Budd-Chiari Syndrome Research Articles

Metabolic dysfunction-associated steatotic liver disease related cirrhosis and incidence of portal vein thrombosis.

There is heterogeneous data on whether metabolic-associated steatohepatitis is an independent risk factor for portal vein thrombosis (PVT). We aim to compare the incidence of PVT in patients with cirrhosis with and without metabolic dysfunction-associated steatotic liver disease (MASLD). This is a single-center retrospective study of patients with cirrhosis seen between 1 January 2016 and 31 January 2021. Patients with a history of hepatocellular cancer, liver transplant, Budd-Chiari syndrome, and intra-abdominal malignancies were excluded. Patients with cirrhosis were followed from their first hepatology visit for 180 days to determine the incidence of PVT. Cox proportional hazard regression was used to determine the relationship between MASLD with PVT. We analyzed data from 2785 patients with cirrhosis who met inclusion and exclusion criteria [mean age: 61.0 ± 12.3 years, 44.3% female, 63.8% Whites and mean model for end-stage liver disease-sodium (MELD-Na) score: 11.7 ± 6.1]. MASLD was present in 21.7% of patients. A total of 89 patients developed PVT during the follow-up, which was fewer in patients with MASLD [2.0% vs. 3.5%, P = 0.04, unadjusted heart rate (HR): 0.60, 95% confidence interval (CI): 0.27-0.96, P = 0.04]. After adjusting for the demographics, MASLD-related comorbid conditions and MELD-Na score, MASLD was associated with a lower incidence of PVT as compared to non-MASLD cirrhosis (HR: 0.44, 95% CI: 0.21-0.92, P = 0.03). After adjusting for the indicators of Child-Pugh Turcotte score, the risk of PVT in patients with MASLD compared to non-MASLD was not statistically significant (HR: 0.50, 95% CI: 0.22-1.13, P = 0.096). PVT incidence was lower in patients with MASLD cirrhosis as compared to non-MASLD cirrhosis. However, the difference was not significantly different after adjusting for liver decompensation.

Budd Chiari Syndrome Revealed by Spinal Cord Compression

Budd-Chiari syndrome results from obstruction of hepatic venous drainage, from the hepatic venules to the terminal part of the inferior vena cava, whatever the cause of the obstruction. It may be of primary or secondary origin. In our case, we report a case of Budd-Chiari syndrome with thrombosis of the terminal part of the inferior vena cava extending to the right suprahepatic vein, revealed by an unusual picture of functional impotence of all 4 limbs due to spinal cord compression caused by collateral venous circulation. Etiological assessment for budd chiari syndrome revealed a protein C deficiency, for which the patient was put on lifelong anticoagulation.

Arnold-Chiari Syndrome – a review

Arnold Chiari syndrome, also known as Chiari malformation, is a group of deformations of the posterior cranial fossa and hindbrain, consisting in the lowering of the cerebellum or its tonsils towards the occipital foramen. This leads to a reduction in the volume reserve within the foramen magnum, pressure on surrounding structures and obstruction of the flow of cerebrospinal fluid. The process is most often asymptomatic. Symptoms include headaches, neck pain, but also fainting, sinus bradycardia, coordination disorders and many others. Chiari malformation is often diagnosed incidentally. The basis of diagnosis is the detection of characteristic morphological features in magnetic resonance imaging. Surgical treatment is successfully used among patients with severe Arnold-Chiari syndrome and progression of clinical symptoms. Materials and methods Literature included in the PubMed, BioMed Central and Polish Medical Platform databases searched by means of the words such as Gamma Knife, CyberKnife, radiosurgery, stereotactic radiotherapy. Sources quoted in selected works were also used. Summary Arnold-Chiari malformation is often discovered incidentally. If the features observed on MRI suggest the above diagnosis, the patient should be referred to a neurologist. After excluding other possible causes of the imaged morphological features and performing a dedicated magnetic resonance imaging examination, the neurosurgeon decides whether to qualify for the procedure. In a selected group of patients, significant improvement can be expected after surgical treatment.

