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Factor IX Deficiency

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141. Factor XIII (Overview)

of a hemorrhagic diathesis. Paradoxically, alterations in FXIII may also predispose to thrombosis. Congenital FXIII deficiency is due principally to defects in the catalytic A subunit of FXIII, with more than 100 mutations throughout the factor XIII A gene identified. [ ] Acquired FXIII deficiencies, which result from autoantibodies against FXIII subunits, are extremely rare but may produce severe bleeding diatheses. [ ] Thrombin, generated by reactions initiated by activated tissue factor VII/factor IX (...) Factor XIII (Overview) Factor XIII Deficiency: Practice Essentials, Background, Pathophysiology Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMjA5MTc5LW92ZXJ2aWV3 processing > Factor XIII Deficiency Updated: Apr

2014 eMedicine.com

142. Factor VIII (Overview)

: Douglass A Drelich, MD; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP Share Email Print Feedback Close Sections Sections Hemophilia A Overview Practice Essentials Hemophilia A is an X-linked, recessive disorder caused by deficiency of functional plasma clotting factor VIII (FVIII), which may be inherited or arise from spontaneous mutation. The development of inhibitory alloantibodies to FVIII can severely complicate the treatment of genetic cases. Rarely, development of autoantibodies to FVIII results (...) ) is now known, it was not appreciated at the time. In 1953, decreased FVIII levels in patients with vWF deficiency was first described. Further research by Nilson and coworkers indicated the interaction between these 2 clotting factors. In 1952, hemophilia B was described and was named Christmas disease after the surname of the first patient who was examined in detail. The differentiation of hemophilia B from hemophilia A followed the observation that mixing plasma from a patient with "true hemophilia

2014 eMedicine.com

143. Factor VII (Overview)

is catalyzed by a number of proteases, including thrombin, factor IXa, factor Xa, factor XIa, and factor XIIa. Comparison of these proteins has shown that factor Xa, in association with phospholipids, has the highest potential to activate factor VII. [ , , ] Factor IXa is responsible for basal levels of plasma factor VIIa in healthy individuals. Patients with hemophilia B (factor IX deficiency), unlike patients with hemophilia A (factor VIII deficiency), have very low concentrations of circulating factor (...) Arg184Gly suggests a structural basis for factor IX binding to factor XIa. J Thromb Haemost . 2013 Apr 26. . Herrmann FH, Wulff K, Auberger K, et al. Molecular biology and clinical manifestation of hereditary factor VIIdeficiency. Semin Thromb Hemost . 2000. 26(4):393-400. . Bernardi F, Patracchini P, Gemmati D, et al. Molecular analysis of factor VII deficiency in Italy: a frequent mutation(FVII Lazio) in a repeated intronic region. Hum Genet . 1993 Nov. 92(5):446-50. . Cutler JA, Patel R, Mitchell MJ

2014 eMedicine.com

144. Factor XIII (Treatment)

Factor XIII (Treatment) Factor XIII Deficiency Treatment & Management: Approach Considerations, Surgical Care, Consultations Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMjA5MTc5LXRyZWF0bWVudA== processing (...) > Factor XIII Deficiency Treatment & Management Updated: Apr 02, 2018 Author: Robert A Schwartz, MD, MPH; Chief Editor: Perumal Thiagarajan, MD Share Email Print Feedback Close Sections Sections Factor XIII Deficiency Treatment Approach Considerations Factor XIII (FXIII) replacement is used to treat bleeding, to prevent perioperative bleeding during elective surgical procedures or, prophylactically, to prevent recurrent bleeding, as in central nervous system (CNS) or joint hemorrhages. Serial

2014 eMedicine.com

145. Factor X (Treatment)

factor X levels. PCCs contain factors II, VII, IX, and X and protein C. PCCs should be used cautiously to avoid factor X levels of more than 50% of normal, which can result in episodes. Perioperative prothrombin complex concentrate has also been employed to treat acquired factor X deficiency associated with systemic light-chain amyloidosis. [ ] Vitamin K administration may be useful in certain patients with acquired factor X deficiency; however, it has been amply demonstrated that patients (...) Factor X (Treatment) Factor X Deficiency Treatment & Management: Medical Care, Surgical Care, Consultations Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMjA5ODY3LXRyZWF0bWVudA== processing > Factor X Deficiency

