Child-Turcotte-Pugh versus MELD-XI identify distinct high-risk populations for heart transplantation following ventricular assist device placement.

BACKGROUND: Patients with end-stage heart failure frequently have significant congestive hepatopathy requiring hepatology assessment prior to heart transplantation listing. An elevated Model for End-stage Liver Disease score with modification to exclude INR (MELD-XI) has been associated with increased mortality following heart transplantation (HT). This study's primary aim was to examine whether Child-Turcotte-Pugh (CTP) classification is associated with post-transplant mortality in patients bridged to transplant with left ventricular assist devices. METHODS AND RESULTS: We conducted a retrospective analysis of 134 patients from our center. Age, CTP class, and MELD-XI at HT were included in the multivariate model for the primary outcome, which demonstrated a significant association between 1-year mortality and CTP class (CTP-A HR: .08, CI .01-.46, P < .01; CTP-B HR: .25, CI .05-1.2, P = .08; reference group CTP-C), and MELD-XI (HR: 1.15; CI: 1.03-1.28; P = .01), but no significant difference for age (HR: .97; CI: .93-1.01; P = .15). Only 13/33 patients with CTP improvement after assist device also had improvement in MELD-XI. CONCLUSIONS: Patients with relatively low MELD-XI scores with discordantly high CTP classification may be a distinct subset for whom MELD-XI underestimates the risk of mortality after heart transplantation compared to CTP.

Effects of eltrombopag on platelet count and platelet activation in Wiskott-Aldrich syndrome/X-linked thrombocytopenia.

UNLABELLED: Because Wiskott-Aldrich syndrome (WAS) and X-linked thrombocytopenia (XLT) patients have microthrombocytopenia, hemorrhage is a major problem. We asked whether eltrombopag, a thrombopoietic agent, would increase platelet counts, improve platelet activation, and/or reduce bleeding in WAS/XLT patients. In 9 WAS/XLT patients and 8 age-matched healthy controls, platelet activation was assessed by whole blood flow cytometry. Agonist-induced platelet surface activated glycoprotein (GP) IIb-IIIa and P-selectin in WAS/XLT patients were proportional to platelet size and therefore decreased compared with controls. In contrast, annexin V binding showed no differences between WAS/XLT and controls. Eltrombopag treatment resulted in an increased platelet count in 5 out of 8 patients. Among responders to eltrombopag, immature platelet fraction in 3 WAS/XLT patients was significantly less increased compared with 7 pediatric chronic immune thrombocytopenia (ITP) patients. Platelet activation did not improve in 3 WAS/XLT patients whose platelet count improved on eltrombopag. IN CONCLUSION: (1) the reduced platelet activation observed in WAS/XLT is primarily due to the microthrombocytopenia; and (2) although the eltrombopag-induced increase in platelet production in WAS/XLT is less than in ITP, eltrombopag has beneficial effects on platelet count but not platelet activation in the majority of WAS/XLT patients. This trial was registered at www.clinicaltrials.gov as #NCT00909363.

Medication adherence and rejection rates in older vs younger adult liver transplant recipients.

A growing number of older adults are undergoing liver transplantation (LT) in the United States. In some settings, it is thought that adherence declines with age. This retrospective study examined adherence and clinical outcomes in older vs younger adult LT recipients. Medical records of adult LT recipients from 2009 to 2012 from a single urban center were reviewed. The medication level variability index (MLVI) was the predefined primary outcome, with nonadherence defined as MLVI >2.5. The secondary outcome was incidence of rejection. Outcomes were evaluated starting 1 year post-LT until 2015. A total of 42 of 248 patients were ≥65 at transplant. Older adults had significantly better adherence than younger ones (65%≥65 were adherent vs 42% younger adults; chi-square two-tailed P=.02). Survival analyses of rejection between age groups censored by time since transplant showed no difference among the four age groups (χ2 =0.84, P=.84). Older age was not found to be a risk factor for reduced adherence or graft rejection in patients surviving at least 1 year post-LT.

Hyper IgM Syndrome: a Report from the USIDNET Registry.

