bluuebunny/crossref_metadata_2025_split huggingface dataset:
996 Disease Characteristics of Acute Hepatic Porphyria Patients: ENVISION, a Phase 3 Global, Multicenter, Randomized, Double-Blind, Placebo-Controlled Trial
Authors: Anderson Karl , Balwani Manisha , Sardh Eliane , Gouya Laurent , Rees David , Stein Penelope , Stolzel Ulrich , Aguilera Paula , Bissell D. Montgomery , Bonkovsky Herbert , Keel Sioban , Parker Charles , Phillips John , Silver Samuel , Windyga Jerzy , D'Avola Delia , Ross Gayle , Stewart Peter , Ritchie Bruce , Oh Jeeyoung , Harper Pauline , Wang Jiaan-Der , Langendonk Janneke , Ivanova Aneta , Horie Yutaka , Minder Elisabeth , Vassiliou Daphne , Kubisch Ilja , Guillen-Navarro Encarna , Coman David , Wang Bruce , Kuo Hung-Chou , Scalera Sushama , Penz Craig , Hua Zoe , Simon Amy , Ko John J. , Ventura Paolo 
Published: October 2019

INTRODUCTION:
Acute Hepatic Porphyria (AHP) is a family of rare genetic diseases due to enzyme deficiencies in heme biosynthesis in the liver that can lead to neurovisceral attacks and chronic symptoms. (1, 2) AHP is also associated with multiple long-term complications, including neuropathy (3,4), hypertension (5,6), chronic kidney disease (7), and hepatocellular carcinoma (8,9).

METHODS:
ENVISION (NCT03338816) was a Phase 3 global, multicenter, randomized, double-blind, placebo-controlled trial with an open label extension to evaluate the efficacy and safety of subcutaneous givosiran in AHP. Eligible patients had an AHP diagnosis and ≥2 attacks within the prior 6 months. At baseline detailed medical history and laboratory assessments were obtained.

RESULTS:
Ninety-four AHP patients enrolled, median (range) age 37.5 (19-65) years, 89% female, and median (range) of 6.5 (0.1–43) years since diagnosis. Patients had a median of 4 composite attacks in the preceding 6 months and 40% were on hemin prophylaxis prior to study. Half the patients experienced daily chronic symptoms and 29% used opioids daily/most days between attacks. Comorbidities reported on medical history included neuropathy (38%), central venous access complications (71%), iron overload (33%), liver disease (28%), and chronic kidney disease (18%). At baseline 17% of patients had liver transaminases > ULN and 34% had an eGFR < 60 mL/min/1.73m2.

CONCLUSION:
The baseline disease characteristics of the Phase 3 study population are consistent with findings reported in the AHP literature, underscoring the high rate of liver disease and central venous access complications in these patients, and highlighting the multi-systemic nature of AHP and the medical challenge it presents for diagnosis and treatment.
1000 Older Adults With Hepatocellular Carcinoma Are More Likely Than Younger Adults to Receive Curative Treatment but Have Worse Overall Survival
Authors: Rich Nicole E. , Murphy Caitlin C. , Yopp Adam C. , Marrero Jorge A. , Singal Amit G. 
Published: October 2019

INTRODUCTION:
Hepatocellular carcinoma (HCC) incidence rates are increasing among older adults in the U.S. Age-related disparities in prognosis and treatment receipt have been described in other cancers; however, there are limited data examining outcomes in older adults with HCC. Our study's aim was to evaluate age-related differences in HCC treatment receipt and prognosis.

METHODS:
We performed a retrospective study of patients diagnosed with HCC between 2008 and 2017 at 2 U.S. hospital systems. Older and younger adults were defined as ≥65 and <65 years old, respectively. Curative therapy was defined as liver transplantation, surgical resection, or local ablation. We used multivariable logistic regression to identify factors associated with curative treatment receipt and Cox proportional hazard models to identify factors associated with overall survival (OS).

