Detailed coagulation studies were done prospectively on 43 patients with
biliary atresia who had undergone Kasai operation (
hepatic portoenterostomy). Patients were divided into three groups based on levels of
factor V,
factor II, and Echis II and/or response to
vitamin K: no coagulopathy (46.5% of patients); coagulopathy of
liver disease (30.2% of patients); and coagulopathy of
vitamin K deficiency (23.3% of patients). Patients with the coagulopathy of
liver disease had significantly lower levels of factors XII, V, and
antithrombin III as well as longer thrombin times than patients with no coagulopathy or
vitamin K deficiency.
Factor V levels were decreased only in patients with more advanced
liver disease; normal levels of
factor V were not usually helpful in differentiating
liver disease and
vitamin K deficiency. The prothrombin time,
factor VII-X levels, and
factor II levels were significantly different for all three groups; the most abnormal values occurred in the
vitamin K-deficient group. Comparison of the Echis II level to
factor II coagulant activity was helpful in deciding whether a coagulopathy was due to
liver disease,
vitamin K deficiency, or both.
Factor VIII levels were elevated in all groups.
Factor VIII coagulant activity was significantly higher by the two-stage (TGT) method than by the one-stage (PTT) method.
Hypersplenism causing
neutropenia and
thrombocytopenia was commonly seen after the age of 5 years.
Vitamin E deficiency was more common than
vitamin K deficiency; however, all
vitamin K-deficient patients were
vitamin E deficient. Coagulation status correlated well with hepatobiliary scan data, but not serum
bilirubin levels. Recommendations for treatment of patients with
vitamin K deficiency and/or
liver disease are discussed.