CHINESE JOURNAL OF ALLERGY & CLINICAL IMMUNOLOGY C1 # * 100730 C1 hereditary angioedemahae C1 C 2 H 5 CO-Lys-Gly-Arg-pNA C1 C1 C1 INH 65 21 HAE C1 HAE 9 4-20 4 8 24 h C1 0. 56 ~ 1. 58 U C1 INH ml HAE 65 65 21 21 100% 24 h C1INH - 20 C1 HAE - 20 24 h C1 R446. 6 R596 A 1673-8705 2013 01-0032-04 Measurement of C1 Inhibitor Function by Colorimetric Method and Its Usage in the Diagnosis of Hereditary Angioedema ZHI Yu-xiang # LIU Hong-xia * XU Ying-yangZHANG Hong-yu Department of AllergyPeking Union Medical College HospitalChinese Academy of Medical Sciences & Peking Union Medical CollegeBeijing 100730China Objective to determine the C1 inhibitor function by using colorimetric method. Sensitivity and specificity in the diagnosis of hereditary angioedema and the impact of the storage condition of the blood sample on the results were evaluated. Methods The excess residual C1-esterase which formed complex with C1 inhibitor was detected by photometrical reaction with the new chromogenic substrate C 2 H 5 CO-Lys- Gly-Arg-pNA. Normal value was determined by detecting the plasma samples from 65 healthy volunteers. The sensitivity and specificity were also evaluated by detecting healthy volunteers and the patients with confirmed diagnosis of hereditary angioedema. Samples from 9 healthy controls were divided into 7 groups and stored at different temperatures room temperature 4-20 for different durations at prime tense4 h 8 h and 24 h and C1 inhibitor function were detected respectivelyfor evaluating the impact factors. Results The normal value of functional C1 INH was 0. 56-1. 58 U C1 INH ml by colorimetric method. The sensitivity and specificity were both 100%. The bio-activity of C1 INH could be influenced by * 150046 # 010-69151601 zhiyuxiang@ yahoo. com. cn 32 March2013
C1 storage durations and temperature. Conclusions Colorimetric method is an effective method to determine the C1 inhibitor functionand sensitivity and specificity was both really high for diagnosis of HAE. The samples should be prepared and stored at - 20 immediatelyotherwise it will be resulted in false positive result. Key words functional C1 INH hereditary angioedema testing method sensitivity specificity Chin J Allergy Clin Immunol20137 1 32-35 C1 C1 inhibitorc1 INH 0. 109 mol L 9 1 3 000 r min 15 min - 1 A 1 C1 INH 1 11 2 C1-1 B 1 4 37 3 0. 1 ml 0. 1 ml C1 Hereditary 37 5 min 4 - angioedemahae C1 INH 0. 6 ml37 3 min 5 50% 0. 2 ml 1 50 000 ~ 1 10 000 2-3 7 C1 INH HAE C1 INH C1 INH Immno Chrom C1 INH C1-1 C 2 H 5 CO-Lys-Gly-Arg-Pna C1 C1 INH A B HAE C1 INH HAE HAE HAE 2010 2012 2 ml HAEⅠ 21 10 11 32. 9 3 4 3-20 3 C1 INH 4 8 24 h C1 INH C4 2012 65 SPSS HAE X 35 30 C1INH 33. 4 C1 INH HAE 2 ml 6 405 nm 10 mm 9 9 25 ~ 31 C1INH P < 0. 05 C1 INH ± Vol. 7 No. 1 33
F 0. 787 1. 710 P > 0. 05 HAE C1 INH 3 C1 INH 478 1 65 C1 INH 1. 07 ± 240 mg L 1 U ml C1INH 0. 26 U C1 INH ml 1U C1 INH 1 ml 28 h 5 C1s C1r αfⅫa βfⅫ C1 INH a FⅪa MASP-1 1. 07 ± 1. 96 MASP-27 C1 0. 26 0. 56 ~ 1. 58 U ml 21 HAE C1 INH P < 0. 001 2 1 Fig 1 0. 11 ± 0. 12 U C1 INH ml C1 INH Functional C1 INH testing standard curve C1 INH C1 65 Lys-Gly-Arg-Pna C1 21 HAE C1 INH C1 INH C1-1 C1 65 65 21 21 100% 4 C1s C1 C1 INH INH-C1s C1 INH C1 INH - 20 3 24 h 4-20 C1 INH F = 5. 038P < 0. 05 4 h 8 h 4-20 C1 INH 4 105 000 INH - C1 INH C1 INH 6 C1 INH HAE 7 HAE C1 INH C1 INH C1 INH HAEⅠ 85% C1 INH HAEⅡ C1 INH C1 INH 15% 8 HAE Ⅰ Ⅱ C1 INH HAE C1INH C1 INH C1 C1 -C1 INH C1-1 C 2 H 5 CO- C1 C1 INH C1 INH HAE 9 10 34 March2013
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Ⅳ C1 32 2 Fig 2 HAE C1 INH Functional C1 INH results of healthy controls and HAE patients C1 INH C1 3 Fig 3 C1 INH Functional C1 INH results that the samples stored at different time and temperature