Abstract Number: PB0717
Meeting: ISTH 2021 Congress
Background: Rare bleeding disorders are group of inherited deficiencies of coagulation factors including, fibrinogen, factor II, FV, FVII, FX, FXI, and FXIII deficiency. They are largely inherited by autosomal recessive genetics and more common in areas with a high rate of consanguineous marriages.
Aims: To study the bleeding profile and outcome for Omani pediatric patients with Rare Bleeding Disorders in the period between 2010-2020.
Methods: This is a retrospective descriptive cohort study that includes all Omani children, less than 13 years of
age, who are diagnosed with rare bleeding disorders at Royal Hospital.
Patient’s data was retrieved from their electronic chart system (AlShifa). Statistical analysis was performed using SPSS 24.0
Results: Forty one patients were included, with a mean age of 4 years (range between 1 day to 12 years ) at presentation. There were 24 (58.5 %) males. The most common presenting complaint was mucocutaneous bleeding (31.7%), however, 22 (53.7%) patients were diagnosed pre- operation. Based on factor level at diagnosis, 34 (83 %) patients have factor VII deficiency, 4 (9.7%) patients have factor XIII deficiency, 1 (2.4 %) patient with fibrinogen deficiency, and 1 patient (2.4 %) has factor X deficiency. In term of treatment, only 4 ( 9.4%) patients required blood transfusion for active bleeding, 15 (36.6 % ) patients were treated on demand with tranexamic acid, and 3 (7.3%) patients were on regular prophylaxis with tranexamic acid. The mean period of follow up was 3.15 years. The majority (87.8%) of patients had no active bleeding , and only 2 (4.9 %) patients developed severe bleeding. The mean ABR outcome was 0.2, 0.5, and 7 for patients with FVII, FXIII and FX respectively.
Conclusions: Omani children with rare bleeding disorders are commonly diagnosed incidentally. They are usually do not require active treatment because their bleeding outcome is apparently favourable.
To cite this abstract in AMA style:AL Saadi M, AL Hasani H, Al Roshdi A, Al Amri A, Al Ghaithi I. Clinical Profile and Bleeding Outcome of Pediatric Rare Bleeding Disorders: The Royal Hospital’s Experience, Oman [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/clinical-profile-and-bleeding-outcome-of-pediatric-rare-bleeding-disorders-the-royal-hospitals-experience-oman/. Accessed January 23, 2022.
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