Chiari type 1.5 malformation as a cause of secondary trigeminal neuralgia: case report and literature discussion

BackgroundChiari syndrome is a very rare cause of secondary trigeminal neuralgia (TN). There are a few cases of TN associated with Chiari syndrome in the literature, and all of these cases were reported as Chiari type 1. In this report, we present a case of secondary trigeminal neuralgia caused by Chiari type 1.5 for the first time unlike the literature.Case presentationA 38-year-old male patient, who had frequent and severe attacks of pain under the orbit, at the chin and rim of the mouth, was evaluated with craniocervical Magnetic Resonance Imaging (MRI) and revealed Chiari type 1.5 malformation and syringomyelia.ConclusionsThe mechanism of TN associated with Chiari malformation is unclear. However, the main concepts emphasized in cases presented in the literature are compression and stretching. The rapid relief of pain after decompression surgery in these cases also supports this situation. It can be predicted that compression and tension forces will become more pronounced in Chiari type 1.5 malformation, in which the brainstem elongation and ventral pressure are more prominent. Decompression of the foramen magnum provides rapid and effective pain control in the treatment of TN accompanying Chiari 1.5 syndrome that does not respond to medical treatment.

Hepatitis isquémica, infarto hepático y colangiopatía isquémica

Hepatic hypoperfusion, whether secondary to tissue hypoperfusion or due to hepatic artery or portal vein thrombosis, can produce ischemic hepatitis or hepatic infarction. The diagnosis of ischemic hepatitis is suspected in patients with sepsis or cardiac or respiratory failure with a very significant transaminase elevation. Hepatic infarction is diagnosed radiologically. The most common cause of ischemic cholangiopathy in patients with liver transplantation is hepatic artery thrombosis.

Rare inherited coagulation disorders in Alexandria University Children’s Hospital

Introduction Rare coagulation disorders (RCD) encompass a diverse range of conditions that differ in terms of frequency and clinical manifestations. Aim To describe clinical and laboratory characteristics of patients with RCD attending Alexandria University Children’s Hospital complaining of bleeding symptoms. Methods This descriptive study enrolled pediatric patients with RCD in Alexandria University Children’s Hospital from December 2021 to June 2022. Results RCD were diagnosed in 64 patients aged 1 day–14 years. Factor ⅤⅠI deficiency was most common, with a prevalence of 29.6%, followed by fibrinogen disorders (23.4%), Factor XⅠ (15.6%), Factor X deficiency (12.5%), Factor V deficiency (10.9%), and Factor XIII deficiency (6.2%). Manifestations ranged from mild to serious presentation. The most common age at presentation was the first year of life. Intracranial hemorrhage occurred in 10/64 patients; it was the initial mode of presentation in one of them. Global developmental delay as a complication occurred in one case. Buddi Chiari syndrome occurred in three cases due to fibrinogen deficiency. The most common rare coagulation disorder was Factor VII deficiency, followed by fibrinogen deficiency due to the high prevalence of consanguineous marriage. The presentation was heterogeneous and life-threatening, with challenges in treatment, including initiation of prophylaxis very early in life. Conclusion There was a significant correlation between factor activity in fibrinogen disorders, Factor X deficiency, and severity of bleeding. There was no correlation between Factor V, Factor VII, Factor XI, Factor XIII activity levels and bleeding severity, so we recommend studying genotype-phenotype correlation.

Analysis of risk factors of short-term prognosis in patients with severe Budd-Chiari syndrome

