Clinical profile of adult and pediatric patients with hepatic Wilson's disease.

BACKGROUND: The clinical profile varies in patients with Wilson's disease (WD). There is paucity of data regarding adult and pediatric patients with hepatic WD. METHODS: As many as 140 consecutive patients diagnosed with hepatic WD between December 2006 and January 2021 were included in the study. Data was collected regarding the demographic parameters, clinical presentation, extrahepatic organ involvement, liver histology and laboratory investigations. Adult and children (0-14 years) with hepatic WD were compared regarding these features. RESULT: Eighty-eight adults and 52 children were included in the study. The median age of presentation was 17 years (range: 1.1-42 years). Male preponderance was seen (adult 68/88, 69%; children 40/52, 77%). Adults as compared to children presented more commonly as cirrhosis (52/88 vs. 15/52, p = 0.0005) and with hepatic decompensation (35/88 vs. 9/52, p = 0.005). Presentation with acute-on-chronic liver failure (ACLF) was more common in children (10/52 vs. 2/88, p = 0.0005). Twenty-eight-day mortality was 50% (5/10) in children and none in adults presenting with ACLF. Nazer's Prognostic Index (≥ 7) and New Wilson Index were more accurate in predicting mortality among children with ACLF with AUROC 1, while AARC (APASL ACLF Research Consortium) was less accurate with AUROC 0.45. Liver histology findings were similar in adults and children. Extrahepatic involvement was also similar. (8/88 in adults vs. 3/52 children, p value 0.48). CONCLUSION: Most patients with WD present as cirrhosis in adulthood. ACLF is more common in children. Nazer's prognostic index and new Wilson Index score are accurate in predicting mortality in children with ACLF.

Outcome of individuals with alcoholic cirrhosis hospitalized with first decompensation and their predictors.

BACKGROUND OBJECTIVES: Alcohol is one of most common aetiologies of cirrhosis and decompensated cirrhosis is linked to higher morbidity and death rates. This study looked at the outcomes and mortality associated risk variables of individuals with alcoholic cirrhosis who had hospitalization with their first episode of decompensation. METHODS: Individuals with alcoholic cirrhosis who were hospitalized with the first episode of decompensation [acute decompensation (AD) or acute-on-chronic liver failure (ACLF)] were included in the study and were prospectively followed up until death or 90 days, whichever was earlier. RESULTS: Of the 227 study participants analyzed, 167 (73.56%) and 60 (26.43%) participants presented as AD and ACLF, respectively. In the ACLF group, the mortality rate at 90 days was higher than in the AD group (48.3 vs 32.3%, P=0.02). In the AD group, participants who initially presented with ascites as opposed to variceal haemorrhage had a greater mortality rate at 90 days (36.4 vs 17.1%, P=0.041). The chronic liver failure-consortium AD score and the lactate-free Asian Pacific Association for the study of the Liver-ACLF research consortium score best-predicted mortality in individuals with AD and ACLF. INTERPRETATION CONCLUSIONS: There is significant heterogeneity in the type of decompensation in individuals with alcoholic cirrhosis. We observed significantly high mortality rate among alcoholic participants hospitalized with initial decompensation; deaths occurring in more than one-third of study participants within 90 days.

Changing Etiological Spectrum of Hepatocellular Carcinoma in India-A Systematic Review and Meta-analysis.

Background: Recent studies from both India and outside India have shown a change in the etiological profile of hepatocellular carcinoma (HCC). We aimed to analyze the etiological spectrum and changing trends of HCC etiology in India using a systematic review of current literature and meta-analysis. Methods: Electronic databases of PubMed/Medline, Scopus, and Embase were searched from inception to July 2023 for studies reporting the data on the etiology of HCC from India. The pooled proportions with 95% confidence interval were calculated using summative statistics. Results: A total of 60 studies (n = 12,327) were included in the final analysis. The pooled proportions of HCC cases with at least one positive and negative viral marker were 56.0 (49.5-62.6) and 43.1% (36.5-49.8), respectively. The pooled proportion of HCC cases with positive hepatitis B virus (HBV) markers was 41.0 (35.8-46.1), while those with positive markers for hepatitis C virus were 20.3 (17.0-23.6). The pooled proportion of cases with HCC with significant alcohol intake was 19.0% (15.6-22.4), and those related to nonalcoholic fatty liver disease (NAFLD) were 16.9% (12.1-21.7). Around 7.9% (5.8-10.0) of the cases had HCC with multiple etiologies. Subgroup analysis showed a significant variation with the location of the study based on zone. Meta-regression analysis based on publication year (1990-2023) showed a significant reduction in the proportion of cases with HBV and an increase in cases with NAFLD. In contrast, the proportion of cases with hepatitis C virus and alcohol did not change significantly. Conclusion: Viral hepatitis is the most common etiology of HCC in India, predominantly HBV. The proportions of cases with HCC related to NAFLD are increasing, and those related to HBV are declining.

