Primary Sclerosing Cholangitis Limited to Intrahepatic Bile Ducts Has Distinctly Better Prognosis.

BACKGROUND: There are two sub-phenotypes of large-duct primary sclerosing cholangitis (PSC): isolated intrahepatic PSC (IIPSC) and extrahepatic disease with or without intrahepatic (extra/intrahepatic). AIMS: This study examined the differences in outcomes in patients with IIPSC compared to extra/intrahepatic and small-duct PSC. METHODS: Patients with PSC treated at our institution from 1998 to 2019 were investigated. Biochemistries, clinical events, and survival were assessed by chart review and National Death Index. Cox-proportional hazards were used to determine the risk of clinical outcomes based on biliary tract involvement. RESULTS: Our cohort comprised 442 patients with large-duct PSC (57 had IIPSC, 385 had extra/intrahepatic PSC) and 23 with small-duct PSC. Median follow-up in the IIPSC group was not significantly different from the extra/intrahepatic group [7 vs. 6 years, P = 0.06]. Except for lower age (mean 37.9 vs. 43.0 years, P = 0.045), the IIPSC group was not different from the extra/intrahepatic. The IIPSC group had longer transplant-free survival (log-rank P = 0.001) with a significantly lower risk for liver transplantation (12% vs. 34%, P < 0.001). The IIPSC group had a lower risk of death or transplantation than the extra/intrahepatic PSC group [HR: 0.34, 95% CI: 0.17-0.67, P < 0.001]. No bile duct or gallbladder cancers developed in patients with IIPSC, compared to 24 in the extra/intrahepatic group. The clinical characteristics and outcomes of IIPSC were similar to 23 individuals with small-duct PSC. CONCLUSIONS: Patients with IIPSC have a favorable prognosis similar to small-duct PSC. These data are important for counseling patients and designing therapeutic trials for PSC.

Multidisciplinary Diagnosis and Management of a Rare Dysplastic Adenoma Involving a Pancreas Transplant.

A 52-year-old man with a history of type 1 diabetes mellitus with diabetic nephropathy who underwent simultaneous pancreas-kidney transplant over a decade ago presented with small bowel obstruction and was found by enteroscopy to have a carpeted lesion encompassing the small bowel anastomosis in the region of the donor pancreas. As endoscopic mucosal resection was impracticable because of technical limitations, the patient was referred to transplant surgical team for surgical exploration and ultimately required organ resection. This represents a unique presentation of an ampullary adenoma with high-grade dysplasia requiring device-assisted enteroscopy requiring multidisciplinary management.

Use of the endoscopic powered resection device for the management of scarred polyps.

Background and Aims: The endoscopic powered resection (EPR) device (EndoRotor, Interscope Inc, Northbridge, Mass, USA) is a novel nonthermal device that can be used for polyp and tissue removal in the GI tract. Here, we review the EPR device and illustrate its use for resection of scarred or fibrotic lesions in the GI tract. Methods: In this article and accompanying video, we describe the EPR device features, provide step-by-step instructions for device setup, and review case examples in which the EPR device was used for scarred polyp resection. We also review the current literature describing the use of the EPR device for scarred or challenging polyps. Results: Four lesions with scarring or fibrosis were successfully resected with the EPR device, either with the EPR device alone or as an adjunct to conventional resection methods. No adverse events occurred. A follow-up endoscopy was available in 1 case, which demonstrated no endoscopic or histologic evidence of residual or recurrent lesion. Conclusions: The endoscopic powered resection device can be used alone or as an adjunct to facilitate resection of lesions with significant fibrosis or scarring. This device serves as a useful addition to endoscopists' toolbox in the management of scarred lesions where other modalities might be technically challenging to use.

Racial differences in primary sclerosing cholangitis mortality is associated with community socioeconomic status.

