Solid organ graft-versus-host disease in a recipient of a COVID-19 positive liver graft.

A 66-year-old male with end-stage liver disease (ESLD) secondary to non-alcoholic fatty liver disease (NAFLD), complicated by hepatocellular carcinoma (HCC), underwent deceased donor liver transplantation from a Coronavirus disease 2019 (COVID-19) positive donor. He presented a month later with fever, diarrhea and pancytopenia which led to hospitalization. The hospital course was notable for respiratory failure, attributed to invasive aspergillosis, as well as a diffuse rash. A bone marrow biopsy revealed hypocellular marrow without specific findings. In the following days, laboratory parameters raised concern for secondary hemophagocytic lymphohistiocytosis (HLH). Clinical concern also grew for solid organ transplant graft-versus-host-disease (SOT-GVHD) based on repeat marrow biopsy with elevated donor-derived CD3+ T cells on chimerism. After, a multidisciplinary discussion, the patient was started on ruxolitinib, in addition to high dose steroids, to address both SOT-GVHD and secondary HLH. Patient developed symptoms concerning for hemorrhagic stroke and was transitioned to comfort care. Although GVHD has been studied extensively in hematopoietic stem cell transplant (HSCT) patients, it is a rare entity in SOT with a lack of guidelines for management. Additionally, whether COVID-19 may play a role in development of SOT-GVDH has not been explored.

Objective Versus Subjective Assessment of Functional Status in Candidates for Liver Transplantation.

INTRODUCTION: and aim. Poor functional status is associated with increased mortality in cirrhosis patients awaiting liver transplantation (LT); however, the optimal assessment of functional status remains unknown. This study sought to determine the relationship between 6-minute walk distance (6MWD) and Karnofsky Performance Status (KPS) and their association with waitlist mortality in LT candidates. MATERIAL AND METHODS: Two hundred seventy-eight consecutive patients listed for LT were included. KPS and 6MWD were assessed at the time of evaluation. KPS was recorded as a percentage from 0 to 100, with 0 representing death and 100 representing no presence of disease. Patients were followed from time of listing until transplantation, death, removal from the waitlist or end of the study period. RESULTS: The mean KPS and 6MWD were 77.4 ± 13.5 and 323.6 ± 163.9 m, respectively. A mild correlation between 6MWD and KPS was demonstrated (Spearman ρ = 0.4317, P < .0001). KPS was significantly lower in patients with 6MWD < 250 meters (P < .0001). The 6MWD was significantly lower in patients who suffered waitlist mortality (266.1 vs 331.8 m, P = .05). CONCLUSION: In conclusion, 6MWD is a better predictor of waitlist mortality than KPS score in candidates for LT. The addition of 6MWD as a standard assessment may help to identify patients at risk of dying on the waitlist.

Review article: the evidence that vancomycin is a therapeutic option for primary sclerosing cholangitis.

BACKGROUND AND AIMS: PSC is an autoimmune biliary inflammatory disorder that is often associated with inflammatory bowel disease (IBD), with 50%-75% of patients with PSC having coexisting IBD, most commonly ulcerative colitis. Currently, no medical therapies have been shown to improve the disease course or slow its progression. However, ongoing research has resulted in a growing interest in the use of antibiotics for treatment of PSC, of which vancomycin is the most studied. In this review, we summarise the current evidence on the use of vancomycin in PSC and comment on future research areas of interest. METHODS: A comprehensive PUBMED and EMBASE literature search for articles on vancomycin, PSC, therapeutic options and microbiome was performed. RESULTS: Two randomised clinical trials, three case series and two case reports were included in the study. These include uncontrolled data from at least 98 patients that include promising improvements in biochemistry and imaging. Optimal dosing regimens are unclear. CONCLUSION: Vancomycin is one of the most studied antibiotics used in the treatment of PSC with promising results. There is not currently sufficient evidence to support treatment recommendations. Further research is needed to establish if vancomycin is a PSC treatment.