Objective: To explore the risk factors of short-term prognosis of severe BCS patients,established and verified the nomogram prediction model for these BCS patients and evaluated its clinical application value. Methods: This study is a retrospective cohort study. The clinical data of 171 patients with severe BCS diagnosed were retrospectively analyzed in the Department of Hepatopancreatobiliary Surgery First Affiliated Hospital of Zhengzhou University from January 2018 to December 2023. There were 105 males and 66 females, aged (52.1±12.8) years (range: 18 to 79 years). The patients were divided into two groups based on whether they died within 28 days: the death group (n=38) and the survival group (n=133). The risk factors for short-term death of patients were analyzed,and independent risk factors were screened by univariate and multivariate analysis. Furthermore,these factors were used to establish the nomogram prediction model. The area under the curve(AUC),the Bootstrap Resampling,the Hosmer-Lemeshow test and the Decision Curve Analysis(DCA) were used to verify the model's differentiation,internal verification,calibration degree and clinical effectiveness,respectively. Results: Univariate and multivariate Logistics regression analysis showed that the history of hepatic encephalopathy,white blood cell,glomerular filtration rate and prothrombin time are independent risk factors (P<0.05). The above factors were used to successfully establish the prediction model with 0.908 of AUC and 0.895 of the internal verification of AUC,indicating that the predictive model was valuable. The 0.663 P-values in the Hosmer-Lemeshow test indicated the high calibration degree of the model. The clinical effectiveness of the model was proved by the 18% clinical benefit population using the DCA curve with the 17% probability threshold. Conclusions: The independent risk factors are the history of hepatic encephalopathy,white blood cell,glomerular filtration rate and prothrombin time. An adequate basis was acquired by establishing a nomogram prediction model of the short-term prognosis of severe BCS,which was helpful for early clinical screening and identification of high-risk patients with severe BCS who could die in the short term and timely providing timely intervention measures for improving the prognosis.

Hepatic Cirrhosis with Esophageal Varices: A Case Report

Background: In adults, portal hypertension is generally caused by hepatic cirrhosis, whereas in children it is more commonly caused by extrahepatic abnormalities with normal liver function. Portal hypertension causes hemodynamic abnormalities. Gastrointestinal bleeding is the most severe clinical manifestation of portal hypertension in both children and adults. Pathogenetically, increased pressure in the portal vein can be caused by increased vascular resistance and increased portal blood flow. The site of obstruction can be prehepatic (portal vein obstruction), intrahepatic (presinusoidal: eg congenital hepatic fibrosis; para sinusoidal: cirrhosis, hepatotoxic drug therapy, vitamin A hepatotoxicity; post sinusoidal: venocclusive disease) and/or post hepatic (Budd-Chiari syndrome, constrictive pericarditis). Case presentation: The study reports the results of observations of a case of a boy, FAA, aged 12 years and 2 months who came to the emergency room at Dr. M. Djamil General Hospital Padang with the main complaint of hematemesis and splenomegaly from physical examination. Non cirrhotic portal fibrosis is a cause that is not uncommon in the population in the early second decade of life. Some children with non-cirrhotic portal fibrosis as adults can end up with end stage liver disease. Conclusion: Patients with noncirrhotic or cirrhotic portal hypertension can be assessed using the Child Pugh instrument as an instrument that is still used to determine the survival rate if patients with portal hypertension.

Hepatic CT Perfusion in Budd-Chiari Syndrome before and after Intervention.

Hepatic CT Perfusion in Budd-Chiari Syndrome before and after Intervention.

Paroxysmal nocturnal hemoglobinuria–related thrombosis in the era of novel therapies: a 2043-patient-year analysis

AbstractThrombophilia is one of the principal features of paroxysmal nocturnal hemoglobinuria (PNH) and constitutes the main cause of disease morbidity/mortality. Anticomplement treatment has revolutionized the natural history of PNH, with control of the hemolytic process and abolition of thrombotic events (TEs). However, no guidelines exist for the management of thromboembolic complications in this setting, with type and duration of anticoagulation depending on individual practices. Besides, a scarcity of data is present on the efficacy of direct oral anticoagulants (DOACs). Herein, we accrued a large real-world cohort of patients with PNH from 4 US centers to explore features, predictors of TE, and anticoagulation strategies. Among 267 patients followed up for a total of 2043 patient-years, 56 (21%) developed TEs. These occurred at disease onset in 43% of cases, involving more frequently the venous system, typically as Budd-Chiari syndrome. Rate of TEs was halved in patients receiving complement inhibitors (21 vs 40 TEs per 1000 patient-years in untreated cases, with a 2-year cumulative incidence of thrombosis of 3.9% vs 18.3%, respectively), and varied according to PNH granulocytes and erythrocytes clone size, type, disease activity parameters, as well as number (≥2 mutations, or less) and variant allelic frequency of PIGA mutations. Anticoagulation with warfarin (39%), DOACs (37%), and low-molecular weight heparin (16%) was administered for a median of 29 months (interquartile range [IQR], 9-61.8). No thrombotic recurrence was observed in 19 patients treated with DOACs at a median observation of 17.1 months (IQR, 8.9-45) whereas 14 cases discontinued anticoagulation without TE recurrence at a median time of 51.4 months (IQR, 29.9-86.8).

