Autoimmune cholangitis mimicking a klatskin tumor: a case report

<p>Abstract</p> <p>Introduction</p> <p>Autoimmune cholangitis remains an elusive manifestation of immunoglobulin G4-associated systemic disease most commonly encountered in patients with autoimmune pancreatitis. No strict diagnostic criteria have been described to date and diagnosis mainly relies on a combination of clinical and histopathologic findings. It is hence even more challenging to diagnose autoimmune cholangitis in patients with late or atypical presentations, such as without concomitant pancreatic involvement. Early diagnosis of this rare disorder can significantly improve outcomes considering high rates of surgical intervention, as well as high relapse rates in the absence of steroid treatment. To the best of our knowledge the literature is quite sparse on cases with atypical presentations of autoimmune cholangitis.</p> <p>Case presentation</p> <p>We report a case of a previously healthy 65-year-old man of Middle-Eastern origin, with a history of pancreatic insufficiency of unknown etiology, evaluated for elevated liver function tests found incidentally on a routine physical examination. Imaging studies revealed an atrophic pancreas and biliary duct dilatation consistent with obstruction. Subsequent endoscopic retrograde cholangiopancreatography showed a bile duct narrowing pattern suggestive of cholangiocarcinoma, but brushings failed to reveal malignant cells. Our patient proceeded to undergo surgical resection. Histological examination of the resected mass revealed lymphoplasmacytic infiltrate with no malignant features. Our patient returned three months later with persistently high liver function tests and no evidence of biliary obstruction on imaging. A presumptive diagnosis of autoimmune cholangitis was made and our patient's symptoms resolved after a short course of an oral steroid regimen. Post factum staining of the resection specimen revealed an immunoglobulin G4 antibody positive immune cell infiltrate, consistent with the proposed diagnosis.</p> <p>Conclusion</p> <p>Our case thus highlights the importance of clinician awareness of the autoimmune spectrum of biliary pathologies when confronted with atypical clinical presentations, the paucity of diagnostic measures and the benefit from long-term steroid and/or immunosuppressive treatment.</p

Liver Transplantation for Hepatic Adenoma: A UNOS Database Analysis and Systematic Review of the Literature

Background. Liver transplantation (LT) has been employed for hepatic adenoma (HA) on a case-oriented basis. We aimed to describe the characteristics, waitlist, and post-LT outcomes of patients requiring LT for HA. Methods. All patients listed or transplanted for HA in the United States were identified in the United Network for Organ Sharing (UNOS) database (1987-2020). A systematic literature review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-analysis statement. Results. A total of 199 HA patients were listed for LT in UNOS and the crude waitlist mortality was 9.0%. A total of 142 HA patients underwent LT; 118 of these were among those listed with an indication of HA who underwent LT, and 24 were diagnosed incidentally. Most did not experience hepatocellular carcinoma transformation (89.4%). Over a median follow-up of 62.9 mo, death was reported in 18.3%. The 1-, 3-, and 5-y patient survival rates were 94.2%, 89.7%, and 86.3% in the UNOS cohort. The systematic review yielded 61 articles reporting on 99 nonoverlapping patients undergoing LT for HA and 2 articles reporting on multicenter studies. The most common LT indications were suspected malignancy (39.7%), unresectable HA (31.7%), and increasing size (27.0%), whereas 53.1% had glycogen storage disease. Over a median follow-up of 36.5 mo, death was reported in 6.0% (n=5/84). The 1-, 3-, and 5-y patient survival rates were all 95.0% in the systematic review. Conclusions. LT for HA can lead to excellent long-term outcomes in well-selected patients. Prospective granular data are needed to develop more optimal selection criteria and further improve outcomes. © 2022 The Author(s)

In a ‘Real-World’, Clinic-Based Community Setting, Sorafenib Dose of 400 mg/day is as Effective as Standard Dose of 800 mg/day in Patients with Advanced Hepatocellular Carcimona, with Better Tolerance and Similar Survival

BACKGROUND: Sorafenib, an oral multityrosine kinase inhibitor, has been approved for treatment of unresectable hepatocellular carcinoma (HCC). British Columbia (BC) was the first province in Canada to provide drug coverage for sorafenib

Supplementary Material for: Investigation of novel urinary biomarkers in hepatocellular carcinoma risk in a predominantly African American population.

