Disseminated Nontuberculous Mycobacterial Disease in NEMO and Iκbα Defects
Abstract Disseminated nontuberculous mycobacterial disease is a severe complication of NEMO (IKBKG) and IκBα (NFKBIA) defects. It may present in adulthood, and diagnosis was often delayed. Despite intensive multidrug therapy and adjunctive IFN-γ, treatment responses were frequently inadequate. Hematopoietic cell transplantation may offer immune reconstitution but carries risks.
Authors
- Elias T. Zambidis (ORCID: https://orcid.org/0000-0001-5452-254X)
- Gülbû Uzel (ORCID: https://orcid.org/0000-0002-3942-1086)
- Mark T. Vander Lugt (ORCID: https://orcid.org/0000-0001-9714-4454)
- Amy P. Hsu (ORCID: https://orcid.org/0000-0001-6841-2122)
- Lavenda Chirombo Kluczynski
- Adrian M. Zelazny (ORCID: https://orcid.org/0000-0003-2847-7428)
- Annalie J Harris (ORCID: https://orcid.org/0009-0007-8231-8002)
- Douglas Byron Kuhns (ORCID: https://orcid.org/0000-0002-9971-0760)
- David Erwin Kleiner (ORCID: https://orcid.org/0000-0003-3442-4453)
- Alyssa R. Letourneau (ORCID: https://orcid.org/0009-0003-1514-2976)
- Howard M. Lederman (ORCID: https://orcid.org/0000-0001-7076-0740)
- Chen Wang (ORCID: https://orcid.org/0000-0003-4055-7391)
- Debra A. Long Priel (ORCID: https://orcid.org/0000-0002-9434-4503)
- Steven M. Holland (ORCID: https://orcid.org/0000-0003-3207-5464)
- Maura M. Manion (ORCID: https://orcid.org/0000-0003-0991-0011)
- Lindsey B. Rosen (ORCID: https://orcid.org/0000-0001-5894-3878)
- Janine R. Daub
- M. Elizabeth M. Younger
- Morgan N. Similuk (ORCID: https://orcid.org/0000-0002-0403-2689)
- Christa S. Zerbe (ORCID: https://orcid.org/0000-0003-4219-0503)
- Luxin Pei (ORCID: https://orcid.org/0000-0003-4865-2473)
- Irini Sereti (ORCID: https://orcid.org/0000-0002-0076-0224)
- Chi A. Ma (ORCID: https://orcid.org/0000-0003-1923-5961)
- Anahita Agharahimi
- Alexandra F. Freeman
- John I. Gallin
- Danielle Fink
- John J. Lipuma
- Amanda Elias Rivera
Institutions
- Leidos (United States) (US)
- National Institutes of Health (US)
- Johns Hopkins University (US)
- University of Michigan (US)
- Johns Hopkins Medicine (US)
- Massachusetts General Hospital (US)
- Michigan Medicine (US)
- National Institute of Allergy and Infectious Diseases (US)
- National Cancer Institute (US)
- Center for Cancer Research (US)
- National Institutes of Health Clinical Center (US)
Publication Details
- Journal
- Clinical Infectious Diseases
- Published
- 2026-10-22
- DOI
- https://doi.org/10.1093/cid/ciag602
- Primary Topic
- Immunodeficiency and Autoimmune Disorders
- Type
- article
- Field-Weighted Citation Impact
- 0.00