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Results for “Factor VII Deficiency”

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PubMed1988

Factor VII activity and antigen in a patient with abnormal factor VII.

A patient with abnormal factor VII, which showed different activity when thromboplastin from different sources was used, is reported. The propositus, who was first seen at routine health examination 1 month after delivery, was a girl with no complaints of bleeding. The coagulation pattern was characterized by an abnormal clotting time using Normotest reagent which did not respond to the administration of vitamin K. Factor VII activity was decreased when measured using rabbit brain and lung thromboplastin, mildly decreased using human brain or human placenta thromboplastin and within the normal range using ox brain thromboplastin. The level of factor VII antigen was normal and revealed a normal mobility on immunoelectrophoresis. The molecular weight of this factor VII was not different from normal factor VII when analysed using autoradiography after sodium dodecyl sulphate-polyacrylamide gel electrophoresis (SDS-PAGE). It seems probable that the propositus had an abnormality similar to the factor VII Padua 1 described previously.

Antigens↗
PubMed1993

Adenotonsillectomy in patients with inherited bleeding disorders.

We describe the management of tonsil or tonsil and adenoid surgery in our hospital in 10 patients with inherited bleeding disorders, over a 10-year-period. The approach to the management of the haemostatic defect is outlined in detail. All underwent successful surgery, two patients had limited secondary haemorrhage. This report demonstrates that patients with inherited bleeding disorders can safely undergo adenotonsillectomy, providing there is close liaison between surgeons and haematologists throughout the peri-operative period.

Adenoidectomy↗
PubMed1983

Morphological and biochemical confirmation of gray platelet syndrome in two siblings.

A case report is presented of gray platelet syndrome in siblings. The absence of platelet alpha-granules in these patients was confirmed by electron microscopy and by analysis of the platelet protein profile using SDS-polyacrylamide gel electrophoresis. This was further confirmed by quantitation of individual alpha-granule protein constituents. The results in these two patients are compared with those of the four reported patients with this syndrome.

Adult↗
PubMedDate not supplied

Mesenteric vein thrombosis. Four cases and review of the literature.

Mesenteric vein thrombosis, an uncommon but important clinical entity, can cause ischemia or infarction of the small intestine. Mesenteric vein thrombosis was first described nearly a century ago, but diagnosis remains difficult because it can affect young individuals without any known predisposing disorder and because patients often present with nonspecific abdominal symptoms. We report 4 cases of small intestinal ischemia secondary to superior mesenteric vein thrombosis. Three were due to hypercoagulable states (protein-S deficiency, factor-VII abnormalities) and one was idiopathic. In recent years, the development of modern imaging techniques (particularly ultrasonography, duplex scanning, and computed tomography) have enabled early recognition of this disease. Anticoagulation is therapeutic acutely unless there are signs of peritonitis which necessitate surgical resection of the infarcted bowel.

Adult↗
PubMed1982

Factor VII Padua defect: the heterozygote population.

14 heterozygote patients, belonging to three families with factor VII Padua defect were investigated. All patients were asymptomatic but presented a mild prolongation of prothrombin time. Factor VII activity varied between 45 and 61% of normal and no overlap was found with the homozygous or the normal populations. On the contrary, factor VII cross-reacting material was normal. A good negative correlation was found between factor VII level and prothrombin times. All patients came from the same valley of northeastern Italy. This valley, the Piave river valley, is not far from the valley where the factor X Friuli defect was found. The significance of this peculiar geographical distribution of the two abnormalities is unknown.

Antigens↗
PubMed1982

Factor VII Padua 1. Another case.

A patient with a peculiar factor VII is described. The propositus is a 70-year-old man without any bleeding tendency. The coagulation pattern is characterized by a prolonged rabbit brain prothrombin time, a normal Stypven cephalin clotting time and a normal thrombotest. Factor VII activity is low when assayed using rabbit brain the thromboplastin but is normal when assayed using ox brain thromboplastin. The neutralization test performed with an antifactor VII antiserum revealed a normal factor VII antigen level. A pedigree study has not been possible, the patient having no living relatives. No differences were observed between the biological results of our patient and those described by Girolami as factor VII + Padua.

Aged↗
PubMed1987

Bronchoalveolar lavage procoagulant activity in bleomycin-induced lung injury in marmosets. Characterization and relationship to fibrin deposition and fibrosis.

