الفهرس | Only 14 pages are availabe for public view |
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 138 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. |