الفهرس 
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Abstract
Biliary atresia is a progressive cholestatic and fibrotic disease of unknown etiology which occurs only in infants. Children often appear normal at birth but become jaundiced in the first weeks of life. Early diagnosis and successful portoenterostomy establishing sufficient bile flow are the key events for extended native liver survival in BA. Even though the majority of BA patients end up with liver transplantation due to progressive hepatic fibrosis and associated portal hypertension.
IL-33 has been found to be an important factor involved in the pathogenesis of liver damage during acute and chronic hepatitis. Cytokine production and activity is usually localized within the target organ, however with increased severity of inflammation and cytokine production, the cytokines may enter the circulation and can be detected within the sera or plasma.
So the aim of this study is to evaluate the value of serum IL-33 levels in infants with cholestasis, correlate serum IL-33 levels with clinic-pathological profile of infants with cholestasis and compare its level with that of healthy infants who served as control.
Methods:
This cross sectional case controlled study was included 60 infants with NC who were recruited from the attendants of the outpatient pediatric hepatology clinic of Benha university hospitals and pediatric hepatology department, national liver institute, Menoufia university and 30 apparently healthy infants as a control group.
These infants were divided into:
1.Patient groups:
•group I (biliary atresia group): included 30 infants diagnosed as neonatal cholestasis with biliary atresia.
•group II (non-biliary atresia group): included 30 infants with cholestasis without biliary atresia.
2.group III (Control Group): included 30 infants apparently healthy infants matching the patient groups for age & sex.
All infants were subjected to the following: - Full history taking, Complete clinical examination. Imaging techniques: Abdominal ultrasonography (US), Investigations( Complete blood count, Serum transaminases (AST-ALT),Serum bilirubin total and direct bilirubin, Total proteins, serum albumin, alkaline phosphatase, gamma glutamyl transpeptidase, prothrombin time, Hepatitis markers (HBV&HCV),TORCH infection(rubella, cytomegalovirus, herpes simplex virus type 1 and 2 and toxoplasma antibodies (both IgM and IgG)), liver biopsy for patient groups only and quantification of serum IL-33 Levels using sandwich ELISA technique.
Results:
There was no statistical significant difference between studied groups regarding age and sex (P>0.05).
There was a statistical significant difference between studied groups regarding weight, length, Head circumference and BMI as all parameters were lower in BA group followed by control group and non-BA group.
There was statistical significant difference between patients groups as clay stool was 56.7 % in BA group while colored stool was 76.7% in non-BA group and also ascites was 20.0 % in BA group, however there was no statistical significant difference between patients group regarding jaundice, abdominal distension, pallor and growth retardation.
Diagnosis of non-BA group was 3 patients with Alagille syndrome ,1 patient with ARC syndrome, 1 patient with ARC syndrome and CMV infection , 2 patients with choledochal cyst , 1 patient with congenital hepatic fibrosis, 2 patients with galactosemia, 2 patients with giant cell hepatitis , 1 patient with GSD , 1 patient with hepatitis A infection, 5 patients with IBS ,5 patients with PFIC , 1 patient with sepsis and 5 patients with TORCH infection
There was a statistical significant difference between studied groups regarding liver span, spleen size and ascites as it was higher in BA group and also regarding liver texture as it was coarse in 93.3% of BA group and smooth in 63.3% of non-BA group. Hepatomegaly was present in all cases of BA group and in 83% of cases of non-BA group .splenomegaly was present in 40% of BA group and 36.7 % of non-BA group.
There was statistical significant difference between studied groups regarding AST and ALT was higher in non-BA group, while GGT, ALP, bilirubin (total and direct), PTT and INR were higher in BA group. Total protein was lower in patient groups, however no statistical significant difference between studied groups regarding albumin and PT.
There was statistical significant difference between studies groups regarding Hb and creatinine were higher in control group, while TLC was higher in non-BA group, however there was no statistical significance between studied groups regarding PLTS.
HAV and HBc igG were positive in only one patients of non-BA group, CMV was positive in only one patient of BA group and 4 patients of the non-BA group, while CMV and Rubella was positive in only one patient of BA group, also CMV, toxoplasma were positive in only one patient of non-BA group and HSV, CMV, Rubella were positive in only one patient of non-BA group, which was statistically non-significant.
Liver biopsy among the studied patients. Mononuclear cells were 66.7% in BA group and 33.3% in non-BA group, Giant cells represents 33.3% in non-BA group and mean± SD of fibrosis stage was 2± 0.8 in BA group, and 2.1± 0.9 in non-BA group, mean ± SD of HAI was 5.6±2.4 in BA group and 5.1±2.1 in non-BA group which was statistically non-significant.
There was no statistically significant difference between the groups regarding Child Pugh score and PELD.
There was statistical significant difference between studied groups regarding serum IL-33 as it was higher in BA group.
There was statistical no significant difference between studied groups regarding serum IL-33 according to diagnosis among non-BA group.
There was positive correlation between serum IL-33 and fibrosis stage, while there was no correlation between serum IL-33 and HAI, PELD, anthropometric measures and age in BA group.
There positive correlation between serum IL-33 and AST, ALT, bilirubin (total and direct) levels while there was no correlation between serum IL-33 and other laboratory findings among BA group.
There was positive correlation between serum IL-33 and fibrosis stage, while there was no correlation between serum IL-33 and HAI, Spleen size, Liver span, PELD, anthropometric measures and age among non-BA group.
There was positive correlation between serum IL-33 and bilirubin (total and direct) levels while there was no correlation between serum IL-33 and other laboratory findings among non-BA group.
There was statistical significant difference between serum IL-33 and fibrosis stage as serum IL-33 increased with increased stage of fibrosis, while there was no statistical significant difference between serum IL-33 and sex, liver texture, ascites, TORCH and child Pugh score among BA group.
There was statistical significant difference between serum IL-33 and fibrosis stage as serum IL-33 increased with increased stage of fibrosis, while there was no statistical significant difference between serum IL-33 and sex, liver texture, ascites, hepatitis markers and child Pugh score among non-BA group.
Serum IL-33 has cut-off value ≥20.8 ng/ml predict biliary atresia with specificity 95%and sensitivity 96.7% (P-value<0.05).
Serum IL-33 has cut-off value ≤ 20.3 ng/ml predict liver fibrosis with specificity 72.2% and sensitivity 66.7% (P value < 0.05).
Factors found to be significantly correlated with IL-33, were entered the multiple linear regression analysis model to detect the significant predictors of IL-33 level. It was found that AST and direct bilirubin are the significant predictors (P<0.05 for both).