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Primary Immunodeficiency Diseases

  • Fayhan Alroqi
  • Abdulrahman Alsultan
  • Mohammed Essa
Chapter

Abstract

Primary immunodeficiency diseases (PIDs) are a heterogeneous group of monogenic disorders in which part of the immune system is deficient or does not function properly. Patients with PID commonly present with recurrent infections, which explains the importance of the immune system in protection from organisms. PIDs are classified according to the affected component of the immune system into innate immunity disorders and adaptive immunity disorders. Early diagnosis and aggressive management ranging from medical treatment to stem cell transplantation are crucial to decrease disease-associated morbidity and mortality. In this review, we will provide a detailed overview of classification, diagnosis, and treatments of PIDs, with particular attention to the critical care management of the common complications of these rare diseases.

Keywords

Primary immunodeficiency diseases Innate immunodeficiencies Humoral immunodeficiencies Cellular immunodeficiencies Hematopoietic cell transplantation Gene therapy 

Notes

Conflict of Interest

The authors declare that they have no conflict of interest.

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Copyright information

© Springer International Publishing 2019

Authors and Affiliations

  • Fayhan Alroqi
    • 1
    • 2
    • 3
  • Abdulrahman Alsultan
    • 1
    • 4
  • Mohammed Essa
    • 1
    • 2
  1. 1.Department of PediatricKing Abdullah Specialized Children’s HospitalRiyadhSaudi Arabia
  2. 2.King Saud bin Abdulaziz University for Health SciencesRiyadhSaudi Arabia
  3. 3.King Abdullah International Medical Research CenterRiyadhSaudi Arabia
  4. 4.Department of Pediatric, College of MedicineKing Saud UniversityRiyadhSaudi Arabia

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