Although pulmonary fibrin deposition and coagulation abnormalities have been observed in acute lung injury in humans, their role in the pathogenesis of pulmonary disorders is unclear. In order to gain further insights into the role of the coagulation in lung injury, we examined the relationship between procoagulant activity in bronchoalveolar lavage (BAL) fluids and the evolution of bleomycin-induced lung injury in marmosets. The BAL procoagulant activity was increased at 1, 2, and 4 wk after bleomycin challenge compared with that in control subjects, and it was capable of shortening the recalcification times of plasmas deficient in factor VII and factor VIII but not in factor X. This profile suggested the presence in BAL of an activator of factor X. Activation of purified human factor X by BAL was demonstrated by measuring the amidolytic activity of the generated factor Xa on its N-benzoyl-L-isoleucyl L-glutamyl-glycyl-L-argenine-p-nitroanilide substrate. Factor X activating activity was increased in BAL at 2 wk after bleomycin challenge. Cleavage of 125I-labeled human factor X by BAL from bleomycin-challenged marmosets yielded a 55,500 Mr product that comigrated with factor Xa, the appearance of which correlated strongly with amidolytic evidence of factor Xa activity. Electron microscopy of the lungs of animals from all groups revealed pulmonary fibrin deposition at 2 wk after bleomycin challenge, at the time of increased BAL procoagulant and factor X activating activity. The BAL procoagulant activity was completely sedimentable by ultracentrifugation and was inhibited by concanavalin A and phospholipase C. Activation of purified factor X by BAL was inhibited by monospecific polyclonal goat and rabbit antibodies to human factor VII as well as antibody to bovine tissue factor, demonstrating that factor X activating activity in BAL was attributable to tissue factor associated with material similar to factors VII or VIIa. We conclude that procoagulant activity in BAL increases after bleomycin challenge in marmosets and is attributable to activation of factor X by tissue factor associated with factors VII or VIIa-like material. Increased BAL procoagulant activity is temporally associated with pulmonary fibrin deposition and pulmonary fibrosis during bleomycin-induced pulmonary injury in the marmoset.

Animals↗
PubMed1990

Estrogen regulation of a tissue factor-like procoagulant in the immature rat uterus.

An estrogen-responsive procoagulant activity is present in the plasma membrane fraction of immature rat uterus. This procoagulant has many of the properties of tissue factor, a widely occurring, integral membrane protein which initiates the intrinsic pathway of coagulation. Procoagulant activity was demonstrated to activate prothrombin in rat uterus, to activate human coagulation factor X, and to cause clot formation by human plasma. Procoagulant activity could be solubilized from the plasma membrane by the detergent octyl glucoside and had an apparent mol wt of 20,000-40,000 by gel filtration. Procoagulant activity was increased 4-fold within 3 h after immature rats were injected with estradiol. The increase was tissue- and hormone specific and was not affected by a warfarin-induced vitamin K deficiency. Coagulation factor VII was required for clot formation by the procoagulant. These properties are consistent with identification of the procoagulant as tissue factor. mRNA for tissue factor was increased in the uterus 3 h after estrogen stimulation. In the preceding paper we showed that prothrombin is increased in the immature uterus within 3 h of estrogen stimulation. The presence of increased amounts of a tissue factor-like procoagulant in the same time period suggests a functional relationship between these two proteins and a possible role for both in uterine development. Thrombin is a growth factor in fibroblasts and endothelial cells. We propose that after estrogen stimulation, prothrombin enters the uterus with the influx of plasma proteins and is activated by the procoagulant to thrombin. We suggest that thrombin might act as a paracrine factor early in the estrogen-stimulated development of the uterus.

Animals↗
PubMed1980

Thromboplastic activity of human arterial walls and its interaction with the plasmatic coagulation system.

Intact arterial vessel wall is not thrombogenic. Disorders of the endothelium in connection with pathological coditions such such as atherosclerosis, hyperlipidaemia, hypertension and hyperuricemia induce interaction of surfaces of high thromboplastic activity with the blood stream. In such situations local formation of thrombin will take place immediately. Evidence is presented for the essential and unique activation of the extrinsic pathway of the plasmatic coagulation system. The local formation of thrombin at pathologically altered arterial wall seems to be an important trigger for arterial thrombosis and haemostasis. It could be that in vivo the initial step of thrombogenesis depends upon the formation of the activator complex between tissue-thromboplastin and factor VII.

Aged↗