Long-term functional and quality-of-life outcomes after knee replacement in patients with inherited bleeding disorders: A 15-year follow-up study

Table 1 Baseline characteristics of 90 arthroplasties operated on patients with inherited bleeding disorder, n (%)

	HA (n = 68)	HB (n = 12)	VWD (n = 10)	All (n = 90)
Subjects, n	38	7	8	53
Bilateral procedure, n	13	2	2	17
Age, mean (SD)	44 (10)	45 (14)	48 (10)	46 (11)
Men	66 (97)	12 (100)	3 (30)	81 (90)
Hospital stay, days, median (IQR)	15 (12-18)	14 (13-18)	17(13-18)	15 (12-18)
C hepatitis positive	46 (68)	8 (67)	1 (10)	55 (61)
BMI, mean (SD)	25.3 (3.7)	24.5 (1.1)	25.1 (3.4)	25.2 (3.4)
Smoking	12 (20)	6 (55)	0 (0)	18 (22)
Components
Demi	3 (4)	0 (0)	0 (0)	3 (3)
Non-constrained	56 (82)	9 (75)	10 (100)	75 (83)
Constrained	9 (13)	3 (25)	0 (0)	12 (13)
Bleeding disorder
Mild	2 (3)	1 (8)	NA	3 (3)
Moderate	8 (12)	2 (17)	NA	10 (11)
Severe	58 (85)	9 (75)	NA	67 (74)
VWD1	NA	NA	10 (100)	10 (11)
Replacement therapy	68 (100)	12 (100)	9 (90)	89 (99)
pdFVIII	52	0	0	52
rFVIII	6	0	0	6
pdVWF/FVIII	0	0	7	7
pdFIX	0	11	0	11
rFIX	0	1	0	1
aPCC and/or rFVIIa	5	0	0	5
Kryo/Kryo-AHG	5	0	2	7
None2	0	0	1	1

¹Type of von Willebrnd disease requiring specific replacement therapy.

²One patient was diagnosed with a bleeding disorder only after the first operation.

BMI: Body mass index; HA: Hemophilia A; HB: Hemophilia B; VWD: Von Willebrnd disease; pdFVIII: Plasma-derived factor VIII; rFVIII: Recombinant factor VIII; pdVWF: Plasma-derived von Willebrand factor; aPCC: Activated prothrombin complex concentrate; rFVIIa: Recombinant activated FVII; FIX: Factor IX; NA: Not available; IQR: Interquartile ranges; pdFIX: Plasma-derived factor IX; FVIII: Factor VIII.

Table 2 Baseline characteristics in walking ability, Hungerford total score, and Hungerford sub-items, n (%)

	HA (n = 68)	HB (n = 12)	VWD (n = 10)	All (n = 90)
Walking ability
Incapable	0 (0)	0 (0)	0 (0)	0 (0)
Inside house	3 (4)	0 (0)	0 (0)	3 (3)
< 200 m	22 (32)	1 (8)	6 (60)	29 (32)
200-2000 m	42 (62)	10 (83)	4 (40)	56 (62)
> 2000 m	1 (1)	1 (8)	0 (0)	2 (2)
Hungerford score
Walking aid	26 (38)	0 (0)	7 (70)	33 (37)
Range of movement, mean (SD)
(0° to 20°)	13 (7)	9 (9)	15 (5)	13 (7)
≤ 30°	7 (10)	4 (33)	0 (0)	11 (12)
31°-60°	12 (18)	2 (17)	1 (10)	15 (17)
61°-90°	14 (21)	0 (0)	2 (20)	16 (18)
91°-105°	5 (7)	3 (25)	3 (30)	11 (12)
> 105°	30 (44)	3 (25)	4 (40)	37 (41)
Pain, (0 to 50), mean (SD)	33 (14)	30 (15)	26 (17)	32 (14)
Flexion contracture (-20 to 0), mean (SD)	-8 (4)	-12 (5)	-5 (4)	-8 (5)
Joint stability (0 to 10), mean (SD)	9 (2)	7 (5)	9 (3)	9 (3)
Deformity (-5 to 10), mean (SD)	6 (4)	5 (3)	8 (3)	6 (4)
Quadriceps strength (0 to 10), mean (SD)	7 (3)	6 (3)	7 (3)	7 (3)
Total score (-25 to 100), mean (SD)	60 (18)	46 (17)	60 (15)	58 (18)

HA: Hemophilia A; HB: Hemophilia B; VWD: Von Willebrand disease.