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

Living related liver transplantation impact on nutritional status of children with chronic liver disease

Chronic liver diseases in infants and children are often associated with poor growth, protein calorie malnutrition and vitamin deficiencies. Although liver transplantation [LTx] has become standard therapy for end stage liver disease in children, growth after living related liver transplantation [LRLT] remains an area of concern, and the present study tries to elucidate this issue. The children included in this study classified into: Transplant group: [Group 1], Awaiting group [Group 2] and Control group [Group 3]. Those patients were assessed by thorough history, anthropometrical measures and complete clinical examination. Also assessed for post transplant infections, graft dysfunction and immunosuppressive regimen. Food intake was registered [24 hour recall] and caloric requirement was calculated according to the Egyptian food composition table. The mean height, weight, [MAC] and caloric intake were significantly lower in the pre transplant cases when compared to the post transplant. The Z-score in the 8 patients included in the transplant group show improvement in height, weight, MAC and caloric intake. The mean height, weight, MAC and caloric intake in the awaiting group were lower after one year follow up than during the preparation for transplant. The Z-score in the 15 patients included in the awaiting group show shift to the more negative side in height, weight, MAC and caloric intake. The mean levels of albumin, Fe, Zn and Se were elevated significantly in the post transplant group when compared to the pre-transplant cases. The mean levels of albumin, Fe, Zn and Se were low in the awaiting group when compared to the control group. Persistent growth failure post transplantation is multifactorial and is related to the preoperative malnutrition, feeding problems and post operative complications. Liver diseases may compromise growth and nutritional status. An improvement occurred in many parameters of growth and nutritional status after LRLT especially MAC and caloric intake

Hepatic involvement in common neonatal problems

Hepatic involvement has been reported to occur in the common neonatal problems as neonatal asphyxia, neonatal sepsis, and neonatal jaundice. However the exact type of involvement, incidence of occurrence, risk factors associated in and the impact outcome are not exactly known. To identify the risk factors, the incidence, and the types of hepatic involvement in the common neonatal problems and to determine the correlation between the types of hepatic involvement and the outcome of all cases. We studied 3 groups of neonates:- group with perinatal asphyxia, group with neonatal sepsis and group with neonatal jaundice and all the 3 groups were subjected to complete perinatal history, clinical examination, and some investigations for the diagnosis of the neonatal affections according to the clinical condition but all groups were subjected to liver function tests at admission and repeated according to clinical indications and hepatic involvement. Only the patients who showed severe hepatic affection will be subjected to liver biopsy. There was hepatic involvement in 20.5% in septic cases [transient in 87.5% and persistent in 12.5%]. There was hepatic involvement in 18% of cases with perinatal asphyxia [40% transient and 60% persistent]. There was hepatic involvement in 16% of cases with neonatal jaundice [80% transient and 20% persistent]. Three newborns with persistent hepatic affection were subjected to liver biopsy which showed galactosemia in one case, neonatal hepatitis syndrome in other case and biliary atresia in the third case