The multiple coagulopathies of biliary atresia

Abstract

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.

Prevention of Vitamin K Deficiency Bleeding in Breastfed Infants: Lessons From the Dutch and Danish Biliary Atresia Registries

“…Moreover, liver dysfunction in biliary atresia is unlikely to cause a PR of Ͼ1.5 during the first months of life. 24 Similarly, a "physiologic" PR of Ͼ1.5 is rare after the first week of life. 22,23 We also analyzed the data using a higher cutoff value of the PR (Ͼ2), but the results were essentially identical ( Table 4).…”

“…22,23 Significant PR elevations in otherwise healthy biliary atresia infants are unlikely to be because of other causes. 24 A PR of Ͼ4 was designated as "severe" VKD. VKDB was defined as bruising, bleeding, or intracranial hemorrhage in combination with a PR of Ͼ4 in any infant between 8 days and 6 months of age and normalizing after administration of vitamin K. 2 The number of bleedings and their locations were noted.…”

Prevalence of Vitamin K Deficiency in Children with Mild to Moderate Chronic Liver Disease

“…Breast feeding without vitamin K supplementation places infants at risk for late-onset hemorrhagic disease of the newborn (10,17,37,38). Several studies suggest clinical and subclinical vitamin K deficiency is prevalent in patients with chronic liver disease (39)(40)(41)(42). However, these studies may have underestimated the prevalence of deficiency because they used less sensitive markers of vitamin K status: PT and plasma levels of phylloquinone (39)(40)(41)(42).…”

Anaesthesia, perioperative management and outcome of correction of extrahepatic biliary atresia in the infant: a review of 50 cases in the King's College Hospital series

“…Vitamin K 1.0 mg is administered for at least 4 days i.m. (45). Oral neomycin (50 mg·kg –1 ) is given for 24 h at 4‐h intervals.…”