الفهرس 
CHRONIC LIVER DISEASES (CLDS) IN CHILDREN
Clinical ManifestationsOnly 14 pages are availabe for public view
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Abstract
Diseases of the liver have been an important health issue since severe
hepatitis diseases could lead to persistent inflammation, necrosis, and even liver
cirrhosis and hepatoma.
Hepatorenal syndrome is a reversible functional renal impairment that
occurs in patients with chronic liver diseases. It is characterized by marked
reduction in GFR with advanced liver cirrhosis or those with fulminant hepatic
failure. So, close monitoring of chronic liver disease is recommended.
In the last few decades, liver transplantation (LTx) has become a
reliable life-saving procedure for patients with chronic end-stage liver diseases.
Patients exposed to nephrotoxic drugs after liver transplantation need early
detection of renal impairment if occurs and to modify immune-suppression.
Cystatin C (Cys C, molecular weight [MW], 13 kDa) is freely filtered
at that level of the glomerulus and virtually all is reabsorbed and metabolized by
proximal tubular cells. An increasing serum Cys C level was found to be related
to decreasing renal function.
Thus, the present study was designed to evaluate the clinical application
of cystatin C as a new marker for non-invasive estimation of glomerular
filtration rate and as a reliable marker for early renal impairment in chronic liver
diseases in children and to know if it is a more sensitive marker than creatinine,
with comparison to radio-isotopic scan of kidney as a gold standard
determination of GFR.
The study groups were composed of: 58 children with chronic liver
diseases, and 20 healthy children of matched age and sex as a control group.
Diagnosis of chronic liver diseases based on clinical, biochemical,
serological and histopathological investigations. All groups included in this
study were subjected to thorough clinical examination, Laboratory
investigations, measurement of serum Cys C concentration by particle induced
immunonephlometry, imaging methods,Histopathological assessment and
statistical analysis.
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Cases of chronic liver diseases without renal impairment were 25 (group 1):
including cholestasis 9 cases, Wilson disease 4 cases, infective hepatitis 3 cases,
autoimmune hepatitis 3 cases, metabolic disease 2 cases, congenital hepatic
fibrosis 2 cases, Budd Chiari syndrome one case and malignancy was one case.
Cases of chronic liver diseases with renal impairment were 25 (group 2):
including infective hepatitis 13 cases, Budd Chiari syndrome were 5 cases,
congenital hepatic fibrosis were 4 cases and autoimmune hepatitis were 3 cases.
Regarding disease duration there were statistical highly significant relation
between chronic liver diseases with renal impairment. On other hand there was
statistically insignificant relation between chronic liver diseases without renal
impairment and after liver transplantation.
According to the age, there was significant relation with cystatin C in
chronic liver diseases with renal impairment, explained by increase the duration
of the disease
Regarding CBC results between studied groups there were statistical
significant relation between control group & chronic liver diseases with and
without renal impairment groups. Regarding to HB and platelets while there
were highly statistical significant relation between chronic Liver diseases with
renal impairment & control group regarding to RBCs, but there were no
significant correlations between studied groups regarding to WBCs.
Regarding liver function test (LFT) total, direct bilirubin, total protein,
prothrombin concentration, albumin, AST and ALT there were statistically
highly significant relation between control group & chronic liver diseases with
and without renal impairment. There was statistically insignificant relation
between control group & chronic liver diseases after liver transplantation. While
in AST and ALT; there were statistically highly significant relation between
control group & chronic liver diseases after liver transplantation.
Regarding kidney function test (KFT) urea and creatinine, there were
statistically highly significant relation between chronic liver diseases with renal
impairment & control group, while in creatinine clearance; there were statistically highly significant relation between chronic liver diseases with renal
impairment & control group (P<0.001), which showed affection of kidney
function.
Regarding Cystatin C there were highly significant relation in chronic liver
diseases with renal impairment & control group. While there was statistically
insignificant relation between chronic liver diseases without renal impairment
and after transplantation& control group.
Regarding Urea, creatinine and creatinine clearance (KFT); there was
statistically significant relation with cystatin C in chronic liver diseases with
renal impairment.
In demonstration the validity of cystatin C in detecting cases of chronic
liver disease with renal impairment the cut- off point detects renal impairment at
1.66 mg/dl with 100% sensitivity and 100% specificity, and the ROC curve is
on longitudinal and vertical axis.
There was no statistically significant relation between cystatin and
abdominal sonographic finding among liver disease patients with and without
renal impairment.
The results of GFRs were determined from renogram, Cystatin C levels and
creatinine clearance showed that cystatin C levels are closely correlated with
99Tc-DTPA renogram finding among liver disease patients with renal
impairment. The correlation between renogram with cystatin c is considerably
better than with creatinine clearance and Schwartz formula using a BlandAltman plot analysis.
Conclusions: Serum creatinine is a crude marker of GFR and the most
widely used measured of GFR. Serum creatinine typically varies slightly from
day to day, age and gender associated differences in creatinine production are
proportional to muscle mass and creatinine generation can vary significantly in
a given individual over time when muscle mass changes. It does not increase
until renal function decrease up to 50%.
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Renogram, a radionuclide technique which using a complex of
99mTc-DTPA for quantification of GFR, is desired in association with renal
imaging. This method is most useful in patients whom the urine collection is
difficult, poorly cooperative patients, children and the ability to asses a single
kidney GFR and do not require urine collection.
This study has shown that a better correlation between renogram with
Cystatin-C than with serum creatinine or creatinine clearance. Furthermore,
Cystatin-C would be better alternative method in case having problems to
obtain closest ideal methods for GFR determination in patients with mild and
moderate stage of chronic kidney disease.
So, Cystatin-C is a simple test that could be used for screening of normal,
early, moderate, and severe renal dysfunction in children with chronic liver
disease with or without renal impairment and after liver transplantation.