Review article: maximising quality of life while aspiring for quantity of life in end-stage liver disease.

BACKGROUND: With recent advances in the management of chronic liver disease and its complications, the long-term survival in cirrhosis has improved. Therefore, the number of individuals who will spend a significant proportion of their life with end-stage liver disease (ESLD) may continue to rise. Thus, more attention to quality of life (QOL) and its integration with traditional clinical endpoints is needed. AIMS: Recently, there have been many studies looking at treatment outcomes and their impact on the QOL in patients with ESLD. The aim of this review was to summarise and compare the insights gained from these intervention studies and to make concise recommendations to further promote and improve QOL in this patient population. METHODS: A literature search was conducted using PubMed and Web of Science. Search terms "Quality of life" "Cirrhosis" and "end-stage liver disease" were used as MeSH terms or searched in the title of the article. RESULTS: These studies uniformly show significant improvement in health-related QOL (HRQOL) with management of malnutrition, hepatic encephalopathy and ascites. Thus, early recognition and management of these complications are keys to better serve our patients. Early involvement of palliative care also leads to improved quality of end-of-life care. CONCLUSIONS: Complications of cirrhosis including malnutrition, encephalopathy, ascites and variceal bleeding lead to a decrease in HRQOL. Assessment of HRQOL has an important implication for the patient. The findings of this review illuminate the importance of using consistent tools to accurately assess QOL in patients with ESLD.

Fulminant hepatitis due to human adenovirus.

PURPOSE: To describe the demographics, clinical manifestations, treatment and outcomes of patients with human adenovirus (HAdV) hepatitis. METHODS: A case of fulminant HAdV hepatitis in a patient with chronic lymphocytic leukemia receiving rituximab and fludarabine is described. We conducted a comprehensive review of the English-language literature through May, 2012 in search of definite cases of HAdV hepatitis. RESULTS: Eighty-nine cases were reviewed. Forty-three (48 %) were liver transplant recipients, 19 (21 %) were bone marrow transplant recipients, 11 (12 %) had received chemotherapy, five (6 %) had severe combined immunodeficiency, four (4 %) were HIV infected, two had heart transplantation, and two were kidney transplant recipients. Ninety percent (46/51) of patients presented within 6 months following transplantation. Fever was the most common initial symptom. Abdominal CT scan revealed hypodense lesions in eight of nine patients. Diagnosis was made by liver biopsy in 43 (48 %), and on autopsy in 46 (52 %). The HAdV was isolated at other sites in 54 cases. Only 24 of 89 patients (27 %) survived: 16 whose immunosuppression was reduced, six with liver re-transplantation, and two who received cidofovir and intravenous immunoglobulin. CONCLUSION: HAdV hepatitis can manifest as a fulminant illness in immunocompromised hosts. Definitive diagnosis requires liver biopsy. Early consideration of a viral etiology, reduction in immunosuppression, and liver transplantation can be potentially life-saving.

Coccidioidomycosis in liver transplant recipients in an endemic area.

Coccidioidomycosis is an infection caused by Coccidioides species, which are endemic for the Southwestern United States and parts of Central America and South America. Most infected individuals are asymptomatic or have mild-to-moderate respiratory illness. Coccidioidomycosis is more severe in patients with depressed cellular immunity, such as organ transplant recipients. We retrospectively reviewed charts of 391 liver transplant recipients (mean follow-up, 38.7 months; range, 2-105 months). Before transplantation, 12 patients had a history of coccidioidomycosis and 13 patients had asymptomatic seropositivity. Of these 25 patients, 23 had no active coccidioidomycosis posttransplantation and 2 had reactivated infection. One of 5 patients with indeterminate serology before transplantation died of disseminated coccidioidomycosis shortly after transplantation. De novo coccidioidomycosis developed in 12 patients (3%) who had no evidence of coccidioidomycosis pretransplantation. Of 15 total episodes of posttransplantation coccidioidomycosis, 10 (66%) occurred during the first year. Dissemination was noted in 33% of active coccidioidomycosis after transplantation; two patients (13%) died of coccidioidomycosis. Because most coccidioidal infections occurred in the first posttransplantation year despite targeted antifungal prophylaxis, we recommend a new strategy of universal antifungal prophylaxis for 6-12 months for liver transplant recipients who reside in the endemic area.

