الفهرس 
CHOLESTATIC LIVER DISEASEOnly 14 pages are availabe for public view
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Abstract
C
holestasis is defined as a pathologic state of reduced bile formation or flow. It results in the retention of substances normally excreted into the bile, such as bilirubin and bile acids (Loomes et al., 2011).
Conjugated bile acids are detergents essential in facilitating the enteric absorption of fats and fat-soluble substances, among them vitamins K (Hadžić et al., 2012).
In vitamin K deficiency, there is an increase in abnormal undercarboxylated or partially carboxylated forms of vitamin K-dependent proteins, which are functionally defective (Booth et al., 2012).
Traditionally, vitamin K status has been assessed by PT & INR which measure activity, rather than vitamin K presence. In addition to that PT &INR have been shown to be an insensitive marker of vitamin K status and are late indicator of vitamin K deficiency and can be normal even when prothrombin concentrations fall to half normal values (Booth et al., 2003).
PIVKA-II (Protein Induced by Vitamin K Absence) is abnormal des-carboxylated prothrombin, which is present in vitamin K deficiency (Chuansumrit et al., 2010).
In light of the underestimation of the actual incidence of vitamin K deficiency, as measured by PT and INR, we aimed in this work to measure (PIVKA-II) as a marker of vitamin K deficiency status in children with chronic cholestatic liver disease and to compare PIVKA-II to traditional marker (INR) and to correlate it to the severity of the disease.
This cross-sectional study was conducted on 25 children and adolescents with chronic cholestatic liver diseases of different etiologies recruited from the Hepatology Clinic. They were 15 males (60%) and 10 females (40%).Their ages ranged from 2 to 14 years with mean 6.5 ± 4.03 years.
All children included in this study were subjected to medical history taking with special emphasis on the onset of the disease, hepatic symptoms and symptoms indicating bleeding tendency, as well as clinical examination including general examination and abdominal examination, in addition to investigations including complete blood count, liver function tests, liver enzymes, Protein induced by vitamin k absence (PIVKA-II).
Among the studied patients in this work, the most common etiology was EHBA (40%) followed by PFIC (24%). The most common clinical symptoms yielded from them were dark urine and clay stool (60%) each and the most common sign was hepatomegaly (84%). As regards Child’s classification of studied patients, (44%) were classified as Child A, (40%) were Child B and (16 %) were Child C.