BACKGROUND AND AIMS: Natural history and outcomes data in PSC are mostly derived from cohorts where Blacks have been underrepresented. It is unknown if there are differences in mortality between Blacks and Whites with PSC. METHODS: PSC patients seen at our institution from June 1988 to Jan 2019 were identified by merging prospective ERCP hepatology-clinic databases and liver-transplant registry. Data on race, clinical events, and death was obtained through chart review. Data on community health were collected using indices from county health rankings. Cumulative incidence of death was calculated using liver transplant (LT) as a competing risk. RESULTS: Of 449 patients, 404 were White and 45 were Black. The median-duration of follow-up was 7 years (IQR:3, 13). Black patients were younger at presentation than White patients (36.3 vs 42.5 years., P = .013). Disease severity as indicated by Mayo Risk Score categories (low 27% vs 31%, intermediate 54% vs 49% and high 19% vs 19%, P = .690), comorbidity burden and frequency of cirrhosis (42% vs 35%, P = .411) were similar between Blacks and Whites. Cumulative incidence of liver-related death, with LT as a competing risk was significantly higher in Blacks compared to Whites (sHR 1.80, 95%CI 1.25, 2.61, P = .002). There was a significant interaction between race and community socioeconomic factors that attenuated the racial difference in mortality (sHR 1.01, 95%CI 0.99, 1.04, P = .345). CONCLUSIONS: Blacks with PSC present at a younger age with a similar disease severity as Whites but have higher liver related mortality that is mediated in part through community health.

Changes in Serum Myostatin Levels in Alcoholic Hepatitis Correlate with Improvement in MELD.

BACKGROUND: Alcoholic hepatitis (AH) is a serious clinical syndrome often associated with muscle wasting. Myostatin, a member of the transforming growth factor-ß superfamily, has been studied in diseases with muscle wasting; however, the role of myostatin in AH is unknown. AIMS: To investigate the association between myostatin, clinical variables, and outcomes in AH. METHODS: We analyzed data for cases of AH and controls of heavy drinkers (HD) in TREAT001 (NCT02172898) with serum myostatin levels (AH: n = 131, HD: n = 124). We compared characteristics between the two groups at baseline, 30, and 90 days and explored correlations between myostatin and clinical variables. We then modeled the relationship of myostatin to other variables, including mortality. RESULTS: Baseline median myostatin was lower in AH compared to HD (males: 1.58 vs 3.06 ng/ml, p < 0.001; females: 0.84 vs 2.01 ng/ml, p < 0.001). In multivariable linear regression, bilirubin, WBC, and platelet count remained negatively correlated with myostatin in AH. AH females who died at 90 days had significantly lower myostatin, but in a multivariable logistic model with MELD and myostatin, only MELD remained significantly associated with 90-day mortality. During 1-year follow-up, AH cases (n = 30) demonstrated an increase in myostatin (mean, 1.73 ng/ml) which correlated with decreasing MELD scores (ρ = - 0.42, p = 0.01). CONCLUSIONS: Myostatin levels are significantly lower in AH compared to HD and are negatively correlated with total bilirubin, WBC, and platelet count. Myostatin increased as patients experienced decreases in MELD. Overall, myostatin demonstrated a dynamic relationship with AH outcomes and future studies are needed to understand the prognostic role of myostatin in AH.

Enhanced Liver Fibrosis Score Can Be Used to Predict Liver-Related Events in Patients With Nonalcoholic Steatohepatitis and Compensated Cirrhosis.

There is a growing need for a noninvasive tool to identify patients at higher risk of hepatic decompensation among individuals with compensated nonalcoholic steatohepatitis (NASH) cirrhosis.1 Hepatic venous pressure gradient (HVPG) has value in risk stratification2 and prediction of mortality among cirrhotics3 but has limitations of being invasive, costly, and a requirement for expertise.4 The Enhanced Liver Fibrosis (ELF) score is based on circulating markers of hepatic matrix turnover and consists of hyaluronic acid, TIMP-1 (tissue inhibitor of metalloproteinases-1), and PIIINP (propeptide of type III collagen). It identifies nonalcoholic fatty liver disease (NAFLD) patients with advanced fibrosis quite reliably.5,6 However, its utility as a prognostic biomarker among individuals with compensated cirrhosis due to NASH is unclear. This study evaluated the prognostic significance of the ELF score for predicting short-term liver-related outcomes among patients with compensated NASH cirrhosis.

