Fulminant Hepatic Failure

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This page is about the pre-ICD10 diagnosis coding schema. See the ICD10 Diagnosis List, or the following for similar diagnoses in ICD10:Liver failure w/wo hepatic encephalopathy, acute or subacute NOS, Liver failure due to toxin/drug NOS, w/wo hepatic encephalopathy, Liver failure, alcoholic, w/wo hepatic encephalopathy

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Category/Organ System:	Category: GI (old)
Type:	Category: Medical Problem (old)
Main Diagnosis:	Fulminant Hepatic Failure
Sub Diagnosis:	FULMINANT HEPATIC FAILURE
Diagnosis Code:	40400 - Fulminant Hepatic Failure 40401 - 2nd to Acetominophen OD 40402 - 2nd to veno occlusive disease post Post Bone Marrow Transplant(BMT) 40490 - 2nd to Other
Comorbid Diagnosis:	No
Charlson Comorbid coding (pre ICD10):	0
Program:	CC & Med
Status:

DEFINITION — Fulminant hepatic failure refers to the rapid development of severe liver injury resulting in impaired function and encephalopathy in a person who previously had a normal liver or had well-compensated liver disease. Several definitions of the time course for which liver failure should be considered fulminant have been proposed but are not standard:

• The development of encephalopathy within eight weeks of the onset of symptoms in a patient with a previously healthy liver.
• The appearance of encephalopathy within two weeks of developing jaundice, even in a patient with previous underlying liver dysfunction.
• Patients who have rapid deterioration of liver function with the development of encephalopathy within six months but fall outside the boundaries of the above time intervals are considered to have "subfulminant" hepatic failure.

Fulminant liver failure (FLF), is a rare condition in which rapid deterioration of liver function results in altered mentation and coagulopathy in previously normal individuals. U.S. estimates are placed at approximately 2,000 cases per year. The most prominent causes include drug-induced liver injury, viral hepatitis, autoimmune liver disease, and shock or hypoperfusion; many cases (20 percent) have no discernible cause. Fulminant liver failure often affects young persons and carries a high morbidity and mortality. Prior to transplantation, most sources suggested less than 15 percent survival. Currently, overall short-term survival with transplantation is greater than 65 percent. Because of its rarity, FLF has been difficult to study in depth and very few controlled therapy trials have been performed. As a result, standards of intensive care for this condition have not been established.

• also see: Non Fulminant Hepatic Failure