Von Willebrand factor and esophageal varices in children with chronic liver diseases.

Aim of the study: To evaluate the role of plasma level of von Willebrand factor antigen (vWF-Ag) as a possible predictor for the presence of esophageal varices (EVs) in children with chronic liver diseases (CLDs). Material and methods: All patients underwent upper esophagogastroduodenoscopy (EGD) and were categorized as group I (had EVs) and group II (had no EVs). The following patient data were determined: Child-Pugh score (CPS), plasma vWF-Ag, vWF-Ag/thrombocyte ratio (VITRO) score, aspartate transaminase (AST) to platelet ratio index (APRI) score, AST/alanine transaminase (ALT), platelet count/spleen diameter, grading of EVs (small, medium and large) and categorizing the stage of liver fibrosis. Results: The analysis included 50 patients with CLD; 30 (60%) were female. The commonest etiological diagnoses were autoimmune hepatitis (AIH) (20%) and extra-hepatic biliary atresia (EHBA) (12%). 26% of cases were categorized as undiagnosed CLD. The CPS showed CPS-A 34%, CPS-B 44% and CPS-C 22%. The vWF-Ag was found at a high level of 243.52 ±195.97, with a highly statistically significant difference in discriminating the EVs with 74% accuracy at a cut-off value of 108.99 IU/ml, p < 0.0001. Also, ROC analysis was performed for discriminating large esophageal varices with 84% accuracy at a cut-off value of 475.85 mg%. The VITRO score at a cut-off value of 1.72 could detect EVs with 70% sensitivity, 86.7% specificity, and 80% accuracy. Conclusions: High vWF-Ag is a valuable prognostic tool for estimating the presence of EVs, and higher vWF-Ag is associated with increased grade of EVs.

Pediatric chronic liver failure-sequential organ failure assessment score and outcome of acute liver failure in children.

AIM OF THE STUDY: Liver transplantation remains the only definitive treatment for children with acute liver failure proven to have irreversible liver injury. Many prognostic models have been used for outcome prediction in pediatric acute liver failure to select patients in a real need of liver transplantation, but unfortunately all have shown inconsistent reproducibility and prognostic accuracy. The aim of this study was to evaluate the pediatric chronic liver failure sequential organ failure assessment (pCLIF-SOFA) score as a predictor of pediatric acute liver failure outcome. MATERIAL AND METHODS: Clinical and laboratory data of 41 children with acute liver failure admitted to the National Liver Institute - Menoufia University were collected retrospectively and used for calculation of both pCLIF-SOFA and Pediatric End-Stage Liver Disease (PELD)/Model for End-Stage Liver Disease (MELD) scores on the day of admission, then statistical analysis was performed to identify the ability of these scores to predict the outcome. RESULTS: According to the outcome, children enrolled in this study were allocated to survived (n = 16) and died (n = 25) groups, which were age and sex matched. The non-survival group had significantly higher values of both pCLIF-SOFA score (11 [7-13]) and PELD/MELD score (36 [18-42]) than those of the survival group (8 [7-11], 27.5 [15-45]; p < 0.001, p = 0.004) respectively. Both pCLIF-SOFA and PELD/MELD scores at cut-off values > 8 and > 30 respectively on admission could predict death in children with acute liver failure (ALF) with high sensitivity, but with higher specificity, positive and negative predictive values for pCLIF-SOFA. CONCLUSIONS: pCLIF-SOFA is a good predictor of death in pediatric acute liver failure.

Histopathological expression of Yes-associated protein in neonatal cholestasis.

BACKGROUND: Biliary atresia (BA) is a common cause of persistent neonatal cholestasis and liver transplantation in the pediatric population. Yes-associated protein (YAP) has also been shown to be necessary for development of bile ducts and adaptive responses within the gastrointestinal tract. We aimed to evaluate the YAP expression in liver tissues of infants with neonatal cholestasis as well as its diagnostic potential in the differential diagnosis of BA. PATIENTS AND METHODS: This prospective study included 100 infants with neonatal cholestasis. After full history taking, thorough clinical examination, routine investigations, and histopathological assessment, the patients were allocated as BA and non-BA; fifty patients in each group. Ten liver biopsies from 10 donors of liver transplant recipients served as controls. Diagnosis of BA was confirmed by operative cholangiography. Hepatic expression of YAP was assessed by immunohistochemical staining. RESULTS: Presence of clay stool, elevated GGT and absence of gall bladder contractility were the main preliminary signs alarming for the possibility of BA. Bile ductular and interlobular biliary epithelium and hepatic lobule expression of YAP in patients with BA was significantly higher than that in Non-BA group (P<0.05). There was no or weak positive YAP expression in normal liver of transplant donors. Positive YAP immunohistochemical had a sensitivity of 80% and a specificity of 94% with accuracy 87% in discrimination between BA and non-BA group (P-value<0.0001). CONCLUSION: Hepatic expression of YAP was significantly higher in BA than in non-BA group and could discriminate BA from other causes of cholestasis.

Glutathione peroxidase and malondialdehyde in children with chronic hepatitis C.

AIM OF THE STUDY: We aimed to assess oxidative stress factors, glutathione peroxidase (GPX) and malondialdehyde (MDA) in children with chronic hepatitis C (CHC) and their relation to treatment response. MATERIAL AND METHODS: The study included 50 children with chronic hepatitis C virus (HCV) before treatment (naïve HCV), 25 children responders to HCV treatment, 25 children non-responders to HCV treatment and 25 healthy controls. All patients and controls were subjected to GPX and MDA measurement by enzyme-linked immunosorbent assay. RESULTS: The average GPX activity in erythrocytes of naïve CHC patients was 29.2 ±10.3 mU/ml. It was statistically significantly lower than the average activity of GPX in erythrocytes of the healthy control group (47.3 ±5.2 mU/ml) (p < 0.05). The average GPX activity in erythrocytes of the responder group was 34.93 ±3.17 mU/ml. It was statistically significantly higher than the average activity of GPX in erythrocytes of the non-responder group (11.7 ±4.2 mU/ml) (p < 0.05). Plasma MDA was significantly higher in naïve CHC patients than in healthy controls (9.7 ±3.7 nmol/ml vs. 3 ±1.1 nmol/ml, p < 0.0001). Furthermore, plasma MDA concentration was significantly decreased in the responder group (5.36 ±0.7 nmol/ml) and elevated in the non-responder group (16.05 ±2.9 nmol/ml). CONCLUSIONS: Lower pretreatment levels of GPX and higher MDA level might be markers of oxidative stress occurring in HCV patients. Reversal of changes of these levels with completion of the treatment may indicate a correlation between oxidative stress and the viral pathogenesis.