Acute liver failure in children: Management
- Robert H Squires, Jr, MD, FAAP
Robert H Squires, Jr, MD, FAAP
- Professor of Pediatrics
- University of Pittsburgh
- Section Editors
- Elizabeth B Rand, MD
Elizabeth B Rand, MD
- Section Editor — Pediatric Hepatology
- Professor of Pediatrics
- University of Pennsylvania School of Medicine
- Adrienne G Randolph, MD, MSc
Adrienne G Randolph, MD, MSc
- Section Editor — Pediatric Critical Care Medicine
- Professor of Anaesthesia and Pediatrics
- Harvard Medical School
Pediatric acute liver failure (PALF) is a complex, rapidly progressive clinical syndrome that is the final common pathway for many disparate conditions, some known and others yet to be identified [1-3]. The estimated frequency of acute liver failure (ALF) in all age groups in the United States is about 17 cases per 100,000 population per year, but the frequency in children is unknown. PALF accounts for 10 to 15 percent of pediatric liver transplants performed in the United States annually.
PALF is a rapidly evolving clinical condition. There are no adequately powered studies to inform diagnostic algorithms, to assess markers of disease severity and trajectory, and to guide decisions about liver transplant. The clinician must construct an individualized diagnostic approach and management strategy. Management requires a multidisciplinary team involving the hepatologist, critical care specialist, and liver transplant surgeon.
Management of PALF and its complications in children are discussed here. An organized approach to diagnosing the cause of PALF is presented separately. (See "Acute liver failure in children: Etiology and evaluation".)
ALF in adults is addressed in separate reviews. (See "Acute liver failure in adults: Etiology, clinical manifestations, and diagnosis" and "Acute liver failure in adults: Management and prognosis".)
GENERAL MANAGEMENT PRINCIPLES
After the initial characterization of the patient presentation, proper patient management needs to be conducted along multiple parallel paths [2,3]:
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- GENERAL MANAGEMENT PRINCIPLES
- Clinical setting
- Laboratory monitoring
- CENTRAL NERVOUS SYSTEM
- Cerebral edema
- Aplastic anemia
- Bowel management
- LIVER SUPPORT
- DISEASE SEVERITY ASSESSMENT
- LIVER TRANSPLANT
- Liver transplant decisions
- Organ allocation in acute liver failure
- Types of grafts
- Hepatocyte transplant
- SUMMARY AND RECOMMENDATIONS