Publications by authors named "F A Ala"
Background: Prophylaxis the current standard care for patients with severe hemophilia should be planned to optimize the replacement therapy and minimize bleeding. We report our single-center experience of tailored prophylaxis in children affected by hemophilia A (HA) and hemophilia B (HB).
Methods: This study was conducted on 55 patients, under 15 years, with HA (PWHA, n: 46) and HB (PWHB, n: 9) between 2015 and 2019.
Article Synopsis
- Congenital combined bleeding disorders (CBDs) are rare and often related to consanguineous marriages, presenting symptoms from minor bleeding to severe complications.
- The study evaluated 450 patients at the Iranian Comprehensive Hemophilia Care Center, identifying FV-FVII deficiency and FVII-FX deficiency as the most prevalent types among the analyzed cases.
- Findings highlighted that a significant number of patients experienced postoperative bleeding, and the majority received on-demand treatment rather than preventative care, indicating a need for better management strategies in Iran.
Type 2 von Willebrand disease (VWD) is the most common congenital bleeding disorder, with variable bleeding tendency and a complex laboratory phenotype. In the current study, we report the clinical and molecular profile of a large number of Iranian patients with type 2 VWD. All exons, intron-exon boundaries, and untranslated regions were sequenced by Sanger sequencing for direct mutation detection.
View Article and Find Full Text PDF: Quantitative and/or qualitative defects of the platelet membrane glycoprotein IIb/IIIa complex lead to the clinical entity of Glanzmann's thrombasthenia. A large variety of mutations and polymorphisms are responsible for the aberrant expression and defective activity of this heterodimeric complex. The current study aimed to determine the pattern of mutations among Iranian population with Glanzmann's thrombasthenia.
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