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Factor IX Deficiency

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121. The carboxyl-terminal region is NOT essential for secreted and functional levels of coagulation factor X. (PubMed)

The carboxyl-terminal region is NOT essential for secreted and functional levels of coagulation factor X. The homologous coagulation factor X (FX), VII (FVII), IX (FIX) and protein C (PC) display striking differences in the carboxyl-terminus, with that of FX being the most extended. This region is essential for FVII, FIX and PC secretion.To provide experimental evidence for the role of the FX carboxyl-terminus.Recombinant FX (rFX) variants were expressed in multiple eukaryotic cell systems (...) contribution to pro-coagulant properties. These findings, which might suggest an involvement of the carboxyl-terminal region in the divergence of the homologous FX, FVII, FIX and PC, help to interpret the mutational pattern of FX deficiency.© 2015 International Society on Thrombosis and Haemostasis.

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2015 Journal of Thrombosis and Haemostasis

122. Preparation of factor VII concentrate using CNBr-activated Sepharose 4B immunoaffinity chromatography (PubMed)

Preparation of factor VII concentrate using CNBr-activated Sepharose 4B immunoaffinity chromatography Factor VII concentrates are used in patients with congenital or acquired factor VII deficiency or treatment of hemophilia patients with inhibitors. In this research, immunoaffinity chromatography was used to purify factor VII from prothrombin complex (Prothrombin- Proconvertin-Stuart Factor-Antihemophilic Factor B or PPSB) which contains coagulation factors II, VII, IX and X. The aim (...) of this study was to improve purity, safety and tolerability as a highly purified factor VII concentrate.PPSB was prepared using DEAE-Sephadex and was used as the starting material for purification of coagulation factor VII. Prothrombin complex was treated by solvent/detergent at 24°C for 6 h with constant stirring. The mixture of PPSB in the PBS buffer was filtered and then chromatographed using CNBr-activated Sepharose 4B coupled with specific antibody. Factors II, IX, VII, X and VIIa were assayed

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2015 Medical journal of the Islamic Republic of Iran

123. Zinc Deficiency

with organ failure) VIII. Labs Serum zinc levels are not a reliable measure of zinc stores IX. Diagnosis Zinc Deficiency is a clinical diagnosis rather than a lab diagnosis Base diagnosis on symptoms, signs, risk factors and overall X. Management See for RDA levels Mild Zinc Deficiency at 2-3 times the RDA for 6 months Severe Zinc Deficiency at 4-5 times the RDA for 6 months Images: Related links to external sites (from Bing) These images are a random sampling from a Bing search on the term "Zinc (...) Zinc Deficiency Zinc Deficiency Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Zinc Deficiency Zinc Deficiency Aka: Zinc Deficiency

2015 FP Notebook

124. Vitamin D Deficiency

Aka: Vitamin D Deficiency , Hypovitaminosis D II. Epidemiology Vitamin D Deficiency is common in U.S. (esp. in northern climates) increasing due to use and less doors Age over 65 are associated worldwide with Vitamin D Deficiency in >50% of people III. Risk Factors: Infants Anticonvulsants Chronic disease with fat malabsorption Exclusively -fed infant without supplementation Low maternal levels Lack of Direct sunlight avoidance is recommended by AAP for those under 6 months Darker skin (...) See Consider in those diagnosed with , , IX. Associated Conditions: Conditions linked to Vitamin D Deficiency Musculoskeletal Conditions Other associated conditions (but insufficient evidence that supplementation prevents these conditions) Fall Risk Cardiovascular Disease X. Labs Serum 25-Hyroxyvitamin D Level Routine screening is not recommended testing or supplementation is not indicated in , , or Preferred test (but expensive) Levels <20 ng/ml suggest deficiency (<30 ng/ml per endocrine society

2015 FP Notebook

125. Vitamin C Deficiency

Aka: Vitamin C Deficiency , Ascorbic Acid Deficiency , Scurvy II. Pathophysiology: Vitamin C Deficiency Increased fragility of capillaries Poor III. Risk factors Elderly edentulous patients who cook for themselves Mental illness Institutionalized patients Infant on processed milk without supplementation Unusual dietary habits Malabsorption syndromes IV. Symptoms Children Irritability Decreased Appetite Leg tenderness External bleeding l erythema and bleeding Other symptoms Mood changes V. Signs (...) Vitamin C Deficiency Vitamin C Deficiency Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Vitamin C Deficiency Vitamin C Deficiency

2015 FP Notebook

126. G6PD Deficiency

A Fava beans (Italian Broad Beans) Bell Beans Broad Beans English Dwarf Beans Haba Beans Horse Beans Pigeon Beans Silkworm Beans Tic Beans V. Symptoms and Signs Usually asymptomatic See for acute episodes VI. Differential Diagnosis See See VII. Labs See See VIII. Complications Increased risk of IX. Prevention Avoid precipitating factors See Keep s up-to-date Treat following episode supplementation Measures that are not recommended (no benefit) Splenectomy X. References Golan in Goldman (2000) Cecil (...) G6PD Deficiency G6PD Deficiency Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 G6PD Deficiency G6PD Deficiency Aka: G6PD Deficiency

2015 FP Notebook

127. Vitamin B12 Deficiency

Deficiency Aka: Vitamin B12 Deficiency , Pernicious Anemia , B12 Deficiency , Cobalamin Deficiency , Cyanocobalamin Deficiency From Related Chapters II. Epidemiology U.S.: 6% in patients under age 60 years (20% in those over age 60 years) Latin America: 40% Kenya: 70% East India: 70-80% III. Pathophysiology See Symptoms may lag Vitamin B12 Deficiency for as many as 10 years until vast hepatic stores are exhausted IV. Causes: Vitamin B12 Deficiency See Decreased intrinsic factor Atrophic Rouz-en-Y (...) and other post-gastrectomy syndromes (see malabsorption below) Pernicious Anemia Autoimmune parietal cell destruction as part of a chronic autoimmune Results in insufficient intrinsic factor production B12 Deficiency is preceded by by 20 years, and asymptomatic by 30 years Elderly with atrophic (10-30% over age 60 years) Can not absorb bound to protein However can absorb crystalline normally Malabsorption Zollinger-Ellison Syndrome ( oma) Whipple Disease Short- Syndrome Intestinal infection (e.g

2015 FP Notebook

128. Thrombin generation and fibrin formation under flow on biomimetic tissue factor-rich surfaces. (PubMed)

and factors VIII, IX and XI deficient plasmas were perfused over the beads coated with 0.08, 0.8 and 8 molecules-TF μm(-2) at shear rates of 50-1000 s(-1) . Fibrin deposition and thrombin generation were measured by fluorescence microscopy in a hydrodynamic focusing microfluidic device.Fibrin deposition was supported on patterned bead spots, but not planar TF substrates at the same surface TF concentration. There was a threshold spot size and a shear rate dependent TF concentration that was necessary (...) Thrombin generation and fibrin formation under flow on biomimetic tissue factor-rich surfaces. Blood flow regulates coagulation and fibrin assembly by controlling the rate of transport of zymogens, enzymes and plasma proteins to and from the site of an injury.The objective of this work was to define the hemodynamic conditions under which fibrin can form under flow on tissue factor (TF)-rich substrates.TF-coated silica beads (~ 800 nm) were patterned into 18-85-μm spots. Normal pooled plasma

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2014 Journal of Thrombosis and Haemostasis

129. 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

130. 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

131. 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

132. 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

133. 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

134. 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

135. 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

136. 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

137. 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

138. 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

139. 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

140. 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

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