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Introduction: Autoantibody testing has contributed to both biological and clinical insights in managing patients with liver disease. These autoantibodies often have clinical value for the diagnosis, disease activity and/or prognosis. Aim of the study: We aimed to investigate the potential application of auto-antibodies in different etiologies of non-autoimmune liver diseases. Material and methods: This study was conducted on 53 infants and children with chronic liver diseases. The patients were subjected to clinical history and examination, laboratory investigations and abdominal ultrasound. Serum of all infants and children was tested for measurement of antiprothrombin antibody and anti-b2-glycoprotein I (ab2GPI) and anticardiolipin (ACL) auto-antibodies using a fully-automated enzyme linked immunosorbent assay (ELISA) system. Results: The mean age of the infants with cholestatic liver diseases was significantly lower than those with metabolic liver diseases, hepatitis C virus (HCV) and vascular liver diseases (p < 0.05). The gender distribution was proportionate in all groups (p = 0.703). Autoantibodies showed significant variations among different etiologies of chronic liver diseases. he incidence of ab2GPI and ACL was significantly increased in both HCV (94.7% and 78.9%, respectively) and vascular liver diseases patients (90.9% and 72.7%, respectively) (p < 0.05). Antiprothrombin antibodies were found in 81.8% of vascular liver disease patients. Interestingly, all types of autoantibodies were deficient in cholestatic and metabolic liver diseases. Conclusions: Testing for liver-related autoantibodies should be included in the workup of patients with chronic liver diseases. Further studies are needed to explain the cause-effect association of ACL, ab2GPI and antiprothrombin with chronic HCV and vascular liver diseases.
ABSTRACT
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.
ABSTRACT
OBJECTIVES: The diagnosis of biliary atresia (BA) can be challenging as its histopathologic features overlap with those of other pediatric cholestatic liver diseases. We aimed to study the diagnostic value of hepatic CD56 immunostaining in the differentiation of BA from other causes of neonatal cholestasis. METHODS: Hepatic CD56 immunostaining was investigated in 30 infants with BA and compared with that in 30 infants with non-BA cholestatic disorders. The expression of positive cells was interpreted semiquantitatively on the basis of the extent (percentage or number) of positive cells on a scale of 0-3. RESULTS: The occurrence of CD56-positive biliary epithelial cells was significantly higher in the BA (83.3%) than in the non-BA group (6.7%), whereas the occurrence of CD56 natural killer cells in hepatic parenchyma was significantly higher in the non-BA group (76.7%) than in the BA group (6.7%; P<0.0001 for both). In contrast, there was no significant difference between both groups in CD56 natural killer cells in portal tracts (P>0.05). Using this differential expression as a discriminative tool between the BA and the non-BA group, positive biliary epithelial cell staining had high specificity, whereas negative parenchymal staining had high sensitivity (93.3% for both) with an accuracy of 88.3 and 84.65%, respectively. The combination of both parameters improved the accuracy up to 91.65%, with 100% specificity in the diagnosis of BA. CONCLUSION: CD56 immunostaining of the liver had a diagnostic value; it can be used to differentiate BA from other neonatal cholestatic disorders and might be useful as an additional stain when investigating infants with neonatal cholestasis.
