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Agammaglobulinemia: Comorbidities and Long-Term Therapeutic Risks Publisher



Rahmani F1, 2 ; Aghamohammadi A1, 3 ; Ochs HD4, 5 ; Rezaei N1, 6, 7
Authors
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Authors Affiliations
  1. 1. Research Center for Immunodeficiencies, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  2. 2. Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran
  3. 3. Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran
  4. 4. Department of Pediatrics, University of Washington and Seattle Children’s Research Institute, Seattle, WA, United States
  5. 5. Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Seattle, WA, United States
  6. 6. Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
  7. 7. Systematic Review and Meta-Analysis Expert Group (SRMEG), Universal Scientific Education and Research Network (USERN), Sheffield, United Kingdom

Source: Expert Opinion on Orphan Drugs Published:2017


Abstract

Introduction: Intravenous Immunoglobulin preparations have long been used as an effective therapeutic approach to patients with predominant antibody deficiencies. The advent of new manufacturing processes and critical control measures have continuously improved the quality of Immunoglobulin preparations, while reducing the rate of adverse events and maintaining their function. Subcutaneous route of administration is becoming a standard of care for safe use of IgG by self-administration at home. Areas covered: Literature search was performed through Pubmed and Scopus using the search term: (‘Therapeutics’ OR ‘Immunoglobulins, Intravenous’ OR ‘IVIG’) AND (‘Agammaglobulinemia tyrosine kinase’ OR ‘Bruton type agammaglobulinemia’ OR ‘Agammaglobulinemia’ OR ‘Agammaglobulinemia, Autosomal Dominant’) with literature search. Expert opinion: Most if not all of the infusion related complications of IVIG can be prevented by closely monitoring the patient, by considering slow infusion and by identifying, and if possible reducing, pre-infusion risk factors such as cardiovascular or renal problems, infections and advanced age and by selecting the safest product accordingly. The choice of IVIG product should be based on patients individual risk factors and, if present, history of previous adverse events with a specific brand. It should be made clear to patients that FDA-approved IVIG brands are not different in efficacy, and patients preference in choice of brand should be considered. © 2017 Informa UK Limited, trading as Taylor & Francis Group.
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