Browsing by Subject "Liver transplant"

Now showing 1 - 5 of 5
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    Hospice Care for End Stage Liver Disease in the United States
    (Taylor & Francis, 2021) Orman, Eric S.; Johnson, Amy W.; Ghabril, Marwan; Sachs, Greg A.; Medicine, School of Medicine
    Introduction: Patients with end-stage liver disease (ESLD) have impaired physical, psychological, and social functions, which can diminish patient quality of life, burden family caregivers, and increase healthcare utilization. For those with a life expectancy of less than six months, these impairments and their downstream effects can be addressed effectively through high-quality hospice care, delivered by multidisciplinary teams and focused on the physical, emotional, social, and spiritual wellbeing of patients and caregivers, with a goal of improving quality of life. Areas Covered: In this review, we examine the evidence supporting hospice for ESLD, we compare this evidence to that supporting hospice more broadly, and we identify potential criteria that may be useful in determining hospice appropriateness. Expert Opinion: Despite the potential for hospice to improve care for those at the end of life, it is underutilized for patients with ESLD. Increasing the appropriate utilization of hospice for ESLD requires a better understanding of patient eligibility, which can be based on predictors of high short-term mortality and liver transplant ineligibility. Such hospice criteria should be data-driven and should accommodate the uncertainty faced by patients and physicians.
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    Incidence, risk factors and outcomes of de novo malignancies post liver transplantation
    (Baishideng Publishing Group Co (World Journal of Hepatology), 2016-04-28) Mukthinuthalapati, Pavan Kedar; Gotur, Raghavender; Ghabril, Marwan; Department of Medicine, IU School of Medicine
    Liver transplantation (LT) is associated with a 2 to 7 fold higher, age and gender adjusted, risk of de novo malignancy. The overall incidence of de novo malignancy post LT ranges from 2.2% to 26%, and 5 and 10 years incidence rates are estimated at 10% to 14.6% and 20% to 32%, respectively. The main risk factors for de novo malignancy include immunosuppression with impaired immunosurveillance, and a number of patient factors which include; age, latent oncogenic viral infections, tobacco and alcohol use history, and underlying liver disease. The most common cancers after LT are non-melanoma skin cancers, accounting for approximately 37% of de novo malignancies, with a noted increase in the ratio of squamous to basal cell cancers. While these types of skin cancer do not impact patient survival, post-transplant lymphoproliferative disorders and solid organ cancer, accounting for 25% and 48% of malignancies, are associated with increased mortality. Patients developing these types of cancer are diagnosed at more advanced stages, and their cancers behave more aggressively compared with the general population. Patients undergoing LT for primary sclerosing cholangitis (particularly with inflammatory bowel disease) and alcoholic liver disease have high rates of malignancies compared with patients undergoing LT for other indications. These populations are at particular risk for gastrointestinal and aerodigestive cancers respectively. Counseling smoking cessation, skin protection from sun exposure and routine clinical follow-up are the current approach in practice. There are no standardized surveillance protocol, but available data suggests that regimented surveillance strategies are needed and capable of yielding cancer diagnosis at earlier stages with better resulting survival. Evidence-based strategies are needed to guide optimal surveillance and safe minimization of immunosuppression.
