Rare clotting factor deficiency among Sudanese children
| Autor: | Elamin Saed, Ishag Adam, Salma Abdallah, Ibrahium Elhadi, Omer Elhory |
|---|---|
| Rok vydání: | 2019 |
| Předmět: |
Male
Pediatrics medicine.medical_specialty Factor V Deficiency Coagulation Protein Disorders Consanguinity 030204 cardiovascular system & hematology Sudan 03 medical and health sciences Rare Diseases 0302 clinical medicine medicine Humans Factor XIII deficiency Family history Child Medical History Taking Retrospective Studies Clotting factor Afibrinogenemia business.industry Retrospective cohort study Hematology General Medicine medicine.disease Factor XIII Deficiency Blood Coagulation Factors Coagulation Female business 030215 immunology |
| Zdroj: | Blood Coagulation & Fibrinolysis. 30:277-280 |
| ISSN: | 0957-5235 |
| DOI: | 10.1097/mbc.0000000000000834 |
| Popis: | Rare clotting factor (F) deficiency is a deficiency of one or more of coagulation factors other than FVIII, FIX and vonWillebrand (FI, FII, FV, FV + FVIII, FVII, FIX, FX, FXI and FXIII) that cause bleeding disorders and are inherited as autosomal recessive. Descriptive study was conducted in Hemophilia Centre, Khartoum, Sudan. The medical files of pediatric patients presented to the center were reviewed retrospectively. Forty-seven patients (male : female ratio = 1.2 : 1) were included. The majority (93.6%) have parental history of consanguinity and around one third (31.9%) have family history of bleeding disorder. FV deficiency was the most common deficient factor (36.2%) followed by FI deficiency (23.4%) and FX111 deficiency (21.3%). Bruising (46.8%) and epistaxis (25.5%) were the most common presenting complains. FV deficiency mainly presented with cutaneous ecchymosis (47.1%). FI deficiency presented with umbilical bleeding (45.5%) and FXIII presented with cutaneous ecchymosis (50%). Rare clotting factor deficiency is an existing disease in Sudan with the male : female ratio was 1.2 : 1. FV deficiency, FI deficiency, FXIII deficiency were the common deficiency encountered. |
| Databáze: | OpenAIRE |
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