Acquired Factor X Deficiency in a Negro Boy

PEDIATRICS ◽  
1969 ◽  
Vol 44 (6) ◽  
pp. 1007-1009
Author(s):  
William L. Bayer ◽  
Dario Curiel C. ◽  
Isabel L. F. Szeto ◽  
Jessica H. Lewis
Keyword(s):  
Coagulation Factor ◽  
Hemorrhagic Disease ◽  
Vitamin K1 ◽  
Factor X ◽  
Factor X Deficiency

A Negro boy suffering from severe hemorrhagic disease associated with a single acquired defect in coagulation factor X is presented. No cause for this deficiency could be found. Vitamin K1 had no effect. He improved spontaneously and is completely healthy 6 months later.

Immunological Studies on the Blood Coagulation Factor X and Its Warfarin-induced Precursor

1974 ◽  
Vol 31 (01) ◽  
pp. 040-051 ◽  
Author(s):  
Gustav Gaudernack ◽  
Åse Gladhaug Berre ◽  
Bjarne Østerud ◽  
Hans Prydz
Keyword(s):  
Coagulation Factor ◽  
Factor X ◽  
Intrinsic Pathway ◽  
Coagulation Activity ◽  
Factor X Deficiency ◽  
Warfarin Treatment ◽  
The Cross ◽  
Monospecific Antisera ◽  
Purified Antigen ◽  
Congenital Factor

SummaryMonospecific antisera against the human coagulation factor X have been raised in rabbits by injections of purified antigen. Such antiserum was used to study the cross-reacting material without factor X activity which is present in the blood of warfarin-treated patients and animals as well as to study the changes in factor X during coagulation. One patient with congenital factor X deficiency was also studied.A complete identity was found between factor X in Macaca mulatta and human blood. During warfarin treatment antigenically cross-reacting material appeared in plasma. This was not adsorbed on BaSO4, and inhibited the coagulation activity of normal factor X.Both this material, normal factor X and the cross-reacting material in plasma from a patient congenitally deficient in factor X gave rise to split products during coagulation by the intrinsic pathway, i. e. all of them served as substrates for the intrinsic activator of factor X.

scholarly journals A Phenome-Wide Association Study Uncovers a Pathological Role of Coagulation Factor X duringAcinetobacter baumanniiInfection

2019 ◽  
Vol 87 (5) ◽  
Author(s):  
Jacob E. Choby ◽  
Andrew J. Monteith ◽  
Lauren E. Himmel ◽  
Paris Margaritis ◽  
Jana K. Shirey-Rice ◽  
...  
Keyword(s):  
Immune Response ◽  
Association Study ◽  
Immune Cell ◽  
Coagulation Factor ◽  
Factor X ◽  
Human Pathogens ◽  
Chemokine Production ◽  
Content Type ◽  
Factor X Deficiency ◽  
Deficient Mice

ABSTRACTCoagulation and inflammation are interconnected, suggesting that coagulation plays a key role in the inflammatory response to pathogens. A phenome-wide association study (PheWAS) was used to identify clinical phenotypes of patients with a polymorphism in coagulation factor X. Patients with this single nucleotide polymorphism (SNP) were more likely to be hospitalized with hemostatic and infection-related disorders, suggesting that factor X contributes to the immune response to infection. To investigate this, we modeled infections by human pathogens in a mouse model of factor X deficiency. Factor X-deficient mice were protected from systemicAcinetobacter baumanniiinfection, suggesting that factor X plays a role in the immune response toA. baumannii. Factor X deficiency was associated with reduced cytokine and chemokine production and alterations in immune cell population during infection: factor X-deficient mice demonstrated increased abundance of neutrophils, macrophages, and effector T cells. Together, these results suggest that factor X activity is associated with an inefficient immune response and contributes to the pathology ofA. baumanniiinfection.

Blood Coagulation Factor X Deficiency Causes Partial Embryonic Lethality and Fatal Neonatal Bleeding in Mice

2000 ◽  
Vol 83 (02) ◽  
pp. 185-190 ◽  
Author(s):  
Mieke Dewerchin ◽  
Zhong Liang ◽  
Lieve Moons ◽  
Peter Carmeliet ◽  
Francis Castellino ◽  
...  
Keyword(s):  
Blood Coagulation ◽  
Coagulation Factor ◽  
Massive Bleeding ◽  
Embryonic Lethality ◽  
Genotype Distribution ◽  
Bleeding Disorders ◽  
Factor X ◽  
Factor X Deficiency ◽  
Effective Models ◽  
The Brain

SummaryMice with a total deficiency in blood coagulation Factor X (FX) were generated by targeted replacement of an 18-kb fragment of the FX gene, comprising all exons encoding the mature FX protein, with a neor cassette. The genotype distribution among the offspring from heterozygous breeding pairs suggested that FX deficiency resulted in partial embryonic lethality, with approximately one-third of the FX −/− embryos dying around embryonic day (E) 11.5-12.5. Two of 44 non-resorbed FX −/− embryos analyzed at these stages showed signs of massive bleeding, one of which into the brain ventricles, but no histological defects in the vasculature of these embryos or their yolk sac were observed. The remainder of the FX −/− embryos appeared normal and survived to term, but the majority of neonates (90%) died within 5 days, most frequently from intraabdominal bleeding. The remaining FX −/− animals succumbed between postnatal day (P)5 and P20 with intraabdominal, subcutaneous, or intracranial bleeding or a combination thereof. The lethal phenotype of the FX −/− mice illustrates the importance of FX function in embryonic and postnatal survival and demonstrates that these mice serve as effective models of the bleeding disorders observed in severe FX deficiency in humans.