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    Mutation Analysis and Disease Features at Presentation in a Multi-Center Cohort of Children With Monogenic Cholestasis
    (Wiley, 2021) Hertel, Paula M.; Bull, Laura N.; Thompson, Richard J.; Goodrich, Nathan P.; Ye, Wen; Magee, John C.; Squires, Robert H.; Bass, Lee M.; Heubi, James E.; Kim, Grace E.; Ranganathan, Sarangarajan; Schwarz, Kathleen B.; Bozic, Molly A.; Horslen, Simon P.; Clifton, Matthew S.; Turmelle, Yumirle P.; Suchy, Frederick J.; Superina, Riccardo A.; Wang, Kasper S.; Loomes, Kathleen M.; Kamath, Binita M.; Sokol, Ronald J.; Shneider, Benjamin L.; Childhood Liver Disease Research Network (ChiLDReN); Pediatrics, School of Medicine
    Objectives: To advance our understanding of monogenic forms of intrahepatic cholestasis. Methods: Analyses included participants with pathogenic biallelic mutations in adenosine triphosphate (ATP)-binding cassette subfamily B member 11 (ABCB11) (bile salt export pump; BSEP) or adenosine triphosphatase (ATPase) phospholipid transporting 8B1 (ATP8B1) (familial intrahepatic cholestasis; FIC1), or those with monoallelic or biallelic mutations in adenosine triphosphate (ATP)-binding cassette subfamily B member 4 (ABCB4) (multidrug resistance; MDR3), prospectively enrolled in the Longitudinal Study of Genetic Causes of Intrahepatic Cholestasis (LOGIC; NCT00571272) between November 2007 and December 2013. Summary statistics were calculated to describe baseline demographics, history, anthropometrics, laboratory values, and mutation data. Results: Ninety-eight participants with FIC1 (n = 26), BSEP (n = 53, including 8 with biallelic truncating mutations [severe] and 10 with p.E297G or p.D482G [mild]), or MDR3 (n = 19, including four monoallelic) deficiency were analyzed. Thirty-five had a surgical interruption of the enterohepatic circulation (sEHC), including 10 who underwent liver transplant (LT) after sEHC. Onset of symptoms occurred by age 2 years in most with FIC1 and BSEP deficiency, but was later and more variable for MDR3. Pruritus was nearly universal in FIC1 and BSEP deficiency. In participants with native liver, failure to thrive was common in FIC1 deficiency, high ALT was common in BSEP deficiency, and thrombocytopenia was common in MDR3 deficiency. sEHC was successful after more than 1 year in 7 of 19 participants with FIC1 and BSEP deficiency. History of LT was most common in BSEP deficiency. Of 102 mutations identified, 43 were not previously reported. Conclusions: In this cohort, BSEP deficiency appears to be correlated with a more severe disease course. Genotype-phenotype correlations in these diseases are not straightforward and will require the study of larger cohorts.
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    Primary Sclerosing Cholangitis-Autoimmune Hepatitis Overlap Syndrome: Significant Barriers in Liver Disease Diagnosis and Treatment Experienced by the Latino Community
    (Springer, 2023-03-14) Guifarro, Daniel A.; De Oliveira-Gomes, Diana; Beas, Renato; Yibirin-Wakim, Marcel J.; Montalvan-Sanchez, Eleazar E.; Medicine, School of Medicine
    Overlap syndrome (OS) is a term that comprises the presentation of multiple hepatic disease characteristics in the same patient, such as the presence of autoimmune hepatitis (AIH) features in addition to primary sclerosing cholangitis (PSC) or primary biliary cholangitis (PBC). Standard therapy for AIH is immunosuppression, while ursodeoxycholic acid is the preferred treatment for PBC. Additionally, liver transplantation (LT) may be considered in severe cases. Hispanics have been found to have a higher prevalence of chronic liver disease and develop more complications associated with portal hypertension at the time of listing for LT. Despite being the fastest-growing population in the USA, Hispanics have a higher probability of not receiving an LT due to issues with social determinants of health (SDOH). It has been reported that Hispanics are more likely to be removed from the transplant list. We report a case of a 25-year-old female immigrant from a Latin American developing country who presented with symptoms consistent with worsening liver disease after years of inappropriate workup and late diagnosis due to barriers within the healthcare system. The patient had a history of unresolved jaundice and pruritus and presented with worsening of her previous symptoms and new onset abdominal distention, bilateral leg edema, and telangiectasias. Laboratory and imaging studies confirmed the diagnosis of AIH and primary sclerosing cholangitis (PSC-AIH syndrome). The patient was started on steroids, azathioprine, and ursodeoxycholic acid, showing improvement. Due to her migratory status, she could not receive an appropriate diagnosis and follow up with a single provider or healthcare institution, putting her at increased risk for life-threatening complications. Although medical management is the first step, the probability of future liver transplants exists. The patient is still undergoing liver transplant evaluation and completing a workup since she was found to have an elevated model for end-stage liver disease (MELD) score. Even with the introduction of new scores and policies that aim to reduce disparities in LT, Hispanic patients are still at higher risk of being removed from the waitlist because of death or clinical deterioration compared to non-Hispanics. To this day, Hispanics have the highest percentage of waitlist deaths (20.8%) of all ethnicities and the lowest overall rate for undergoing LT. Understanding and addressing the causes that could contribute to and explain this phenomenon is essential. Increasing awareness of this problem is vital to promote more research on LT disparities.
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    Therapeutic Use of Viruses: Newcastle Disease Virus HK84 Oncolytic Treatment for Hepatocellular Carcinoma
    (Xia & He Publishing, 2022) Baiocchi, Leonardo; Francis, Heather; Alpini, Gianfranco; Medicine, School of Medicine