Correlation of the oxygendissociation curves of adult and fetal hemoglobin solutions with the H + ion concentration gradients of adult and fetal red blood cells

Abstract

children has any history of hemorrhagic tendencies, a prolonged whole-blood clotting time in an l l -year-old girl led to the findings of a markedIy prolonged partial thromboplastin time ( P T T ) , abnormal thromboplastin generation test (TGT) , and a normal prothrombin time (PT) in the patient and in 3 of her 10 siblings. The abnormal PTT and T G T were corrected by aluminum hydroxide adsorbed fresh plasma and by serum. Using the kaot in-PTT system, equal mixtures of plasma from the patients and normal plasma produced a normal time. In addition, plasmas deficient in plasma thromboplastin antecedent (PTA) , Hageman factor (HF) , antihemophilic factor (AHF) , or plasma thromboplastin component (PTC) corrected the abnormality. Specific assays of the patients' plasmas (confirmed by two other coagulation laboratories) for fibrinogen (I) , prothrombin ( I I ) , proaccelerin (V), AHF (VI I I ) , PTC ( IX) , Stuart factor (X) , PTA (XI ) , HF ( X I I ) , and anticoagulant activity were all within normal limits. Physical and chemical properties of plasma correcting the defect in vitro ( temperature and storage stability, glass activation, chemical adsorption, and chemical fraetionation) indicate that the defeet is closely related to that found in PTAand HF-deficient plasma. These findings suggest the existence of a previously undeseribed coagulation factor which is similar to the known glass contact factors, PTA and HF.