Resilience against misbehaving nodes in asynchronous networks

When dealing with network systems, a fundamental challenge is to ensure their functioning even when some of the network nodes do not operate as intended due to faults or attacks. The objective of this paper is to address the problem of resilient consensus in a context where the nodes have their own clocks, possibly operating in an asynchronous way, and can make updates at arbitrary time instants. The results represent a first step towards the development of resilient event-triggered and self-triggered coordination protocols. (C) 2019 Elsevier Ltd. All rights reserved

Barriers to ideal outcomes after pediatric liver transplantation

Long‐term survival for children who undergo LT is now the rule rather than the exception. However, a focus on the outcome of patient or graft survival rates alone provides an incomplete and limited view of life for patients who undergo LT as an infant, child, or teen. The paradigm has now appropriately shifted to opportunities focused on our overarching goals of “surviving and thriving” with long‐term allograft health, freedom of complications from long‐term immunosuppression, self‐reported well‐being, and global functional health. Experts within the liver transplant community highlight clinical gaps and potential barriers at each of the pretransplant, intra‐operative, early‐, medium‐, and long‐term post‐transplant stages toward these broader mandates. Strategies including clinical research, innovation, and quality improvement targeting both traditional as well as PRO are outlined and, if successfully leveraged and conducted, would improve outcomes for recipients of pediatric LT.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/151257/1/petr13537.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/151257/2/petr13537_am.pd

Natural History of Liver Disease in a Large International Cohort of Children with Alagille syndrome:Results from The GALA Study

BACKGROUND: Alagille syndrome (ALGS) is a multisystem disorder, characterized by cholestasis. Existing outcome data are largely derived from tertiary centers and real-world data are lacking. This study aimed to elucidate the natural history of liver disease in a contemporary, international, cohort of children with ALGS.METHODS: Multicenter retrospective study of children with a clinically and/or genetically confirmed ALGS diagnosis, born Jan-1997 - Aug-2019. Native liver survival (NLS) and event-free survival rates were assessed. Cox models were constructed to identify early biochemical predictors of clinically evident portal hypertension (CEPH) and NLS.RESULTS: 1433 children (57% male) from 67 centers in 29 countries were included. 10 and 18-years NLS rates were 54.4% and 40.3%. By 10 and 18-years, 51.5% and 66.0% of ALGS children experienced ≥1 adverse liver-related event (CEPH, transplant or death). Children (&gt;6 and ≤12 months) with median total bilirubin (TB) levels between ≥5.0 and &lt;10.0 mg/dL had a 4.1-fold (95% CI 1.6 - 10.8) and those ≥10.0 mg/dL had an 8.0-fold (95% CI 3.4 - 18.4) increased risk of developing CEPH compared with those &lt;5.0 mg/dL. Median TB levels between ≥5.0 and &lt;10.0 mg/dL and &gt;10.0 mg/dL were associated with a 4.8 (95% CI 2.4 - 9.7) and 15.6 (95% CI 8.7 - 28.2) increased risk of transplantation relative to &lt;5.0 mg/dL. Median TB &lt;5.0 mg/dL were associated with higher NLS rates relative to ≥5.0 mg/dL, with 79% reaching adulthood with native liver (p&lt;0.001).CONCLUSIONS: In this large international cohort of ALGS, only 40.3% of children reach adulthood with their native liver. A TB &lt;5.0 mg/dL between 6-and-12-months of age is associated with better hepatic outcomes. These thresholds provide clinicians with an objective tool to assist with clinical decision-making and in the evaluation of novel therapies.</p

