Baseline level of functional C1-inhibitor correlates with disease severity scores in hereditary angioedema

Clinical Immunology

Volumn 134, Issue 3, 2010, Pages 354-358

  Kelemen, Zsuzsanna ^a   Moldovan, Dumitru ^b   Mihály, Eniko ^c   Visy, Beáta ^d   Széplaki, Gábor ^a   Csuka, Dorottya ^a   Füst, George ^a   Farkas, Henriette ^a   Varga, Lilian ^a  

^a SEMMELWEIS UNIVERSITY OF MEDICINE   (Hungary)

^b UNIVERSITY OF MEDICINE AND PHARMACY   (Romania)

^c Romanian Haereditary Angioedema Network   (Romania)

^d HEIM PÁL CHILDREN S HOSPITAL   (Hungary)

Author keywords

C1 inhibitor; Complement; Diagnosis; Disease severity; Hereditary angioedema

Indexed keywords

COMPLEMENT COMPONENT C1S INHIBITOR; COMPLEMENT COMPONENT C4;

ADOLESCENT; ADULT; AGED; ANGIONEUROTIC EDEMA; ARTICLE; CHILD; CLINICAL ASSESSMENT; CONTROLLED STUDY; CORRELATION ANALYSIS; DIAGNOSTIC ACCURACY; DIAGNOSTIC VALUE; DISEASE ASSOCIATION; DISEASE COURSE; DISEASE SEVERITY; ENZYME LINKED IMMUNOSORBENT ASSAY; FEMALE; HEMOLYSIS; HUMAN; MAJOR CLINICAL STUDY; MALE; PATHOPHYSIOLOGY; PRESCHOOL CHILD; PRIORITY JOURNAL; RETROSPECTIVE STUDY; SCHOOL CHILD; SCORING SYSTEM; SEX DIFFERENCE; STATISTICAL SIGNIFICANCE;

ADOLESCENT; ADULT; AGED; ANGIOEDEMAS, HEREDITARY; CHILD; CHILD, PRESCHOOL; COMPLEMENT C1; COMPLEMENT C1 INHIBITOR PROTEIN; COMPLEMENT C4; COMPLEMENT HEMOLYTIC ACTIVITY ASSAY; FEMALE; HUMANS; INFANT; MALE; MIDDLE AGED; RETROSPECTIVE STUDIES; SEVERITY OF ILLNESS INDEX; STATISTICS, NONPARAMETRIC; YOUNG ADULT;

EID: 76049125173     PISSN: 15216616     EISSN: 15217035     Source Type: Journal    
DOI: 10.1016/j.clim.2009.11.002     Document Type: Article

References (20)

