Rare clotting factor deficiency among Sudanese children

Blood Coagul Fibrinolysis. 2019 Sep;30(6):277-280. doi: 10.1097/MBC.0000000000000834.

Abstract

: 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.

MeSH terms

  • Afibrinogenemia
  • Blood Coagulation Factors / analysis*
  • Child
  • Coagulation Protein Disorders / epidemiology*
  • Coagulation Protein Disorders / etiology
  • Consanguinity
  • Factor V Deficiency
  • Factor XIII Deficiency
  • Female
  • Humans
  • Male
  • Medical History Taking
  • Rare Diseases / blood*
  • Retrospective Studies
  • Sudan / epidemiology

Substances

  • Blood Coagulation Factors