الفهرس 
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Abstract
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This study included 50 infants with NC recruited from the pediatric hepatology department, National Liver Institute, Menofiya University from August 2013 to February 2015. Another 25 infants with sepsis without cholestasis were recruited from the pediatric hospital, Zagazig University as a control group for SF.
All cholestatic infants were subjected to:
1- Full history taking:
2- Thorough clinical examination:
3- The following investigations:
- Liver function tests.
- Prothrombin time and international normalized ratio (INR).
- Complete blood count.
- Abdominal ultrasound and Doppler on hepatic vessels.
- Serum ferritin, iron and total iron binding capacity.
- C-reactive protein
- TORCHs.
- Liver biopsy to detect :
Hepatic iron deposition grades
Liver fibrosis grades in liver tissue
Hepatic necroinflammatory activity
Data were collected, coded and processed by SPSS version 21 and the results were presented in tables and graphs.
Summary and conclusion
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The results of this study showed that:
1- Both cholestasis group and non-cholestatic (sepsis group) were age and sex matched (P > 0.05 for both).
2- There was no significant statistical difference between cholestasis group and non-cholestatic (sepsis group) regarding head circumference, weight, and length (P > 0.05 for all).
3- BA followed by PFIC had the highest percentage in the cholestasis group.
4- Total bilirubin, direct bilirubin, ALT, AST, ALP, and GGT were significantly higher in the cholestasis group than that in the non-cholestatic (sepsis group) (P < 0.0001 for all), while total protein, hemoglobin (P < 0.0001 for both), and albumin (P = 0.001) were significantly lower in the cholestasis group than non-cholestatic (sepsis group). On the other hand, platelets were significantly higher in the cholestasis group than non-cholestatic (sepsis group) (P < 0.0001). Regarding the other parameters (PT, PTT, INR, WBCS), there was no significant statistical difference between both groups (P > 0.05 for all).
5- Serum ferritin was elevated in cholestasis group and non-cholestatic sepsis group with no significant difference between both groups.
6- Serum iron and TIBC were within the normal ranges for age in both cholestasis and non-cholestatic sepsis groups. However, serum iron and TIBC were significantly higher in cholestasis group than non-cholestatic sepsis group (P < 0.0001 and P = 0.005; respectively).
7- Serum ferritin was significantly higher in NC with sepsis group than NC without sepsis group (P1 = 0.019) and it was higher in NC with sepsis group than non-cholestatic sepsis group with borderline significance (P2 = 0.055). On the other hand, there was no significant difference between NC without sepsis group and non-cholestatic sepsis group (P3 = 0.872).
8- Serum iron was significantly higher in NC with sepsis group than non-cholestatic sepsis group (P2= 0.004) and in NC without sepsis group than
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non-cholestatic sepsis group (P3 < 0.0001). TIBC was borderline higher in NC with sepsis group than non-cholestatic sepsis group (P2=0.056) and TIBC was significantly higher in NC without sepsis group than non-cholestatic sepsis group (P3 = 0.006). On the other hand, there was no significant difference between NC with sepsis group and NC without sepsis group regarding serum iron and TIBC (P1 > 0.05).
9- Serum ferritin was significantly higher in non-BA cholestasis (intrahepatic cholestasis) group than BA group (P < 0.0001).
10- Serum ferritin was significantly higher in non-BA cholestasis (intrahepatic cholestasis) without sepsis group than BA without sepsis group (P = 0.007).
11- Serum iron and TIBC were within the normal range for age in BA group and non-BA cholestasis group and there was no significant difference between both groups (P > 0.05 for each).
12- Total protein and GGT were significantly higher in BA group than non-BA cholestasis group (P = 0.048 and P < 0.0001; respectively) while Hb and AFP were significantly lower in BA group than non-BA cholestasis group (P = 0.014 and P = 0.01; respectively). On the other hand, there was no significant difference between BA group and non-BA cholestasis group regarding total bilirubin, direct bilirubin, albumin, ALT, AST, ALP, PT, INR, PTT, WBCS, and platelets.
