[Home ] [Archive]   [ فارسی ]  
:: Main :: About us :: Current Issue :: Archive :: Search :: Submit :: Contact ::
Main Menu
Home::
Journal Information::
Articles archive::
For Authors::
For Reviewers::
Subscription::
News& Events::
Contact us::
Site Facilities::
::
Search in website

Advanced Search
..
Receive site information
Enter your Email in the following box to receive the site news and information.
..
Indexing
                        
..
:: Volume 10, Issue 3 (Autumn 2013) ::
Sci J Iran Blood Transfus Organ 2013, 10(3): 282-288 Back to browse issues page
Factor XIII deficiency in Sistan and Baluchistan province
M. Naderi , M. Imani , P. Eshghi , A, Dorgalaleh , Sh. Tabibian , Sh. Alizadeh , E. Sanei Moghaddam , E. Miri-Moghaddam
Abstract:   (13510 Views)

  Abstract

 Background and Objectives

 Factor XIII deficiency is one of the rarest bleeding disorders with an estimated prevalence of 1 in 1-3 million in the general population. The main clinical manifestations of the disease are delayed wound bleeding, recurrent miscarriage, intracranial bleeding, and umbilical cord bleeding. The prevalence of the disease in the regions such as Sistan and Baluchistan with consanguinity marriages is higher. The aim of this study was to assess the diagnosis and treatment of factor XIII deficiency in Sistan and Baluchistan province.

 

 Materials and Methods

 This descriptive study was performed on 205 patients with the severe factor XIII deficiency. The diagnosis of the disease was done by a wide spectrum of characteristics including family history, clinical manifestations, laboratory tests, clot solubility in 5 M urea or monochloroacetic acid 1% environments.

 

 Results

 It seems that Khash city has the highest prevalence of the disease worldwide. The molecular analysis of the patients showed that an identical homozygote mutation TGG —› CGG at codon 187 in exon 4 of catalytic A subunit is responsible of this deficiency. The common manifestations of the disease at time of diagnosis were umbilical bleeding (84.4%), deep soft tissue haematoma (54.4%), and less frequently gum bleeding and finally ecchymosis.

  

 Conclusions

 It seems that Sistan and Baluchistan province has the highest prevalence of factor XIII deficiency worldwide with a dramatic increase of 46 cases in 2008 to 205 patients in 2012 that alarmed the absence of a screening test in this region.

 

 

Keywords: Factor XIII Deficiency, Blood Coagulation Disorders, Bleeding
Full-Text [PDF 294 kb]   (2534 Downloads) |   |   Full-Text (HTML)  (7676 Views)  
Type of Study: Research | Subject: Hematology
Published: 2013/10/28
Send email to the article author

Add your comments about this article
Your username or Email:

CAPTCHA


XML   Persian Abstract   Print


Download citation:
BibTeX | RIS | EndNote | Medlars | ProCite | Reference Manager | RefWorks
Send citation to:

Naderi M, Imani M, Eshghi P, Dorgalaleh A, Tabibian S, Alizadeh S, et al . Factor XIII deficiency in Sistan and Baluchistan province. Sci J Iran Blood Transfus Organ 2013; 10 (3) :282-288
URL: http://bloodjournal.ir/article-1-797-en.html


Rights and permissions
Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Volume 10, Issue 3 (Autumn 2013) Back to browse issues page
فصلنامه پژوهشی خون Scientific Journal of Iran Blood Transfus Organ
The Scientific Journal of Iranian Blood Transfusion Organization - Copyright 2006 by IBTO
Persian site map - English site map - Created in 0.05 seconds with 39 queries by YEKTAWEB 4645