U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Hemophilia A with vascular abnormality

MedGen UID:
336105
•Concept ID:
C1844137
•
Disease or Syndrome
Synonym: Hemophilia A with Vascular Abnormality
 
Monarch Initiative: MONDO:0010603
OMIM®: 306800

Recent clinical studies

Etiology

The hypertension of hemophilia is associated with vascular remodeling in the joint.
Barnes RFW, Cramer TJ, Hughes TH, von Drygalski A
Microcirculation 2017 Oct;24(7) doi: 10.1111/micc.12387. PMID: 28627086
Procoagulant activity in hemostasis and thrombosis: Virchow's triad revisited.
Wolberg AS, Aleman MM, Leiderman K, Machlus KR
Anesth Analg 2012 Feb;114(2):275-85. Epub 2011 Nov 21 doi: 10.1213/ANE.0b013e31823a088c. PMID: 22104070Free PMC Article
Vascular haemostasis.
Key NS, DE Paepe A, Malfait F, Shovlin CL
Haemophilia 2010 Jul;16 Suppl 5:146-51. doi: 10.1111/j.1365-2516.2010.02313.x. PMID: 20590874
Future directions in hemostasis: normalizing the lives of patients with hemophilia.
Carr ME Jr
Thromb Res 2010 Apr;125 Suppl 1:S78-81. Epub 2010 Feb 16 doi: 10.1016/j.thromres.2010.01.044. PMID: 20163836
Inherited bleeding disorders.
Blanchette VS, Sparling C, Turner C
Baillieres Clin Haematol 1991 Apr;4(2):291-332. doi: 10.1016/s0950-3536(05)80162-3. PMID: 1912663

Diagnosis

Joint status, pain and quality of life in elderly people with haemophilia: A case-control study.
Chantrain VA, Foubert A, Meeus M, Lambert C, Lobet S, Maes P, Fransen E, Durnez L, Hermans C, Roussel NA
Haemophilia 2023 Nov;29(6):1621-1632. Epub 2023 Oct 20 doi: 10.1111/hae.14890. PMID: 37861076
Severe haemophilia a in a preterm girl with turner syndrome - a case report from the prenatal period to early infancy (part I).
Berendt A, Wójtowicz-Marzec M, Wysokińska B, Kwaśniewska A
Ital J Pediatr 2020 Sep 7;46(1):125. doi: 10.1186/s13052-020-00892-7. PMID: 32894158Free PMC Article
Vascular haemostasis.
Key NS, DE Paepe A, Malfait F, Shovlin CL
Haemophilia 2010 Jul;16 Suppl 5:146-51. doi: 10.1111/j.1365-2516.2010.02313.x. PMID: 20590874
Molecular mechanisms of mild and moderate hemophilia A.
Jacquemin M, De Maeyer M, D'Oiron R, Lavend'Homme R, Peerlinck K, Saint-Remy JM
J Thromb Haemost 2003 Mar;1(3):456-63. doi: 10.1046/j.1538-7836.2003.00088.x. PMID: 12871450
Inherited bleeding disorders.
Blanchette VS, Sparling C, Turner C
Baillieres Clin Haematol 1991 Apr;4(2):291-332. doi: 10.1016/s0950-3536(05)80162-3. PMID: 1912663

Therapy

Perioperative Hemostatic Management of a Newborn With Hereditary Hemophilia A Undergoing Emergent Surgery for Dextro-Transposition of the Great Arteries.
Torfs A, Chardonnal L, Meunier S, Désage S, Henaine R, Lilot M
J Cardiothorac Vasc Anesth 2022 Oct;36(10):3855-3858. Epub 2022 Jun 19 doi: 10.1053/j.jvca.2022.06.018. PMID: 35879147
Newly Diagnosed Acquired Hemophilia A Manifesting as Massive Intracranial Hemorrhage Following a Neurosurgical Procedure.
Akamatsu Y, Hayashi T, Yamamoto J, Karibe H, Kameyama M, Tominaga T
World Neurosurg 2018 Mar;111:175-180. Epub 2017 Dec 15 doi: 10.1016/j.wneu.2017.12.016. PMID: 29253693
Desmopressin-related myocardial infarction in a patient with Wegener's granulomatosis: a case report and review of the literature.
Pape E, Béné J, Buchdahl AL, Gautier S, Hatron PY, Lambert M
J Mal Vasc 2013 Feb;38(1):43-6. Epub 2012 Dec 21 doi: 10.1016/j.jmv.2012.11.001. PMID: 23265678
Future directions in hemostasis: normalizing the lives of patients with hemophilia.
Carr ME Jr
Thromb Res 2010 Apr;125 Suppl 1:S78-81. Epub 2010 Feb 16 doi: 10.1016/j.thromres.2010.01.044. PMID: 20163836
Inherited bleeding disorders.
Blanchette VS, Sparling C, Turner C
Baillieres Clin Haematol 1991 Apr;4(2):291-332. doi: 10.1016/s0950-3536(05)80162-3. PMID: 1912663

