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Factor IX Deficiency

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61. Factor XI Deficiency (Diagnosis)

. Zivelin A, Bauduer F, Ducout L, et al. Factor XI deficiency in French Basques is caused predominantly by an ancestral Cys38Arg mutation in the factor XI gene. Blood . 2002 Apr 1. 99(7):2448-54. . Zivelin A, Ogawa T, Bulvik S, et al. Severe factor XI deficiency caused by a Gly555 to Glu mutation (factor XI-Glu555): a cross-reactive material positive variant defective in factor IX activation. J Thromb Haemost . 2004 Oct. 2(10):1782-9. Media Gallery Factor XI deficiency. Graph depicts factor deficiencies (...) Factor XI Deficiency (Diagnosis) Factor XI Deficiency: Background, Pathophysiology, Epidemiology 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=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMjA5OTg0LW92ZXJ2aWV3 processing > Factor XI Deficiency Updated: May

2014 eMedicine.com

62. Factor XI Deficiency (Treatment)

XI deficiency caused by a Gly555 to Glu mutation (factor XI-Glu555): a cross-reactive material positive variant defective in factor IX activation. J Thromb Haemost . 2004 Oct. 2(10):1782-9. Media Gallery Factor XI deficiency. Graph depicts factor deficiencies. of 1 Tables Contributor Information and Disclosures Author Jamie E Siegel, MD Director, Cardeza Foundation Hemophilia Treatment Center, Thomas Jefferson University Jamie E Siegel, MD is a member of the following medical societies (...) Factor XI Deficiency (Treatment) Factor XI Deficiency Treatment & Management: Approach Considerations 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=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMjA5OTg0LXRyZWF0bWVudA== processing > Factor XI Deficiency

2014 eMedicine.com

63. Factor XI Deficiency (Follow-up)

. . Zivelin A, Ogawa T, Bulvik S, et al. Severe factor XI deficiency caused by a Gly555 to Glu mutation (factor XI-Glu555): a cross-reactive material positive variant defective in factor IX activation. J Thromb Haemost . 2004 Oct. 2(10):1782-9. Media Gallery Factor XI deficiency. Graph depicts factor deficiencies. of 1 Tables Contributor Information and Disclosures Author Jamie E Siegel, MD Director, Cardeza Foundation Hemophilia Treatment Center, Thomas Jefferson University Jamie E Siegel, MD is a member (...) Factor XI Deficiency (Follow-up) Factor XI Deficiency Follow-up: Prognosis, Patient Education 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=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMjA5OTg0LWZvbGxvd3Vw processing > Factor XI Deficiency Follow-up Updated

2014 eMedicine.com

64. Factor VII Deficiency (Treatment)

in congenital hemophilia with inhibitors and congenital factor VII deficiency. J Blood Med . 2018. 9:227-39. . . Media Gallery Intrinsic and extrinsic pathways of coagulation. Factor VII/tissue factor complex activates factor IX and factor X. Factor IXa along with factor VIIIa results in formation of more factor Xa. Factor Xa along with factor Va converts prothrombin to thrombin. of 1 Tables Contributor Information and Disclosures Author Helge Dirk Hartung, MD Attending Physician, Division of Hematology (...) Factor VII Deficiency (Treatment) Pediatric Factor VII 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=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvOTYwNTkyLXRyZWF0bWVudA

2014 eMedicine Pediatrics

65. Factor XIII Deficiency (Overview)

with mild to moderate bleeding disorders found that out of 418 individuals with these conditions, just one (0.2%) had factor XIII deficiency. Factor VIII, IX, and XI deficiencies occurred in 11 (2.6%), 3 (0.7%), and 3 (0.7%) patients, respectively. [ ] Mortality/Morbidity The mortality and morbidity are primarily related to bleeding; intracranial hemorrhage can be life threatening. The spontaneous abortion rate in women with severe factor XIII deficiency approaches 80%. Sex This is an autosomal (...) Factor XIII Deficiency (Overview) Pediatric Factor XIII Deficiency: Background, Pathophysiology, Epidemiology 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=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvOTYwNTE1LW92ZXJ2aWV3 processing > Pediatric Factor XIII