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

BACKGROUND: There is scant literature on hepatocellular carcinoma (HCC) in patients with Budd-Chiari syndrome (BCS). AIM: To assess the magnitude, clinical characteristics, feasibility, and outcomes of treatment in BCS-HCC. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

APASL clinical practice guidelines on the management of acute kidney injury in acute-on-chronic liver failure.

Acute-on-chronic liver failure (ACLF) is a syndrome that is characterized by the rapid development of organ failures predisposing these patients to a high risk of short-term early death. The main causes of organ failure in these patients are bacterial infections and systemic inflammation, both of which can be severe. For the majority of these patients, a prompt liver transplant is still the only effective course of treatment. Kidneys are one of the most frequent extrahepatic organs that are affected in patients with ACLF, since acute kidney injury (AKI) is reported in 22.8-34% of patients with ACLF. Approach and management of kidney injury could improve overall outcomes in these patients. Importantly, patients with ACLF more frequently have stage 3 AKI with a low rate of response to the current treatment modalities. The objective of the present position paper is to critically review and analyze the published data on AKI in ACLF, evolve a consensus, and provide recommendations for early diagnosis, pathophysiology, prevention, and management of AKI in patients with ACLF. In the absence of direct evidence, we propose expert opinions for guidance in managing AKI in this very challenging group of patients and focus on areas of future research. This consensus will be of major importance to all hepatologists, liver transplant surgeons, and intensivists across the globe.

Ammonia is associated with liver-related complications and predicts mortality in acute-on-chronic liver failure patients.

The relationship between ammonia and liver-related complications (LRCs) in acute-on-chronic liver failure (ACLF) patients is not clearly established. This study aimed to evaluate the association between ammonia levels and LRCs in patients with ACLF. The study also evaluated the ability of ammonia in predicting mortality and progression of LRCs. The study prospectively recruited ACLF patients based on the APASL definition from the ACLF Research Consortium (AARC) from 2009 to 2019. LRCs were a composite endpoint of bacterial infection, overt hepatic encephalopathy (HE), and ascites. A total of 3871 cases were screened. Of these, 701 ACLF patients were enrolled. Patients with LRCs had significantly higher ammonia levels than those without. Ammonia was significantly higher in patients with overt HE and ascites, but not in those with bacterial infection. Multivariate analysis found that ammonia was associated with LRCs. Additionally, baseline arterial ammonia was an independent predictor of 30-day mortality, but it was not associated with the development of new LRCs within 30 days. In summary, baseline arterial ammonia levels are associated with 30-day mortality and LRCs, mainly overt HE and ascites in ACLF patients.

Risk of tuberculosis with anti-TNF therapy in Indian patients with inflammatory bowel disease despite negative screening.

BACKGROUND AND STUDY AIMS: Tuberculosis (TB) is a well-recognized adverse effect associated with using biological therapy to manage various autoimmune conditions. There is a dearth of information about the development of TB after using anti-TNF agents in patients with inflammatory bowel disease (IBD) from TB-endemic countries like India. This study aimed to estimate the risk of TB and its predictors after treatment with anti-TNF agents in patients with IBD. PATIENTS AND METHODS: The present study is a retrospective analysis of data of patients with IBD from two tertiary care centers in India receiving anti-TNF therapy. Patients who had undergone chest X-ray, high-resolution computed tomography of the chest, and tuberculin skin test, with a follow-up duration of at least 6 months, were included in the analysis. RESULTS: In this multi-center study, 95 patients on anti-TNF agents for IBD (Median age of onset: 27 years, 62.1 % males) were followed up for a median duration of 9 (6-142) months. Among patients with IBD, 79 (83.2 %) had Crohn's disease, and 16 (16.8 %) had ulcerative colitis. Infliximab was the commonest biological, used in 82.1 % of cases, followed by adalimumab (17.9 %). On follow-up, 8.4 % (8/95) of the patients developed TB, among which the majority had extrapulmonary tuberculosis (5/8). On multivariate analysis, the duration of biological (Odds ratio: 1.047, 95 % confidence interval 1.020-1.075; p = 0.001) use was the only independent predictor of the development of TB with biologicals. CONCLUSION: Among Indian patients with IBD, there is a high risk of TB with anti-TNF agents, which increases with the duration of therapy. The current methods for latent TB screening in Indians are ineffective, and predicting TB after initiating biological therapy is difficult.

