Impact of the combined low dose prednisolone regimen on liver fibrosis in children with autoimmune hepatitis who responded to treatment

Autoimmune hepatitis [AIH] is a chronic liver disease characterized histologically by interface hepatitis. Hepatic fibrosis and cirrhosis can occur in many types of chronic liver injury including AIH. Recent studies have reported that hepatic fibrosis and cirrhosis may be reversible in some patients with AIH who respond to treatment. To assess the effects of treatment on fibrosis in liver biopsies of children with AIH who responded to the combined low dose prednisolone and azathioprine regimen. Twenty children with median age 8 +/- 3.5 yrs [9 girls, 11 boys], 18 AIH type I and two AIH type II, who were in clinical and biochemical remission for at least 6 months, and who had a diagnostic and a follow-up liver biopsy were included in this study. Different histological stains were used for assessing the grade of necroinflammatory activity [HAI] and for evaluating the stage of fibrosis according to Ishak scoring system. Morphometric analysis using LeicaQ500IS image analyzer was applied on Peri's stained liver sections to assess the percentage of liver fibrosis. Data revealed significant decrease in the median HAI from 8.85 to 3.6 [p=0.001]. The median fibrosis score showed significant reduction from 3.9 to 2.4 [p=0.001] and the median fibrosis percentage decreased from 28.7 to 12.8 [p=0.001]. These data provide evidence for regression of fibrosis in AIH in children who responded to the combined low dose immunosuppressive prednisolone and azothioprine regimen. Fibrosis control is associated with regression of necroinflammatory activity, which is the main treatment component in AIH

Fulminant hepatic failure in pediatric: an Egyptian experience

Fulminant hepatic failure [FHF] is one of the most dramatic and challenging syndromes in pediatric clinical medicine. In fact it is considered to be a true medical emergency that affect multiple organs and can lead to serious damage and multi-organ failure. Assessment of this challenging syndrome in children is still in need for highlights and we try to elucidate this issue and its interrelating factors. A retrospective review study of all patients younger than 17 years who presented to the National Liver Institute with FHF over a period of two years from 2003 - 2004 were investigated. Upon presentation and every 6 hours: blood glucose, arterial blood gases, electrolyte levels, prothrombin time and hematocrit were assessed. Upon presentation and daily: serum bilirubin, AST, ALT, serum albumin, creatinine and CBC, urine analysis total serum protein were assessed. All serum samples were tested for: HAV IgM antibodies, HBs Ag, IgM-anti HBc and IgM antibodies to HEV. All samples were screened for HCV RNA in nested PCR employing primers. Special investigations like alpha fetoprotein, copper studies, blood culture, drug levels, other metabolic studies were carried out whenever indicated. Detailed clinical evaluations including history, physical signs of liver cell failure and staging of encephalopathy were done. The mean age of patients presented with FHF was 55.667 +/- 66.12 months and, the age of the non survivals is younger than survivals though it was not significant. Males were affected more than females. The onset of coma was 14.791 +/- 12.43 days from the onset of the first symptom. Hepatomegaly was present in 66.7% of all the patients and ascites was present in 66.7 of them. Nearly 66.7% of the patients got bleeding tendency. 18/24 died while only six patients had survived with mortality rate of 75%. AST levels in these patients is higher on admission and on discharge than ALT, however, the rate of descent of ALT was higher than that of AST. Prothrombin time on admission was 39.6 sec +/- 18.36] and on discharge was 18.88. The mean level of total serum bilirubin on admission was 19.39 +/- 13.93 mg/dl, it reached a peak level of 38.5 +/- 15.592 mg/dl. On discharge it became 23.42 +/- 14.08 mg/dl. Regarding the etiologies in those patients, viral etiology was the main cause and hepatitis A virus was the commonest virus, among non survivals 44.4% of the patients got acute viral hepatitis. Others causes were discussed. None of the survivals had developed ascites. Encephalopathy was significantly present among non survivals, also, grades of coma tend to be significantly severer among them. Patients who did not survive tend to have leuckocytosis and anemia. They also had low pH and low serum sodium level. Mean total serum bilirubin, mean direct serum bilirubin [on admission and on discharge] and the peak level of both [total serum bilirubin and direct serum bilirubin] were significantly higher among nonsurvivals those patients who survived had got significantly lower level of prothrombin time on admission and on discharge. They also got significantly lower rate of descent of prothrombin time than the non-survivals. Regarding the etiologies in FHF hepatitis A virus was the commonest. The peak level of total serum bilirubin and the rate of change of the prothrombin time/day were found to be significant predictors of mortality in FHF