Clinical profile and outcomes of hepatocellular carcinoma in primary Budd-Chiari syndrome.

There is scant literature on hepatocellular carcinoma (HCC) in patients with Budd-Chiari syndrome (BCS). To assess the magnitude, clinical characteristics, feasibility, and outcomes of treatment in BCS-HCC. A total of 904 BCS patients from New Delhi, India and 1140 from Mumbai, India were included. The prevalence and incidence of HCC were determined, and among patients with BCS-HCC, the viability and outcomes of interventional therapy were evaluated. In the New Delhi cohort of 35 BCS-HCC patients, 18 had HCC at index presentation (prevalence 1.99%), and 17 developed HCC over a follow-up of 4601 person-years, [incidence 0.36 (0.22-0.57) per 100 person-years]. BCS-HCC patients were older when compared to patients with BCS alone (P = 0.001) and had a higher proportion of inferior vena cava block, cirrhosis, and long-segment vascular obstruction. The median alpha-fetoprotein level was higher in patients with BCS-HCC at first presentation than those who developed HCC at follow-up (13029 ng/mL vs 500 ng/mL, P = 0.01). Of the 35 BCS-HCC, 26 (74.3%) underwent radiological interventions for BCS, and 22 (62.8%) patients underwent treatment for HCC [transarterial chemoembolization in 18 (81.8%), oral tyrosine kinase inhibitor in 3 (13.6%), and transarterial radioembolization in 1 (4.5%)]. The median survival among patients who underwent interventions for HCC compared with those who did not was 3.5 years vs 3.1 mo (P = 0.0001). In contrast to the New Delhi cohort, the Mumbai cohort of BCS-HCC patients were predominantly males, presented with a more advanced HCC [Barcelona Clinic Liver Cancer C and D], and 2 patients underwent liver transplantation. HCC is not uncommon in patients with BCS. Radiological interventions and liver transplantation are feasible in select primary BCS-HCC patients and may improve outcomes.

Protocolo diagnóstico de la afectación hepática en las enfermedades autoinmunes sistémicas

Systemic autoimmune diseases are conditions in which there is an alteration of the immune system that is chronic and with multisystemic involvement. Due to its central role in metabolism, the liver is often affected to varying degrees. There are different mechanisms that can explain this involvement. First, immune dysregulation itself can lead to direct liver toxicity (antibody formation, T cell-mediated injury), as well as the development of primary liver disease (autoimmune hepatitis, primary biliary cholangitis). Second, patients with autoimmune diseases can develop different forms of hepatic vascular involvement such as porto-sinusoidal vascular disease, sinusoidal obstruction syndrome, Budd-Chiari syndrome, or the onset of hepatic ischemia within vasculitis. Finally, the drugs used in the treatment of systemic autoimmune disease can produce hepatotoxicity through different mechanisms, some of which are not fully known.

Liver imaging and pregnancy: what to expect when your patient is expecting.

Liver diseases in pregnancy can be specific to gestation or only coincidental. In the latter case, the diagnosis can be difficult. Rapid diagnosis of maternal-fetal emergencies and situations requiring specialized interventions are crucial to preserve the maternal liver and guarantee materno-fetal survival. While detailed questioning of the patient and a clinical examination are highly important, imaging is often essential to reach a diagnosis of these liver diseases and lesions. Three groups of liver diseases may be observed during pregnancy: (1) diseases related to pregnancy: intrahepatic cholestasis of pregnancy, pre-eclampsia, eclampsia, hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome, and acute fatty liver of pregnancy; (2) liver diseases that are more frequent during or exacerbated by pregnancy: acute herpes simplex hepatitis, Budd-Chiari syndrome, hemorrhagic hereditary telangiectasia, hepatocellular adenoma, portal vein thrombosis, and cholelithiasis; (3) coincidental conditions, including acute hepatitis, incidental focal liver lesions, metabolic dysfunction-associated steatotic liver disease, cirrhosis, hepatocellular carcinoma, liver abscesses and parasitosis, and liver transplantation. Specific knowledge of the main imaging findings is required to reach an early diagnosis, for adequate follow-up, and to avoid adverse consequences in both the mother and the fetus.Critical relevance statement Pregnancy-related liver diseases are the most important cause of liver dysfunction in pregnant patients and, in pregnancy, even common liver conditions can have an unexpected turn. Fear of radiations should never delay necessary imaging studies in pregnancy.Key points• Pregnancy-related liver diseases are the most frequent cause of liver dysfunction during gestation.• Fear of radiation should never delay necessary imaging studies.• Liver imaging is important to assess liver emergencies and for the diagnosis and follow-up of any other liver diseases.• Common liver conditions and lesions may take an unexpected turn during pregnancy.• Pregnancy-specific diseases such as pre-eclampsia and HELLP syndrome must be rapidly identified. However, imaging should never delay delivery when it is considered to be urgent for maternal-fetal survival.