Prognosis

Severe haemophilia a in a preterm girl with turner syndrome - a case report from the prenatal period to early infancy (part I).
Berendt A, Wójtowicz-Marzec M, Wysokińska B, Kwaśniewska A
Ital J Pediatr 2020 Sep 7;46(1):125. doi: 10.1186/s13052-020-00892-7. PMID: 32894158Free PMC Article
Vascular Permeability and Remodelling Coincide with Inflammatory and Reparative Processes after Joint Bleeding in Factor VIII-Deficient Mice.
Cooke EJ, Zhou JY, Wyseure T, Joshi S, Bhat V, Durden DL, Mosnier LO, von Drygalski A
Thromb Haemost 2018 Jun;118(6):1036-1047. Epub 2018 May 30 doi: 10.1055/s-0038-1641755. PMID: 29847841Free PMC Article
Desmopressin-related myocardial infarction in a patient with Wegener's granulomatosis: a case report and review of the literature.
Pape E, Béné J, Buchdahl AL, Gautier S, Hatron PY, Lambert M
J Mal Vasc 2013 Feb;38(1):43-6. Epub 2012 Dec 21 doi: 10.1016/j.jmv.2012.11.001. PMID: 23265678
Procoagulant activity in hemostasis and thrombosis: Virchow's triad revisited.
Wolberg AS, Aleman MM, Leiderman K, Machlus KR
Anesth Analg 2012 Feb;114(2):275-85. Epub 2011 Nov 21 doi: 10.1213/ANE.0b013e31823a088c. PMID: 22104070Free PMC Article
Molecular mechanisms of mild and moderate hemophilia A.
Jacquemin M, De Maeyer M, D'Oiron R, Lavend'Homme R, Peerlinck K, Saint-Remy JM
J Thromb Haemost 2003 Mar;1(3):456-63. doi: 10.1046/j.1538-7836.2003.00088.x. PMID: 12871450

Clinical prediction guides

Joint status, pain and quality of life in elderly people with haemophilia: A case-control study.
Chantrain VA, Foubert A, Meeus M, Lambert C, Lobet S, Maes P, Fransen E, Durnez L, Hermans C, Roussel NA
Haemophilia 2023 Nov;29(6):1621-1632. Epub 2023 Oct 20 doi: 10.1111/hae.14890. PMID: 37861076
Vascular Permeability and Remodelling Coincide with Inflammatory and Reparative Processes after Joint Bleeding in Factor VIII-Deficient Mice.
Cooke EJ, Zhou JY, Wyseure T, Joshi S, Bhat V, Durden DL, Mosnier LO, von Drygalski A
Thromb Haemost 2018 Jun;118(6):1036-1047. Epub 2018 May 30 doi: 10.1055/s-0038-1641755. PMID: 29847841Free PMC Article
The hypertension of hemophilia is associated with vascular remodeling in the joint.
Barnes RFW, Cramer TJ, Hughes TH, von Drygalski A
Microcirculation 2017 Oct;24(7) doi: 10.1111/micc.12387. PMID: 28627086
Procoagulant activity in hemostasis and thrombosis: Virchow's triad revisited.
Wolberg AS, Aleman MM, Leiderman K, Machlus KR
Anesth Analg 2012 Feb;114(2):275-85. Epub 2011 Nov 21 doi: 10.1213/ANE.0b013e31823a088c. PMID: 22104070Free PMC Article
Molecular mechanisms of mild and moderate hemophilia A.
Jacquemin M, De Maeyer M, D'Oiron R, Lavend'Homme R, Peerlinck K, Saint-Remy JM
J Thromb Haemost 2003 Mar;1(3):456-63. doi: 10.1046/j.1538-7836.2003.00088.x. PMID: 12871450

Supplemental Content

Table of contents

    Clinical resources

    Consumer resources

    Reviews

    Related information

    • GTR
      Related information in GTR
    • GTR(Clinical)
      Clinical tests in GTR
    • MeSH
      Related Medical Subject Headings
    • OMIM
      Related records in OMIM
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed
    • PubMed (OMIM)
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...