Abstract

Background: Acute liver failure (ALF) is a dramatic event. 5% of pediatric cases are determined by autoimmune hepatitis (AIH). When AIH does not respond to immunosuppressive treatment, liver transplantation remains the only therapeutic option. However, after transplantation AIH may recur in 20% of patients.Aim: To report the third pediatric case of ALF due to AIH successfully treated with auxiliary liver transplantation (ALT).Case report and results: A 5-year-old girl was referred to our Center of Pediatric Hepatology for the sudden onset (<7 days) of jaundice and lethargy. Elevated liver enzymes (AST 999 U/L, ALT 1583 U/L), severe coagulopathy (international normalized ratio 3.3) and hyperammoniemia (158 μmol/L) were present at admission. High titers of anti-nuclear and anti-smooth muscle antibodies were retrieved suggesting the presence of type 1 AIH. Despite a prompt steroid treatment, the girl showed a progressive neurological deterioration leading to hepatic coma in less than 24 h after admission. Cerebral edema/hemorrhages were excluded by neurological imaging. 72 h after admission the girl underwent a native left hepatectomy and received a left auxiliary graft implanted orthotopically. Histology of the explanted left lobe confirmed the diagnosis of AIH. Immunosuppression was based on tacrolimus, azathioprine and steroids. The girl showed a complete neurological recovery in the first week after ALT. Liver tests normalized 3 months after transplant. Liver biopsies and volumetric CT scans, performed at 1 and 6 months after ALT, respectively demonstrated a complete remission of AIH and a full volume recovery of the right native lobe. Steroid treatment was interrupted 6 months after ALT. Gradual withdrawal of tacrolimus was started 7 months after ALT. No significant complications have been observed at 10 months of follow up.Conclusions: ALT is an option for the treatment of ALF when the chances for the native liver to recovery are high but temporary support is critical for survival. Less than 50 cases of ALT have been reported in children with ALF so far; the main indications consisted of seronegative non-A- non-G hepatitis, drug toxicity and mushroom poisoning. We have described the third pediatric case of AIH treated by ALT with rapid restoration of liver function and reversal of hepatic encephalopathy. ALT should be always considered as a therapeutic option for AIH in the setting of a rapidly deteriorating liver function despite maximal medical treatment. Background: Acute liver failure (ALF) is a dramatic event. 5% of pediatric cases are determined by autoimmune hepatitis (AIH). When AIH does not respond to immunosuppressive treatment, liver transplantation remains the only therapeutic option. However, after transplantation AIH may recur in 20% of patients. Aim: To report the third pediatric case of ALF due to AIH successfully treated with auxiliary liver transplantation (ALT). Case report and results: A 5-year-old girl was referred to our Center of Pediatric Hepatology for the sudden onset (<7 days) of jaundice and lethargy. Elevated liver enzymes (AST 999 U/L, ALT 1583 U/L), severe coagulopathy (international normalized ratio 3.3) and hyperammoniemia (158 μmol/L) were present at admission. High titers of anti-nuclear and anti-smooth muscle antibodies were retrieved suggesting the presence of type 1 AIH. Despite a prompt steroid treatment, the girl showed a progressive neurological deterioration leading to hepatic coma in less than 24 h after admission. Cerebral edema/hemorrhages were excluded by neurological imaging. 72 h after admission the girl underwent a native left hepatectomy and received a left auxiliary graft implanted orthotopically. Histology of the explanted left lobe confirmed the diagnosis of AIH. Immunosuppression was based on tacrolimus, azathioprine and steroids. The girl showed a complete neurological recovery in the first week after ALT. Liver tests normalized 3 months after transplant. Liver biopsies and volumetric CT scans, performed at 1 and 6 months after ALT, respectively demonstrated a complete remission of AIH and a full volume recovery of the right native lobe. Steroid treatment was interrupted 6 months after ALT. Gradual withdrawal of tacrolimus was started 7 months after ALT. No significant complications have been observed at 10 months of follow up. Conclusions: ALT is an option for the treatment of ALF when the chances for the native liver to recovery are high but temporary support is critical for survival. Less than 50 cases of ALT have been reported in children with ALF so far; the main indications consisted of seronegative non-A- non-G hepatitis, drug toxicity and mushroom poisoning. We have described the third pediatric case of AIH treated by ALT with rapid restoration of liver function and reversal of hepatic encephalopathy. ALT should be always considered as a therapeutic option for AIH in the setting of a rapidly deteriorating liver function despite maximal medical treatment.

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