scholarly journals Congenital Factor Deficiencies in Children: A Report of a Single-Center Experience

2017 ◽  
Vol 24 (6) ◽  
pp. 901-907 ◽  
Author(s):  
Zafer Şalcıoğlu ◽  
Cengiz Bayram ◽  
Hülya Şen ◽  
Gizem Ersoy ◽  
Gönül Aydoğan ◽  
...  
Keyword(s):  
Hemophilia A ◽  
Coagulation Factors ◽  
Von Willebrand Disease ◽  
Hemophilia B ◽  
Pediatric Hematology ◽  
Single Center Experience ◽  
Von Willebrand ◽  
Collaborative Efforts ◽  
Congenital Factor

Congenital factor deficiencies (CFDs) refer to inherited deficiency of coagulation factors in the blood. A total of 481 patients with CFDs, who were diagnosed and followed at our Pediatric Hematology and Oncology Clinic between 1990 and 2015, were retrospectively evaluated. Of the 481 cases, 134 (27.8%) were hemophilia A, 38 (7.9%) were hemophilia B, 57 (11.8%) were von Willebrand disease (vWD), and 252 (52.3%) were rare bleeding disorders (RBDs). The median age of the patients at the time of diagnosis and at the time of the study was 4.1 years (range: 2 months to 20.4 years) and 13.4 years (range: 7 months to 31.3 years), respectively. The median duration of the follow-up time was 6.8 years (range: 2.5 months to 24.8 years). One hundred nineteen (47.2%) of 252 patients with RBDs were asymptomatic, 49 (41.1%) of whom diagnosed by family histories, 65 (54.6%) through preoperative laboratory studies, and 5 (4.2%) after prolonged bleeding during surgeries. Consanguinity rate for the RBDs was 47.2%. Prophylactic treatment was initiated in 80 patients, 58 of whom were hemophilia A, 7 were hemophilia B, 13 were RBDs, and 2 were vWD. Significant advances have been achieved during the past 2 decades in the treatment of patients with CFDs, particularly in patients with hemophilias. The rarity and clinical heterogeneity of RBDs lead to significant diagnostic challenges and improper management. In this regard, multinational collaborative efforts are needed with the hope that can improve the management of patients with RBDs.

scholarly journals Dental extractions in patients of mild level hemophilia A and hemophilia B and von Willebrand disease without clotting factor supplementation

2018 ◽  
Author(s):  
Bogumił Lewandowski ◽  
Joanna Wojnar ◽  
Robert Brodowski ◽  
Marta Mucha ◽  
Ewelina Czenczek-Lewandowska ◽  
...  
Keyword(s):  
Hemophilia A ◽  
Von Willebrand Disease ◽  
Hemophilia B ◽  
Clotting Factor ◽  
Von Willebrand

scholarly journals Protein Replacement Therapy and Gene Transfer in Canine Models of Hemophilia A, Hemophilia B, von Willebrand Disease, and Factor VII Deficiency

ILAR Journal ◽  
2009 ◽  
Vol 50 (2) ◽  
pp. 144-167 ◽  
Author(s):  
T. C. Nichols ◽  
A. M. Dillow ◽  
H. W. G. Franck ◽  
E. P. Merricks ◽  
R. A. Raymer ◽  
...  
Keyword(s):  
Gene Transfer ◽  
Replacement Therapy ◽  
Hemophilia A ◽  
Von Willebrand Disease ◽  
Hemophilia B ◽  
Factor Vii ◽  
Factor Vii Deficiency ◽  
Canine Models ◽  
Von Willebrand

Transient Ischemic Attack in a Hemophilia Patient with Severe Preeclampsia after Preoperative Administration of Tranexamic Acid and Factor VIII Replacement for Cesarean Section

2021 ◽  
Vol 9 (2) ◽  
Author(s):  
D’Onofrio JD ◽  
◽  
Hoffman CR ◽  
Goldberg SF ◽  
◽  
...  
Keyword(s):  
Tranexamic Acid ◽  
Transient Ischemic Attack ◽  
Hemophilia A ◽  
Coagulation Factors ◽  
Background Information ◽  
Preoperative Administration ◽  
Increased Risk ◽  
Pharmacologic Prophylaxis ◽  
Ischemic Attack ◽  
Von Willebrand

Hemophilia A in females accounts for few cases due to hemophilia A and B having X-linked recessive inheritance patterns. Hemostatic changes in pregnancy include an increase in coagulation factors and von Willebrand activity, placing hemophilia patients at an increased risk for Postpartum Hemorrhage (PPH). General recommendations include considering pharmacologic prophylaxis, including tranexamic acid and factor replacement when necessary. The ultimate goal is to prevent uncontrolled bleeding during vaginal or operative delivery. Benefits of prophylactic therapies must be weighed with relevant risk profiles of each intervention. We present a case where a parturient with hemophilia prophylactically treated with TXA and FVIII experienced a transient ischemic attack. We discuss the background information known regarding tranexamic acid and factor replacement, and the subsequent recommendations for their use in this patient population. We consider recommendations to expand the multidisciplinary team incorporated in the assessment and planning for the peripartum care of such a patient.

The Most Prominent Hemophilia Clotting Factors in the Province of Nineveh

2021 ◽  
pp. 85-91
Author(s):  
Salih khudhair Abdullah ◽  
Asmaa Mohammed Khaleel ◽  
Khalid Satam Sultan
Keyword(s):  
Von Willebrand Disease ◽  
Clotting Factor ◽  
Recessive Mutation ◽  
Clotting Factors ◽  
Ibn Sina ◽  
Genetic History ◽  
Study Results ◽  
Different Types ◽  
Von Willebrand

Background: Hemophilia is a recessive mutation in X-linked chromosome. Hemophilia A is characterized by a deficiency of clotting factor F-VIII. Hemophilia B is characterized by a deficiency of clotting factor F-IX. Fibrin Stabilizer is a deficiency of F-XIII. Alexander's disease is a deficiency of clotting factor F-VII. Von Willebrand disease is a deficiency of clotting factor VWF. Afibrinogenemia is a deficiency of clotting factor F-I. Aim: This study amid to find out prevalence of deficiency clotting factors in Nineveh province. Methods: This research was conducted at Ibn-Sina Teaching Hospital. Staco special kits were used to determine factors under the study. Results: 365 out of 829 total patients have been detected deficiency in one or more of different types of factors. The most prevalence of deficiency factors in Nineveh are F-VIII, FIX and VWF. Infected males are more than females. The ages between 1-20 years and blood groups (A⁺, B⁺, and O⁺) are most prevalent. Conclusions: It is necessary to monitor patients during the initial disease, follow it up, and use effective treatment methods to limit the increased number of cases. Moreover, it is necessary to follow up on the family's genetic history to avoid new infections.

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