Chylous pericardial effusion with cardiac tamponade in a child treated with imatinib.

Chylous pericardial effusions are extremely rare outside of thoracic and cardiac surgery patients. We report the case of an 8-year-old girl with history of recurrent benign giant cell granulomas who developed a large chylous pericardial effusion with cardiac tamponade soon after beginning therapy with imatinib. In this article, we discuss the presentation, diagnosis, and management and review the published literature of this rarely reported side effect of this medication.

Genetic screening of relatives of decedents experiencing sudden unexpected death: medical examiner's office referrals to a multi-disciplinary cardiogenetics program.

Currently, no standardized system exists for evaluating and testing at-risk family members of decedents with abnormal post-mortem genetic testing in cases of sudden unexpected death (SUD). The goal of this study was to evaluate the outcomes of referrals made by an urban medical examiner's office to a multi-disciplinary cardiogenetics clinic. Relatives of decedents with pathogenic/likely pathogenic (P/LP) variants or variants of unknown significance (VUS) in genes known to be associated with cardiomyopathies and/or arrhythmias were identified by the New York City Office of Chief Medical Examiner and referred to the Cardiogenetics Clinic at Montefiore Medical Center. Familial referrals of 15 decedents (median 15 years, range 2 days to 57 years) were evaluated. Variants in 13 genes were identified among decedents (9 arrhythmia, 5 cardiomyopathy). P/LP variants were identified in both arrhythmia (RYR2, SCN5A) and cardiomyopathy syndrome (MYBPC3 (2), MYH7) genes. Thirty-two family members were referred, and 14 variants were detected. One pathogenic (MYBPC3) and two likely pathogenic (RYR2, MYH7) mutations were identified. Referral of at-risk family members of decedents who experienced SUD based on informative post-mortem genetic testing for cardiac and genetic evaluation is warranted, as family studies help to reclassify variants and prevent additional sudden death.

Kidney dysfunction in the low-birth weight murine adult: implications of oxidative stress.

Maternal undernutrition (MUN) during pregnancy leads to low-birth weight (LBW) neonates that have a reduced kidney nephron endowment and higher morbidity as adults. Using a severe combined caloric and protein-restricted mouse model of MUN to generate LBW mice, we examined the progression of renal insufficiency in LBW adults. Through 6 mo of age, LBW males experienced greater albuminuria (ELISA analysis), a more rapid onset of glomerular hypertrophy, and a worse survival rate than LBW females. In contrast, both sexes experienced a comparable progressive decline in renal vascular density (immunofluorescence analysis), renal blood flow (Laser-Doppler flowmetry analysis), glomerular filtration rate (FITC-sinistrin clearance analysis), and a progressive increase in systemic blood pressure (measured via tail-cuff method). Isolated aortas from both LBW sexes demonstrated reduced vasodilation in response to ACh, indicative of reduced nitric oxide bioavailability and endothelial dysfunction. ELISA and immunofluorescence analysis revealed a significant increase of circulating reactive oxygen species and NADPH oxidase type 4 (NOX4) expression in both LBW sexes, although these increases were more pronounced in males. Although more effective in males, chronic tempol treatment did improve all observed pathologies in both sexes of LBW mice. Chronic NOX4 inhibition with GKT137831 was more effective than tempol in preventing pathologies in LBW males. In conclusion, despite some minor differences, LBW female and male adults have a reduced nephron endowment comparable with progressive renal and vascular dysfunction, which is associated with increased oxidative stress and subsequent endothelial dysfunction. Tempol treatment and/or NOX4 inhibition attenuates renal and vascular dysfunction in LBW adults.

Postoperative Elevated Resistive Indices Do Not Predict Hepatic Artery Thrombosis in Extended Criteria Donor Livers.

Postoperative transplant liver ultrasounds were analyzed in standard criteria donor (SCD), extended criteria donor (ECD), and donation after cardiac death (DCD) liver allografts to determine if elevated resistive indices (RIs) are consistently present and if they are pathological. Postoperative transplant liver ultrasounds were reviewed from 115 consecutive patients. Hepatic arterial RIs were stratified based on the type of donor: DCD, macrosteatosis (>30%), or standard criteria. In all patients with elevated RI, subsequent ultrasounds were reviewed to demonstrate RI normalization. The mean RI for all 115 patients was 0.64, DCD was 0.67, macrosteatosis was 0.81, and SCD was 0.61 ( p = 0.033). The RI on subsequent liver ultrasounds for DCD and macrosteatosis normalized without any intervention. There were no incidences of early hepatic artery thrombosis (HAT) observed in the cohort. Hepatic arterial RI in ECDs and DCDs are elevated in the immediate postoperative period but are not predictive of HAT. It represents interparenchymal graft stiffness and overall graft edema rather than an impending technical complication. The results of our study do not support the routine use of anticoagulation or routine investigation with computed tomography angiography for elevated RIs as these findings are self-limiting and normalize over a short period of time. We hope that this information helps guide the clinical management of liver transplant patients from expanded criteria donors.