1. Donaldson V.H., and Evans R.R. A biochemical abnormality in hereditary angioneurotic edema: absence of serum inhibitor of C' 1-esterase. Am. J. Med. 35 (1963) 37-44
2. Rosen F.S., Pensky J., Donaldson V., and Charache P. Hereditary angioneurotic edema: two genetic variants. Science 148 (1965) 957-958
3. Bowen T., Cicardi M., Bork K., Zuraw B., Frank M., Ritchie B., Farkas H., Varga L., Zingale L.C., Binkley K., Wagner E., Adomaitis P., Brosz K., Burnham J., Warrington R., Kalicinsky C., Mace S., McCusker C., Schellenberg R., Celeste L., Hebert J., Valentine K., Poon M.C., Serushago B., Neurath D., Yang W., Lacuesta G., Issekutz A., Hamed A., Kamra P., Dean J., Kanani A., Stark D., Rivard G.E., Leith E., Tsai E., Waserman S., Keith P.K., Page D., Marchesin S., Longhurst H.J., Kreuz W., Rusicke E., Martinez-Saguer I., Aygoren-Pursun E., Harmat G., Fust G., Li H., Bouillet L., Caballero T., Moldovan D., Spath P.J., Smith-Foltz S., Nagy I., Nielsen E.W., Bucher C., Nordenfelt P., and Xiang Z.Y. Hereditary angioedema: a current state-of-the-art review, VII: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema. Ann. Allergy Asthma Immunol. 100 (2008) S30-S40
4. Cugno M., Cicardi M., Bottasso B., Coppola R., Paonessa R., Mannucci P.M., and Agostoni A. Activation of the coagulation cascade in C1-inhibitor deficiencies. Blood 89 (1997) 3213-3218
5. Cugno M., Zanichelli A., Bellatorre A.G., Griffini S., and Cicardi M. Plasma biomarkers of acute attacks in patients with angioedema due to C1-inhibitor deficiency. Allergy 64 (2009) 254-257
6. Bork K., Meng G., Staubach P., and Hardt J. Hereditary angioedema: new findings concerning symptoms, affected organs, and course. Am. J. Med. 119 (2006) 267-274
7. Agostoni A., Aygoren-Pursun E., Binkley K.E., Blanch A., Bork K., Bouillet L., Bucher C., Castaldo A.J., Cicardi M., Davis A.E., De Carolis C., Drouet C., Duponchel C., Farkas H., Fay K., Fekete B., Fischer B., Fontana L., Fust G., Giacomelli R., Groner A., Hack C.E., Harmat G., Jakenfelds J., Juers M., Kalmar L., Kaposi P.N., Karadi I., Kitzinger A., Kollar T., Kreuz W., Lakatos P., Longhurst H.J., Lopez-Trascasa M., Martinez-Saguer I., Monnier N., Nagy I., Nemeth E., Nielsen E.W., Nuijens J.H., O'Grady C., Pappalardo E., Penna V., Perricone C., Perricone R., Rauch U., Roche O., Rusicke E., Spath P.J., Szendei G., Takacs E., Tordai A., Truedsson L., Varga L., Visy B., Williams K., Zanichelli A., and Zingale L. Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. J. Allergy Clin. Immunol. 114 (2004) S131-S151
8. Bowen T., Cicardi M., Farkas H., Bork K., Kreuz W., Zingale L., Varga L., Martinez-Saguer I., Aygoren-Pursun E., Binkley K., Zuraw B., Davis III A., Hebert J., Ritchie B., Burnham J., Castaldo A., Menendez A., Nagy I., Harmat G., Bucher C., Lacuesta G., Issekutz A., Warrington R., Yang W., Dean J., Kanani A., Stark D., McCusker C., Wagner E., Rivard G.E., Leith E., Tsai E., MacSween M., Lyanga J., Serushago B., Leznoff A., Waserman S., de Serres J., and Canadian 2003. International consensus algorithm for the diagnosis, therapy, and management of hereditary angioedema. J. Allergy Clin. Immunol. 114 (2004) 629-637
9. Kalmar L., Bors A., Farkas H., Vas S., Fandl B., Varga L., Fust G., and Tordai A. Mutation screening of the C1 inhibitor gene among Hungarian patients with hereditary angioedema. Hum. Mutat. 22 (2003) 498
10. Frank M.M., Gelfand J.A., and Atkinson J.P. Hereditary angioedema: the clinical syndrome and its management. Ann. Intern. Med. 84 (1976) 580-593
11. Agostoni A. Inherited C1 inhibitor deficiency. Complement Inflamm. 6 (1989) 112-118
12. Gelfand J.A., Sherins R.J., Alling D.W., and Frank M.M. Treatment of hereditary angioedema with danazol. Reversal of clinical and biochemical abnormalities. N. Engl. J. Med. 295 (1976) 1444-1448
13. Cicardi M., Bergamaschini L., Cugno M., Hack E., Agostoni G., and Agostoni A. Long-term treatment of hereditary angioedema with attenuated androgens: a survey of a 13-year experience. J. Allergy Clin. Immunol. 87 (1991) 768-773
14. Cedzynski M., Madalinski K., Gregorek H., Swierzko A.S., Nowicka E., Obtulowicz K., Dzierzanowska-Fangrat K., Wojda U., Rabczenko D., and Kawakami M. Possible disease-modifying factors: the mannan-binding lectin pathway and infections in hereditary angioedema of children and adults. Arch. Immunol. Ther. Exp. (Warsz) 56 (2008) 69-75
15. Wagenaar-Bos I.G., Drouet C., Aygoren-Pursun E., Bork K., Bucher C., Bygum A., Farkas H., Fust G., Gregorek H., Hack C.E., Hickey A., Joller-Jemelka H.I., Kapusta M., Kreuz W., Longhurst H., Lopez-Trascasa M., Madalinski K., Naskalski J., Nieuwenhuys E., Ponard D., Truedsson L., Varga L., Nielsen E.W., Wagner E., Zingale L., Cicardi M., and van Ham S.M. Functional C1-inhibitor diagnostics in hereditary angioedema: assay evaluation and recommendations. J. Immunol. Methods 338 (2008) 14-20
16. Gompels M.M., and Lock R.J. Laboratory testing for C1 inhibitor deficiency: a comparison of two approaches to C1 inhibitor function. Ann. Clin. Biochem. 44 (2007) 75-78
17. Gompels M.M., Lock R.J., Morgan J.E., Osborne J., Brown A., and Virgo P.F. A multicentre evaluation of the diagnostic efficiency of serological investigations for C1 inhibitor deficiency. J. Clin. Pathol. 55 (2002) 145-147
18. Gompels M.M., Lock R.J., Unsworth D.J., Johnston S.L., Archer C.B., and Davies S.V. Misdiagnosis of hereditary angio-oedema type 1 and type 2. Br. J. Dermatol. 148 (2003) 719-723
19. Tarzi M.D., Hickey A., Forster T., Mohammadi M., and Longhurst H.J. An evaluation of tests used for the diagnosis and monitoring of C1 inhibitor deficiency: normal serum C4 does not exclude hereditary angio-oedema. Clin. Exp. Immunol. 149 (2007) 513-516
20. Späth P.J., and Wütrich B. Inherited and acquired deficiencies of C1 esterese deficiencies in humans. In: Rother K., Till G.O., and Hänsch G.M. (Eds). The Complement System (1998), Springer-Verlag, Berlin, Heidelberg 353-409