2014 eMedicine Pediatrics

66. Factor VII Deficiency (Overview)

and extrinsic pathways of coagulation. Factor VII/tissue factor complex activates factor IX and factor X. Factor IXa along with factor VIIIa results in formation of more factor Xa. Factor Xa along with factor Va converts prothrombin to thrombin. Previous Next: Epidemiology Frequency United States Inherited factor VII deficiency is rare. Incidence is 1 case per 500,000 population. International The frequency is higher in countries where consanguineous marriage is more common. For example, the reported (...) with congenital factor VII deficiency: A single center experience. Korean J Hematol . 2012 Dec. 47(4):281-5. . . Rajpurkar M, Cooper DL. Continuous infusion of recombinant activated factor VII: a review of data in congenital hemophilia with inhibitors and congenital factor VII deficiency. J Blood Med . 2018. 9:227-39. . . Media Gallery Intrinsic and extrinsic pathways of coagulation. Factor VII/tissue factor complex activates factor IX and factor X. Factor IXa along with factor VIIIa results in formation

2014 eMedicine Pediatrics

67. Factor VII Deficiency (Follow-up)

. Korean J Hematol . 2012 Dec. 47(4):281-5. . . Rajpurkar M, Cooper DL. Continuous infusion of recombinant activated factor VII: a review of data in congenital hemophilia with inhibitors and congenital factor VII deficiency. J Blood Med . 2018. 9:227-39. . . Media Gallery Intrinsic and extrinsic pathways of coagulation. Factor VII/tissue factor complex activates factor IX and factor X. Factor IXa along with factor VIIIa results in formation of more factor Xa. Factor Xa along with factor Va converts (...) Factor VII Deficiency (Follow-up) Pediatric Factor VII Deficiency Follow-up: Further Outpatient Care, Deterrence/Prevention, Complications 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=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvOTYwNTkyLWZvbGxvd3Vw

2014 eMedicine Pediatrics

68. Laboratory Diagnosis of Functional Iron Deficiency

ferritin values), a Ret‐He cut‐off of 25 pg may also help to distinguish iron deficiency (values <25 pg) from ACD (values >25 pg). A Ret‐He cut‐off value of 30·6 pg is a better predictor of response to intravenous iron than baseline serum ferritin or transferrin saturation values in CKD patients undergoing thrice‐weekly haemodialysis (Buttarello et al , ). Red blood cell size factor (Rsf) This variable is derived from the square root of the product of the MCVs of mature RBC and reticulocytes. It shows (...) being this technique remains a research investigation. Functional iron deficiency and the anaemia of chronic disease The failure of adequate iron incorporation into the developing erythron is just one component of ACD and cancer‐related anaemia. With these disorders the actions of γ‐interferon, transforming growth factor‐β and tumour necrosis factor produce a down‐regulation of the erythron, early erythroid precursor cell death and reduced Epo sensitivity. Increased levels of both hepcidin and IL6

2013 British Committee for Standards in Haematology

69. Membrane carbonic anhydrase IX expression and relapse risk in resected stage I-II non-small-cell lung cancer. (Full text)

Membrane carbonic anhydrase IX expression and relapse risk in resected stage I-II non-small-cell lung cancer. Adjuvant chemotherapy reduces recurrences of non-small-cell lung cancer (NSCLC). To determine which patients need adjuvant chemotherapy, we assessed factors associated with time to relapse (TTR).In 230 resected stage I-II NSCLCs, we correlated immunohistochemistry scores for factors associated with cell growth rate, growth regulation, hypoxia, cell survival, and cell death with TTR.With (...) a median follow-up of 82 months (1-158) for those alive and relapse free at last follow-up, median time to recurrence was not reached. The 2- and 5-year probabilities of maintaining freedom from recurrence were 80.7% (95% confidence interval, 75.3%, 86.4%) and 74.6% (95% confidence interval, 68.6%, 81.2%), respectively. TTR curves flattened at an apparent cure rate of 70%. In multicovariate Cox models, factors correlating with shorter TTR were membranous carbonic anhydrase IX (mCAIX) staining (any

2014 Journal of Thoracic Oncology PubMed

70. Prevention and Reversal of Antibody Responses Against Factor IX in Gene Therapy for Hemophilia B (Full text)

Prevention and Reversal of Antibody Responses Against Factor IX in Gene Therapy for Hemophilia B Intramuscular (IM) administration of an adeno-associated viral (AAV) vector represents a simple and safe method of gene transfer for treatment of the X-linked bleeding disorder hemophilia B (factor IX, F.IX, deficiency). However, the approach is hampered by an increased risk of immune responses against F.IX. Previously, we demonstrated that the drug cocktail of immune suppressants rapamycin, IL-10