Quantitative assessment of disease severity of primary sclerosing cholangitis with T1 mapping and extracellular volume imaging.

PURPOSE: Assess the relationship between liver T1 relaxation time and extracellular volume (ECV) fraction and the disease severity of primary sclerosing cholangitis (PSC). METHODS: This retrospective study included 93 patients with PSC and 66 healthy patients in the control group. T1 relaxation times were measured in the right and left lobe, as well as in the area of stricture. T1PSC and ECVPSC were calculated by averaging T1 and ECV of both lobes and stricture site. T1 and ECV were compared between the two groups and according to PSC phenotypes and severity based on Mayo Risk Score (MRS). We also examined the relationship between T1 and ECV with non-invasive measures of fibrosis such as Fibrosis-4 index (FIB-4) and liver stiffness measurement (LSM) by transient elastography. RESULTS: Mean liver T1 (774 ± 111 ms, p < 0.001) and liver ECV (0.40 ± 0.14, p < 0.05) were significantly higher with both large-duct and small-duct-type PSC which may lack classic imaging findings on MRCP compared to the control group (p < 0.001). T1PSC and ECVPSC showed weak-moderate correlation with LSM, FIB-4, and MRS (p < 0.05). Cut-off values of liver T1 to detect patients in low-risk and high-risk MRS groups were 677 ms (AUC: 0.68, sensitivity: 76%, specificity: 53%, p = 0.03) and 743 ms (AUC: 0.83, sensitivity: 79%, specificity: 76%, p < 0.001), respectively. CONCLUSION: T1 relaxation time and ECV fraction can be used for quantitative assessment of disease severity in patients with PSC.

Improving Outcomes of Bariatric Surgery in Patients With Cirrhosis in the United States: A Nationwide Assessment.

INTRODUCTION: With increasing burden of obesity and liver disease in the United States, a better understanding of bariatric surgery in context of cirrhosis is needed. We described trends of hospital-based outcomes of bariatric surgery among cirrhotics and determined effect of volume status and type of surgery on these outcomes. METHODS: In this population-based study, admissions for bariatric surgery were extracted from the National Inpatient Sample using International Classification of Diseases, 9th and 10th Revision, Clinical Modification codes from 2004 to 2016 and grouped by cirrhosis status, type of bariatric surgery, and center volume. In-hospital mortality, complications, and their trends were compared between these groups using weighted counts, odds ratios [ORs], and logistic regression. RESULTS: Among 1,679,828 admissions for bariatric surgery, 9,802 (0.58%) had cirrhosis. Cirrhosis admissions were more likely to be in white men, had higher Elixhauser Index, and higher in-hospital complications rates including death (1.81% vs 0.17%), acute kidney injury (4.5% vs 1.2%), bleeding (2.9% vs 1.1%), and operative complications (2% vs 0.6%) (P < 0.001 for all) compared to those without cirrhosis. Overtime, restrictive surgeries have grown in number (12%-71%) and complications rates have trended down in both groups. Cirrhotics undergoing bariatric surgery at low-volume centers (<50 procedures per year) and nonrestrictive surgery had a higher inpatient mortality rate (adjusted OR 4.50, 95% confidence interval 3.14-6.45, adjusted OR 4.00, 95% confidence interval 2.68-5.97, respectively). DISCUSSION: Contemporary data indicate that among admissions for bariatric surgery, there is a shift to restrictive-type surgeries with an improvement in-hospital complications and mortality. However, patients with cirrhosis especially those at low-volume centers have significantly higher risk of worse outcomes (see Visual abstract, Supplementary Digital Content, http://links.lww.com/AJG/B648).

Herpes Simplex Virus Hepatitis: A Brief Review of an Oft-overlooked Pathology.

