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Article
A FAMILY WITH FACTOR XIII DEFICIENCY CASE REPORT AND LITERATURE REVIEW

Author: Zuhair Al-Barazanchi
Journal: Basrah Journal of Surgery مجلة البصرة الجراحية ISSN: 16833589 / ONLINE 2409501X Year: 2006 Volume: 12 Issue: 1 Pages: 92
Publisher: Basrah University جامعة البصرة

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Abstract

Factor XIII deficiency is a rare disorder of coagulation characteri-zed by moderate to severe bleeding tendency with increased susceptibility to intracranial hemorrhages, almost normal coagulation screening tests, clot lysis in 5 M urea solution & a tendency to abnormal wound healing. It is inherited as an autosomal recessive trait. However, acquired causes for its deficiency are rarely encountered. We describe here an inherited form of F XIII deficiency in two members (a boy & a girl) in a one family in Basrah.


Article
Review of Congenital Factor XIII Deficiency in Single Iraqi Teaching Hospital

Authors: Lubna Foad Hussain --- Obeida Amir Abid
Journal: Iraq Joural of Hematology المجلة العراقية لامراض الدم ISSN: 20728069/25432702 Year: 2016 Volume: 5 Issue: 1 Pages: 81-89
Publisher: Al-Mustansyriah University الجامعة المستنصرية

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Abstract

Background: Factor XIII deficiency is one of the rarest bleeding disorders with an estimatedprevalence of 1in 3 million populations worldwide. The main clinical manifestations of thedisease are delayed wound healing, recurrent miscarriage, intracranial bleeding, andprolonged umbilical cord bleeding.Objectives: The aim of this study was to assess the diagnosis and treatment of factor XIIIdeficiency in Children Welfare teaching hospital in Baghdad.Patients and Methods: This retrospective study was performed on thirty three patients withthe severe factor XIII deficiency. The diagnosis of the disease was done by a wide spectrumof characteristics which is part of inclusion criteria and including family history, clinicalmanifestations, laboratory tests, clot solubility in 5 M urea or monochloroacetic acidenvironments.Results: the common manifestations of the disease at time of diagnosis were bleeding aftertrauma (42.4%), umbilical cord bleeding (21.2%)and less frequently gum bleeding andecchymosis.Conclusions: factor XIII deficiency is a rare disease in Iraq, most patients are diagnosed atthe age of 1-10 years, family history was positive in more than half of the patients.


Article
Molecular Study of the Correlation between Hemophilia and the Thrombophilic Risk Factors in Dohuk Province by using CVD Strip Assay.

Author: Rana Adel Hanoon
Journal: Medical Journal of Babylon مجلة بابل الطبية ISSN: 1812156X 23126760 Year: 2017 Volume: 14 Issue: 4 Pages: 670-675
Publisher: Babylon University جامعة بابل

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Abstract

Haemophilia A is an X-linked, recessively inherited bleeding disorder which results from deficiency of procoagulant factor VIII (FVIII). Forty hemophilic patients were enrolled in the current study. Blood sample were collected and DNA was extracted and subjected to multiplex PCR amplification using specific primers which resulted in bands containing all the suspected mutations associated with hemophilia; CVD Strip Assay was also used to differentiate the obtained mutations. The results showed that out of the (40) hemophilic individuals, the higher incidence (55%) was for the MTHFR (A1298C) heterozygotic type of mutation while the lower incidence (5%) for a mutation recorded for factor V (G1691A, leiden), factor V (H1299R, R2), prothrombin (G20210A), and facto XIII (V34L). For the Apo type mutation, B type hasn’t been detected while E type observed in all cases; A1/A2 mutation created the higher incidence with 25% on the other hand, A3/A3 hasn’t been detected and thus it represented 0% of all the Apo mutations

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