Case Report: Budd-Chiari Syndrome (BCS) on Hepatocellular Carcinoma (HCC): Primary or Secondary Cause?

This is the case of a 63-year-old female patient admitted for management of a liver mass discovered incidentally on imaging. The patient presented with weight loss and prutitus, and on clinical examination had collateral venous circulation and scraping lesions. Abdominal ultrasound coupled with Doppler showed a hepatic tissue mass with left-sided portal thrombosis. Hepatic MRI confirmed the diagnosis and also revealed SBC with infiltration of the left and median hepatic veins. Liver biopsy confirmed diagnosis of hepatocellular carcinoma (HCC)in cirrhotic liver. Etiological work-up for prothrombotic factors and cirrhosis was negative. The patient was a candidate for palliative treatment. But our dilemma: is this a case of primary or secondary BCS? Scholars Middle East Publishers

Evaluation of potential hepatic recompensation criteria in patients with PBC and decompensated cirrhosis.

Aetiological therapy improves liver function and may enable hepatic recompensation in decompensated cirrhosis. We explored the potential for recompensation in patients with decompensated primary biliary cholangitis (PBC) - considering a biochemical response to ursodeoxycholic acid (UDCA) according to Paris-II criteria as a surrogate for successful aetiological treatment. Patients with PBC were retrospectively included at the time of first decompensation. Recompensation was defined as (i) resolution of ascites and hepatic encephalopathy (HE) despite discontinuation of diuretic/HE therapy, (ii) absence of variceal bleeding and (iii) sustained liver function improvement. In total, 42 patients with PBC with decompensated cirrhosis (age: 63.5 [IQR: 51.9-69.2] years; 88.1% female; MELD-Na: 13.5 [IQR: 11.0-15.0]) were included and followed for 41.9 (IQR: 11.0-70.9) months after decompensation. Seven patients (16.7%) achieved recompensation. Lower MELD-Na (subdistribution hazard ratio [SHR]: 0.90; p = 0.047), bilirubin (SHR per mg/dL: 0.44; p = 0.005) and alkaline phosphatase (SHR per 10 U/L: 0.67; p = 0.001) at decompensation, as well as variceal bleeding as decompensating event (SHR: 4.37; p = 0.069), were linked to a higher probability of recompensation. Overall, 33 patients were treated with UDCA for ≥1 year and 12 (36%) achieved Paris-II response criteria. Recompensation occurred in 5/12 (41.7%) and in 2/21 (9.5%) patients with vs. without UDCA response at 1 year, respectively. Recompensation was linked to a numerically improved transplant-free survival (HR: 0.46; p = 0.335). Nonetheless, 4/7 recompensated patients presented with liver-related complications after developing hepatic malignancy and/or portal vein thrombosis and 2 eventually died. Patients with PBC and decompensated cirrhosis may achieve hepatic recompensation under UDCA therapy. However, since liver-related complications still occur after recompensation, patients should remain under close follow-up.

Recent outcomes of liver transplantation for Budd-Chiari syndrome: A study of the European Liver Transplant Registry (ELTR) and affiliated centers.

