• Users Online: 594
  • Home
  • Print this page
  • Email this page
Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Subscribe Contacts Login 
ORIGINAL ARTICLE
Year : 2021  |  Volume : 10  |  Issue : 1  |  Page : 23-27

Congenital fibrinogen deficiency in Hemophilia Center Medical City/Baghdad


Department of Pediatrics, Children Welfare Teaching Hospital, Baghdad, Iraq

Correspondence Address:
Dr. Afrah A Salih
Department of Pediatrics, Children Welfare Teaching Hospital, Baghdad
Iraq
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijh.ijh_2_21

Rights and Permissions

CONTEXT: Congenital fibrinogen deficiency is a rare inherited coagulation disorder with an estimated prevalence of 1:1,000,000 which is characterized by bleeding that varies from mild to severe and by an extremely low level or complete absence of plasma and platelet fibrinogen. AIMS: This study aims to assess prevalence, demographic parameters, clinical presentation, and hemostatic values in patients diagnosed with congenital fibrinogen deficiency. SETTINGS AND DESIGN: A retrospective descriptive study of patients diagnosed with congenital fibrinogen deficiency in Hemophilia Center/Medical City/Baghdad over a period of 7 years (from August 2008 to August 2015). SUBJECTS AND METHODS: The study included patients diagnosed with congenital fibrinogen deficiency. The confirmation of diagnosis was done based on a low fibrinogen assay. All the fibrinogen deficient patients were diagnosed on basis of having fibrinogen level (<150 mg/dl). RESULTS: There were 111 cases of other coagulation factors deficiency (rare bleeding disorders) registered in the center, congenital fibrinogen deficiency represented 25 (22.5%) of them. Out of 25 patients with congenital fibrinogen deficiency, 8 patients (32%) had fibrinogen level <20 mg/dl (afibrinogenemia) and 17 patients (68%) had fibrinogen level between (20 and 150 mg/dl) (hypofibrinogenemia). The males were 17 patients (68%) and females were 8 patients (32%). The consanguinity was present in 16 (64%) and 11 patients (44%) who had a positive family history of the diseases. The patients with afibrinogenemia mostly presented with hemarthrosis (22.7%) while patients with hypofibrinogenemia mostly presented with menorrhagia (50.0%). The patients who were their symptoms manifested before the 1st year of their age most frequently developed life-threatening bleeding (central nervous system and gastrointestinal bleeding) which were observed in 4 cases. Most of the patients included in this study were without complications (80%) while just one patient (4%) died due to gastrointestinal bleeding others were complicated with viral hepatitis, hepatitis B in one patient (4%) and 3 patients (12%) developed hepatitis C. CONCLUSIONS: Congenital fibrinogen deficiency founded to be one of the most frequent rare bleeding disorders among Iraqi patients, with a high rate of consanguineous marriage and positive family history. There was a high frequency of life-threatening bleeding among patients who develop symptoms before the 1st year of their age, without notable major complications among the majority of patients.


[FULL TEXT] [PDF]*
Print this article     Email this article
 Next article
 Previous article
 Table of Contents

 Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
 Citation Manager
 Access Statistics
 Reader Comments
 Email Alert *
 Add to My List *
 * Requires registration (Free)
 

 Article Access Statistics
    Viewed1092    
    Printed16    
    Emailed0    
    PDF Downloaded93    
    Comments [Add]    

Recommend this journal