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Published on: September 13, 2014
Corticosteroid Therapy for Indeterminate Pediatric Acute Liver Failure and Aplastic Anemia with Acute Hepatitis
Catherine A Chapin1, Simon P Horslen2, James E Squires3
1Department of Pediatrics, Ann and Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL.
Insights
Corticosteroid treatment showed improvement in pediatric patients with indeterminate pediatric acute liver failure (IND-PALF) and aplastic anemia with acute hepatitis. However, outcomes may not differ from historical data, warranting further research.
Area of Science:
- Pediatric Hepatology
- Immunology
- Hematology
Background:
- Indeterminate pediatric acute liver failure (IND-PALF) and aplastic anemia with acute hepatitis are severe conditions often linked to immune dysregulation.
- Corticosteroids are frequently used to manage presumed immune-mediated liver injury in children.
Purpose of the Study:
- To evaluate the characteristics and outcomes of pediatric patients diagnosed with IND-PALF and aplastic anemia with acute hepatitis treated with corticosteroids.
- To assess the efficacy and safety of corticosteroid therapy in this specific patient population.
Main Methods:
- A retrospective multicenter study was conducted involving patients aged 1-17 years diagnosed between 2009 and 2018.
- Patients received varying doses of intravenous methylprednisolone for presumed immune dysregulation.
Main Results:
- Of 28 IND-PALF patients, 50% underwent liver transplantation, 46% recovered with their native liver, and 1 died. Patients recovering with native liver required prolonged corticosteroid therapy.
- All 6 patients with aplastic anemia with acute hepatitis recovered with their native liver following corticosteroid treatment.
- Serious infections occurred in 1 IND-PALF patient and 2 patients with aplastic anemia within 90 days of treatment initiation.
Conclusions:
- Corticosteroid therapy demonstrated improvement in some pediatric patients with IND-PALF and aplastic anemia with acute hepatitis.
- Native liver survival rates may not significantly differ from historical data, suggesting a need for cautious interpretation of these results.
- A randomized controlled trial is recommended to definitively establish the benefits and risks of steroid therapy before widespread adoption.
Objective:
To examine the characteristics and outcomes of a multicenter patient cohort with indeterminate pediatric acute liver failure (IND-PALF) and with aplastic anemia with acute hepatitis treated with corticosteroids.
Study Design:
Retrospective study of patients age 1-17 years with IND-PALF and aplastic anemia with acute hepatitis who presented between 2009 and 2018 to 1 of 4 institutions and were treated with corticosteroids for presumed immune dysregulation.
Results:
Of 28 patients with IND-PALF (median of 4.0 years of age [range 1-16] and 71% male) 71% (n = 20) were treated with 0.5-4 mg/kg/day of intravenous methylprednisolone, and 8 patients received 10 mg/kg/day followed by a taper. By 21 days postcorticosteroid initiation, 14 patients (50%) underwent liver transplantation, 13 patients (46%) recovered with their native liver, and 1 patient (4%) died. Patients who recovered with their native liver received a median of 139 days (range 19-749) of corticosteroid therapy, with a median of 12 days (range 1-240) to international normalized ratio ≤1.2. Patients with aplastic anemia with acute hepatitis (n = 6; median of 9.5 years of age [range 1-12], 83% male), received 1-2 mg/kg/day of methylprednisolone for a median of 100 days (range 63-183), and all recovered with their native liver. One patient with IND-PALF and 2 patients with aplastic anemia with acute hepatitis developed a serious infection within 90 days postcorticosteroid initiation.
Conclusions:
Many patients with IND-PALF or aplastic anemia with acute hepatitis that were treated with corticosteroids improved, but survival with native liver may not be different from historical reports. A randomized controlled trial exploring the benefits and risks of steroid therapy is needed before it is adopted broadly.
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