2014 eMedicine.com

146. Factor VII (Treatment)

or as prophylaxis for surgery. Unlike factor VIII and factor IX deficiencies, for which levels of 100% are required before surgery, factor VII deficiency requires levels in the range of 10-15% to produce efficient hemostasis. For major surgery, trough levels of factor VII must not fall to less than 20 U/dL. Prothrombin complex concentrates are also a source of factor VII but carry the risk of infectious complications and thrombosis. When prothrombin concentrates are used, doses of 50 U/kg every 8 hours for 24 (...) . 2002. 32 Suppl 1:37-40. . Geng Y, Verhamme IM, Sun MF, Paul Bajaj S, Emsley J, Gailani D. Analysis of the factor XI variant Arg184Gly suggests a structural basis for factor IX binding to factor XIa. J Thromb Haemost . 2013 Apr 26. . Herrmann FH, Wulff K, Auberger K, et al. Molecular biology and clinical manifestation of hereditary factor VIIdeficiency. Semin Thromb Hemost . 2000. 26(4):393-400. . Bernardi F, Patracchini P, Gemmati D, et al. Molecular analysis of factor VII deficiency in Italy

2014 eMedicine.com

147. Factor II (Treatment)

, IX, and X, along with protein C; however, PCCs should be used judiciously because of the risk of thromboembolic complications Vitamin K administration may be useful in patients with acquired factor II deficiency Consultations to consider in cases of factor II deficiency include hematologists and, in patients with congenital factor II deficiency, genetic counselors. Next: Diet and Activity No dietary restrictions are necessary in individuals with factor II deficiency. Patients should be advised (...) Factor II (Treatment) Factor II Deficiency Treatment & Management: Appproach Considerations, Diet and Activity Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMjA5NzQyLXRyZWF0bWVudA== processing > Factor II

2014 eMedicine.com

148. Wound Healing, Growth Factors

be continually assessed and adjusted according to the stage of healing and injury. This is particularly true for burn patients who require multiple debridements and grafting. Vitamin supplementation has not been proven to increase wound healing unless a specific deficiency exists. [ ] Vitamin A is an exception to this rule and is detailed below. Steroids: Corticosteroids can blunt the response of macrophages, the most essential cell in wound healing. [ ] Vitamin A, insulinlike growth factor (IGF (...) Wound Healing, Growth Factors Wound Healing and Growth Factors: Overview, Types of Wound Healing, Phases of Wound Healing Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTI5ODE5Ni1vdmVydmlldw== processing > Wound

2014 eMedicine Surgery

149. Factor XIII (Follow-up)

Factor XIII (Follow-up) Factor XIII Deficiency Follow-up: Further Outpatient Care, Further Inpatient Care, Inpatient & Outpatient Medications Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMjA5MTc5LWZvbGxvd3Vw (...) processing > Factor XIII Deficiency Follow-up Updated: Apr 02, 2018 Author: Robert A Schwartz, MD, MPH; Chief Editor: Perumal Thiagarajan, MD Share Email Print Feedback Close Sections Sections Factor XIII Deficiency Follow-up Further Outpatient Care Clinic-supervised outpatient care is an extremely important part of treatment. Complete annual physical examinations and laboratory testing for inhibitors, hepatitis, and HIV infection, as well as other tests, should be performed as needed. As in persons

2014 eMedicine.com

150. Factor VII (Follow-up)

manifestations, management, and molecular genetics in congenitalfactor VII deficiency: the International Registry on Congenital Factor VII Deficiency (IRF7). Blood . 2000 Jul 1. 96(1):374. . Roberts HR, Monroe DM, White GC. The use of recombinant factor VIIa in the treatment of bleeding disorders. Blood . 2004 Dec 15. 104(13):3858-64. . Media Gallery Factor VII. Intrinsic and extrinsic pathways of coagulation. Factor VII/tissue factor complex activates factor IX and factor X. Factor IXa along with factor (...) Factor VII (Follow-up) Factor VII Deficiency Follow-up: Further Outpatient Care, Complications, Prognosis Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMjA5NTg1LWZvbGxvd3Vw processing > Factor VII Deficiency

2014 eMedicine.com

151. Factor VII (Diagnosis)

is catalyzed by a number of proteases, including thrombin, factor IXa, factor Xa, factor XIa, and factor XIIa. Comparison of these proteins has shown that factor Xa, in association with phospholipids, has the highest potential to activate factor VII. [ , , ] Factor IXa is responsible for basal levels of plasma factor VIIa in healthy individuals. Patients with hemophilia B (factor IX deficiency), unlike patients with hemophilia A (factor VIII deficiency), have very low concentrations of circulating factor (...) Arg184Gly suggests a structural basis for factor IX binding to factor XIa. J Thromb Haemost . 2013 Apr 26. . Herrmann FH, Wulff K, Auberger K, et al. Molecular biology and clinical manifestation of hereditary factor VIIdeficiency. Semin Thromb Hemost . 2000. 26(4):393-400. . Bernardi F, Patracchini P, Gemmati D, et al. Molecular analysis of factor VII deficiency in Italy: a frequent mutation(FVII Lazio) in a repeated intronic region. Hum Genet . 1993 Nov. 92(5):446-50. . Cutler JA, Patel R, Mitchell MJ