Do patients need more frequent colonoscopic surveillance after liver transplantation?

BACKGROUND: Solid-organ transplant recipients are at higher risk for developing malignancies believed to be due to the use of immunosuppression. The aim of our study was to determine the risk of development of colon polyps with advanced features and colon carcinoma in liver transplant (LT) recipients when compared to individuals with chronic liver disease (CLD) and individuals without liver disease. METHODS: Case-control analyses of 82 LT recipients who underwent posttransplant colonoscopy, matched for age, gender, and year of colonoscopy, were compared to 82 patients with chronic liver disease and 82 patients without liver disease undergoing screening colonoscopy. Incidence of advanced adenomas (polyps >1 cm, high-grade dysplasia, villous histology) and colon carcinoma was documented. RESULTS: The groups were similar in age and gender, but there were more Hispanic patients in the LT group. Six patients (7.3%) of the LT group, 3 patients (3.6%) of the chronic liver disease group, and 1 patient (1.2%) of the no-liver-disease group had the outcome of interest, but the P value was not significant. Immunosuppression used was tacrolimus and mycophenolate in 2 patients, tacrolimus-only in 2 patients, and cyclosporine in 2 patients. CONCLUSIONS: There is a trend toward increased incidence of advanced colon polyps and colon carcinoma in immunosuppressed patients after liver transplantation. Larger studies are needed to determine whether posttransplant colon cancer surveillance should be more frequent than currently recommended for nontransplant patients.

The yield of capsule endoscopy in patients with abdominal pain or diarrhea.

BACKGROUND AND STUDY AIMS: Capsule endoscopy, proven effective for evaluation of obscure gastrointestinal bleeding and suspected Crohn's disease, is increasingly used to investigate other small-intestine disorders, but its yield for other indications is not well known. We sought to evaluate its yield and findings for abdominal pain or diarrhea. PATIENTS AND METHODS: Medical records of patients with abdominal pain or diarrhea (> 6 weeks' duration) who underwent capsule endoscopy between August 2001 and June 2004 were retrospectively reviewed for demographic data, indications, findings, diagnoses, complications, and radiologic studies. All patients had previous endoscopic or radiologic examinations (colonoscopy, enteroscopy, upper endoscopy, small-bowel series, computed tomography enterography, or computed tomography) demonstrating no abnormalities sufficient for diagnosis. RESULTS: 64 patients (26 men; 38 women; mean age, 43 years; age range, 19 - 83 years) who met study criteria had 68 capsule endoscopy studies. Indications were abdominal pain (35 patients), diarrhea (14), or both (15). Complete small-bowel visualization with identification of the cecum was achieved in 81 %; yield of positive findings was 9 % (6 patients). By indications, the yield was 6 % for abdominal pain, 14 % for diarrhea, and 13 % for both. Diagnoses included Crohn's disease (3), enteropathy induced by nonsteroidal anti-inflammatory drugs (2), and submucosal tumor (1). Capsule retention occurred in two patients, requiring surgical removal. CONCLUSIONS: Capsule endoscopy had a low yield for evaluation of abdominal pain or diarrhea and cannot be recommended as a first-line test without further study. Nonetheless, it facilitated diagnosis in 9 % of patients with negative endoscopic and radiologic examinations.

Familial polyarticular osteochondritis dissecans masquerading as juvenile rheumatoid arthritis.

A boy with polyarticular osteochondritis dissecans presented with clinical features of juvenile rheumatoid arthritis. Examination of the synovial fluid of an involved joint demonstrated inflammatory characteristics. Osteochondritis dissecans was present in the patient's mother and brother. Human leukocyte antigens HLA A-2, BW-15 and CW-4 were identified in the affected individuals.