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Hemophilia A

Aka: Hemophilia A, Hemophilia, Factor VIII Deficiency
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  1. Epidemiology
    1. Most common hereditary coagulation disorder (1:10,000)
  2. Pathophysiology
    1. Sex linked recessive deficiency of factor VIII
  3. Symptoms
    1. Excessive bleeding from slight trauma
      1. Dental extraction
      2. Surgery
    2. Visceral bleeding
    3. Hemarthrosis
      1. Contractures
      2. Degenerative arthritis
  4. Labs
    1. Partial Thromboplastin Time (PTT) elevated
    2. ProTime normal
  5. Management: Factor VIII replacement
    1. Indications
      1. Acute Bleeding
      2. Before surgical procedure
    2. Dosing
      1. Mild Bleeding
        1. Give Factor VIII to obtain 15% Factor VIII level
      2. Severe Bleeding
        1. Give Factor VIII to obtain 50% Factor VIII level

Hemophilia A (C0019069)

Definition (NCI) An inherited deficiency of coagulation factor VIII characterized by the tendency to spontaneous or exaggerated post-traumatic hemorrhage. Inherited as an x-linked recessive disease, hemophilia A is the most common hemophilia, occurring in approximately 1 in 10,000 male births.
Definition (MSH) The classic hemophilia resulting from a deficiency of factor VIII. It is an inherited disorder of blood coagulation characterized by a permanent tendency to hemorrhage.
Definition (CSP) classic hemophilia resulting from a deficiency of factor VIII; an inherited disorder of blood coagulation characterized by a permanent tendency to hemorrhage.
Concepts Disease or Syndrome (T047)
MSH D006467
ICD9 286.0
ICD10 D66
SnomedCT 28293008, 234440005
English Hemophilia, Congenital factor VIII disorder, Sex-linked factor VIII deficiency disease, Hemophilia A, AHG DEFICIENCY, ANTIHEMOPHILIC FACTOR A DEFICIENCY, ANTIHEMOPHILIC GLOBULIN DEFICIENCY, Deficiencies, Factor VIII, Deficiency, Factor VIII, Factor VIII Deficiencies, Factor VIII Deficiency, HEMOPHILIA A, Hemophilia A, NOS, Cong factor VIII deficiency, Haemophilia A, NOS, Hereditary factor VIII deficiency, hemophilia A, FACTOR VIII DEFICIENCY, AUTOSOMAL HEMOPHILIA A, DEFIC FACTOR VIII, FACTOR VIII DEFIC, Haemophilia, factor VIII deficiency (hemophilia), clotting factor deficiency factor VIII (hemophilia A), factor VIII deficiency, factor VIII deficiency (diagnosis), Haemophilia A (Factor VIII), Hemophilia A (Factor VIII), FACTOR VIII DISORDER CONGENITAL, Hemophilia A (disorder), Hereditary factor VIII deficiency disease (disorder), Haemophilia A, Hereditary factor VIII deficiency disease, Cong factor viii diord, Hemophilias, Classic, Factor 8 Deficiency, Congenital, Factor VIII Deficiency, Congenital, Congenital Hemophilia A, Classic Hemophilia, Classic Hemophilias, Congenital Hemophilia As, Hemophilia A, Congenital, Hemophilia, Classic, Hemophilia As, Congenital, Hemophilia A [Disease/Finding], haemophilia a, congenital factor viii disorder, hemophilia a, factor viii deficiency, factor viii hemophilia, Hemophilia NOS, A, Hemophilia, As, Hemophilia, Hemophilia As, Classical