ArticleInternational journal of hematology2014
Clinical manifestations and management of life-threatening bleeding in the largest group of patients with severe factor XIII deficiency.
Article in International journal of hematology, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers.
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Who cites it
27 citing papers in PubMed, 74 citations in OpenAlex.
- Identification of an F13A1 frameshift variant associated with factor XIII deficiency in a Coonhound dog with severe coagulopathy.Journal of veterinary internal medicine · 2026Article
- Exploration of the Pathogenic Mechanism of the Factor XIII A Subunit in a Patient With Congenital Factor XIII Deficiency.Molecular genetics & genomic medicine · 2026Article
- Recurrent Subdural Hematoma Revealing Undiagnosed Mild Hemophilia A and Factor XIII Deficiency in an Elderly Patient.Cureus · 2025Article
- Clinical Profile of Congenital Factor XIII Deficiency in Children.Indian journal of pediatrics · 2024Article
- Neurological Complications Associated with Hereditary Bleeding Disorders.Current neurology and neuroscience reports · 2023Review
- Identification of a novel mutation in the factor XIII A subunit in a patient with inherited factor XIII deficiency.International journal of hematology · 2023Article
- A multicenter, real-world experience with recombinant FXIII for the treatment of patients with FXIII deficiency: from pharmacokinetics to clinical practice. The Italian FXIII Study.Blood transfusion = Trasfusione del sangue · 2023Article
- Delayed and prolonged umbilical stump bleeding in a Caucasian newborn as a presenting feature of factor XIII deficiency.BMJ case reports · 2022Article
- Immunoglobulin A vasculitis without purpura in an elderly female patient: a case report.Clinical journal of gastroenterology · 2021Article
- Factor XIII-A: An Indispensable "Factor" in Haemostasis and Wound Healing.International journal of molecular sciences · 2021Review
- Hemorrhagic Shock after Neonatal Circumcision: Severe Congenital Factor XIII Deficiency.Case reports in pediatrics · 2021Article
- A case of life-threatening small intestinal bleeding accompanied by lower coagulation factor XIII activity.Clinical journal of gastroenterology · 2020Article
- How to Assess Founder Effect in Patients with Congenital Factor XIII Deficiency.International journal of hematology-oncology and stem cell research · 2020Review
- Pubertal Menorrhagia - A Rare Presentation of Congenital Factor XIII Deficiency.Indian pediatrics · 2020Article
- Management of Neuraxial Analgesia in a Parturient with Factor XIII Deficiency: A Case Report and Proposed Management Algorithm.Case reports in anesthesiology · 2020Article
- Cellular Factor XIII, a Transglutaminase in Human Corneal Keratocytes.International journal of molecular sciences · 2019Article
- Novel Factor XIII variant identified through whole-genome sequencing in a child with intracranial hemorrhage.Cold Spring Harbor molecular case studies · 2018Article
- Molecular Basis of Congenital Factor XIII Deficiency in Iran.Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis · 2018Article
- Advances of Coagulation Factor XIII.Chinese medical journal · 2017Review
- Recurrent Post Tonsillectomy Secondary Hemorrhage in Patients with Factor XIII Deficiency: A Case Series and Review of Literature.The American journal of case reports · 2016Article
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Authors and funding
7 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Factor XIII (FXIII) deficiency is a rare hemorrhagic disorder for which the highest incidence occurs in southeast Iran. The aim of this study was to assess molecular characteristics, clinical manifestations and management of life-threatening diathesis in FXIII deficiency. This study was conducted on 190 patients with FXIII deficiency. Genotype analysis for the most frequent mutation of FXIII-A subunit gene in Iranian, Trp187Arg, was performed for all patients. Clinical manifestations and management of patients with intracranial hemorrhage (ICH), miscarriage and neonates with FXIII deficiency were documented. Neonates were divided in two groups: Group 1 received a standard dose of Fibrogammin P(®) (10-26 IU/Kg) and group 2 received a high dose of this drug (60-80 IU/Kg) for 36 months. Bleeding episodes in both groups were recorded, and neonates of group 2 were regularly checked for thrombotic events. Molecular analysis revealed that all patients were homozygous for Trp187Arg mutation. Umbilical bleeding, hematoma and prolonged wound bleeding were common presentations. ICH was another common presentation leading to behavioral and developmental disorders and aphasia. ICH was managed by Fibrogammin P(®) at a dose of 10-26 IU/Kg, and miscarriage was managed by Fibrogammin P(®) at a dose of 10 IU/Kg every 2 weeks during pregnancy, and the same dose administered as prophylaxis before gestation every 4 weeks. Neonates of group 2 received 60-80 IU/kg dose of Fibrogammin P(®). This higher dose did not trigger thrombotic events but significantly decreased bleeding episodes and prevented the occurrence of major bleeding. Trp187Arg is the most common mutation of FXIII-A subunit in Iran, and Fibrogammin P(®) is effective in the management of FXIII deficiency, and higher dose of this drug is safe and effective in neonates.
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