Table 1. Frequency of ARBDs in different... | American Society of Hematology

Table 1.

Frequency of ARBDs in different provinces of Pakistan

ARBD	Sindh	Punjab	Federal capital	KPK	Total	Percentage	Previously reported cases from Pakistan*	Total	Local prevalence per million	International prevalence per million†
Type 3 VWD 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
FXIII deficiency	7	2	4	0	13	4.6	29	42	0.3	0.5
FVII deficiency	4	6	1	1	12	4.3	84	96	0.6	2
FV deficiency	0	9	0	0	9	3.2	28	37	0.2	1
Vitamin K–dependent clotting factor 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
FX deficiency	1	1	0	0	2	0.7	41	43	0.3	1
FII deficiency	0	2	0	0	2	0.7	10	12	0.07	0.5
FXI deficiency	1	0	0	0	1	0.4	1	2	0.01	∼1
Combined FV and FVIII deficiency	1	0	0	0	1	0.4	0	1	0.006	1

ARBD	Sindh	Punjab	Federal capital	KPK	Total	Percentage	Previously reported cases from Pakistan*	Total	Local prevalence per million	International prevalence per million†
Type 3 VWD 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
FXIII deficiency	7	2	4	0	13	4.6	29	42	0.3	0.5
FVII deficiency	4	6	1	1	12	4.3	84	96	0.6	2
FV deficiency	0	9	0	0	9	3.2	28	37	0.2	1
Vitamin K–dependent clotting factor 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
FX deficiency	1	1	0	0	2	0.7	41	43	0.3	1
FII deficiency	0	2	0	0	2	0.7	10	12	0.07	0.5
FXI deficiency	1	0	0	0	1	0.4	1	2	0.01	∼1
Combined FV and FVIII deficiency	1	0	0	0	1	0.4	0	1	0.006	1

There were no patients from the provinces of Baluchistan, Gilgit-Baltistan, Azad Jammu, and Kashmir because of a lack of health and diagnostic facilities.

†

International prevalence data from world hemophilia database and Orphanet Journal of Rare Diseases.

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