Management of Budd-Chiari syndrome (BCS) has improved over the last decades. The main aim was to evaluate the contemporary post-liver transplant (post-LT) outcomes in Europe. Data from all patients who underwent transplantation from 1976 to 2020 was obtained from the European Liver Transplant Registry (ELTR). Patients < 16 years, with secondary BCS or HCC were excluded. Patient survival (PS) and graft survival (GS) before and after 2000 were compared. Multivariate Cox regression analysis identified predictors of PS and GS after 2000. Supplemental data was requested from all ELTR-affiliated centers and received from 44. In all, 808 patients underwent transplantation between 2000 and 2020. One-, 5- and 10-year PS was 84%, 77%, and 68%, and GS was 79%, 70%, and 62%, respectively. Both significantly improved compared to outcomes before 2000 ( p < 0.001). Median follow-up was 50 months and retransplantation rate was 12%. Recipient age (aHR:1.04,95%CI:1.02-1.06) and MELD score (aHR:1.04,95%CI:1.01-1.06), especially above 30, were associated with worse PS, while male sex had better outcomes (aHR:0.63,95%CI:0.41-0.96). Donor age was associated with worse PS (aHR:1.01,95%CI:1.00-1.03) and GS (aHR:1.02,95%CI:1.01-1.03). In 353 patients (44%) with supplemental data, 33% had myeloproliferative neoplasm, 20% underwent TIPS pre-LT, and 85% used anticoagulation post-LT. Post-LT anticoagulation was associated with improved PS (aHR:0.29,95%CI:0.16-0.54) and GS (aHR:0.48,95%CI:0.29-0.81). Hepatic artery thrombosis and portal vein thrombosis (PVT) occurred in 9% and 7%, while recurrent BCS was rare (3%). LT for BCS results in excellent patient- and graft-survival. Older recipient or donor age and higher MELD are associated with poorer outcomes, while long-term anticoagulation improves both patient and graft outcomes.

Splanchnic Vein Thrombosis: The State-of-the-Art on Anticoagulant Treatment.

Splanchnic vein thrombosis (SVT) is a rare type of venous thromboembolism occurring within the splanchnic venous system. Portal vein thrombosis is the most common presentation, while Budd-Chiari syndrome is the least common. Liver cirrhosis and abdominal solid cancer are the main local risk factors for SVT, whereas myeloproliferative neoplasms are the predominant systemic risk factors. Signs and symptoms of SVT are nonspecific and include abdominal pain, gastrointestinal bleeding, and ascites. Asymptomatic SVT is not uncommon, and the majority would be detected incidentally on routine abdominal imaging performed for the follow-up of liver diseases and tumors. The management of SVT aims to prevent thrombus progression, promote vessel recanalization, and prevent recurrent venous thromboembolism. Anticoagulation should be started early in order to increase the chances of vessel recanalization and reduce the risk of portal hypertension-related complications. Direct oral anticoagulants have been included in recent guidelines, as alternatives to vitamin K antagonists, after clinical stability has been reached; however, caution is required in patients with liver or kidney dysfunction. Treatment duration is based on the presence (or absence) and type (transient vs. permanent) of risk factors. This narrative review aims to summarize the latest evidence on SVT, with a particular focus on the anticoagulant treatment in special categories of patients (e.g., liver cirrhosis, solid cancer, myeloproliferative neoplasms, pancreatitis, incidentally detected SVT, Budd-Chiari syndrome, and chronic SVT).

Innovations in endoscopic ultrasound for portal hypertension and its role in managing complications in clinical practice: Lessons learned from a tertiary referral public hospital

AbstractPortal hypertension (PH) poses significant challenges. This paper presents an innovative study on the utilization of endoscopic ultrasound (EUS) for both the diagnosis and management of PH. Conducted at Dr. Cipto Mangunkusumo National General Hospital in Jakarta, this retrospective case series included patients diagnosed with PH through clinical examination, imaging evaluation, and esophagogastroduodenoscopy. Exclusion criteria comprised a history of reduced blood consumption within the last 5 days, hepatocellular carcinoma, massive ascites, or elevated international normalized ratio (&gt;1.4). EUS‐guided portal pressure gradient (PPG) measurements were performed using an innovative standard manometer. The study involved 15 patients, with 14 having liver cirrhosis and 1 diagnosed with Budd–Chiari syndrome. Among them, nine patients experienced bleeding due to gastroesophageal varices. Small and large esophageal varices were identified in four and eight patients, respectively. Gastroesophageal varices type 1 were observed in two patients, and type 2 in four patients. Isolated gastric fundal varices type 1 were present in one patient. Based on EUS‐PPG measurements, 14 patients exhibited clinically significant portal hypertension. Seven patients underwent endoscopic band ligation and three underwent EUS‐guided cyanoacrylate injection during the same session as the EUS‐PPG measurement procedure. Notably, no adverse events, such as abdominal pain, perforation, or bleeding were observed during or after the procedure. EUS emerges as a promising and accurate tool for both diagnosis and management.