A Rare Case of Congenital Factor X Mild Deficiency Presenting as Life-Threatening Hemorrhagic Shock in a Neonate

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S151-S151
Author(s):  
Rachelle Mendoza ◽  
Tahmineh Haidary ◽  
Steven Kang
Keyword(s):  
Bleeding Event ◽  
Coagulation Factor ◽  
Hemodynamic Instability ◽  
Fresh Frozen Plasma ◽  
Bleeding Disorder ◽  
Activity Level ◽  
Factor X ◽  
Factor X Deficiency ◽  
Life Threatening ◽  
Congenital Factor

Abstract Introduction Congenital factor X deficiency is one of rarest bleeding disorders, occurring in 1 out of 1 million births. Its rarity limits its consideration in newborns presenting with hemodynamic instability. It is autosomal recessive and seen frequently in the consanguineous population. Patients with factor X deficiency are classified into three groups, based on factor X activity level: severe (<1%), moderate (1%-4%), and mild (6%-10%). Severe deficiency presents with bleeding diathesis early in life. Methods A 3-day-old male newborn, delivered at term via spontaneous vaginal delivery, presented in a well-baby clinic for a routine bilirubin check. Family history was negative for bleeding disorder or consanguinity. Nurse noted persistent blood oozing at heel stick site and oral, nasal, and umbilical stump bleeding. The patient immediately developed respiratory distress and shock. Sepsis, vitamin K deficiency, and congenital metabolic syndrome were considered. Liver function, WBC count, and other chemistry were normal, and cultures were negative. Hemoglobin was low and platelet count was elevated. PT (30.3 seconds) and aPTT (53.3 seconds) were both prolonged. Mixing patient’s sample with normal plasma corrected PT (13.1 seconds) and aPTT (25.7 seconds), indicating a factor deficiency. Results Coagulation factor assays revealed normal levels of factors VII, VIII, IX, and II. Factor X activity (12.9%) was low. The patient’s condition improved after multiple pRBC and plasma transfusions. He was placed on a daily 25-mL/kg dose of fresh-frozen plasma, which maintained his PT at 17.6 to 19.1 seconds and aPTT at 34.1 to 48.0 seconds. Factor X level increased to 20% after plasma transfusion. Conclusion Congenital bleeding disorder should be considered for neonates presenting with bleeding and shock. Factor X deficiency is suspected when both PT and aPTT are prolonged and corrected with mixing studies. Although factor levels of 10% to 40% are considered adequate for hemostasis, our patient with 12% factor X activity presented with a life-threatening bleeding event.

Congenital coagulation factor X deficiency: Genetic analysis of five patients and functional characterization of mutant factor X proteins

Haemophilia ◽  
2018 ◽  
Vol 24 (5) ◽  
pp. 774-785 ◽  
Author(s):  
Satomi Nagaya ◽  
Masashi Akiyama ◽  
Morika Murakami ◽  
Akiko Sekiya ◽  
Hidesaku Asakura ◽  
...  
Keyword(s):  
Genetic Analysis ◽  
Functional Characterization ◽  
Coagulation Factor ◽  
Factor X ◽  
Factor X Deficiency

scholarly journals Successful use of BPL Factor X concentrate in a child with severe factor X deficiency

2013 ◽  
Vol 1 (2) ◽  
pp. 8-10 ◽  
Author(s):  
Kate Khair ◽  
Poornima Kumar ◽  
Mary Mathias ◽  
Jemma Efford ◽  
Ri Liesner
Keyword(s):  
Central Venous Access ◽  
Coagulation Factor ◽  
Venous Access ◽  
Fresh Frozen Plasma ◽  
Factor X ◽  
Post Dose ◽  
Thrombotic Risk ◽  
Factor X Deficiency ◽  
Fresh Frozen ◽  
The Right

Abstract Introduction: Severe factor X deficiency is a rare serious bleeding disorder historically treated with fresh frozen plasma (FFP) and more recently with prothrombin complex concentrate (PCC) which contains activated factors II, VII, IX and X. The infusion volume of PCC is smaller than FFP, but there is a risk of thromboembolic complications given the presence of activated forms of vitamin K-dependent factor concentrates when treating an isolated coagulation factor deficiency. Methods: We describe the case of a nine-year-old girl of consanguineous origin with co-existent congenital merosin deficient muscular dystrophy and severe factor X deficiency treated with twice-weekly PCC prophylaxis via an indwelling central venous access device (CVAD). Infusion occlusion of her fifth CVAD occurred 24-months post-insertion; thrombus within the right subclavian and brachiocephalic veins was seen on radiological imaging. She started peripheral treatment with BPL Factor X concentrate as infusion volumes were smaller and given her immobility further thrombotic risk was predicted to be reduced. A sixth CVAD was inserted seven months later and BPL Factor X prophylaxis was continued. Results:BPL Factor X concentrate was effective in maintaining trough levels of 13IU/ml 72-hours post-dose, with no intercurrent bleeding episodes or further problems in terms of occlusion of her portacath. Further radiological screening has not been undertaken. Conclusion: BPL Factor X has been shown to be a safe and effective alternative to PCC for treatment of severe factor X deficiency in this case.

scholarly journals Anesthetic Management for the Patient with Coagulation Factor X Deficiency.

1998 ◽  
Vol 18 (1) ◽  
pp. 71-74 ◽  
Author(s):  
Koichi MORI ◽  
Kazunori KAWAMURA ◽  
Shinya HORI ◽  
Akira TANAKA
Keyword(s):  
Anesthetic Management ◽  
Coagulation Factor ◽  
Factor X ◽  
Factor X Deficiency
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