084, 95% confidence interval 0.488-34.158, P = 0.194) in multivariate logistic regression analyses.

Conclusions: NSBBs may play a role in the progression of PVT in liver cirrhosis. The benefits and risks of NSBBs in the management of liver cirrhosis should be fully weighed.
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http://dx.doi.org/10.1007/s12325-020-01250-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7140745PMC
April 2020
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