Fibrosis Progression in Patients with Budd–Chiari Syndrome and Transjugular Intrahepatic Portosystemic Shunt (TIPS): A Long-Term Study Using Transient Elastography

Hepatic vein outflow obstruction causes congestion of the liver, leading to necrosis, fibrosis, and portal hypertension (PH). A transjugular intrahepatic portosystemic shunt (TIPS) reduces congestion and PH by providing artificial outflow. The aim of the study was to investigate fibrosis progression in patients with Budd–Chiari syndrome (BCS) and TIPS using transient elastography (TE). From 2010 to 2022, 25 patients received 80 TEs using FibroScan®, Echosens, Paris, France (3.2 ± 2.1 per patient). TIPS function was assessed via Doppler ultrasound or radiological intervention. At the time of TE examination, 21 patients had patent shunts. Four patients had occluded shunts but normal pressure gradients during the intervention. The first TE measurement performed 9.8 ± 6.8 years after the BCS diagnosis showed stiffness values of 24.6 ± 11.5 kPa. A second or last measurement performed 7.0 ± 2.9 years after the first measurement showed similar stiffness values of 24.1 ± 15.7 kPa (p = 0.943). Except for three patients, the liver stiffness was always &gt;12 kPa, indicating advanced fibrosis. Stiffness values obtained &lt;5 years (n = 8, 23.8 ± 9.2 kPa) or &gt;5 years after the BCS diagnosis (24.9 ± 12.7 kPa) did not differ (p = 0.907). In addition, stiffness was not related to the interval between BCS and TIPS implantation (p = 0.999). One patient received liver transplantation, and two patients died from non-hepatic causes. Most patients developed mild to moderate cirrhosis, possibly during the early phase of the disease. Timing of TIPS did not influence fibrosis progression. This and the release of portal hypertension may argue in favor of a generous TIPS implantation practice in patients with BCS.

Safety and effectiveness of SARS-CoV-2 vaccines for patients with intractable hepatobiliary diseases: A multicenter, questionnaire-based, cross-sectional study.

There are few data regarding the safety and effectiveness of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccines in patients with intractable hepatobiliary diseases. We conducted a multicenter, questionnaire-based, cross-sectional study to determine the safety and effectiveness of the SARS-CoV-2 vaccines in Japanese patients with intractable hepatobiliary disease. Patients aged ≥18years with autoimmune hepatitis (AIH), primary biliary cholangitis, primary sclerosing cholangitis, Budd-Chiari syndrome, idiopathic portal hypertension, and extrahepatic portal vein obstruction at each center were consecutively invited to join the study. Participants were asked to complete a questionnaire regarding their characteristics, vaccination status, post-vaccination adverse effects, and SARS-CoV-2 infection. Additionally, liver disease status, treatment regimens, and liver function test values pre- and post-vaccination were collected. The survey was conducted from September 2021 to May 2022, and 528 patients (220 AIH, 251 primary biliary cholangitis, 6 AIH- primary biliary cholangitis/primary sclerosing cholangitis overlap, 39 primary sclerosing cholangitis, 4 Budd-Chiari syndrome, 5 idiopathic portal hypertension, and 3 extrahepatic portal vein obstruction) participated in the study. Post-vaccination adverse effects were comparable to those observed in the general population. Post-vaccination liver injuries classified as grade 1 or higher were observed in 83 cases (16%), whereas grades 2 and 3 were observed in only six cases (1.1%); AIH-like liver injury requiring treatment was not observed. Overall, 12 patients (2.3%) were infected with SARS-CoV-2, and only one patient was infected 6months after the second vaccination. SARS-CoV-2 vaccines demonstrated satisfactory safety and effectiveness in Japanese patients with intractable hepatobiliary diseases.