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Table 2 Frequency of ARBDs from different provinces of Pakistan

From: Autosomal recessive inherited bleeding disorders in Pakistan: a cross-sectional study from selected regions

ARBD Sindh Punjab Federal capital KPK Total Percentage Previously reported cases from Pakistan Total Local prevalence~
Per million
International prevalence*
Per million
VWD type 3 disorder 05 62 21 7 95 (33.8%) 61 156 1.0 0.5
Fibrinogen deficiency 11 20 3 0 34 (12%) 9 43 0.3 0.5
Glanzmann Thrombasthenia 18 9 0 0 27 (9.6%) 50 77 0.5 1
Factor XIII deficiency 7 2 4 0 13 (4.6%) 29 42 0.3 0.5
Factor VII deficiency 4 6 1 1 12 (4.3%) 84 96 0.6 2
Factor V deficiency 0 9 0 0 9 (3.2%) 28 37 0.2 1
Vitamin K dependent clotting factors deficiency 0 7 0 1 8 (2.8%) 0 8 0.04 1
Bernard Soulier syndrome 3 0 4 0 7 (2.5%) 5 12 0.07 1
Factor X deficiency 1 1 0 0 2 (0.7%) 41 43 0.3 1
Factor II deficiency 0 2 0 0 2 (0.7%) 10 12 0.07 0.5
Factor XI deficiency 1 0 0 0 1 (0.4%) 1 2 0.01 1~
Combined Factor V & VIII deficiency 1 0 0 0 1 (0.4%) 0 1 0.006 1
  1. There were no patients from the province of Baluchistan, Gilgit Baltistan and Azad Jammu & Kashmir due to lack of health and diagnostic facilities
  2. *International prevalence data from world hemophilia database and orphanet journal of rare diseases
  3. ~Frequency is 1 in 450 in Ashkenazi Jews
  4. [6, 37, 38]
  5. ~Period Prevalence calculations were based on CDC formulation