The use of venous jump grafts in pancreatic transplantation - no difference in patient or allograft outcomes - an update of the UNOS database.

Venous jump grafts are used in pancreas transplantation to salvage a pancreas with a short portal vein or to facilitate an easier anastomosis. There have been no large studies evaluating the safety of venous jump grafts in pancreas transplantation. We analyzed the UNOS database to determine whether venous jump grafts are associated with graft loss or patient death. Data from UNOS on all adult pancreas transplant recipients 1996-2012 were analyzed. Venous extension grafts were used in 2657 cases; they were not in 18 124. Kaplan-Meier/product-limit estimates analysis demonstrated similar patient survival (p < 0.641) and death-censored graft survival (p < 0.351) at one, three, five,10, and 15 yr between subjects with and without venous jump grafts. There was a statistically significant difference in one-yr unadjusted patient survival between the venous extension graft (94.9%) and the no-venous extension graft (95.8%) groups (p < 0.045) and a borderline difference in one-yr graft survival between the venous extension graft (84.1%) and the no-venous extension graft (82.6%) groups (p < 0.055). There was no significant difference in patient survival or allograft survival at the three-, five-, 10-, and 15-yr intervals. The use of venous jump grafts is not associated with increased graft loss or mortality.

An analysis of pancreas transplantation outcomes based on age groupings--an update of the UNOS database.

INTRODUCTION: Previously, increasing age has been a part of the exclusion criteria used when determining eligibility for a pancreas transplant. However, the analysis of pancreas transplantation outcomes based on age groupings has largely been based on single-center reports. METHODS: A UNOS database review of all adult pancreas and kidney-pancreas transplants between 1996 and 2012 was performed. Patients were divided into groups based on age categories: 18-29 (n = 1823), 30-39 (n = 7624), 40-49 (n = 7967), 50-59 (n = 3160), and ≥60 (n = 280). We compared survival outcomes and demographic variables between each age grouping. RESULTS: Of the 20 854 pancreas transplants, 3440 of the recipients were 50 yr of age or above. Graft survival was consistently the greatest in adults 40-49 yr of age. Graft survival was least in adults age 18-29 at one-, three-, and five-yr intervals. At 10- and 15-yr intervals, graft survival was the poorest in adults >60 yr old. Patient survival and age were found to be inversely proportional; as the patient population's age increased, survival decreased. CONCLUSION: Pancreas transplants performed in patients of increasing age demonstrate decreased patient and graft survival when compared to pancreas transplants in patients <50 yr of age.

Pancreas transplantation from donors after cardiac death: an update of the UNOS database.

OBJECTIVE: There is reluctance to use donation after cardiac death (DCD) organs for fear of worse outcomes due to increased warm ischemia time. Extensive evidence to confirm the quality of DCD pancreas transplants is not manifest. METHODS: A united network for organ sharing database review of pancreas transplants performed between 1996 and 2012 was conducted. We compared outcomes and all demographic variables between donors after cardiac death and donors after brain death in pancreas transplantation. RESULTS: There were 320 DCD pancreas transplants and 20,448 donation after brain death pancreas transplants performed in the United States between 1996 and 2012. There was no statistically significant difference in graft survival or patient survival in pancreas transplantation in DCD versus donation after brain death donors measured at 1-year, 3-year, 5-year, 10-year, and 15-year intervals. There was no significant difference between donor and recipient age, race, sex, and body mass index (BMI) between the groups. There was no significant difference between the recipient ethnicity or time on wait list between the groups. CONCLUSIONS: Pancreata procured by DCD have comparable outcomes to those procured after brain death. Donation after cardiac death pancreas transplant is a viable method of increasing the donor pool, decreasing wait list mortality, and improving the quality of life for type 1 diabetic patients.