RESULTS:
Of 1110 patients with HCC, 261 (23.5%) were ≥65 and 849 (76.5%) were <65 years old; the cohort was racially/ethnically diverse (33.3% white, 32.4% black, 27.5% Hispanic). A higher proportion of older adults had non-viral cirrhosis compared to younger adults (46.1% vs 21.8%, P < 0.001). A similar proportion of HCC were detected by surveillance in both groups (41.0% vs 40.0%, P = 0.80), but a higher proportion of older adults presented with early stage HCC (BCLC 0/A; 44.8% vs 40.6%, P < 0.001) compared to younger adults; further, a higher proportion of older adults had Child A cirrhosis (61.1% vs 43.4%, P < 0.001) compared to younger adults. Among treated patients (n = 750), older adults were more likely to receive curative therapy (OR 1.71, 95% CI 1.06–2.77) even after adjusting for sex, race, Child Pugh score, BCLC stage and ECOG performance status; there was no significant difference in complete response rates (31.0% vs 28.9%, P = 0.58) between older and younger adults. Among those who received curative therapy, median OS was significantly lower among older compared to younger adults (37.9 vs 57.5 months, P = 0.04) while survival was similar among older and younger adults receiving palliative HCC therapy (12.6 vs 12.2 months, P = 0.46) and best supportive care (1.6 vs 2.4 months, P = 0.05). Older adults had higher mortality than younger adults (HR 1.28, 95%CI 1.05–1.56) after adjusting for sex, race, Child Pugh score, BCLC stage and ECOG performance status.

CONCLUSION:
Despite being more likely than younger adults to receive curative treatment, older adults with HCC had worse overall survival, independent of BCLC stage or cirrhosis severity.
1001 Significant Differences in Liver Transplant (LT) Waitlist Mortality, Receiving LT, and Post-LT Survival Among Adults With Primary Sclerosing Cholangitis, Primary Biliary Cholangitis, and Autoimmune Hepatitis in the U.S.
Authors: Bayable Asnakech , Liu Benny , Bhuket Taft , Wong Robert 
Published: October 2019

INTRODUCTION:
Given few studies specifically focusing on waitlist (WL) and liver transplant (LT) outcomes among adults with autoimmune liver diseases, there is a need to better understand epidemiology and outcomes among patients with primary sclerosing cholangitis (PSC), primary biliary cholangitis (PBC), and autoimmune hepatitis (AIH). We aim to evaluate disparities in LT outcomes among adults with PSC, PBC, and AIH using data from a comprehensive U.S. LT registry.

METHODS:
We retrospectively evaluated U.S. adults (age ≥18 y) listed for LT using the 2004-2017 United Network for Organ Sharing database, focusing on patients with PSC, PBC, and AIH. Overall WL survival, probability of receiving LT, and post-LT survival were evaluated using Kaplan Meier methods and adjusted multivariate Cox proportional hazards models.

RESULTS:
Among 11,742 patients listed for LT (34.7 % PSC, 32.9 % PBC 32.2 % AIH), PSC patients had higher proportion of male patients (69.3% vs. 14.3% in PBC and 26.9% in AIH, P < 0.001), and PBC patients were older at time of WL registration (mean age: 46.7 PSC vs. 56.5 PBC vs. 48.1 AIH, P < 0.001). While majority of patients were non-Hispanic white, AIH patients had higher proportion of African Americans, and PBC and AIH had higher proportion of Hispanics compared to PSC. Compared to PSC, those with PBC had lower risk of WL death (HR 0.76, 95% CI 0.63-0.92, P < 0.01). Compared to non-Hispanic whites, Asians had significantly higher risk of WL death (HR 1.61 95% CI 1.05-2.45, P < 0.03). When evaluating probability of receiving LT, AIH patients had lower likelihood of receiving LT compared to PSC (HR 0.82, 95% CI 0.76-0.89, P < 0.001). Hispanics had lower likelihood of LT compared to non-Hispanic whites (HR 0.77, 95% CI 0.70-0.85, P < 0.001). Among LT recipients, AIH patients had significantly higher risk of death post-LT compared to PSC patients (HR 1.29 95% CI 1.09-1.51, P < 0.01). African Americans had significantly higher risk of death post-LT compared to non-Hispanic whites.

