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Subarachnoid Hemorrhage in Congenital Factor X Deficiency: A Case Study and Literature Review Publisher



Mohammadi S1 ; Torab Z2 ; Aghakhani S3 ; Ghalandari M4 ; Mohammadimanesh R5 ; Asgary V6 ; Aligoudarzi SL7 ; Younesi MR8
Authors
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Authors Affiliations
  1. 1. Central Medical Laboratory, Ayatollah Taleghani Hospital, Tehran, Iran
  2. 2. Hematology and Oncology Research Center, Tabriz University of Medical Sciences, Tabriz, Iran
  3. 3. Faculty of Biological Science, Islamic Azad University, North-Tehran Branch, Tehran, Iran
  4. 4. Emergency Medicine Specialist, Department of Emergency Medicine, Ayatollah Taleghani Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  5. 5. Department of Chemical Engineering, Biotechnology Faculty of Engineering, Payame Noor University, Tehran, Iran
  6. 6. Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
  7. 7. Mehr General Laboratories, Tehran, Iran
  8. 8. Department of Hematology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran

Source: Iranian Red Crescent Medical Journal Published:2016


Abstract

Background: Inborn factor X deficiency (FXD) is a very rare (1: 500,000) hereditary coagulation disorder, which is characterized by clinical manifestations including hematoma, epistaxis, menorrhagia, ecchymosis, and central nervous system (CNS) or gastrointestinal (GI) bleeding (depending on the zygosity). In homozygote patients, the risk of spontaneous intracranial hemorrhage (ICH) is high. Objectives: The aim of this investigation was to study and long-term follow-up of the patients with FXD and ICH. In addition, we investigated their frequent bleeding symptoms throughout their life and the results were compared with results of other studies. Patients and Methods: This study investigated2 cases with spontaneous intracranial hemorrhage in patients with severe congenital (factor X) FX deficiency including a3-year-old boy and a1-month-old female neonate. The world literature was explored through the PubMed Medline and Scopus using appropriate and pertinent key words. Results: The Patients referred to the hematology department due to the neurological complications such as vomiting, unconsciousness, prolonged nasal bleeding for recent12 hours. They had no familial history of spontaneous CNS bleeding. The blood coagulation test analysis indicated a prolonged activated partial thromboplastin time (APTT) and also revealed a prolonged prothrombin time (PT) and the low levels of coagulation factor X implicating severe congenital FX deficiency. They followed up by our hematologists to prevent intracranial hemorrhage. Discussions: As one ICH patient whose PT and aPTT suggest a coagulation disorder secondary to vitamin K deficiency or coagulation factor deficiency, unresponsiveness to vitamin K therapy should be useful to take FX deficiency into consideration. © 2016, Iranian Red Crescent Medical Journal.
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