SummaryHemorrhage is one of the most serious complications of chronic liver disease. Deficiency of plasma coagulation factors, thrombocytopenia, and increased vascular fragility have been reported in many patients with Laennec’s cirrhosis. Factors V, VII, IX, X and prothrombin were deficient in a majority of the 25 patients included in this study.Six tests of liver function and seven of plasma coagulation factors were evaluated, using the method of statistical regression analysis. The intercorrelation of tests in each group was evaluated as well as the correlation between liver function tests and plasma coagulation factors. Fibrinogen deficiency was not felt to play a significant role in hemorrhagic diathesis in our cases.

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High fat diets were given ad libitum or force-fed to rats. The incidence of thrombosis in animals fed a cow butter diet was higher than in those fed a cocoa butter diet. No thrombosis was found in rats fed a diet containing peanut oil. Coagulation studies performed at weekly intervals on blood samples obtained from a tail vein of rats fed the diets ad libitum showed that there was a gradual but marked increase in the levels of Factors II, VII, and X of animals fed a diet containing cow or cocoa butter, but not one containing peanut oil. Several weeks prior to death, however, the level of all coagulation factors, including Factors I and V, fell. A significant prolongation of the values for the partial thromboplastin time was found in animals killed at the time when levels of plasma coagulation factors decreased. The pattern of changes in concentration of plasma coagulation factors was similar in the groups of animals dying with proven thrombosis and in those in which thrombosis was not found. All rats force-fed the above two diets died within two weeks, showing a marked fall in all coagulation factors and no thrombosis. Weight loss was more pronounced in the groups of animals fed the diet containing cow butter and cocoa butter than in animals fed Purina chow. Autopsy findings were: intra-ventricular thrombi with myocardial infarction, lung hemorrhage, pneumonia, and rarely jaundice. No cause of death other than inanition was discernible in some of the animals. Marked fatty infiltration of the liver was a feature of all animals autopsied.

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The case of a four year old white girl with a bleeding tendency characterized by the spontaneous occurrence of petechiae, ecchymoses and hemarthroses is presented. Laboratory studies indicate a deficiency of factor VII, with normal levels of all other clotting components, and a vascular deficiency possibly related to frequent upper respiratory infection. Serum from the patient and from four close relatives having reduced levels of factor VII is shown to correct the abnormal thromboplastin generation of Stuart factor-deficient serum. The rate of conversion of prothrombin into thrombin is markedly delayed in the patient’s plasma but essentially normal in that of other family members. The patient’s prothrombin conversion is corrected by the addition of normal or Stuart-deficient plasma. The partial thromboplastin time of the patient is somewhat prolonged. It is concluded that factor VII deficiency is inherited in this family as an incompletely recessive autosomal characteristic. The penetrance of the deficiency allele in the heterozygous individual appears to be variable. Analyses of the distributions of clotting factor levels in the family and in samples from the normal population indicate that an individual may vary greatly in his clotting factor levels, the average variation being nearly as great as the variation among individuals. The magnitude of the variation among siblings suggests that the penetrance of the factor VII deficiency allele is affected by extrinsic factors and that the observed population variation does not result from the occurrence of multiple alleles. It is suggested that sex may be a factor determining clotting factor levels. The bleeding of the patient is controlled by transfusion of plasma and by injection of a plasma preparation called ACC-76 (Behringwerke, Germany), the latter followed by a transient elevation of blood factor VII.