CONCLUSION:
Compared to U.S. adults with PSC listed for LT, PBC patients had significantly lower risk of WL death and AIH patients had significantly lower likelihood of receiving LT. Among patients that underwent LT, AIH patients had significantly higher risk of post-LT death compared to PSC. While the majority of patients were non-Hispanic white, comparatively, Asians had significantly higher risk of WL death and African Americans had significantly higher risk of post-LT death.
1002 Clostridioides difficile Infection in Liver Transplant Recipients
Authors: Brindise Elizabeth , Kobeszko Matthew , Kuruvilla Aneesh , Elkhatib Imad , Silva Rogelio 
Published: October 2019

INTRODUCTION:
Clostridium Difficile Infection (CDI) has been increasing in prevalence within the United States. The CDC estimates that approximately half a million people are infected every year. Patients with liver transplantation tend to require more hospitalizations with increased frequency of antibiotics and higher risk for CDI. We sought to evaluate outcomes of patients with liver transplantation developing CDI.

METHODS:
The study population was derived from the HCUP-National Inpatient Sampling (NIS) database for the years 2010 to the third quarter of 2015. ICD-9 codes were used to identify patients with history of liver transplant and CDI. In-hospital all-cause mortality, total cost of hospitalization, mean length of stay and outcomes rates were assessed. Propensity score matching was used to adjust for baseline confounders.

RESULTS:
A total of 8,567 individuals had a history of liver transplantation and co-existing CDI. Age and race were similar between the two groups; 43.7% of the CDI cohort were female. Patient’s in the control group had higher baseline clinical risk (Table 1). All-cause mortality (3.2% vs 1.7%, P < 0.0001), length of stay (10.4 ± 15.4 vs 5.5 ± 7.4, P < 0.0001), cost of hospitalization ($88,866 ± 166,834 vs $50,724 ± 94,482, P < 0.0001) and blood transfusion rates (13.6% vs 19%, P < 0.001) were higher in the CDI and liver transplant population. Colonoscopy and bowel resection were also performed significantly more frequently in the CDI and liver transplantation cohort (7.1% vs 2.9%, P < 0.0001 and 0.8% vs 1.1%, P = 0.012, respectively) (Table 2). After propensity score matching analysis, these statistically significant differences remained; bowel resection rates were similar between the two groups (1.3% vs 1.1%, P = 0.324). (Table 3).

CONCLUSION:
CDI in liver transplant recipients is associated with significantly higher rates of in-hospital mortality, blood transfusions, length of stay and total cost of hospitalization. Colonoscopy is used more frequently in the management of this population.
1006 Non-Alcoholic Fatty Liver Disease Is an Increasing Reason for Referral to Gastroenterology
Authors: Aggarwal Pankaj , Thomas Aaron , Andrews Christine , Kundrotas Leon , Govani Shail 
Published: October 2019

INTRODUCTION:
Non-Alcoholic Fatty Liver Disease (NAFLD) is increasing in prevalence. In integrated healthcare systems with limited clinic access, prior studies have utilized care pathways to triage patients to clinic in situations of suspected advanced fibrosis. Our aim was to study clinic utilization for NAFLD over time and assess how many patients could avoid specialty care using predictive scores such as the FIB4 and NAFLD fibrosis score (NFS).

METHODS:
Consecutive consultations to GI, 200 from 2012 and 300 from 2017, at a large Veterans Administration Hospital in Texas were retrospectively reviewed to determine reason for consultation, final diagnosis, additional workup, and follow up utilization. Weight 1 year after consultation was compared to the initial weight. Chi square testing and Student’s t test were used to compare categorical and quantitative data, respectively, using R.