hemophilia, AHG deficiency disease, Congenital factor VIII deficiency disease, Sex-linked factor VIII deficiency, Congenital factor VIII deficiency, Classical haemophilia, Factor VIII deficiency, Factor VIII deficiency (disorder), A; hemophilia, classical; hemophilia, deficiency; anti-hemophilic globulin, hemophilia; A, hemophilia; classical, anti-hemophilic globulin; deficiency, AHG deficiency, Antihemophilic globulin deficiency, Hemophilia, classical, Functional factor VIII deficiency, Hemophilia, familial, Hemophilia, hereditary, Subhemophilia
Italian Deficit di Fattore VIII, Disturbi congeniti del fattore VIII, Emofilia A (Fattore VIII), Deficienza del fattore VIII, Emofilia, Emofilia A
Dutch hemofilie A (factor VIII), hemofilie A, congenitale factor VIII-deficiëntie, A; hemofilie, anti-hemofilieglobuline; deficiëntie, deficiëntie; anti-hemofilieglobuline, hemofilie; A, hemofilie; klassiek, klassiek; hemofilie, Hereditaire factor VIII-deficiëntie, factor VIII-deficiëntie, Hemofilie, Hemofilie A, Factor-VIII-deficiëntie
French Hémophilie A (facteur VIII), Trouble congénital du facteur VIII, Déficit en facteur anti-hémophilique A, Hémophilie A, Déficit en FVIII, Déficit en facteur VIII, Déficit en facteur antihémophilique A, Déficit héréditaire en facteur VIII, Hémophilie classique
German Haemophilie A, Haemophilie A (Faktor VIII), kongenitaler Faktor VIII-Mangel, Hereditaerer Faktor-VIII-Mangel, Faktor VIII-Mangel, Faktor-VIII-Mangel, Hämophilie A, Hämophilie
Portuguese Hemofilia A (factor VIII), Hemofilia A (Factor VIII), Perturbação congénita do factor VIII, Carência de factor VIII, Deficiência do Fator VIII, Hemofilia A, Hemofilia
Spanish Hemofilia A (Factor VIII), Trastorno congénito del factor VIII, hemofilia A, enfermedad de deficiencia del factor congénito VIII, enfermedad por deficiencia hereditaria de factor VIII (trastorno), enfermedad por deficiencia hereditaria de factor VIII, deficiencia congénita de factor VIII, hemofilia A (trastorno), deficiencia de factor VIII (trastorno), deficiencia de factor VIII, deficiencia hereditaria de factor VIII ligada al sexo, deficiencia hereditaria de factor VIII, hemofilia clásica, Déficit de factor VIII, Hemofilia A, Deficiencia de Factor VIII, Hemofilia
Japanese 第ＶＩＩＩ因子欠乏症, 血友病Ａ, 先天性第ＶＩＩＩ因子障害, 血友病Ａ（第ＶＩＩＩ因子）, ﾀﾞｲ8ｲﾝｼｹﾂﾎﾞｳｼｮｳ, ｾﾝﾃﾝｾｲﾀﾞｲ8ｲﾝｼｼｮｳｶﾞｲ, ｹﾂﾕｳﾋﾞｮｳAﾀﾞｲ8ｲﾝｼ, ｹﾂﾕｳﾋﾞｮｳA, 血友病, A型血友病, 第8因子欠乏症, 第VIII因子欠乏症, 血友病A, 血友病症, 血液凝固第8因子欠乏, 血液凝固第8因子欠乏症, 血液凝固第VIII因子欠乏症
Swedish Blödarsjuka
Czech hemofilie A, Deficit faktoru VIII, Hemofilie A (deficit faktoru VIII), Vrozená porucha faktoru VIII, Hemofilie A
Finnish Hemofilia A
Russian GEMOFILIIA A, FAKTORA VIII NEDOSTATOCHNOST', GEMOFILIIA, ГЕМОФИЛИЯ A, ГЕМОФИЛИЯ, ФАКТОРА VIII НЕДОСТАТОЧНОСТЬ
Korean 유전성 제8인자 결핍증
Croatian HEMOFILIJA A
Polish Hemofilia A, Krwawiączka, Niedobór czynnika VIII, Hemofilia
Hungarian A-típusú haemophilia, A-típusú haemophilia (VIII-faktor), Veleszületett VIII-faktor-betegség, Haemophilia A (faktor VIII), Haemophilia A, Faktor VIII hiány
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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