13
Treatment
Î SS 10. After diagnosis of a PIDD, it is important to proceed quickly
with preventive therapy, replacement therapy, or both. (C)
Î SS 11. Immunoglobulin replacement therapy is indicated for all
disorders with significantly impaired antibody production. (B)
Î SS 12. In association with low IgG levels, IgA deficiency is not a
contraindication to IgG therapy. (C)
Î SS 13. Patients receiving IgG therapy should have regular monitoring
of IgG trough levels, blood cell counts, and serum chemistry. (D)
Î SS 14. The placement of permanent central venous access solely for
the purpose of IVIG administration should be discouraged. (F)
Î SS 15. Aggressive and prolonged antimicrobial therapy should be
considered for immunodeficient patients. (C)
Î SS 16. Short- or long-term antimicrobial prophylaxis should be
considered for patients with immunodeficiency. (C)
Î SS 17. Lung imaging and function should be monitored regularly in
patients with a history of or who are at risk for recurrent pneumonia
and/or other chronic lung damage or disease. (C)
Î SS 18. Surgical procedures undertaken with the aim of reducing
infection susceptibility should be approached with caution in patients
with known or suspected PIDDs. (F)
Î SS 19. The recommended definitive therapy of cellular or combined
PIDD is reconstitution by hematopoietic stem cells. (B)
Î SS 20. Only irradiated, CMV-negative, lymphocyte-depleted cellular
blood products should be administered to patients with cellular or
combined PIDDs. (C)
Î SS 21. Live vaccines should not be administered to patients with
severely impaired specific immunity. (C)
Î SS 22. Inactivated or subunit vaccines can be administered to
immunocompromised patients. (C)
Î SS 23. Education for patients and families with PIDDs is
recommended for optimal outcomes. (F)
Î SS 24. Patients with suspected or diagnosed PIDDs are recommended
to have evaluation and follow-up by a clinical immunologist with
experience with these disorders. (F)
Î SS 25. A coordinated multidisciplinary approach to management
should be considered in patients with PIDDs. (F)