Safety and efficacy of endoscopic retrograde cholangiopancreatography in pediatric pancreatic and biliary disorders.

INTRODUCTION: There is sparse data from India on indications, technical success, safety and outcomes of endoscopic retrograde cholangiopancreatography (ERCP) using standard adult duodenoscope in the pediatric population. METHODS: Retrospective analysis of prospectively maintained electronic endoscopy and clinical database was performed to identify pediatric patients (age ≤ 18 years) who underwent ERCP between January 2017 and December 2022. Demographics and procedural details including indications, cholangio-pancreatogram findings, endotherapy type performed, technical and clinical success and complications were noted. RESULTS: As many as 150 pediatric patients were included of whom 88 had pancreatic (mean age-13.7 years) and 62 had biliary disease (mean age- 14.9 years). Common pancreatic ERCP indications were chronic pancreatitis (n = 45 [51.1%]), pancreatic duct disruption fistula (n = 21 [23.9%]) and recurrent acute pancreatitis (n = 16 [18.2%]). Among biliary indications were choledocholithiasis (n = 29 [46.8%]), benign bile duct strictures (n = 13 [21%]), bile duct injury/leak and biliary stent removal (n = 7 [11.3%]) , choledochal cyst (n = 5  [8.1%]) and pancreatic mass causing biliary compression (n = 1 [1.6%]). Technical success in pancreatic and biliary ERCP was 94.3% and 95.2%, respectively, and clinical success was 84.1% and 93.5%, respectively. Most common complications following pancreatic ERCPs were acute pancreatitis (n = 9 [10.2%]) (mild = 5, moderate = 4) patients and post sphincterotomy bleed in one (1.1%). Among biliary ERCPs, post ERCP pancreatitis was seen in (n = 3 [4.8%]) (mild = 2, moderate = 1). CONCLUSION: ERCP can be safely and effectively performed in children using standard duodenoscope. Chronic pancreatitis, choledocholithiasis and pancreatic divisum are common pediatric ERCP indications.

Upfront tofacitinib in patients with biological-naïve ulcerative colitis - An Indian multicentric experience.

OBJECTIVES: Tofacitinib is a Janus Kinase inhibitor used for treating moderate to severe ulcerative colitis (UC), mainly after the failure of biological therapy. There is a paucity of data on the outcome of tofacitinib in biological-naïve UC patients. The present study was aimed at analyzing the safety and efficacy of tofacitinib in biological-naïve Indian patients with UC. METHODS: The present study retrospectively evaluated consecutive patients with biological-naïve moderate-to-severe active UC from six tertiary care centers in India receiving tofacitinib from September 2020 to September 2022. Clinical remission or response assessment was based on partial Mayo score (PMS) calculated at baseline and weeks eight, 16 and 24. RESULTS: Total 47 cases (57.4% male, median age: 32 years) were included. After eight weeks of therapy, 33 (70.2%) achieved clinical remission and eight (17.0%) had a primary failure. The baseline serum albumin at treatment initiation was the only independent predictor of remission at eight weeks (Odds ratio: 11.560, 95% CI: 1.478 - 90.404), but not at 16 weeks. By 24 weeks, 59.6% (28/47) of the patients were in remission and 29.8% (14/47) had stopped tofacitinib either due to failure (27.6%) or adverse events (AEs) (2.1%). Among the 47 patients, 10 (21.2%) cases developed AEs during follow-up, including two tuberculosis (4.2%), one cytomegalovirus (CMV) colitis (2.1%) and one herpes zoster (2.1%). Four patients with infection required temporary drug discontinuations. One required permanent discontinuation (mania). CONCLUSION: Upfront tofacitinib is effective in biologic-naïve Indian patients with moderate-severe UC. Further randomized studies are required to validate the study findings.

Navigating portal hypertension: Unlocking safe passage to healthy pregnancy in EHPVO.