Outcomes after liver transplantation in young infants

Background: Liver transplantation in infants younger than 90 days is increasingly common. These infants typically arrive for transplantation in fragile medical condition. It is commonly assumed that they may experience high complication rates, difficult postoperative courses, and poor graft and patient survival. Objectives: We sought to understand whether graft and patient survival rates in these young infants were lower than in older children, these patients experienced more complications than older children, and health care resource utilization was higher in this population. Patients and Methods: Data queried from the Study of Pediatric Liver Transplantation (SPLIT) database were limited to infants ages 0 to 90 days who had received their primary liver transplant between February 1996 and May 2004. Patients older than 90 days registered in the SPLIT database were used for comparison. Results: Thirty-eight patients, ages 0 to 90 days, were included in the analysis. Their severity of illness was reflected by a median calculated Pediatric Endstage Liver Disease score of 34.8 at transplant. A majority (89.5%) of infants received cadaveric liver grafts, of which 47% were reduced organs. The infants experienced prolonged hospitalizations, spending an average of 50.9 +/- 7.6 days in the hospital after transplant. Long stays in the intensive care unit (average 22.1 +/- 1.5 days) and need for mechanical ventilation (average 16.2 +/- 2.7 days) also occurred. Length of hospitalization, intensive care, and mechanical ventilation were significantly higher than in older children (P <0.0001). The reoperation rate (60.5%) was high and significantly greater than in older children (P = 0.007), with 10 patients (26.3%) needing 3 or more early reoperations. Reoperations occurred for bleeding, wound complications, biliary complications, and sepsis. There was no difference in vascular or biliary tract complications compared with older children. Bacterial infections were also common (52.6%) and were seen with greater frequency than in older children (P <0.04). This infant cohort had an overall graft survival of 76.1% and overall patient survival of 87.8% at 1 year, with median follow-up of 12.5 months (range 0.6-84.0 months). Graft and patient survival in infants younger than 90 days was similar to that in older infants and children (P = NS). Conclusions: Young infants experience graft and patient survival similar to that in older cohorts of liver transplant recipients. Posttransplant complication rates, including the reoperation rate, were higher in this younger group, and the duration of hospitalization and intensive support were significantly longer. Future studies to better examine the factors, including age, that may contribute to the need for reoperation in children are warranted. Recognition and further analysis of the cost of care in this age group is also needed

Outcomes after liver transplantation in young infants

Background: Liver transplantation in infants younger than 90 days is increasingly common. These infants typically arrive for transplantation in fragile medical condition. It is commonly assumed that they may experience high complication rates, difficult postoperative courses, and poor graft and patient survival. Objectives: We sought to understand whether graft and patient survival rates in these young infants were lower than in older children, these patients experienced more complications than older children, and health care resource utilization was higher in this population. Patients and Methods: Data queried from the Study of Pediatric Liver Transplantation (SPLIT) database were limited to infants ages 0 to 90 days who had received their primary liver transplant between February 1996 and May 2004. Patients older than 90 days registered in the SPLIT database were used for comparison. Results: Thirty-eight patients, ages 0 to 90 days, were included in the analysis. Their severity of illness was reflected by a median calculated Pediatric Endstage Liver Disease score of 34.8 at transplant. A majority (89.5%) of infants received cadaveric liver grafts, of which 47% were reduced organs. The infants experienced prolonged hospitalizations, spending an average of 50.9 +/- 7.6 days in the hospital after transplant. Long stays in the intensive care unit (average 22.1 +/- 1.5 days) and need for mechanical ventilation (average 16.2 +/- 2.7 days) also occurred. Length of hospitalization, intensive care, and mechanical ventilation were significantly higher than in older children (P <0.0001). The reoperation rate (60.5%) was high and significantly greater than in older children (P = 0.007), with 10 patients (26.3%) needing 3 or more early reoperations. Reoperations occurred for bleeding, wound complications, biliary complications, and sepsis. There was no difference in vascular or biliary tract complications compared with older children. Bacterial infections were also common (52.6%) and were seen with greater frequency than in older children (P <0.04). This infant cohort had an overall graft survival of 76.1% and overall patient survival of 87.8% at 1 year, with median follow-up of 12.5 months (range 0.6-84.0 months). Graft and patient survival in infants younger than 90 days was similar to that in older infants and children (P = NS). Conclusions: Young infants experience graft and patient survival similar to that in older cohorts of liver transplant recipients. Posttransplant complication rates, including the reoperation rate, were higher in this younger group, and the duration of hospitalization and intensive support were significantly longer. Future studies to better examine the factors, including age, that may contribute to the need for reoperation in children are warranted. Recognition and further analysis of the cost of care in this age group is also needed

Higher Mortality in Pediatric Liver Transplant Candidates With Sarcopenia

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/168284/1/lt26027.pdfhttp://deepblue.lib.umich.edu/bitstream/2027.42/168284/2/lt26027_am.pd