الفهرس 
Neonatal CholestasisOnly 14 pages are availabe for public view
 |  | from | 174 |  |  |
| | | from | 174 | | |
Abstract
Neonatal cholestasis (NC) is defined as persistence of direct bilirubin more than 20% of total serum bilirubin for more than 14 days. Cholestasis in infancy is caused by a wide range of causes; biliary atresia (BA) is the commonest cause of neonatal cholestasis.
BA is a progressive destruction of the extra hepatic and intrahepatic bile ducts, with scarring and obliteration with mostly unknown etiology and pathogenesis. It is important to be differentiated from other causes of neonatal cholestasis as early as possible for the success of Kasai portoenterostomy. If portoenterostomy is not successful or not performed, liver transplantation is the only life-saving alternative
If left untreated, these disorders produce biliary cirrhosis and eventual hepatic failure; thus it is anticipated that several biochemical metabolic pathways would be affected through the disease progression. However, it is still difficult to differentiate BA from other causes of neonatal cholestasis due to limitations in conventional approaches.
As the liver plays a central role in amino acid metabolism, it is of great importance to investigate the metabolic profiles in BA and other causes of neonatal cholestasis. Carnitine plays an important role in fatty acid oxidation and energy production. It helps in b- oxidation of fatty acids by entering into the mitochondria. The carnitine bound with acyl-CoA to form acyl carnitine.
In the previous studies, there were different amino acid and acyl carnitine metabolite profiles corresponding to the different forms of liver disease. However, there were only a few studies on BA using liquid chromatography-Mass Spectrometry based methods to investigate the acyl carnitine profiles.
This study aims toto compare different levels of amino acids and acyl carnitines profile to give an insight about differential expression and metabolic pathways in cholestasis and biliary atresia in pediatric patients compared to controls and for differentiation of BA from other causes of NC for early operative intervention by kasai operation postponing live transplantation in BA pediatric patients.
To achieve this goal, it was conducted on 35 diagnosed as biliary atresia and 35 neonatal cholestasis rather than biliary atresia randomly selected from National Liver Institute inpatients wards of paediatric department. In addition to 35 apparently healthy infants matching age and gender with the patients were used as a control group.
All patients and control groups were subjected to the following:
1. Full history taking.
2. Complete clinical examination.
3. Laboratory investigations
4. Abdominal ultrasonography.
5. Liver biopsy.
Laboratory investigations including: Liver function tests and amino acid and acyl carnitine assay using high performance liquid chromatography tandem mass spectrometry (LC MS/MS).