XIII Deficiency (xiii + deficiency)

Distribution by Scientific Domains

Kinds of XIII Deficiency

  • factor xiii deficiency


  • Selected Abstracts


    Long-term prophylaxis in patients with factor XIII deficiency complicated by intracranial haemorrhage in Iran

    HAEMOPHILIA, Issue 2 2010
    P. ESHGHI
    No abstract is available for this article. [source]


    Intracranial haemorrhage in patients with congenital haemostatic defects

    HAEMOPHILIA, Issue 5 2008
    P. MISHRA
    Summary., We investigated 52 of 457 patients with congenital factor deficiencies with 57 episodes of intracranial haemorrhage (ICH) between 1998 and 2007. There were 38 severe haemophiliacs, 6 with factor XIII deficiency, 5 with factor X deficiency, 2 factor V-deficient patients, and 1 with type 3 von Willebrand disease (VWD). The median age was 8 years (range 1 month,22 years). Most patients were below 15 years of age (86.5%). All patients with factor X deficiency were between 1 and 5 months of age. ICH was the primary bleeding episode leading to detection of factor deficiency in 19.2% (five patients with severe haemophilia and all patients with factor X deficiency). Trauma caused bleeding in 66%. None of the patients with factor X deficiency had history of prior trauma. Surgery was performed in five patients with subdural haematomas, all of whom survived. Conservative factor replacement with 100% correction for 3 days followed by 50,60% correction for 7 days was possible in 60% patients. Seizures requiring prolonged therapy were noted in eight patients. Death was recorded in 15 patients (29%). Inadequate therapy in the form of delay or insufficient replacement was noted in 7/15 deaths. ICH was seen in 11.3% of all patients with coagulation factor deficiencies. Factor X deficiency presented with ICH at an earlier age. Inadequate replacement therapy including delayed treatment caused nearly 50% of all deaths. Most patients can be managed satisfactorily with adequate replacement therapy alone, with surgery being reserved for those with worsening neurological conditions. [source]


    A novel compound heterozygous mutation in the F13A gene causing hereditary factor XIII deficiency in a Chinese family

    JOURNAL OF THROMBOSIS AND HAEMOSTASIS, Issue 1 2006
    S. WU
    No abstract is available for this article. [source]


    Prophylactic and perioperative replacement therapy for acquired factor XIII deficiency: reply to a rebuttal

    JOURNAL OF THROMBOSIS AND HAEMOSTASIS, Issue 1 2005
    C. P. M. HAYWARD
    [source]


    Prophylactic and perioperative replacement therapy for acquired factor XIII deficiency: a rebuttal

    JOURNAL OF THROMBOSIS AND HAEMOSTASIS, Issue 11 2004
    É. Ajzner
    [source]


    Prevalence of factor XIII deficiency in patients presenting with a bleeding disorder in Pakistan

    JOURNAL OF THROMBOSIS AND HAEMOSTASIS, Issue 12 2003
    M. Anwar
    No abstract is available for this article. [source]


    Spontaneous splenic rupture in a patient with factor XIII deficiency and a novel mutation

    PEDIATRIC BLOOD & CANCER, Issue 1 2008
    Hassan Khalife MD
    Abstract We report a novel mutation in factor XIIIA gene that caused severe congenital factor XIII deficiency in a 6 year and 8 month old male. The mutation is a GA deletion in the core domain leading to a premature stop at codon 502. The child had severe deficiency with two episodes of intracerebral hemorrhage. He also developed spontaneous splenic rupture, an unusual complication of this disorder. Pediatr Blood Cancer 2008;50:113,114. © 2006 Wiley-Liss, Inc. [source]


    Spinal anaesthesia and caesarean section in a patient with hypofibrinogenaemia and factor XIII deficiency,

    ANAESTHESIA, Issue 6 2010
    A. A. Hanke
    Summary We report the peri-operative management of a 32-year-old patient suffering from symptomatic hypofibrinogenaemia and factor XIII deficiency scheduled for caesarean section. Starting with an impaired fibrinogen (1.04 g.l,1) and factor XIII level (48%), fibrinogen and factor XIII administration was guided by point-of-care rotational thrombelastometry (ROTEM®) to achieve normal whole blood coagulation, which allowed uncomplicated spinal anaesthesia and an uneventful surgical procedure. We conclude that rotational thrombelastometry may be suitable to guide administration of coagulation factors in patients with hereditary bleeding disorders and allow otherwise contraindicated neuraxial anaesthesia and surgery to proceed without increased risk of blood loss. [source]


    Valproate induces reversible factor XIII deficiency with risk of perioperative bleeding

    ACTA NEUROLOGICA SCANDINAVICA, Issue 2 2003
    B. Pohlmann-Eden
    The antiepileptic drug valproic acid (VPA) induces subclinical changes in both the intrinsic and extrinsic coagulation system. However, fatal bleeding is very rare. This study reports a 39-year-old patient who underwent selective amygdalohippocampectomy because of drug-resistant temporal lobe epilepsy. Preoperatively, the patient was on a combined therapy with VPA and topiramate, and routine coagulation laboratory parameters were entirely normal. Epilepsy surgery was immediately followed by severe intracranial bleeding events which promped repeated craniectomy. Extensive laboratory analyses revealed a factor XIII activity level of 17%, indicating factor XIII deficiency confirmed by a reduced XIIIA-antigen. After termination of treatment with VPA, factor XIII levels returned to normal. Control examinations after 9 and 24 months showed normal range values for all coagulation parameters, including factor XIII, platelet function, and von Willebrand factor. To our knowledge, this case is the first description of a well-documented, clinically relevant transient factor XIII-deficiency syndrome related to VPA treatment. [source]