الفهرس 
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Abstract
SUMMARY
he aim of the present study is to search for associated
congenital anomalies in pediatric patients with chronic
liver disease and to correlate these anomalies with the severity
of the disease.
This study was carried out to evaluate the effect of
associated anomalies on the severity of the chronic liver disease
in children and adolescents with chronic liver disease in the
Hepatology Clinic Children’s Hospital– Ain Shams University.
Fifty patients of children and adolescents following up at
the Pediatric Hepatology Clinic Children’s Hospital - Ain
Shams University in the period from December 2012 to
December 2013 they comprised 23 females and 27 males, the
mean age was 9.94+4.84year.
The current study included 11 patients (22%) had
metabolic causes (3Glycogen storage diseases, 3 Wilson
disease and 5 Nieman pick diseases), 12 patients (24%) had
congenital hepatic fibrosis, 5 patient (10%) had Autoimmune
hepatitis, 1 patient (2%) had hepatitis C, 2 patient (4%) had
Alagille syndrome, 5 patient (10%) had budd chiari syndrome,
2 patient (4%) had caroli syndrome, 7 patient (14%) billiary
atrasia, 3 patient (6%) had criggler nejar syndrome, paucity
intra hepatic bile duct and alpha one anti trypsin deficiency and
2 patient (4%) un diagnosed.
T
Summary
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Fourteen patients (28%) showed history of jaundice, 12
patients (28%) showed history of hematemesis, 46 patients
(92%) showed history of fever, 6 patients (12%) showed
symptoms of hepatic cell failure, 3 patients (6%) showed
symptoms of hepatic encephalopathy, 4 patients (8%) showed
symptoms of billiary obstruction.
On radiology 45 patients (90%) showed abnormal sonar,
48 patients (96%) showed abnormal ct abdomen, 3 patients
(6%) showed abnormal CT brain and 14 patients (28%) showed
abnormal echo.
In conclusion, 19 patients (38%) showed congenital
anomalies in heart, abdomen or brain.
Child score are 40 patients (80%) are good, 8 patients
(16%) are fair and 2 patients (4%) are bad.