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Primary Immunodeficiency

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60 Classification of PPID Disclaimer is Guideline attempts to define principles of practice that should produce high-quality patient care. It is applicable to specialists, primary care, and providers at all levels. is Guideline should not be considered exclusive of other methods of care reasonably directed at obtaining the same results. e ultimate judgment concerning the propriety of any course of conduct must be made by the clinician aer consideration of each individual patient situation. Neither IGC, the medical associations, nor the authors endorse any product or service associated with the distributor of this clinical reference tool. Source Bonilla FA, Khan DA, Ballas ZK, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. J Allerg y Clin Immunol. 2015;136(5): 1186-205.e1-78. 106 Commerce Street, Suite 105 Lake Mary, FL 32746 TEL: 407.878.7606 • FAX: 407.878.7611 Order additional copies at Copyright © 2016 All rights reserved AAAAIPRI16023 Immunodeficiency associated with autoantibodies Acquired angioedema Anti–C1 inhibitor Neutropenia/Felty syndrome Anti–G-CSF Cryptococcal meningitis/PAP Anti–GM-CSF Disseminated varicella-zoster/APECED Anti–IFN-α/β Disseminated infections (virus, bacteria, fungi) Anti–IFN-γ Recurrent bacterial skin infections/sepsis Anti–IL-6 Disseminated Burkholderia gladioli infection Anti–IL-12p70 CMCC/APECED Anti–IL-17, anti–IL-22 a e classification is based on the format used by the WHO/IUIS (Al-Herz W, Bousfiha A, Casanova JL, et al. Front Immunol. 2014;5:162.) e authors have attempted to use the Human Genome Organization name for each gene current at the time of publication of this document. e reader should be aware that this nomenclature is fluid, and some names might have changed. Table 11. Classification of Primary Immunodeficiencies a (cont'd) Defect or disease(s) Gene(s)

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