Liver function, paraclinical tests, and mortality risk factors in pediatric liver transplant candidates

Abstract Liver transplantation has become a well-recognized transplant modality for children with end-stage liver disease. The first indicator to evaluate its efficiency is mortality rate. The aim of this study is to evaluate the complications and mortality of liver disease in children waiting for transplantation and understanding the risk factors that predict liver transplant waiting list death. This is a single-center prospective cohort study of all patient ≤18 years of age with chronic liver disease listed for liver transplantation. We analyzed medical records of 130 children for mortality risk factor. There were 52.3 % boys and 47.7 % girls with mean age of 8.6 ± 6.1 year (range, 3 months–18 year). The most common causes of cirrhosis were biliary atresia (21.5 %). The most common complication while awaiting transplantation was failure to thrive (76.2 %). Up to end of study, 20 patients (15.4 %) died without transplantation, 22 (16.9 %) of them transplanted, and 88 (67.7 %) of patients were alive while waiting for a liver transplantation. Mortality rate and transplantation ratio were 15.4 and 16.9 % with survival time waiting list 67.7 %. Factors which significantly associated with waiting list mortality included encephalopathy (p = 0.006), child score ≥10 (p = 0.036), model for end-stage liver disease (MELD) score ≥20 (p =&thinsp...
Source: Comparative Clinical Pathology - Category: Pathology Source Type: research

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CONCLUSIONS: Anesthetic preconditioning is a promising strategy to prevent I-R injury associated with transplantation. Our results suggest that sirtuin 2 is involved in the protective mechanisms of some commonly used anesthetics against I-R damage. PMID: 31726818 [PubMed - as supplied by publisher]
Source: Minerva Urologica e Nefrologica - Category: Urology & Nephrology Tags: Minerva Urol Nefrol Source Type: research
We report an optic atrophy spectrum disorder, including retinal macular dystrophy and kidney insufficiency leading to transplantation, associated with mitochondrial DNA (mtDNA) depletion without accumulation of multiple deletions. By whole-exome sequencing, we identified mutations affecting the mitochondrial single-strand binding protein (SSBP1) in 4 families with dominant and 1 with recessive inheritance. We show that SSBP1 mutations in patient-derived fibroblasts variably affect the amount of SSBP1 protein and alter multimer formation, but not the binding to ssDNA. SSBP1 mutations impaired mtDNA, nucleoids, and 7S-DNA am...
Source: Journal of Clinical Investigation - Category: Biomedical Science Authors: Source Type: research
Sustained, indolent immune injury of the vasculature of a heart transplant limits long-term graft and recipient survival. This injury is mitigated by a poorly characterized, maladaptive repair response. Vascular endothelial cells respond to proangiogenic cues in the embryo by differentiation to specialized phenotypes, associated with expression of apelin. In the adult, the role of developmental proangiogenic cues in repair of the established vasculature is largely unknown. We found that human and minor histocompatibility–mismatched donor mouse heart allografts with alloimmune-mediated vasculopathy upregulated express...
Source: Journal of Clinical Investigation - Category: Biomedical Science Authors: Source Type: research
Abstract Live imaging of neutrophils within optically transparent larval zebrafish has proved a powerful technique to investigate how specific gene products control neutrophil function. To resolve whether a gene contributes to neutrophil function in a cell-autonomous manner necessitates a way to examine gene-deficient neutrophils in an otherwise wild type background. To this end, here we describe methods to harvest fluorescent neutrophils from larval donor zebrafish and transplant them into age-matched recipients. We show that transplanted neutrophils can survive in recipient larvae for at least 3 days provid...
Source: Mol Biol Cell - Category: Molecular Biology Authors: Tags: Methods Mol Biol Source Type: research
Authors: Ghemame M, Charpentier P, Mouriaux F Abstract Developments in corneal topography allow for increasingly precise, detailed analysis of the corneal surface. This test is becoming indispensable in the treatment of complex corneas: keratoconus, corneal transplants, orthokeratology, etc. In refractive surgery, the combined analysis of the anterior and posterior cornea has permitted better screening for forme fruste keratoconus at risk for post-LASIK ectasia. Topography also assists in the calculation of premium intraocular lenses. Topography is an indispensable test for analyzing and following corneal disease. ...
Source: Journal Francais d Ophtalmologie - Category: Opthalmology Tags: J Fr Ophtalmol Source Type: research
Liver cirrhosis (LC) is the final usual outcome of liver damage induced by various chronic liver diseases. Because of asymptomatic nature of LC, it is usually diagnosed at late and advanced stages, and patients are easy to miss the best timing for treatment. Thus, the early detection of LC is needed. In the prospective Korean Cancer Prevention Study-II (K-II), we aimed to identify valuable biomarkers for LC using metabolomics to distinguish subjects with incident LC (LC group) from subjects free from LC (control group) during a mean 7-year follow-up period. Metabolic alterations were investigated using baseline serum speci...
Source: Frontiers in Physiology - Category: Physiology Source Type: research
ino Worldwide, approximately 10% of all human immunodeficiency virus (HIV)-infected people are also chronically coinfected with hepatitis B virus (HBV). HBV infection has a poor prognosis in HIV-positive people and has been documented by an increased risk of developing chronic HBV infection (CHB), progression to liver fibrosis and end-stage liver disease (ESLD) and evolution of hepatocellular carcinoma (HCC). Furthermore, in HIV patients, HBV-resolved infection is often associated with the appearance of HBV-DNA, which configures occult HBV infection (OBI) as a condition to be explored in coinfected patients. In this na...
Source: Viruses - Category: Virology Authors: Tags: Review Source Type: research
AbstractBiliary atresia (BA) is a rare neonatal cholestatic disease which leads to progressive obliterative cholangiopathy, resulting in biliary obstruction and jaundice. The standard surgical treatment is hepatoportoenterostomy (Kasai operation). Although approximately 50% of the affected infants would require liver transplantation within the first 2  years of life, the other 50% of the patients can live for years with their native liver, despite the progression of cirrhosis and chronic liver disease. Many of these patients will be affected by long-term complications such as repeated cholangitis, portal hypertension,...
Source: Pediatric Surgery International - Category: Surgery Source Type: research
Objectives: Biliary atresia (BA) is the leading cause of neonatal cholestasis and can progress to biliary cirrhosis. Portal hypertension and risk of Gastrointestinal bleeding (GB) are well-known complications for children awaiting liver transplant (LT). Primary prophylaxis remains controversial in children. The aim of our study was to identify the risk factors and outcomes for GB in patients with BA evaluated for LT.
Source: Digestive and Liver Disease - Category: Gastroenterology Authors: Tags: P48 Source Type: research
Abstract AIM: To evaluate the outcomes of patients with end-stage biliary disease (ESBD) who underwent liver transplantation, to define the concept of ESBD, the criteria for patient selection and the optimal operation for decision-making. METHODS: Between June 2002 and June 2014, 43 patients with ESBD from two Chinese organ transplantation centres were evaluated for liver transplantation. The causes of liver disease were primary biliary cirrhosis (n = 8), cholelithiasis (n = 8), congenital biliary atresia (n = 2), graft-related cholangiopathy (n = 18), Caroli's disease (n = 2), iatrogenic bile duct injury (n ...
Source: World Journal of Gastroenterology : WJG - Category: Gastroenterology Authors: Tags: World J Gastroenterol Source Type: research
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