Plasma-derived C1-INH, this prophylactic infusion regimen seems justified only forPlasma-derived C1-INH, this prophylactic infusion regimen

Plasma-derived C1-INH, this prophylactic infusion regimen seems justified only for
Plasma-derived C1-INH, this prophylactic infusion regimen seems justified only for those patients with two or more severe attacks per week, a condition that we did not find in any of our 42 AAE patients. We reserve plasma-derived C1-INH infusions for ondemand treatment of severe angioedema events and do not use this for prophylaxis.Acknowledgements Supported by Telethon grant n. GGP08223 and a grant from Invernizzi Foundation Authors’ contributions MC wrote the review and AZ gave critical revision and collected the personal data that are reported. Both authors have read and approved the final manuscript. Competing interests MC has consultancy agreement with Dyax, Pharming and Shire, is in advisory board and invited speaker of Dyax, Shire. CSL Behring. AZ has been Invited speaker for CSL Behring and Shire Received: 26 April 2010 Accepted: 28 July 2010 Published: 28 July 2010 References 1. Caldwell JR, Ruddy S, Schur PH, Austen KF: Acquired C1 PubMed ID:https://www.ncbi.nlm.nih.gov/pubmed/27321907 inhibitor deficiency in lymphosarcoma. Clin Immunol Immunopathol 1972, 1:39-52. 2. Zingale LC, Castelli R, Zanichelli A, Cicardi M: Acquired deficiency of the inhibitor of the first complement component: presentation, diagnosis, course, and conventional management. Immunol Allergy Clin North Am 2006, 26:669-90. 3. Bygum A: Hereditary angio-oedema in Denmark: a nationwide survey. Br J Dermatol 2009, 161:1153-8. 4. Zuraw BL: Clinical practice. Hereditary angioedema. N Engl J Med 2008, 359:1027-36. 5. Agostoni A, Ayg en-P s E, Binkley KE, Blanch A, Bork K, Bouillet L, Bucher C, Castaldo AJ, Cicardi M, Davis AE, De Carolis C, Drouet C, Duponchel C, Farkas H, F K, Fekete B, Fischer B, Fontana L, F t G, Giacomelli R, Gr er A, Hack CE, Harmat G, Jakenfelds J, Juers M, Kalm L, Kaposi PN, Kar i I, Kitzinger A, Koll T, et al: Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. J Allergy Clin Immunol 2004, 114:S51-131. 6. Nussberger J, Cugno M, Amstutz C, Cicardi M, Pellacani A, Agostoni A: Plasma bradykinin in angio-oedema. Lancet 1998, 351:1693-7. 7. Davis AE: C1 inhibitor and hereditary angioneurotic edema. Annu Rev Immunol 1988, 6:595-628. 8. Zingale LC, Zanichelli A, Thonzonium (bromide)MedChemExpress Thonzonium (bromide) Deliliers DL, Rondonotti E, De Franchis R, Cicardi M: Successful resolution of bowel PubMed ID:https://www.ncbi.nlm.nih.gov/pubmed/27663262 obstruction in a patient with hereditary angioedema. Eur J Gastroenterol Hepatol 2008, 20:583-7.Cicardi and Zanichelli Allergy, Asthma Clinical Immunology 2010, 6:14 http://www.aacijournal.com/content/6/1/Page 5 of9.10.11.12.13.14.15. 16. 17. 18.19.20. 21.22.23.24.25.26.27.28.29. 30.Bouillet-Claveyrolas L, Ponard D, Drouet C, Massot C: Clinical and biological distinctions between type I and type II acquired angioedema. Am J Med 2003, 115:420-1. Eck SL, Morse JH, Janssen DA, Emerson SG, Markovitz DM: Angioedema presenting as chronic gastrointestinal symptoms. Am J Gastroenterol 1993, 88:436-9. Schreiber AD, Zweiman B, Atkins P, Goldwein F, Pietra G, Atkinson B, Abdou NI: Acquired angioedema with lymphoproliferative disorder: association of C1 inhibitor deficiency with cellular abnormality. Blood 1976, 48:567-80. Hauptmann G, Lang JM, North ML, Oberling F, Mayer G, Lachmann P: Acquired c1-inhibitor deficiencies in lymphoproliferative diseases with serum immunoglobulin abnormalities. A study of three cases. Blut 1976, 32:195-206. Castelli R, Deliliers DL, Zingale LC, Pogliani EM, Cicardi M: Lymphoproliferative disease and acquired C1 inhibitor deficiency. Haematologica 20.