RESULTS:
Among the 500 patients, the mean age was 57.4 years (+/- 13.9), 86.2% were male and 18.4% Hispanic. Of the 200 consults in 2012, 18 (9.0%) were for NAFLD compared to 53 (17.7%) of 300 consults from 2017 (P < 0.01). Comparatively, the number of consultations for Hepatitis C was not significantly different from 2012 to 2017 (19.5% vs. 16.7%, P = 0.42). Neither liver biopsy (27.8% vs. 17.0%, P = 0.32) nor MR elastography (11.1% vs. 28.9%, P = 0.20) use was significantly different between 2012 and 2017. Using the FIB4 score, 31 (43.7%) were classified as low risk and 11 (15.5%) were classified as high risk for advanced fibrosis. Using the NFS, 18 (25.4%) were classified as both low and high risk. 27 patients underwent MR elastography or liver biopsy and 13 (48%) were found to have advanced fibrosis. The performance characteristics of NFS and FIB4 compared to results of liver biopsy or MR elastography is presented in Table 1 and 2, respectively. Mean weight change 1 year later was -0.8% (+/-4.7%). There was no difference in weight change between those found to have advanced fibrosis or not (-0.3% [95%CI: -3.0- 2.3%] vs. -2.0% [95%CI:-4.1- 0.1], P = 0.29).

CONCLUSION::
One of every 6 consultations to gastroenterology is for NAFLD, now exceeding Hepatitis C referrals in 2017. Using the NFS could save 25% of these referrals without missing cases of advanced fibrosis. GI care was not associated with weight loss. Prospective studies are needed to determine the efficacy of these strategies to triage NAFLD patients for specialty care.
1014 The Majority of Patients With Primary Biliary Cholangitits Do Not Achieve the Ideal Treatment Response in a Real World Clinical Practice
Authors: Cummings-John Oladuni D. , Geistweidt Stephenie , Ritchie Michael , Angueira Candice , Okubote Toluwalase C. , Ascher Bartlett Johanna M. , Filo Jean , Barbara Mary , Lawitz Eric , Hanouneh Ibrahim , Alkhouri Naim 
Published: October 2019

INTRODUCTION:
The currently accepted criteria to define therapeutic response to primary biliary cholangitis (PBC) consists of alkaline phosphatase (ALP) (<180 U/L) and bilirubin (<1.0 mg/dL). New data suggests that normalization of ALP (<120 U/L) and bilirubin (<0.5 mg/dL) is associated with better overall outcomes. PBC patients are usually maintained on first line therapy of ursodeoxycholic acid (UDCA), obeticholic acid (OCA) or combination of both agents. The aim of the study was to determine the percentage of PBC patients that achieve the accepted (ALP < 180, bilirubin < 1) and ideal (ALP < 120, bilirubin < 0.5) treatment responses in a real-world setting.

METHODS:
All adult patients in this multi-center study had a diagnosis of PBC via ICD-9 and ICD-10 codes. Other chronic liver disease cases and those with significant alcohol consumption were excluded. Baseline demographics, clinical characteristics and laboratory data were collected. Therapy starting time was noted, assessing their lab values at baseline compared to a recent workup. A two-sided t test was done to compare continuous variables and a P value < 0.05 was considered statistically significant.

RESULTS:
76 adult PBC patients were included in the final analysis. The mean age at the last follow up was 61.8 years (±11.3). 96% were female, 47% were White and 51% were of Hispanic ethnicity. 93% were on UDCA therapy alone with daily dosages between 750-1500 mg. Only 7% received both UDCA and OCA. The mean baseline ALP was 286 U/L and the mean follow up ALP was 172 U/L (±84.6), 52 U/L above the ideal value (P < 0.05). The mean baseline bilirubin was 1.04 mg/dL and the mean follow up bilirubin was 0.8 mg/dL (±1.2), 0.3 mg/dL above the ideal value (P < 0.05). 62% reached the accepted ALP value, 86% reached the accepted bilirubin value, and 57% reached the accepted level. 30% of patients achieved the ideal ALP value and 26% achieved the ideal bilirubin level with 20% reaching the ideal response. The rates for accepted and ideal responses were 80% and 40%, respectively, for those on UDCA and OCA.