2011 Frontiers in microbiology PubMed

71. Nonredundant Roles of IL-10 and TGF-β in Suppression of Immune Responses to Hepatic AAV-Factor IX Gene Transfer (Full text)

growth factor-β (TGF-β) in the development of tolerance to human coagulation factor IX (hF.IX). Interestingly, IL-10 deficient C57BL/6 mice receiving gene transfer remained tolerant to hF.IX and generated Treg that suppressed anti-hF.IX formation. Effects of TGF-β blockade were also minor in this strain. In contrast, in C3H/HeJ mice, a strain known to have stronger T-cell responses against hF.IX, IL-10 was specifically required for the suppression of CD8(+) T-cell infiltration of the liver (...) Nonredundant Roles of IL-10 and TGF-β in Suppression of Immune Responses to Hepatic AAV-Factor IX Gene Transfer Hepatic gene transfer using adeno-associated viral (AAV) vectors has been shown to efficiently induce immunological tolerance to a variety of proteins. Regulatory T-cells (Treg) induced by this route suppress humoral and cellular immune responses against the transgene product. In this study, we examined the roles of immune suppressive cytokines interleukin-10 (IL-10) and transforming

2011 Molecular Therapy PubMed

72. A Safety and Efficacy Study of a Recombinant Factor IX in Patients With Severe Hemophilia B

A Safety and Efficacy Study of a Recombinant Factor IX in Patients With Severe Hemophilia B A Safety and Efficacy Study of a Recombinant Factor IX in Patients With Severe Hemophilia B - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more (...) studies before adding more. A Safety and Efficacy Study of a Recombinant Factor IX in Patients With Severe Hemophilia B The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our for details. ClinicalTrials.gov Identifier: NCT01361126 Recruitment Status : Completed First Posted : May 26, 2011 Results First Posted : May 9, 2016 Last Update Posted : May 9, 2016

2011 Clinical Trials

73. A Safety and Efficacy Study of a Recombinant Fusion Protein Linking Coagulation Factor IX With Albumin (rIX-FP) in Patients With Hemophilia B

A Safety and Efficacy Study of a Recombinant Fusion Protein Linking Coagulation Factor IX With Albumin (rIX-FP) in Patients With Hemophilia B A Safety and Efficacy Study of a Recombinant Fusion Protein Linking Coagulation Factor IX With Albumin (rIX-FP) in Patients With Hemophilia B - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save (...) this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. A Safety and Efficacy Study of a Recombinant Fusion Protein Linking Coagulation Factor IX With Albumin (rIX-FP) in Patients With Hemophilia B The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. Read our for details

2011 Clinical Trials

74. Surgical Operation in Hemophilia B—Use of Factor IX Concentrate (Full text)

Surgical Operation in Hemophilia B—Use of Factor IX Concentrate A concentrate containing plasma clotting factors II, VII, IX and X was used to secure hemostasis for a herniorrhaphy, an osteotomy of a femur, a cup arthroplasty of a hip, and a tonsillectomy in patients with factor IX deficiency. After single infusions of concentrate, the net increase in plasma factor IX activity was 0.7 to 1.0 percent for each in-vitro unit of factor IX infused per kilogram of body weight. After large infusions (...) of concentrate in two patients, the disappearance pattern of factor IX had two phases: a first component with half-disappearance times of 4.4 and 6 hours, and a second component with half-disappearance times of 26 and 32.6 hours.

1970 California Medicine PubMed

75. 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

2018 FP Notebook

76. 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

2018 FP Notebook

77. 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

2018 FP Notebook

78. 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

2018 FP Notebook

79. 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

2018 FP Notebook

80. How to Optimize Activated Partial Thromboplastin Time (APTT) Testing: Solutions to Establishing and Verifying Normal Reference Intervals and Assessing APTT Reagents for Sensitivity to Heparin, Lupus Anticoagulant, and Clotting Factors. (PubMed)

in this pathway, most typically factors VIII, IX, and XI. The APTT is also sensitive to contact factor deficiencies, including factor XII, prekallikrein, and high-molecular-weight kininogen. The APTT may also be elevated in a variety of conditions, including liver disease, vitamin K deficiency, and disseminated intravascular coagulation. The APTT can also be used for monitoring unfractionated heparin (UFH) therapy, as well as for screening lupus anticoagulant (LA) or for assessing thrombosis risk. Which (...) How to Optimize Activated Partial Thromboplastin Time (APTT) Testing: Solutions to Establishing and Verifying Normal Reference Intervals and Assessing APTT Reagents for Sensitivity to Heparin, Lupus Anticoagulant, and Clotting Factors. The activated partial thromboplastin time (APTT) assay is a very common coagulation test, used for several reasons. The test is conventionally used for assessing the contact factor (intrinsic) pathway of blood coagulation, and thus for screening deficiencies

2019 Seminars In Thrombosis And Hemostasis

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