Literature DB >> 16126062

Successful use of the new immune-suppressor sirolimus in IPEX (immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome).

Lutz Bindl1, Troy Torgerson, Lucia Perroni, Nelly Youssef, Hans D Ochs, Olivier Goulet, Frank M Ruemmele.   

Abstract

IPEX (immune-dysregulation, polyendocrinopathy, enteropathy, X-linked) syndrome is an autoimmune disorder with an often lethal outcome in spite of immunosuppressive therapy. We report the successful use of sirolimus in 3 patients with IPEX. The efficacy of sirolimus is probably due to its different mode of action compared to calcineurin-dependent agents.

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Year:  2005        PMID： 16126062     DOI： 10.1016/j.jpeds.2005.04.017

Source DB:  PubMed          Journal:  J Pediatr        ISSN： 0022-3476            Impact factor:   4.406


  25 in total

1.  Mammalian target of rapamycin activation underlies HSC defects in autoimmune disease and inflammation in mice.

Authors:  Chong Chen; Yu Liu; Yang Liu; Pan Zheng
Journal:  J Clin Invest       Date:  2010-10-25       Impact factor: 14.808

2.  Medium-term survival without haematopoietic stem cell transplantation in a case of IPEX: insights into nutritional and immunosuppressive therapy.

Authors:  Andrea Taddio; Elena Faleschini; Erica Valencic; Marilena Granzotto; Alberto Tommasini; Loredana Lepore; Marino Andolina; Egidio Barbi; Alessandro Ventura
Journal:  Eur J Pediatr       Date:  2007-01-05       Impact factor: 3.183

Review 3.  An Update on the Use of Immunomodulators in Primary Immunodeficiencies.

Authors:  Pandiarajan Vignesh; Amit Rawat; Surjit Singh
Journal:  Clin Rev Allergy Immunol       Date:  2017-04       Impact factor: 8.667

Review 4.  An appraisal of the Wilson & Jungner criteria in the context of genomic-based newborn screening for inborn errors of immunity.

Authors:  Jovanka R King; Luigi D Notarangelo; Lennart Hammarström
Journal:  J Allergy Clin Immunol       Date:  2021-02       Impact factor: 10.793

Review 5.  Mechanism-Based Strategies for the Management of Autoimmunity and Immune Dysregulation in Primary Immunodeficiencies.

Authors:  Jolan E Walter; Jocelyn R Farmer; Zsofia Foldvari; Troy R Torgerson; Megan A Cooper
Journal:  J Allergy Clin Immunol Pract       Date:  2016 Nov - Dec

Review 6.  Regulatory T cells in human autoimmune diseases.

Authors:  Troy R Torgerson
Journal:  Springer Semin Immunopathol       Date:  2006-08-11

Review 7.  Cytopenia and autoimmune diseases: a vicious cycle fueled by mTOR dysregulation in hematopoietic stem cells.

Authors:  Pan Zheng; Xing Chang; Qianjin Lu; Yang Liu
Journal:  J Autoimmun       Date:  2013-02-01       Impact factor: 7.094

Review 8.  IPEX, FOXP3 and regulatory T-cells: a model for autoimmunity.

Authors:  Hans D Ochs; Eleonora Gambineri; Troy R Torgerson
Journal:  Immunol Res       Date:  2007       Impact factor: 2.829

Review 9.  Diagnosis and treatment of gastrointestinal disorders in patients with primary immunodeficiency.

Authors:  Shradha Agarwal; Lloyd Mayer
Journal:  Clin Gastroenterol Hepatol       Date:  2013-03-13       Impact factor: 11.382

10.  Immunodysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome: an unusual cause of proteinuria in infancy.

Authors:  Asha Moudgil; Paige Perriello; Brett Loechelt; Ronald Przygodzki; Wendy Fitzerald; Naynesh Kamani
Journal:  Pediatr Nephrol       Date:  2007-07-13       Impact factor: 3.714
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