CONCLUSION:
The majority of PBC patients in real-life settings did not achieve the ideal treatment response of ALP < 120 U/L and bilirubin < 0.5 mg/dL. Furthermore, a significant percentage did not achieve the accepted treatment response of ALP < 180 U/L and bilirubin < 1.0 mg/dL. Only a small percentage received combination therapy with UDCA/OCA. There's a need to increase awareness among healthcare providers on the optimal responses to PBC treatment.
1024 A Retrospective Analysis of Hepatitis-B Reactivation in Hepatitis-C Patients Treated With Direct-Acting Antivirals Therapy
Authors: Tahir Muhammad , Gopalakrishnan Ravikumar Naveen Prasad , Chathura Wijewardena , Martinez Anthony 
Published: October 2019

INTRODUCTION:
Hepatitis B virus/hepatitis C virus (HBV/HCV) co-infection has a prevalence of 5%-10% in patients with chronic HCV patients. Direct-acting antiviral agent (DAA) have replaced Interferon (IFN)-based treatment for HCV infection given the increased HCV sustained virological response (SVR). However, the Food and Drug Administration (FDA) has issued warnings about HBV reactivation (HBV-R) in chronic HCV patients treated with DAA. We aimed to assess the HBV reactivation rate in our chronic HCV patient population treated with DAA (Ledipasvir / Sofosbuvir).

METHODS:
We conducted a retrospective study involving HCV patients treated with DAA at our outpatient hepatology clinic located in Erie county medical center. The goal as discussed above was to find the HBV reactivation which was defined as reverse seroconversion of HBsAg-negative patients at baseline becoming HBsAg-positive post DAA therapy with a DNA level >0 IU/mL. Patient characteristics and laboratory results at baseline and at the end of follow-up were retrospectively analyzed from electronic medical records. A chart review was completed for a total of 260 patients fulfilling the inclusion criteria. The mean age of the study population was 56.5 years. Inclusion criteria: 1-DAA therapy for HCV with confirmed SVR. 2-HBsAg-negative/HbcAb+. 3-HBV/HCV coinfection. Exclusion criteria: 1- Coinfection of HAV/HDV/HEV. 2- Decompensated cirrhosis with CPT class B/C. 3- Active HBV coinfection. 4- Undergoing immunosuppressive therapy or chemotherapy.

RESULTS:
Post-treatment serum samples of 260 patients were analyzed. Of these, 19.60% (51/260) of the total population was positive for HbcAb. Out of these 51 patients, 13 post-DAA treatment patients were checked for hepatitis-B DNA level to rule out the concern for reactivation. None of the patients showed any elevation in Hepatitis-B virus DNA level, i.e. undetectable. Also, no seroconversion to HbsAg+ was noticed in our population post-DAA therapy. Further detailed results will be presented in the poster during meeting.

CONCLUSION:
To conclude, we found no hepatitis-B virus reactivation or seroconversion to HBsAg positive state in our cohort. Our study supports the latest literature that although it has been observed in the past, HBV reactivation in exposed patients with HbcAb without positive HbsAg is very rare.
1034 Efficacy and Safety of Saroglitazar in Nonalcoholic Fatty Liver Disease Patients at 1 Year: An Investigator Initiated Study
Authors: Dadhich Sunil K. 
Published: October 2019

INTRODUCTION:
Currently there is no drug or therapy approved for NAFLD. Although there are many drugs which have been explored for their usage in NAFLD/NASH. Saroglitazar is one of such novel dual peroxisome proliferator-activated receptors-α/γ agonist drug approved for diabetic dyslipidemia in India and is being investigated for treatment of NAFLD. In this current observational study we have tried to explore the efficacy of Saroglitazar in NAFLD patients with dyslipidemia.

