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NIK deficiency

Related gene: NIK

Inheritance pattern: Autosomal recessive

NF-κB-inducing kinase (NIK) deficiency is a rare immunodeficiency resulting in impaired function of T cells, B cells, and natural killer (NK) cells. T cells are generally normal in number, whereas B cells and NK cells are decreased. Affected individuals are susceptible to a wide range of infections, including opportunistic parasites such as Cryptosporidium. Development of secondary lymphatics may be impaired. NIK deficiency may be amenable to hematopoietic stem cell transplant (HSCT), though the ideal methods remain unclear.

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This page contains general medical and/or legal information that cannot be applied safely to any individual case. Medical and/or legal knowledge and practice can change rapidly. Therefore, this page should not be used as a substitute for professional medical and/or legal advice.

Adapted from the IDF Patient & Family Handbook for Primary Immunodeficiency Diseases, Sixth Edition 
Copyright ©2019 by Immune Deficiency Foundation, USA