Læknablaðið - 15.06.1995, Blaðsíða 36

480
LÆKNABLAÐIÐ 1995; 81
Table II. Results of laboratory investigations of members infamilies A, B and C.
F VIII F VIII Ag vWF
BT activity ELISA lU/ml* vWF Ag assay
Simplate II APTT Normal Normal Normal Normal
Pedigree Normal Normal ref. range ref. value ref. range ref. range
Family no. Age Sex <7 min <35 Sec 60-140% 0.83 60-140% 60-140%
A IV,5 62 M 5,0 55 21 0.04 101 102
IV,9 58 F 3,0 36 88 **nd 208 187
IV, 10 56 M 3,0 51 22 0.04 127 106
IV,16 53 M 3,0 53 20 0.06 82 109
IV,17 47 M 4,0 51 19 0.06 163 81
V,3 40 F 5,5 39 70 nd 75 81
V,4 36 F 4,0 46 35 0.26 115 101
V,5 34 F 5,5 36 107 0.91 70 67
V, 6 31 F 6,0 41 50 nd 103 111
V,7 25 F nd 51 40 nd 157 nd
V,8 30 F 4,0 39 63 nd 130 100
V,10 29 F 3,0 35 130 nd 165 152
V,15 26 M 4,5 56 15 0.03 133 105
V,18 32 F 6,0 38 60 nd 63 57
V,27 25 F 5,0 38 84 nd 223 114
V,28 26 F 9,0 41 43 nd 150 151
V,30 15 F 3,5 37 58 nd 240 113
VI,4 15 M 3,5 57 12 0.03 80 74
B IV,1 66 M 4,0 51 19 0.03 145 81
IV,2 61 F nd 41 59 0.60 115 68
IV,3 54 M 4,5 51 13 0.03 87 126
V,1 38 F 5,5 37 47 nd 163 88
V, 2 36 F 6,5 40 55 nd 98 68
V,3 33 F 18,0 44 57 nd 84 80
V,4 31 F 6,5 43 36 nd 160 76
V,10 31 M 4,5 50 18 0.03 145 73
V,11 29 M 5,5 48 14 0.03 84 55
V,13 25 F 11,0 39 49 0.37 85 97
V,14 21 F nd 50 33 0.24 56 114
V,16 16 F 5,5 37 40 0.28 59 70
VI,1 19 M 5,0 52 13 0.02 60 53
VI,4 14 F 6,5 47 40 nd 49 100
VI,5 13 M 3,5 51 18 0.03 155 nd
VI,6 11 F 9,5 46 nd nd 122 107
VI,7 9 M 9,5 56 15 0.02 120 86
VI,8 13 F 5,5 46 41 nd 75 124
VI,9 12 M 7,5 55 14 nd 123 70
VI,16 3 M nd 54 11 0.02 90 nd
C V,1 65 M nd 41 8 nd 320 89
V,3 54 M 10,0 46 15 nd 76 54
VI,2 38 M 8,0 49 14 nd 37 38
VI,5 44 F 12,5 34 46 <0.01 78 68
VI,6 30 F nd 42 36 nd 64 54
VII,7 21 M 20,0 43 12 0.02 29 28
VII,8 20 F 6,0 35 103 nd 45 55
VII,9 17 F 8,0 34 68 nd 54 47
VII,11 14 M 15,0 46 11 <0.01 50 56
VII,12 10 M nd 47 20 nd 128 71
VII,13 3 M nd 42 27 nd 130 70
'Coating E-IGG, conjungate Z-lgG, F VIII Ag ELISA, with polyclonal F VIII inhibitor IgG,
**nd= not done
BT: Bleeding Time
APTT: Activated Partial Thromboplastier Time
F VIII: Factor VIII
vWF: von Willebrand Factor