METHODS:
This is a single centre, single arm, open label study of Saroglitazar conducted in northern India. Obese/overweight patients having Fatty Liver (on USG), elevated transaminases and dyslipidemia with or without type 2 diabetes were enrolled. All the patients were given Saroglitazar 4 mg once daily for at least 1 year. Patients with concomitant liver disease due to other etiologies (alcohol, viral etc.) were excluded. The laboratory parameters and LSM reports at baseline and 1 year were compared using t test.

RESULTS:
A total of 64 patients with mean age of 41.95 ± 8.56 years were enrolled in the study. The ratio of male to female was 84.38 (N:54): 15.62 (N:10). Out of 64 patients 17 Diabetic, 30 Non- diabetic and 17 were Prediabetic. The baseline and follow up of aspartate aminotransferase (AST), alanine aminotransferase (ALT), Total Cholesterol, triglycerides (TG) and liver stiffness measurement values were given in Table 1. There was a consistent and significant reduction in Total Cholesterol, TG, ALT, AST and LSM. There were no reported adverse events pertaining to Hypoglycemia.

CONCLUSION:
Saroglitazar was found to be safe and effective in management of NAFLD patients in this interim analysis of 12 months follow up. It is a potential option and need further exploration for management of dyslipidemia and NAFLD in non diabetic patients.
1040 Quality Improvement Initiative to Improve Receipt of Timely Surveillance Endoscopy After Variceal Bleeding
Authors: Moon Andrew M. , Kim Hannah P. , Barritt A. Sidney , Darling Jama , Arora Shifali 
Published: October 2019

INTRODUCTION:
Among patients with cirrhosis who develop esophageal variceal bleeding, surveillance endoscopy (EGD) with variceal ligation within 4 weeks of index bleed decreases the risk of re-bleeding. However, many of these patients do not receive timely surveillance EGD. Therefore, we aimed to assess the rates of timely surveillance EGD after an episode of variceal hemorrhage (VH) before and after a quality improvement (QI) initiative to improve provision of surveillance EGD.

METHODS:
We identified patients with cirrhosis hospitalized at the University of North Carolina Medical Center from 7/1/2017-6/30/2018 with variceal bleeding. We excluded all patients with a transjugular intrahepatic portosystemic shunt placement or death during hospitalization, discharge to hospice, or patient preference for local follow-up. Following the initial EGD, we determined if surveillance EGD was ordered and scheduled, and if scheduled, whether or not patients underwent timely surveillance EGD, defined as <4 weeks from the initial EGD. Based on these results, we designed a QI intervention to reserve two open scheduling slots dedicated to surveillance EGD after VH. Post-intervention surveillance EGD rates were then calculated for hospitalized patients with cirrhosis who underwent EGD for VH from 12/1/2018-4/15/2019.

RESULTS:
We identified 33 patients with cirrhosis hospitalized from 7/2017-6/2018 with VH. Of these patients, 5 (15%) patients underwent surveillance EGD within 4 weeks from initial EGD. Of the 28 patients who did not undergo timely surveillance, the most common reason was the EGD being scheduled for a date >4 weeks after the index bleed (75%). Other reasons included inability to contact the patient (14%), cancellations/no-shows (7%), and failure to place an EGD order (4%). In the post-intervention period, 18 patients had a VH and met other inclusion criteria. Of these patients, 8 (44%) underwent timely surveillance EGD. The reasons for failure to undergo timely surveillance EGD were cancellations (50%), EGD scheduled >4 weeks from initial bleed (40%), and failure to place an EGD order (10%).

CONCLUSION:
A QI intervention to reserve post-VH scheduling slots was associated with a 29% absolute increase in timely surveillance EGDs. Steps to further increase procedure capacity and improve both patient and scheduler education on the importance of surveillance EGDs within 4 weeks of VH may further increase rates of timely surveillance EGDs.