Evaluation of transforming growth factor alpha [TGFn] and tumor necrosis factor alpha [TGFn] in children with acute hepatitis

The present study included 60 children who were recruited from Pediatric Department of National Liver Institute [NLI], Menoufiya University and 20 apparently healthy children from the relatives of patients as a control group. Their ages ranged from 2 to 15 years, they were 49 males and 31 females. The studied children were divided according to their diagnosis into groups each of 20; Group [I] acute hepatitis, Group [II] acute fulminant hepatitis [F. H], Group [III] chronic hepatitis and Group [IV] control group. The mean level of both transforming growth factor alpha [TGF I] and tumor necrosis factor alpha [TNF I] were higher in group II [32.74 +/- 9.98 pg/mL and 116.41 +/- 49.70 pg/ml, respectively] more than the other groups and the difference was statistically highly significant [[P<0.001] and [<0.05], respectively]. The mean of level of [TGFD in FH survivors was [29.23 +/- 5.51pg/mL] compared to deceased ones [20.93 +/- 4.60 pg/mL] but the difference was statistically insignificant [p>0.05]. On the other hand the mean of [TNFI] in the deceased cases was 132.76 +/- 48.81pg/mL compared to 117.93 +/- 19.15pg/mL in survivors but the difference was statistically insignificant [P > 0.05]. Case fatality rate for all FH cases was 80%. In conclusion, the levels of both TGFI and TNFI are significantly higher in fulminant hepatitis more than acute hepatitis cases but with no significance in the progress of the disease. Further studies is recommended to uncover predictors that can differentiate between acute hepatitis and fulminant hepatitis and the progress of the disease

Living related liver transplantation pediatric patients [a single center study]

This study included all pediatric cases that had done Living related liver transplantation [LRLT] in National Liver Institute [NLI], Menoufiya University from April 2003 to August 2007; they were 15 children, 8 were males and 7 were females. Their ages ranged between 1 year and 14 with a mean of 6 +/- 5.5 years. The indications for LRLT were Biliary atresia [BA]: 6 cases [40%], Budd Chiari syndrome [BCS]: 3 cases [20%], Bylers disease; 2 cases [13.3%], congenital hepatic fibrosis [CHF]: 2 cases [13.3%] [cirrhosis due to hepatitis C [HCV]: one case [6.7%] and hepatoplastoma: one case [6.7%]. The mean of follow-up was 30 +/- 20 months [range: 1-52 months]. The mean of pediatric end stage liver disease [PELD] score in deceased patients [20 +/- 11] was higher than the survivors [9 +/- 8] [P<0.05]. Seven mortalities [46.7%] occurred and the main causes of death were sepsis, vascular complications and chronic rejection, The over all patient survival in six month, first and second year were 73.3%, 667% and 53.3%, respectively The best first year patient survival was in BA cases [100%] while the worst was in BCS patients [33.3%]. In conclusion, LRLT is a successful therapy for infants and children with end stage liver diseases. It seems that the primary liver disease has no impact on the early results of LRLT except BCS where the cause of early mortalities where related to the recurrence of the disease. Implementation of education program for donors [the family] and candidates for LRLT is necessary to fulfill the criteria required for successful LRLT. Appropriate timing, meticulous surgical procedures, and comprehensive management of complications are crucial for successful outcome with LRLT

Evaluation of autoimmune scoring system in autoantibodies seropostive and seronegative children with autoimmune hepatitis

The scoring system of autoimmune hepatitis that was defined by the International Autoimmune Hepatitis Group [IAHG] in 1999 was applied on thirty seven children with autoimmune hepatitis [AIH] from the attendants of Pediatric Department of National Liver Institute [NLI], Menofiya University. Thirty three cases [89.2%] were seropositive to one or more of conventional autoimmune antibodies of AIH [antinuclear antibodies [ANA], smooth muscle antibodies [SMA], and antibodies to liver / kidney microsome type 1 [anti-LKM-1]] and 4 cases [10.8%] were seronegative. No statistically significant difference were found between the two groups as regards clinical presentations, the results of liver function tests, histopathological findings, hypergammopathy mean of scoring system before and after treatment as well as the mean of scoring system after response and / or relapse after treatment. These results point to the reliability of using the autoimmune scoring system to uncover cases of autoimmune hepatitis in patients seronegative for autoimmune markers in children

Billiary atresia cases among attendants of Menoufiya National Liver Institute: [a retrospective study]

This study included 126 cases of neonatal cholestasis syndrome [NCS] from the attendants of Pediatric Department of the National Liver Institute, Menoufiya University from 1994 up to 2004 . They were divided into two groups: First group[1stGr.] included 58 biliary atresia [BA] cases [46%] and the second group [2ndGr.] included 68 [54%] were due to other causes of NCS . The etiology of NCS due to causes other than BA were as follows: 28 cases [22.22%] neonatal hepatitis, 11 cases [8.73%] septicemia, 8 cases [6.35%] paucity of intrahepatic bile ducts, 5 cases [3.97%] inspessated bile syndrome, 4 cases [3.17%] choledochal cyst, 2 cases [1.59%] Byler's disease, 2 cases [1.59%] galactosemia, 2 cases ? 1 antitrypsin deficiency [1.59%], 1 case [0.79%] Alagille syndrome, and 5 cases [3.97%] due to unknown causes. Onset of jaundice whether early or late does not differentiate cholestatic cases due to BA from other causes of NCS.Clay coloured [acholic] stools were more frequently detected in BA cases [77.6%] than 2ndGr. [27.9%] [p<0.05]. Triangular cord sign [Tc sign] and absence of and/or gall bladder abnormality were detected in significantly higher proportion of BA group more than the 2ndGr.[p<0.05]. By histopathological examination portal tract fibrosis, bile duct proliferation, bile plugs in portal ductules and preservation of hepatic lobular architecture were detected more frequently among BA cases than the other group, while interface hepatitis and giant cell detection were observed more frequently among 2ndGr. than BA cases [p < 0.05]. The mean of alkaline phosphatase and gamma glutamyl transpeptidase was found to be statistically higher among BA group than that of the 2ndGr. [p<0.05] . Kasai operation was done for only 20 cases of BA cases [34.5%] and the mean of age of patients at time of operation was 75 days +/- 17.8. Complications encountered in BA cases were recurrent cholangitis, ascites, itching, coagulopathy, hematemesis and end-stage liver failure in 48%, 50%, 41.7%, 39.6, 18.8% and 54.2% of cases respectively. In conclution, the results of the present study indicate that clinical evaluation by an experienced pediatric hepatologist and liver biopsy together with careful ultrasonographic evaluation are considered as the most reliable methods for early differentiation of BA from other causes of neonatal cholestasis. Management of BA cases will be improved by public and professional education to encourage early referral of infants with neonatal cholestasis [>14 days] to specilised liver centers for early diagnosis to facilitate initial surgery before 8 weeks of age