Measurement of C1 Inhibitor Function by Colorimetric Method and Its Usage in the Diagnosis of Hereditary Angioedema

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1 CHINESE JOURNAL OF ALLERGY & CLINICAL IMMUNOLOGY C1 # * C1 hereditary angioedemahae C1 C 2 H 5 CO-Lys-Gly-Arg-pNA C1 C1 C1 INH HAE C1 HAE h C ~ U C1 INH ml HAE % 24 h C1INH - 20 C1 HAE h C1 R R596 A 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 China 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 U C1 INH ml by colorimetric method. The sensitivity and specificity were both 100%. The bio-activity of C1 INH could be influenced by * # zhiyuxiang@ yahoo. com. cn 32 March2013

2 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 Immunol C1 C1 inhibitorc1 INH mol L r min 15 min - 1 A 1 C1 INH C1-1 B ml 0. 1 ml C1 Hereditary 37 5 min 4 - angioedemahae C1 INH 0. 6 ml37 3 min 5 50% 0. 2 ml ~ 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 ml HAEⅠ C1 INH h C1 INH C SPSS HAE X C1INH C1 INH HAE 2 ml nm 10 mm ~ 31 C1INH P < C1 INH ± Vol. 7 No. 1 33

3 F P > HAE C1 INH 3 C1 INH C1 INH ± 240 mg L 1 U ml C1INH U C1 INH ml 1U C1 INH 1 ml 28 h 5 C1s C1r αfⅫa βfⅫ C1 INH a FⅪa MASP ± MASP-27 C ~ U ml 21 HAE C1 INH P < Fig ± 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 C % 4 C1s C1 C1 INH INH-C1s C1 INH C1 INH h 4-20 C1 INH F = P < h 8 h 4-20 C1 INH 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 March2013

4 C1 Engl J Med Ugochukwu CEvangelo FSWilliam J. Hereditary HAE angioedema a broad review forclinicians J. Arch Intern Med HAE 11 4 Schapira MDe Pagostini ASichifferli JAet al. Biochemistry and pathophysiology of human C1 inhibitor Current issues J. Complement Nuijins JHEeenberg-Belmer AJMHuijbtrgts CCet al. Proteolytic inactivation of plasm C1 inhibiter in C1 INH HAE C1 INH h 8 h 7 Bork K. Current management options for hereditary - 20 angioedema J. Curr Allergy Asthma Rep Ineke 10 C1 INH 8 Zuraw BLChristiansen SC. Pathophysiology of hereditary 2 h angioedema J. Am J Rhinol Allergy dc1 INH HAE 9 Gompels MMLock RJ. Laboratory testing for C1 4 inhibitor deficiency a comparison of two approaches to C1 inhibitor function J. Ann Clin Biochem2007 HAE C1 INH HAE C1 10Ineke GA. Wagenaar-BosChristian Drouetet al. INH C1 INH HAE angioedema assay evaluation and recommendations J. Journal of Immunological Methods Gompels MMLock RJMogan JEet al. A multicenter h evaluation of the diagnostic efficiency of serological 2 3 Ⅳ 1 Davis AE 3rd. C1 inhibitor and hereditary angioneurotic edema J. Annu Rev Immuml Cicardi MAgostoni A. Hereditary angioedema J. N sepsis J. J Clin Invest Caiezi CWuiemin WAZeerleder Set al. C1- C1 INH Esterase inhibitor An anti- inflammatory agent and its potential use in the treatment of disease other than 4 24 h hereditary angioedema J. Pharmacological Reviews Functional C1-Inhibitor diagnostics in hereditary investigations for C1 inhibitor deficiency J. J Clin Pathol Nielsen EWJohansen HTStraume Bet al. Effect of time and additives on a functional assay of C1 inhibitor J. J Immuno Methods Vol. 7 No. 1 35

5 Ⅳ C 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

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