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Tang, P. IPEX Syndrome. Encyclopedia. Available online: https://encyclopedia.pub/entry/6057 (accessed on 20 September 2026).
Tang P. IPEX Syndrome. Encyclopedia. Available at: https://encyclopedia.pub/entry/6057. Accessed September 20, 2026.
Tang, Peter. "IPEX Syndrome" Encyclopedia, https://encyclopedia.pub/entry/6057 (accessed September 20, 2026).
Tang, P. (2021, January 04). IPEX Syndrome. In Encyclopedia. https://encyclopedia.pub/entry/6057
Tang, Peter. "IPEX Syndrome." Encyclopedia. Web. 04 January, 2021.
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Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome primarily affects males and is caused by problems with the immune system. The immune system normally protects the body from foreign invaders, such as bacteria and viruses, by recognizing and attacking these invaders and clearing them from the body. However, the immune system can malfunction and attack the body's own tissues and organs instead, which is known as autoimmunity. IPEX syndrome is characterized by the development of multiple autoimmune disorders in affected individuals. Although IPEX syndrome can affect many different areas of the body, autoimmune disorders involving the intestines, skin, and hormone-producing (endocrine) glands occur most often. IPEX syndrome can be life-threatening in early childhood.
genetic conditions
References
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- Bin Dhuban K, Piccirillo CA. The immunological and genetic basis of immunedysregulation, polyendocrinopathy, enteropathy, X-linked syndrome. Curr OpinAllergy Clin Immunol. 2015 Dec;15(6):525-32. doi: 10.1097/ACI.0000000000000214.Review.
- Fuchizawa T, Adachi Y, Ito Y, Higashiyama H, Kanegane H, Futatani T, KobayashiI, Kamachi Y, Sakamoto T, Tsuge I, Tanaka H, Banham AH, Ochs HD, Miyawaki T.Developmental changes of FOXP3-expressing CD4+CD25+ regulatory T cells and their impairment in patients with FOXP3 gene mutations. Clin Immunol. 2007Dec;125(3):237-46.
- Nik Tavakoli N, Hambly BD, Sullivan DR, Bao S. Forkhead box protein 3:essential immune regulatory role. Int J Biochem Cell Biol. 2008;40(11):2369-73.
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- Peterson RA. Regulatory T-cells: diverse phenotypes integral to immunehomeostasis and suppression. Toxicol Pathol. 2012;40(2):186-204. doi:10.1177/0192623311430693.
- Tan QKG, Louie RJ, Sleasman JW. IPEX Syndrome. 2004 Oct 19 [updated 2018 Jul19]. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K,Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University ofWashington, Seattle; 1993-2020. Available fromhttp://www.ncbi.nlm.nih.gov/books/NBK1118/
- Torgerson TR, Ochs HD. Immune dysregulation, polyendocrinopathy, enteropathy, X-linked: forkhead box protein 3 mutations and lack of regulatory T cells. JAllergy Clin Immunol. 2007 Oct;120(4):744-50; quiz 751-2. Review.
- van der Vliet HJ, Nieuwenhuis EE. IPEX as a result of mutations in FOXP3. ClinDev Immunol. 2007;2007:89017. doi: 10.1155/2007/89017. Review.
- Verbsky JW, Chatila TA. Immune dysregulation, polyendocrinopathy, enteropathy,X-linked (IPEX) and IPEX-related disorders: an evolving web of heritableautoimmune diseases. Curr Opin Pediatr. 2013 Dec;25(6):708-14. doi:10.1097/MOP.0000000000000029. Review.
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