We report the case of a middle-aged man who died from multiorgan failure 3 weeks after orthotopic liver transplant for fulminant hepatic failure, associated with a rare, often fatal, hematologic condition that usually presents in childhood. We discuss the importance of its diagnosis, treatment, and implications for liver transplant.

Acute fulminant liver failure and acute renal failure are devastating complications caused by many drugs. The use of N-acetylcysteine has been well established in acetaminophen toxicity, but it remains controversial in other cases. Dialysis is a very effective method of removing certain drugs from the system. With the invention of new street drugs such as "synthetic marijuana," it may be benefi...

Fulminant hepatic failure (FHF) is defined as a syndrome of acute liver failure with the development of hepatic encephalopathy and severe hypoprothrombinemia occurring within 2 months of the onset of symptoms or jaundice in a person without preexisting liver disease. Total orthotopic liver transplantation (OLTX) is a lifesaving therapeutic option for patients with FHF, but currently requires li...

The etiology and prognosis of individuals with various forms of fulminant hepatic failure are reviewed. Special techniques of clinical management and decision making as to when and to whom to transplant in cases of fulminant hepatic failure are reviewed.

In 25 patients with fulminant hepatic failure the prognostic value of a quantitative liver function test, the galactose elimination capacity, was assessed and comapred with routine liver function tests and clinical features. The galactose elimination capacity was significantly higher (P less than 0-05) in the five patients who survived than in the 20 patients who died. None of the other liver f...

Fulminant hepatic failure' is defined as severe, acute impairment of hepatic function culminating in hepatic encephalopathy, the encephalopathy being the result of hepatocellular failure and supervening within three weeks of the onset of symptoms*. This definition excludes the hepatic encephalopathy of hepatic cirrhosis and of chronic hepatitis, which produces a slightly different clinical pict...

The clinically reported case of liver involvement with multiple myeloma (MM) is rare. Amyloidosis, defined as a tissue deposition of clonal light-chain fibrils, has been reported in 10-15% of the MM patients. We described a rare MM patient with the primal presentation of fulminant hepatic failure and biliary system involvement due to amyloidosis. Our patient had the primal symptoms of hyperbili...

Wilson's disease is a rare but treatable condition with variable clinical presentations. Its diagnosis depends on a combination of clinical and laboratory findings. We evaluated the clinical and laboratory findings in children with Wilson's disease (WD). Twenty -seven children (4-14 years, 59.2 % male, 40.7% female) with confirmed WD were evaluated between 1994 and 2003 at Imam Khomeini Hos...

Although the incidence of fulminant hepatic failure appears to have decreased worldwide, the management of this dreaded condition remains unsatisfactory (1). Despite ingenious methods of treatment, the mortality in patients with grade IV coma is still between 80 and 90% in most major series (15, 20). Furthermore the mortality is nearly 100% in patients ov~r the age of 45 who develop grade IV co...