Introduction African Americans are at increased risk of hepatocellular carcinoma (HCC) compared to other racial and ethnic groups. We investigated the associations of four urinary biomarkers of prostaglandin E2(PGE-M), prostacyclin (PGI-M), and thromboxane (11dTxB2) synthesis and the ratio of PGI-M to 11dTXB2 with HCC risk in a cohort of predominantly African American population. Methods We conducted a nested case-control study (50 cases; 43 with HCC, 151 controls) in the Southern Community Cohort Study (SCCS), a large prospective cohort study including over 80,000 study participants of whom two-thirds are African Americans. Urine samples were collected at enrollment and subsequently analyzed to assess biomarker levels. Multivariable regression models adjusted for age, race, sex, BMI, smoking status, NSAID use, education level, income and alcohol consumption were used to assess the relationship between the biomarker and HCC risk. Results Only 11dTxB2 (OR=11.50; 95%CI [2.34-56.47] for highest tertile vs lowest tertile, p=0.004) and the PGI-M/11dTXB2 ratio of the second quartile (0.25-0.49) (OR=5.16; 95%CI[1.44-18.47];p=0.01) were significantly associated with increased risk of liver cancer. Conclusion 11dTXB2 and PGI-M/11dTXB2 ratio may be urinary markers of HCC risk, particularly among African Americans and future prospective studies are needed to evaluate this finding further and to develop accessible methods

Structured patient handoff on an internal medicine ward: A cluster randomized control trial

<div><p>Background</p><p>The effect of a multi-faceted handoff strategy in a high volume internal medicine inpatient setting on process and patient outcomes has not been clearly established. We set out to determine if a multi-faceted handoff intervention consisting of education, standardized handoff procedures, including fixed time and location for face-to-face handoff would result in improved rates of handoff compared with usual practice. We also evaluated resident satisfaction, health resource utilization and clinical outcomes.</p><p>Methods</p><p>This was a cluster randomized controlled trial in a large academic tertiary care center with 18 inpatient internal medicine ward teams from January-April 2013. We randomized nine inpatient teams to an intervention where they received an education session standardizing who and how to handoff patients, with practice and feedback from facilitators. The control group of 9 teams continued usual non-standardized handoffs. The primary process outcome was the rate of patients handed over per 1000 patient nights. Other process outcomes included perceptions of inadequate handoff by overnight physicians, resource utilization overnight and hospital length of stay. Clinical outcomes included medical errors, frequency of patients requiring higher level of care overnight, and in-hospital mortality.</p><p>Results</p><p>The intervention group demonstrated a significant increase in the rate of patients handed over to the overnight physician (62.90/1000 person-nights vs. 46.86/1000 person-nights, p = 0.002). There was no significant difference in other process outcomes except resource utilization was increased in the intervention group (26.35/1000 person-days vs. 17.57/1000 person-days, p-value = 0.01). There was no significant difference between groups in medical errors (4.8% vs. 4.1%), need for higher level of care or in hospital mortality. Limitations include a dependence of accurate record keeping by the overnight physician, the possibility of cross-contamination in the handoff process, analysis at the cluster level and an overall low number of clinical events.</p><p>Conclusions</p><p>Implementation of a multi-faceted resident handoff intervention did not result in a significant improvement in patient safety although did improve number of patients handed off. Novel methods to improve handoff need to be explored.</p><p>Trial registration</p><p>Registered at ClinicalTrials.gov: <a href="https://clinicaltrials.gov/ct2/show/NCT01796756" target="_blank">NCT01796756</a>.</p></div

Process, healthcare resource utilization and patient clinical outcomes.

<p>Process, healthcare resource utilization and patient clinical outcomes.</p

Baseline characteristics of intervention and control groups.

<p>Baseline characteristics of intervention and control groups.</p

Handoff satisfaction of clinical associates and trainees, comparison between intervention and control.

<p>Handoff satisfaction of clinical associates and trainees, comparison between intervention and control.</p