Herpes simplex virus (HSV) is a rarely reported cause of viral hepatitis. Aggressive in nature, most cases of HSV hepatitis rapidly progress to fulminant hepatic failure. Present day, its pathogenesis is yet to be elucidated, but its complications and associated high mortality rate are clear. Clinically, its symptoms mimic those of other causes of acute hepatic failure thus making the diagnosis of HSV hepatitis a precarious task. Although treatment in the form of acyclovir is readily available, most cases have a poor prognosis due to late initiation of therapy. This makes the early identification of HSV essential in improving outcomes and potentially preventing mortality.

Hepatocellular carcinoma is leading in cancer-related disease burden among hospitalized baby boomers.

INTRODUCTION AND OBJECTIVES: Three fourths of chronic hepatitis C virus (HCV) infected adult patients in the United States (US) are born between 1945 and 1965, also known as baby boomers (BB). Prevalence of hepatocellular carcinoma (HCC) is raising in BB due to their advancing age and prolonged HCV infection. We evaluated inpatient hospitalization and mortality in BB associated with HCC. MATERIALS AND METHODS: It is a retrospective cohort study utilizing the Healthcare Utilization Project-National Inpatient Sample (HCUP-NIS) database. From 2003 to 2012, top five primary cancer related hospitalization and mortality among BB were studied. RESULTS: Among 48,733 hospitalizations related to HCC in HCUP-NIS database from 2003 to 2012, BB accounted for 49.6% (24,210) whereas non-BB 50.4% (24,523). Within BB cohort, the top five cancers with the highest proportion of hospitalizations were HCC (46%), prostate (43%), kidney (41%), pancreas (33%), and bladder (21%). From 2003 to 2012, the proportion of HCC related hospitalizations represented by BB almost doubled (33.5 to 57.8%) whereas there was one-third reduction (66.4 to 42.1%) among non-BB. Similarly, HCC-related inpatient mortality in BB decreased by 28% (6.1 to 2.7 per 100,000 hospitalization) but it remained unchanged in non-BB (11.1 to 10.6). HCC accounted for 2nd highest mortality (4960 total deaths) among hospitalized BB behind pancreatic cancer. HCC related to HCV was disproportionately higher in BB compared to non-BB (50.6% vs. 19%; P<0.001). CONCLUSION: HCC ranks number one among the top five cancers with highest proportion of inpatient burden. Future studies should focus on understanding the underlying reasons for this ominous trend.

Predictors of acute gastrointestinal bleeding in diabetic ketoacidosis: a retrospective observational study in minority population.

BACKGROUND: Diabetic ketoacidosis (DKA) is a common acute complication of diabetes mellitus requiring aggressive medical management. We attempted to study the incidence and various clinical and laboratory variables associated with acute gastrointestinal bleeding (AGIB) and acute upper AGIB (AUGIB) in patients with DKA. METHODS: We conducted a retrospective chart review of all the patients admitted to our hospital with DKA between January 2010 and December 2015. We collected demographic, clinical, laboratory, endoscopy and hospitalization details using an electronic medical-record database. Patients were divided into two groups based on the occurrence of gastrointestinal bleeding. RESULTS: A total of 234 patients with DKA were admitted during this period, of which 27 (11.5%) patients had documented AGIB. The majority of patients had hematemesis (n=22, 9.4%) except two had rectal and three had occult bleeding. We did not notice any difference in age, gender and ethnicity distribution between the two groups. There was no difference in the serum levels of electrolytes, anion gap, pH and hemoglobin A1C between the two groups. However, patients with AGIB had significantly higher initial blood glucose levels (738 vs 613 mg/dL, p =0.014). There was also increased mortality (7.4% vs 4.8%) in patients with AGIB, but this did not reach statistical significance. CONCLUSION: We conclude that higher initial serum blood glucose was associated with increased incidence of AGIB in patients admitted with DKA. We also noted increased in-patient mortality in patients with DKA who had AGIB, even though statistically insignificant. More aggressive measures to correct blood glucose levels may result in decreased incidence of AGIB, thereby reducing mortality during hospitalization in patients with DKA.