Primary Immunodeficiency

Published: September 2015
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  • Key Points 
  • General Diagnostic Considerations 
  • Treatment Recommendations 
    • Combined B- and T-Cell Immunodeficiencies 
    • Well-Defined Syndromes with Immunodeficiency 
    • Predominantly Antibody Deficiencies 
    • Diseases of Immune Dysregulation 
    • Phagocytic Cell Defects 
    • Defects of Innate Immunity 
    • Autoinflammatory Disorders 
    • Complement Deficiencies & Anti-Cytokine Autoantibodies 
  • Figures  
    • General Approach for the Diagnosis of Primary Immunodeficiency 
    • Diagnosis of Combined or Syndromic Immunodeficiencies 
    • Diagnosis of Diseases of Immune Dysregulation 
    • Diagnosis of Phagocyte Defects 
    • Diagnosis of Innate Immune Defects 
    • Diagnosis of Autoinflammatory Syndromes 
  • Tables 
    • Characteristic Clinical Presentations of Some Immunodeficiency Disorders 
    • Laboratory Tests of Immune Function 
    • Summary of Laboratory Findings in the Diagnosis of Antibody Deficiencies 
    • Summary of Therapeutic Considerations for Primary Immunodeficiencies and their Complications 
    • Regimens for Prophylaxis of Bacterial Respiratory Tract Infections 
    • Clinical and Laboratory Manifestations of Selected Combined Immunodeficiencies and Syndromes 
    • Lymphocyte Phenotype Classification of SCID 
    • Assessing Serotype-specific Responses to Pneumococcal Capsular Polysaccharides 
    • Summary of Screening Laboratory Findings and Diagnosis of Complement Deficiencies 
    • Clinical Associations with Complement Deficiency 
    • Classification of Primary Immunodeficiencies 
    • Categories of PIDD 
  • Internet Resources for PIDDs
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This pocket guide 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. This pocket guide should not be considered exclusive of other methods of care reasonably directed at obtaining the same results. The ultimate judgment concerning the propriety of any course of conduct must be made by the clinician after 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.


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