PURPOSE: The United States Immunodeficiency Network (USIDNET) patient registry was used to characterize the presentation, genetics, phenotypes, and treatment of patients with Hyper IgM Syndrome (HIGM). METHODS: The USIDNET Registry was queried for HIGM patient data collected from October 1992 to July 2015. Data fields included demographics, criteria for diagnosis, pedigree analysis, mutations, clinical features, treatment and transplant records, laboratory findings, and mortality. RESULTS: Fifty-two physicians entered data from 145 patients of ages 2 months to 62 years (median 12 years); 131 were males. Using patients' age at last entry, data from 2072 patient years are included. Mutations were recorded for 85 subjects; 82 were in CD40LG. Eighteen subjects had non-X-linked HIGM. 40 % had a normal serum IgM and 15 %, normal IgA. Infections were reported for 91 %, with pulmonary, ear, and sinus infections being the most common. 42 % had Pneumocystis jirovecii pneumonia; 6 % had Cryptosporidium. 41 % had neutropenia. 78 % experienced non-infectious complications: chronic diarrhea (n = 22), aphthous ulcers (n = 28), and neoplasms (n = 8) including colon cancer, adrenal adenoma, liver adenocarcinoma, pancreatic carcinoid, acute myeloid leukemia, hepatoma, and, in a female with an autosomal dominant gain of function mutation in PIK3CD, an ovarian dysgerminoma. Thirteen patients had a hematopoietic marrow or stem cell transplant; three had solid organ transplants. Thirteen were known to have died (median age = 14 years). CONCLUSIONS: Analysis of the USIDNET Registry provides data on the common clinical features of this rare syndrome, and in contrast with previously published data, demonstrates longer survival times and reduced gastrointestinal manifestations.

Recall patterns and risk of primary liver cancer for subcentimeter ultrasound liver observations: a multicenter study.

BACKGROUND: Patients with cirrhosis and subcentimeter lesions on liver ultrasound are recommended to undergo short-interval follow-up ultrasound because of the presumed low risk of primary liver cancer (PLC). AIMS: The aim of this study is to characterize recall patterns and risk of PLC in patients with subcentimeter liver lesions on ultrasound. METHODS: We conducted a multicenter retrospective cohort study among patients with cirrhosis or chronic hepatitis B infection who had subcentimeter ultrasound lesions between January 2017 and December 2019. We excluded patients with a history of PLC or concomitant lesions ≥1 cm in diameter. We used Kaplan Meier and multivariable Cox regression analyses to characterize time-to-PLC and factors associated with PLC, respectively. RESULTS: Of 746 eligible patients, most (66.0%) had a single observation, and the median diameter was 0.7 cm (interquartile range: 0.5-0.8 cm). Recall strategies varied, with only 27.8% of patients undergoing guideline-concordant ultrasound within 3-6 months. Over a median follow-up of 26 months, 42 patients developed PLC (39 HCC and 3 cholangiocarcinoma), yielding an incidence of 25.7 cases (95% CI, 6.2-47.0) per 1000 person-years, with 3.9% and 6.7% developing PLC at 2 and 3 years, respectively. Factors associated with time-to-PLC were baseline alpha-fetoprotein >10 ng/mL (HR: 4.01, 95% CI, 1.85-8.71), platelet count ≤150 (HR: 4.90, 95% CI, 1.95-12.28), and Child-Pugh B cirrhosis (vs. Child-Pugh A: HR: 2.54, 95% CI, 1.27-5.08). CONCLUSIONS: Recall patterns for patients with subcentimeter liver lesions on ultrasound varied widely. The low risk of PLC in these patients supports short-interval ultrasound in 3-6 months, although diagnostic CT/MRI may be warranted for high-risk subgroups such as those with elevated alpha-fetoprotein levels.

Enhanced Communication for Interhospital Transfers Increases Preparedness in an Academic Tertiary Care Center.

OBJECTIVES: This quality improvement project sought to enhance clinical information sharing for interhospital transfers to an inpatient hepatology service comprised of internal medicine resident frontline providers (housestaff) with the specific aims of making housestaff aware of 100% of incoming transfers and providing timely access to clinical summaries. INTERVENTIONS: In February 2020, an email notification system to senior medicine residents responsible for patient triage shared planned arrival time for patients pending transfer. In July 2020, a clinical data repository ("Transfer Log") updated daily by accepting providers (attending physicians and subspecialty fellows) became available to senior medicine residents responsible for triage. METHODS: Likert scale surveys were administered to housestaff before email intervention (pre) and after transfer log intervention (post). The time from patient arrival to team assignment (TTA) in the electronic medical record was used as a proxy for time to patient assessment and was measured pre- and postinterventions; >2 hours to TTA was considered an extreme delay. RESULTS: Housestaff reported frequency of access to clinical information as follows: preinterventions 4/31 (13%) sometimes/very often and 27/31 (87%) never/rarely; postinterventions 11/26 (42%) sometimes/very often and 15/26 (58%) never/rarely (p = 0.02). Preinterventions 12/39 (31%) felt "not at all prepared" versus 27/39 (69%) "somewhat" or "adequately"; postinterventions 2/24 (8%) felt "not at all prepared" versus 22/24 (92%) somewhat/adequately prepared (p = 0.06). There was a significant difference in mean TTA between pre- and posttransfer log groups (62 vs. 40 minutes, p = 0.01) and a significant reduction in patients with extreme delays in TTA post-email (18/180 pre-email vs. 7/174 post-email, p = 0.04). CONCLUSION: Early notification and increased access to clinical information were associated with better sense of preparedness for admitting housestaff, reduction in TTA, and reduced frequency of extreme delays in team assignment.