الفهرس 
Neonatal CholestasisOnly 14 pages are availabe for public view
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Abstract
Biliary atresia is a severe neonatal liver disease resulting from sclerosing cholangiopathy. Definitive diagnosis requires surgery, cholangiogram, and liver biopsy. Early diagnosis is particularly important for BA because early surgical intervention by Kasai portoenterostomy correlates with good long-term outcome. Thus, there is real need for novel blood tests that facilitate differentiation between BA and other neonatal cholestatic diseases.Developing rapid non-invasive diagnostic tests that clearly distinguish BA from other forms of neonatal cholestasis is important, Definitive diagnosis may require a combination of serum biomarkers, clinical criteria and imaging results to provide an unambiguous diagnosis without liver biopsy or cholangiogram. The main target of the current study was to evaluate serum apolipoprotein CII levels in infants with BA in comparison with infants with other forms of neonatal cholestasis Apolipoprotein C-II is a single polypeptide chain consisting of amino acids. Apolipoprotein C-II is present in plasma at a concentration of 4 mg/dl and its prime metabolic function appears to be associated with its ability to act as a cofactor in activating lipoprotein lipase. Apolipoprotein C-II has also been reported to activate lethicin cholesterol acyl transferase (LCAT). It is reported to act as a possible receptor for hepatitis C virus Apolipoprotein CII is linked on chromosome 19 and its
expression is regulated by two hepatic control region cis-acting liver enhancers that are activated by the farnesoid X-activated receptor (FXR), a nuclear hormone receptor that induces gene expression in response to several bile acids. FXR activity is elevated in liver of
infants with BA, since FXR is activated by bile acids that accumulate more significantly in cholestatic than inflammatory liver disease Elevated apolipoprotein C-II in infants with BA correlates with old
observations about dyslipoproteinemia in cholestatic disease.
Seventy five infants were enrolled in this study, 25 infants with
BA and 25 infants with other causes of neonatal cholestasis,
underwent full history taking and thorough clinical examination and
diagnostic tools including LFTs, PT, CBC, renal functions, abdominal
US with stress on TC sign and gall bladder contractility, Doppler on
hepatic veins, portal vein and hepatic arteries, hepatic subcapsular
flow, duodenal tube aspiration, intraoperative cholangiography and
investigations according to other cause of neonatal cholestasis and 25
apparently healthy infants, with no history or clinical evidence of liver
disease or any other disease, served as a control group. All cases were
subjected to measurement of serum apolipoprotein CII level.
Data were collected, coded and processed by statistical analysis
(SPSS) statistical package version 13 on IBM compatible computer,
and the results were put in tables and graphs.
Our results showed that:
1. Both patients and controls were age and sex matched (P>0.05)
2. It was found that clay coloured stools was significantly higher in
BA group than in non- BA group with P-value < 0.05.
3. Total proteins was significantly higher in BA group than in non-
BA group (P< 0.05).
4. There was statistically significant difference between BA group
and non-BA group regarding GGT with p-value <0.05.
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Summary
5. There was no significant difference between BA group and non-
BA group regarding platelet count.
6. Ultrasonographic hepatic size and splenic size were significantly
higher in BA group than in non-BA group with P-value < 0.05.
7. The contractility of gall bladder is significant in BA group than in
non-BA group with P-value < 0.05.
8. Triangular cord sign was statistically significant higher in BA
group than in non-BA group with P-value < 0.05.
9. Subcapsular flow was statistically significant higher in BA group
than in non-BA group with P-value < 0.05.
10. Duodenal tube aspiration was statistically significant higher in BA
group than in non-BA group with p-value < 0.05.
11. The current study showed a significant correlation between BA
group and non-BA group regarding intra-operative
cholangiography with p-value < 0.05.
12. There was significant difference between BA group and non-BA
group regarding bile ductular proliferation and bile plugs with pvalue
< 0.05.
13. Apo CII was statistically significant higher in BA group than in
non-BA and control groups (P < 0.05).
14. Apo CII was correlated with maximum velocity of hepatic veins.
15. Apo CII at a cutoff value of 35.5 ng/ml could discriminate between
BA and non-BA group and had 81.2 % AUROC with 80%
sensitivity, 76% specificity, 77% PPV and 79.1% NPV.
16. Apo CII AUROC of 0.81 is indicative of an excellent predictive
biomarker to differentiate between BA group and in non-BA