الفهرس 
Cholestatic Liver DiseaseOnly 14 pages are availabe for public view
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Abstract
SUMMARY
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).C
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.
The results of our study can be summarized in the following:
1. Despite vitamin K supplementation, elevation of plasma
PIVKA-II indicating vitamin K deficiency is common in
cholestatic liver disease.
2. Elevated PIVKA-II levels occurred in children with a
normal INR (40%) of studied children. Moreover all
patients without bleeding symptoms had increased PIVKAII,
while only (28%) of them had increased INR and the
remaining 72% had subclinical vitamin K deficiency
indicating that PIVKA-II is a more sensitive markers of
vitamin K status that could be used in children with chronic
cholestatic liver disease.
3. Elevation of PIVKA II was significantly more frequent in
studied patient than elevation of INR.
4. Plasma PIVKA-II was positively correlated with PT and
INR and negatively correlated with hemoglobin and
albumin.
5. Plasma PIVKA-II level increased in children with chronic
cholestatic liver disease of various etiologies and it was
correlated with worsening of the clinical stage of chronic
cholestatic liver disease and cirrhosis from Child A to
Child B and C classes