Hip Replacement Surgery in 14-Year-Old Girl with Factor V Deficiency: Haemostatic Treatment and Thromboprophylaxis

Table 2

FV deficiency surgeries identified in literature.

Subject	Age	Basal; Fv levels; (IU mL^−1)	Surgery	Treatment	FV; postsurgery pick; (IU mL^−1)	Clinical outcome
1 [3]	6 days	Undetectable	Central line	(i) 15 mL kg^−1 FFP TD 5 days, then OD 3 days, EOD till resolution.	0.32	Good
			Hickman	(ii) 19 mL kg^−1 platelets, TD 2 days.
				(iii) rFVIIa 90 mcg Kg^−1 every 2 hours, 8 doses.
1 [3]	5 months	Undetectable	Central line	(i) 15 mL kg^−1 FFP TD 5 days, then OD 3 days, EOD till resolution.	No reference	Good
			Hickman	(ii) 19 mL kg^−1 platelets, TD 2 days.
				(iii) rFVIIa 90 mcg kg^−1 every 2 hours, 8 doses.
1 [3]	3 years	Undetectable	Tetralogy Fallot repairment	(i) 15 mL kg^−1 FFP TD 2 days, then OD 11 days,; (ii) 19 mL kg^−1 platelets, TD 1 day,; (iii) rFVIIa 90 mcg Kg^−1, 1 dose,; (iv) tranexamic .	0.39	Good
1 [3]	3 years	Undetectable	Central line	(i) .; (ii) .	No reference	Good
			Port-a-cath	(iii) rFVIIa 90 mcg mL^−1, 2 doses.
				(iv) Tranexamic .
1 [3]	4 years	Undetectable	Central line	(i) .; (ii) .	No reference	Good
			Port-a-cath	(iii) Tranexamic .
				(iv) rFVIIa 90 mcg mL^−1, 2 doses.
1 [3]	5 years	Undetectable	Central line	(i) .; (ii) .	No reference	Good
			Port-a-cath	(iii) rFVIIa 90 mcg mL^−1, 2 doses.; (iv) Tranexamic .
2 [3]	6 weeks	Undetectable	Central line	(i) 15 mL Kg^−1 FFP, TD 12 days, OD until resolution.	0.4	Good
			Hickman	(ii) 10 mL Kg^−1 platelets, OD, 4 days.
				(iii) rFVIIa 90 mcg Kg^−1, 3 doses.
2 [3]	8 months	Undetectable	Central line	(i) 15 mL Kg^−1 FFP, TD 12 days, OD until resolution.	No reference	Good
			Port-a-cath	(ii) 10 mL Kg^−1 platelets, OD, 4 days.
				(iii) rFVIIa 90 mcg Kg^−1, 3 doses.
2 [3]	4 years	Undetectable	Central line	(i) 15 mL Kg^−1 FFP, TD 12 days, OD until resolution.	No reference	Good
			Port-a-cath	(ii) 10 mL Kg^−1 platelets, OD, 4 days.
				(iii) rFVIIa 90 mcg Kg^−1, 3 doses.
2 [3]	6 years	Undetectable	Central line	(i) 15 mL Kg^−1 FFP, TD 12 days, OD until resolution.	No reference	Good
			Port-a-cath	(ii) 10 mL Kg^−1 platelets, OD, 4 days.
				(iii) rFVIIa 90 mcg Kg^−1, 3 doses.
3 [3]	11 weeks	Severe	Craniotomy	(i) 15 mL Kg^−1 FFP, TTD initial, progressive reducing. Total 10 days.; (ii) rFVIIa 90 mcg Kg^−1, 2 doses.	0.28	Good
3 [3]	12 weeks	Severe	Central line	(i) 15 mL Kg^−1 FFP, TTD initial, progressive reducing.	No reference	Good
			Hickman	(ii) rFVIIa 90 mcg Kg^−1, 2 doses.
3 [3]	1 year	Severe	Central line	Not specified.	No reference
			Port-a-cath
4 [4]	60 years	0.15	Arthroscopic	(i) rFVIIa 120 mcg kg^−1, 2 doses.	No reference	Good
			Synovectomy	(ii) 80 mcg kg^−1 every 2 hours, 8 doses.; (iii) Progressive reducing frequency 5 days.; (iv) Tranexamic acid.
4 [4]	60 years	0.15	Arteriography embolization	(i) rFVIIa 120 mcg kg^−1, 2 doses.; (ii) 80 mcg kg^−1 every 2 hours, 6 doses.; (iii) Progressive reducing frequency 3 days.	No reference	Good
5 [5]	27 years	0.05	Intrauterine insemination	.	No reference	Good

other information. EOD: every other day; FFP: fresh frozen plasma; OD: once a day; rFVIIa: recombinant factor VII activated; TD: twice a day; TTD: three times a day.