BACKGROUND AND AIMS: Pregnancy is associated with hyperdynamic circulatory state and increased risk of portal hypertension related complications in patients with extra-hepatic portal vein obstruction (EHPVO). We aim to study the impact of EHPVO on pregnancy-related outcomes with focus on subset of patients with UGIB (upper GI bleed). METHODS: Retrospective analysis of obstetric, maternal and neonatal outcomes of patients with EHPVO registered between January 2006 and December 2022. Forty-five patients were included. Forty-five healthy females with low-risk pregnancies formed the control group. RESULTS: Adverse obstetric and neonatal outcomes were comparable between EHPVO and control group (22% vs. 28.6%; p > .05; low birth weight/ small for gestational age 17.8% vs. 36%, p = .0918 and 14.2% vs. 10%, p = .5698 respectively). Adverse outcomes were similar in patients with and without history of UGIB (26.3% vs. 19.4%, p = .0814; 17.8% vs. 36%, p = .0918; 14.2% vs. 10%, p = .5698). There was no maternal mortality in both the groups. A total of 7% pregnancies in EHPVO patients were complicated by ascites. CONCLUSIONS: EHPVO pregnancies have successful obstetric and neonatal outcomes with adequate management of portal hypertension.

Exploring the utility of EUS in Budd-Chiari syndrome: a multicenter multiobserver prospective cohort study.

BACKGROUND AND AIMS: The diagnosis of Budd-Chiari syndrome (BCS) is essentially radiologic and is needed to plan appropriate therapy. We therefore conducted this proof of concept study to assess the utility of EUS in assessing the anatomy of BCS. METHODS: This prospective, multicenter, observational study enrolled 50 consecutive patients with a diagnosis of BCS. All patients underwent a detailed EUS examination by 3 independent endosonographers, blinded to the anatomic details of BCS and others' findings. The EUS examination was compared between the endosonographers and with conventional angiography (where available) or magnetic resonance venography (MRV). Outcomes assessed were interobserver agreement, sensitivity, specificity, positive predictive value, negative predictive value, and overall accuracy in diagnosing pathologic lesions of BCS. RESULTS: Fifty BCS patients (mean age, 34 years [range, 13-65]) underwent EUS. Results showed good agreement among endosonographers for diagnosing right hepatic vein (κ = .716) and left hepatic vein lesions (κ = .722), moderate agreement for middle hepatic vein lesions (κ = .660), and very good agreement for inferior vena cava (IVC) lesions (κ = .823). EUS demonstrated high sensitivity and positive predictive value, low interobserver variability, and overall diagnostic accuracy for BCS lesions. CONCLUSIONS: EUS is a safe and accurate diagnostic tool for BCS. It can provide accurate mapping of hepatic veins, intrahepatic collaterals, and the IVC.

2023 Update of Indian National Association for Study of the Liver Consensus on Management of Intermediate and Advanced Hepatocellular Carcinoma: The Puri III Recommendations.

Hepatocellular carcinoma (HCC) presents significant treatment challenges despite considerable advancements in its management. The Indian National Association for the Study of the Liver (INASL) first published its guidelines to aid healthcare professionals in the diagnosis and treatment of HCC in 2014. These guidelines were subsequently updated in 2019. However, INASL has recognized the need to revise its guidelines in 2023 due to recent rapid advancements in the diagnosis and management of HCC, particularly for intermediate and advanced stages. The aim is to provide healthcare professionals with evidence-based recommendations tailored to the Indian context. To accomplish this, a task force was formed, and a two-day round table discussion was held in Puri, Odisha. During this event, experts in their respective fields deliberated and finalized consensus statements to develop these updated guidelines. The 2023 INASL guidelines offer a comprehensive framework for the diagnosis, staging, and management of intermediate and advanced HCC in India. They represent a significant step forward in standardizing clinical practices nationwide, with the primary objective of ensuring that patients with HCC receive the best possible care based on the latest evidence. The guidelines cover various topics related to intermediate and advanced HCC, including biomarkers of aggressive behavior, staging, treatment options, and follow-up care.

Utility of gastroscopy in the left lateral semi-recumbent position: A blood-free coup!