13- Clay stool and splenomegly were significantly higher in the BA group than the non-BA cholestasis group (P < 0.0001 and P = 0.006; respectively) than non-B.A cholestasis. On the other hand, there was no significant statistical difference regarding jaundice, abdominal enlargement, encephalopathy, edema, bleeding diathesis, dark urine, hepatomegly, and ascites (P > 0.05 for all).
14- Abnormal GB (non-contractile/ non-visualized) and splenomegly were statistically significantly higher in the BA group than the non-BA cholestasis group (P = 0.004 and 0.016; respectively). While, there was no significant
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statistical difference in ultrasonic findings of hepatomegaly, and ascites between BA and non-BA cholestasis groups (P > 0.05).
15- Hepatic iron deposition grades were present with no significant difference in both NC with sepsis group and NC without sepsis group (P > 0.05). All 5 grades of hepatic deposition were present in neonatal cholestasis without sepsis group [grade 0 (56.3%), grade 1 (12.5%), grade 2 (15.6%), grade 3 (6.3%), grade 4 (9.4%)] while only 4 grades present in neonatal cholestasis with sepsis group [ grade 0 (22.2%), grade 1 (27.8%), grade 2 (22.2%), grade 3 (0%), grade 4 (27.8%)].
16- Hepatic iron deposition with its higher grades (≥ grades 3) were significantly higher in non-BA cholestasis group (intrahepatic cholestasis) than BA group (P = 0.016). All 5 grades of hepatic deposition were present in non-BA cholestasis group [grade 0 (20.8%), grade 1 (20.8%), grade 2 (25%), grade 3 (8.3%), grade 4 (25%)] while only 4 grades present in BA group [ grade 0 (65.4%), grade 1 (15.4%), grade 2 (11.5%), grade 3 (0%), grade 4 (7.7%)].
17- Serum ferritin at a cutoff value of ≥ 803.5 ng/ml can predict higher grades of iron deposition in liver tissue (≥ grade 3) in NC significantly (P = 0.0004) with a sensitivity of 100% and a specificity of 70%.
18- High grades of liver fibrosis (≥ grade 3) were significantly higher in the BA group than the non-BA cholestasis (intrahepatic cholestasis) group (P < 0.0001). ). All 5 grades of liver fibrosis were present in non-BA group [grade 0 (50%), grade 1 (33.3%), grade 2 (4.2%), grade 3 (8.3%), grade 4 (4.2%)] while only 4 grades present in BA group [ grade 0 (3.8%), grade 1 (11.5%), grade 2 (23.1%), grade 3 (61.5%), grade 4 (0%).
19- Ductular proliferation, bile plugs, porto-portal bridging, and NCHS total score were statistically significantly higher in the BA group than in non-BA cholestasis (intrahepatic cholestasis) group (P <0.0001 for all). While, diffuse hepatocyte swelling was statistically significantly higher in non-BA cholestasis group than BA group (P = 0.01).
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20- Neonatal cholestasis histopathological score at a cutoff value of 9.5 can predict BA diagnosis significantly (P < 0.0001) with a sensitivity of 80.8% and a specificity of 100%.
21- Neonatal cholestasis histopathological score diagnosed 21 cases of BA and only 5 cases failed to be diagnosed by NCHS. On the other hand, all the case of non-BA cholestasis were excluded by NCHS at a cutoff value = 9.5.
22- Serum ferritin had significant positive correlation with serum iron and hepatic iron deposition grades, while significant negative correlation with liver fibrosis grades and NCHS.
23- Serum ferritin had significant negative correlation with the age of cholestatic infants (P =0.0002).
24- Hepatic iron deposition grades had negative correlation with the age of cholestatic infants but without significance (P = 0.410).
25- Liver fibrosis grades had significant positive correlation with the age of cholestatic infants (P = 0.026).
26- Serum ferritin had positive correlation with ALT and AST, while negative correlation with ALP and GGT but without significance.
In conclusion, SF which is elevated in all the cholestatic disorders is not unique for certain neonatal disorder like HLH and NH. The elevated SF ≥ 803.5 ng/ml can predict higher grades of hepatic iron deposition irrespective of the etiology of the cholestasis. Pathological diagnosis of the cholestatic infants using NCHS can exclude all the cases of non-BA cholestasis.