2014 eMedicine.com

152. Factor XIII (Diagnosis)

of a hemorrhagic diathesis. Paradoxically, alterations in FXIII may also predispose to thrombosis. Congenital FXIII deficiency is due principally to defects in the catalytic A subunit of FXIII, with more than 100 mutations throughout the factor XIII A gene identified. [ ] Acquired FXIII deficiencies, which result from autoantibodies against FXIII subunits, are extremely rare but may produce severe bleeding diatheses. [ ] Thrombin, generated by reactions initiated by activated tissue factor VII/factor IX (...) Factor XIII (Diagnosis) Factor XIII Deficiency: Practice Essentials, Background, Pathophysiology Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMjA5MTc5LW92ZXJ2aWV3 processing > Factor XIII Deficiency Updated: Apr

2014 eMedicine.com

153. Factor VIII (Diagnosis)

: Douglass A Drelich, MD; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP Share Email Print Feedback Close Sections Sections Hemophilia A Overview Practice Essentials Hemophilia A is an X-linked, recessive disorder caused by deficiency of functional plasma clotting factor VIII (FVIII), which may be inherited or arise from spontaneous mutation. The development of inhibitory alloantibodies to FVIII can severely complicate the treatment of genetic cases. Rarely, development of autoantibodies to FVIII results (...) ) is now known, it was not appreciated at the time. In 1953, decreased FVIII levels in patients with vWF deficiency was first described. Further research by Nilson and coworkers indicated the interaction between these 2 clotting factors. In 1952, hemophilia B was described and was named Christmas disease after the surname of the first patient who was examined in detail. The differentiation of hemophilia B from hemophilia A followed the observation that mixing plasma from a patient with "true hemophilia

2014 eMedicine.com

154. Recurrence of axial malalignment after surgical correction in congenital femoral deficiency and fibular hemimelia Full Text available with Trip Pro

Recurrence of axial malalignment after surgical correction in congenital femoral deficiency and fibular hemimelia Recurrent genu valgum deformity complicates treatment of congenital femoral deficiencies (CFD) and fibular hemimelia (FH). We analysed factors influencing recurrence.Patients who underwent limb lengthening or deformity correction for CFD and/or FH were reviewed. Radiographs after surgery and after a minimum of a further six months were analysed. Change in parameters of mechanical (...)  mm regarding MAD in the first radiograph and patient age. CFD cases Pappas types VII and VIII showed a ∆ MAD/month of 1.6 mm, whereas milder cases of Pappas IX showed a ∆ MAD/month of 0.8. Mild FH (type Ia) showed a mean ∆ MAD/month of 0.39 mm, whereas mean ∆ MAD/month for FH type Ib/II was 0.72 mm. In FH type II cases, mean ∆ MAD/month was 0.79 mm after resection of the fibular anlage compared with 1.98 mm in those without resection.Recurrence in FH and CFD was not dependent on patient age

2011 International orthopaedics

155. Coagulation Factors

and IX that occur almost exclusively in men (women are usually who are or have mild bleeding). Other inherited factor deficiencies, not associated with the X chromosome, are found equally in both men and women. The severity of symptoms experienced by a patient with an inherited factor deficiency depends on the factor involved and amount available. Symptoms may vary from episode to episode, from excessive bleeding after dental procedures to severe recurrent bleeding into joints or muscles. Patients (...) with a modest reduction in coagulation factor level may experience few symptoms and may discover their deficiency as an adult - after a surgical procedure or trauma or during screening that includes a or . Those with severe factor deficiencies may have their first bleeding episode very early; for example, a male infant with a deficiency of Factor VIII, IX, or XIII may bleed excessively after circumcision. Acquired deficiencies may be due to diseases, such as or cancer; to an condition such as , which uses

2012 Lab Tests Online UK

156. The effect of repeated freezing and thawing on levels of vitamin K-dependent coagulation factors and fibrinogen in fresh frozen plasma Full Text available with Trip Pro