Background and study aims The utility of esophagogastroduodenoscopy (EGD) in the left lateral semi-recumbent position in the setting of acute upper gastrointestinal bleeding (UGIB) with a persistent fundal pool of blood for adequate visualization and effective endotherapy has not been studied. This study aimed to evaluate the feasibility, efficacy, and safety of this position in such settings. Patients and methods A prospective study of patients presenting with acute UGIB with an uncleared fundal pool of blood was conducted. All underwent EGD in the left lateral decubitus and secondary left lateral semi-recumbent positions. Outcomes in secondary position in terms of adequate visualization of the fundus, identification of new or additional sources of bleeding, and effectiveness of endotherapy were studied. Results We screened 860 patients and included 44 patients (5.11%) with a persistent fundal pool of blood. Endoscopy in the primary position revealed the source of bleeding in 37 of 44 patients (84%). The source of the bleeding was not identified in seven of 44 patients (16%). Endoscopy in the secondary position showed clearance of fundal pool in all 44 patients (100%). A new source of bleeding was identified in all seven patients (100%) and an additional source could be identified in another five patients (13.6%). Endotherapy was performed in the secondary position for all 44 patients with 100% technical success and 94% clinical success. Conclusions These data show that endotherapy in the left lateral semi-recumbent position is feasible, safe, and effective. It should be done when endoscopy in the left lateral decubitus position reveals a pool of blood in the fundus and there is inadequate visualization of the fundus.

Predictors of proximal migration of straight biliary plastic stents.

BACKGROUND/AIMS: Proximal biliary plastic stent migration (PSM) remains a challenging complication. The study aims at determining the PSM rate, retrieval outcomes and factors associated with PSM. METHODS: Endoscopy database was analyzed from January 2016 to January 2021 to identify 1137 patients, who underwent stent removal or repeat endoscopic retrograde cholangiopancreatography (ERCP). Demography, methods of stent retrieval, outcomes and complications were noted. Logistic regression was performed to determine risk factors for PSM. Propensity score matching was done in a 1:1 manner using age, sex, comorbidities and indication to assess endoscopy-related factors. Clinical trial registration done (CTRI/2022/02/040516). RESULTS: PSM was noted in 74 (6.5%) cases. Stent retrieval was successful in 94.59% (70/74) of cases. A balloon catheter (46/74) was commonly used. Technical failure was due to an impacted stent (2) and stent above the stricture (2). Complications were seen in 2.7% of cases. On multi-variate regression, sphincteroplasty at index ERCP (Odds ratio [OR] = 5.68, 95% confidence interval [CI] = 2.7-11.89), stent length < 10 cm (OR = 8.53, 95% CI = 3.2-22.47), 7-Fr stent (OR = 18.25, 95% CI = 6.5-50.64), dilated bile duct (mean diameter- 9.2 ± 3.94 mm) (OR = 0.384, 95% CI = 0.18-0.72) and delayed ERCP by > 3 months from index ERCP (OR = 15.28, 95% CI = 8.1-28.49). After performing propensity score matching for age, sex, comorbidities and indication to determine endoscopy-related factors, 7-Fr stent size (OR 3.495; 95% CI-1.23-9.93) and duration of indwelling stent for more than three months (OR-3.37; 95% CI-1.646-6.76) were significantly associated with proximal stent migration. CONCLUSION: Proximally migrated straight stents can be successfully retrieved using standard accessories. The use of 7-Fr size stent, stents indwelling for more than three months, sphincteroplasty at index ERCP, stent length < 10 cm and dilated bile duct were associated with increased risk of proximal migration of straight biliary plastic stents. After propensity score matching, the use of 7-Fr size stents and stent indwelling for over three months were endoscopy-related factors associated with proximal migration.

Survival of Trial-Like and Non-Trial-Like Patients With Immunotherapy in Advanced Hepatocellular Carcinoma in Real World: A Collaborative Multicenter Indian Study (IMHEP).

