Liver Disease

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Chapter 475 Liver Disease

J. Paul Scott, Robert R. Montgomery

Because all of the clotting factors except factor VIII are produced exclusively in the liver, coagulation abnormalities are very common in patients with severe liver disease. Only 15% of such patients have significant clinical bleeding states. The severity of the coagulation abnormality appears to be directly proportional to the extent of hepatocellular damage. The most common mechanism causing the defect is decreased synthesis of coagulation factors. Patients with severe liver disease characteristically have normal to increased (not reduced) levels of factor VIII activity in plasma. In some instances, disseminated intravascular coagulation (Chapter 477) or hyperfibrinolysis may complicate liver disease, making laboratory differentiation of severe liver disease from disseminated intravascular coagulation difficult.

Treatment of the coagulopathy of liver disease consists of replacement with fresh frozen plasma (FFP) or cryoprecipitate. FFP (10-15 mL/kg) contains all clotting factors, but replacement of fibrinogen for severe hypofibrinogenemia may require cryoprecipitate at a dose of 1 bag/5 kg body weight. Because a reduction in vitamin K–dependent coagulation factors is common in those with acute or chronic liver disease, vitamin K therapy can be given as a trial. Vitamin K can be given orally, subcutaneously, or IV (not IM) at a dose of 1 mg/24 hr for infants, 2-3 mg/24 hr for children, and 5-10 mg/24 hr for adolescents and adults. Inability to correct coagulopathy with vitamin K indicates that the coagulopathy may be caused by reduced levels of clotting factors that are not vitamin K–dependent and/or by inadequate production of precursor vitamin K proteins. In severe liver disease, it is often difficult to attain correction of abnormal clotting studies despite vigorous therapy with FFP and cryoprecipitate. Some patients with bleeding due to liver disease have responded to therapy with desmopressin, and others have responded to treatment with recombinant factor VIIa.

Frequently, severe liver disease is associated with moderate prolongation of bleeding time that is not corrected by either vitamin K or plasma replacement. Desmopressin (0.3 µg/kg IV) has been found to be effective in shortening bleeding time and has been used effectively to augment hemostasis before liver biopsy. In clinical trials of adults, recombinant factor VIIa has not been shown to be effective for the treatment of bleeding caused by severe liver disease.

Atkison PR, Jardine L, Williams S, et al. Use of recombinant factor VIIa in pediatric patients with liver failure and severe coagulopathy. Transplant Proc. 2005;37:1091-1093.

Kaul V, Munoz SJ. Coagulopathy of liver disease. Curr Treat Options Gastroenterol. 2000;3:433-438.

Monagle P, Andrew M. Acquired disorders of hemostasis. In: Nathan DG, Orkin SH, Ginsburg D, et al, editors. Hematology of infancy and childhood. ed 6. Philadelphia: WB Saunders; 2003:1631-1668.

Pipe SW, Goldenberg NA. Acquired hemostatic defects. In: Orkin SH, Nathan DG, Ginsberg D, et al, editors. Nathan and Oski’s hematology of infancy and childhood. ed 7. Philadelphia: Saunders Elsevier; 2009:1591-1622.

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