The effect of repeated freezing and thawing on levels of vitamin K-dependent coagulation factors and fibrinogen in fresh frozen plasma Fresh frozen plasma (FFP) is considered adequate for transfusion immediately after thawing or for up to 24 hours if kept at 1-6°C, and is currently used very often to replace deficient clotting factors. If factor levels in refrozen FFP are within normal limits, then this component can possibly be transfused, thus avoiding wastage of FFP.To study the fate (...) of vitamin K-dependent coagulation factors (F II, F VII, F IX, F X) and fibrinogen activity levels in repeatedly (twice) frozen and thawed FFP.Two hundred FFP units comprising 50 units of each major blood group (A, B, AB, and O) were thawed at 37°C and 10-20 mL of FFP transferred to transfer bags with the help of a sterile connecting device (SCD). The FFP samples were taken into tubes (first sampling), and then the transfer bags were kept for 24 hours at 4°C. After 24 hours, repeat samples were taken

2013 Asian journal of transfusion science

157. Rational Use of Recombinant Factor VIIa in Clinical Practice Full Text available with Trip Pro

Rational Use of Recombinant Factor VIIa in Clinical Practice In the United States, the FDA-approved indications for recombinant factor VIIa is for bypassing inhibitors to factors VIII and IX in patients with hemophilia A and B respectively and for treatment of congenital factor VII deficiency. In European countries, rFVIIa is licensed for the above indications as well as for Glanzmann's thrombasthenia. In absence of high-quality data favoring off-label use of this agent and laboratory test (...) to predict response to this agent, and in view of high cost of rFVIIa, off-label use of recombinant factor VIIa should be restricted to only when hemorrhage has not responded to transfusion or other conventional therapy. It appears, two such conditions where recombinant factor VIIa may be beneficial are traumatic and postpartum hemorrhages.

2013 Indian Journal of Hematology & Blood Transfusion

158. Future of coagulation factor replacement therapy. (Abstract)

Future of coagulation factor replacement therapy. Over a million patients worldwide currently suffer from hemophilia and other congenital clotting factor deficiencies. Patients affected with hemophilia A and B are treated by intravenous replacement therapy of factor VIII and factor IX, respectively. Current hemophilia treatments have favorably supported their efficacy, tolerability, and safety profiles. The onset of alloantibodies inactivating the infused coagulation factor is the main problem (...) in hemophilia patients rendering replacement therapies ineffective; another disadvantage is the short half-life of the infused clotting factors with the need for multiple and frequent infusions to manage a bleeding episode. Now, the challenge in the management of hemophilia treatment is the prolongation of the half-life and reduction in the immunogenicity of recombinant clotting factors. The bioengineering strategies, previously applied successfully to other therapeutic proteins, encourage the current

2013 Journal of Thrombosis and Haemostasis

159. Thromboembolism Risk Factors

) increase after acute therapy completed (2.2) Persistent risk factors - see above (2) Idiopathic VTE (2) Protein C,Protein S and deficiency (1.8) Prothrombin mutation - G20210A (1.7) for (1.6) Second VTE (1.5) Mild (0.9) Distal VTE (0.5) Transient risk factors (0.5) VII. Risk Factors: Venous Stasis Prolonged immobility Long leg or other limb immobilization Paralysis (CVA) Spinal cord injury s High risk for DVT in surgery without Cardiac Disease VIII. Risk Factors: Hypercoagulable state See Inherited (...) cause found in up to one third of DVT cases Prior (DVT) Medications Increased or Pregnancy (Nolvadex) Phenothiazines Major Recent Surgery (e.g. ) Cancer Consider evaluation for occult cancer in DVT Polycythemia History of thromboembolic disease Deep Venous Thrombosis Type A Blood IX. Risk Factors: Intimal damage Local Surgery (Especially ral and Orthopedic Surgery) ral anesthesia is an independent risk factor Consider Penetrating vessel injury Especially femoral X. References Images: Related links

2015 FP Notebook

160. Von Willebrand Factor

Factor Aka: Von Willebrand Factor , VWF , Von Willebrand Factor Antigen , Von Willebrand Factor Ristocetin Cofactor Activity , VWF:Ag , VWF:RCo From Related Chapters II. Physiology Von Willebrand Factor synthesis Vascular endothelium s Von Willebrand Factor release Platelet activation Endothelial cells Von Willebrand Factor activity Binds factor VIII in circulation (prolongs Factor VIII half-life) Releases factor VIII in response to bleeding Factor VIII in turn is a or in the conversion of Factor IX (...) to IXa in the initrinsic to form thrombin Bridges exposed collagen and platelets when vascular injury occurs Von Willebrand Factor mediates platelet adhesion Von Willebrand Factor is a large protein that binds damaged vasculature and traps, binds platelets to form a platelet plug Analogous to 6-pack plastic ring holder that traps wildlife Von Willebrand Factor deficiency Results in delayed platelet plug formation Results in mucocutaneous bleeding III. Labs Normal VWF range: 50-200 IU/dl IV. Causes

2015 FP Notebook

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