PURPOSE: Immune checkpoint inhibitors (ICIs) is the initial line of management in advanced hepatocellular carcinoma (HCC), but survivals in the real world are not known. MATERIALS AND METHODS: A retrospective study of patients with advanced HCC receiving ICIs (as first-line therapy or as later lines of therapy) across 11 Indian institutions was conducted. Patients were divided into either cohort 1 (trial-like receiving ICI as first-line therapy), with a Child Pugh score (CTP) of ≤6, an Eastern Cooperative Oncology Group performance status (ECOG PS) of 0/1, and no VP4 (main portal vein thrombosis [MPVT]) or cohort 2 (trial unlike) who did not satisfy at least one of the above criteria. The primary end point was 12-month overall survival (OS). RESULTS: Between January 2017 and January 2022, 133 patient data were analyzed. The presence of MPVT was seen in 33 patients (25%). The ICIs used were atezolizumab-bevacizumab, nivolumab, and pembrolizumab in 89 (66%), 44 (33%), and one (1%) patients, respectively. With a median follow-up of 13.8 months, the 12-month OS for the entire cohort was 33.4% (95% CI, 23.6 to 43.2). Patients in cohort 1 (n = 31) had a significantly improved OS compared with patients in cohort 2 (n = 102; 12-month OS, 57.9% [95% CI, 38.5 to 77.3] v 24% [95% CI, 13.4 to 34.6]; P = .005). Patients with CTP A as compared with CTP B (9.7 v 4.3 months; P < .001) and an ECOG PS of 0/1 as compared with a PS of ≥2 (8.7 v 7.2 months; P = .04) and without MPVT (9.4 v 4.0; P < .001) had superior survivals. CONCLUSION: Patients with advanced HCC in the real world, trial-like have survivals in consonance with trial data, whereas patients with features excluding them from trials, such as main portal vein thrombosis, poor ECOG PS, and child Pugh B status, have markedly inferior survivals, despite good tolerance to immunotherapy.

Management of Established Small-for-size Syndrome in Post Living Donor Liver Transplantation: Medical, Radiological, and Surgical Interventions: Guidelines From the ILTS-iLDLT-LTSI Consensus Conference.

Small-for-size syndrome (SFSS) following living donor liver transplantation is a complication that can lead to devastating outcomes such as prolonged poor graft function and possibly graft loss. Because of the concern about the syndrome, some transplants of mismatched grafts may not be performed. Portal hyperperfusion of a small graft and hyperdynamic splanchnic circulation are recognized as main pathogenic factors for the syndrome. Management of established SFSS is guided by the severity of the presentation with the initial focus on pharmacological therapy to modulate portal flow and provide supportive care to the patient with the goal of facilitating graft regeneration and recovery. When medical management fails or condition progresses with impending dysfunction or even liver failure, interventional radiology (IR) and/or surgical interventions to reduce portal overperfusion should be considered. Although most patients have good outcomes with medical, IR, and/or surgical management that allow graft regeneration, the risk of graft loss increases dramatically in the setting of bilirubin >10 mg/dL and INR>1.6 on postoperative day 7 or isolated bilirubin >20 mg/dL on postoperative day 14. Retransplantation should be considered based on the overall clinical situation and the above postoperative laboratory parameters. The following recommendations focus on medical and IR/surgical management of SFSS as well as considerations and timing of retransplantation when other therapies fail.

Hepatocellular Carcinoma with Hepatic Vein and Inferior Vena Cava Invasion.

Hepatocellular carcinoma (HCC) invades intrahepatic vessels causing tumor thrombosis. Infrequently, there is involvement of the hepatic vein (HV) and inferior vena cava (IVC). In this review, we summarize the epidemiology, classification, clinical features, and management of HCC with HV and IVC invasion. While the involvement of HV and IVC usually portends an overall poor survival, selected patients may be candidates for aggressive treatment and thus improving outcomes.

Maternal and fetal outcomes in pregnant patients with non-cirrhotic portal hypertension: A systematic review and meta-analysis.

Background: Concerned studies with respect to the outcome of pregnant patients with non-cirrhotic portal hypertension are limited. Thus, a systematic review and meta-analysis of the available literature was conducted. Methods: A literature search was conducted from 1999 to December 2021 for studies evaluating pregnancy outcomes in patients with non-cirrhotic portal hypertension. Results: Twelve studies were included in the meta-analysis. The pooled rate of variceal bleeding, ascites and severe anemia requiring blood transfusion were 9.6%, 2.3%, and 14.9%, respectively. The pooled rate of spontaneous miscarriage, gestational hypertension, delivery by cesarean section, and postpartum hemorrhage were 11.9%, 4.5%, 36.7%, and 4.7%, respectively. The pooled stillbirth rate was 2.5% and among the live births, the pooled rates of preterm birth, low birth weight, intensive care unit admission, and neonatal mortality were 21.6%, 18.7%, 15.5%, and 1.8%, respectively. Conclusion: Pregnancy in patients with non-cirrhotic portal hypertension is associated with increased maternal & fetal morbidity but mortality remains low.