Literature DB >> 34143686

Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked Syndrome Manifesting as Lymphocytic Interstitial Pneumonia and Treatment-Resistant Bullous Pemphigoid.

Stine Maria Andersen1, Simon Fage1, Sune Leisgaard Rubak2, Mette Holm2, Jens Magnus Bernth Jensen3, Trine Mogensen4, Mette Deleuran1.   

Abstract

Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare immune deficiency with a broad clinical presentation. IPEX syndrome causes dysfunctional regulatory T cells, increasing the risk of autoimmune diseases. In this case report, we describe a 7-year-old boy with lymphocytic interstitial pneumonia and bullous pemphigoid who was recently diagnosed with IPEX syndrome.

Entities:  

Keywords:  IPEX; bullous pemphigoid; lymphocytic interstitial pneumonia

Mesh：

Substances：

Year:  2021        PMID： 34143686      PMCID： PMC8329723          DOI： 10.1089/ped.2020.1307

Source DB:  PubMed          Journal:  Pediatr Allergy Immunol Pulmonol        ISSN： 2151-321X            Impact factor:   0.885


  16 in total

1.  Regulatory T-cell dysfunction induces autoantibodies to bullous pemphigoid antigens in mice and human subjects.

Authors:  Ken Muramatsu; Hideyuki Ujiie; Ichiro Kobayashi; Wataru Nishie; Kentaro Izumi; Takamasa Ito; Norihiro Yoshimoto; Ken Natsuga; Hiroaki Iwata; Hiroshi Shimizu
Journal:  J Allergy Clin Immunol       Date:  2018-04-26       Impact factor: 10.793

Review 2.  Paediatric interstitial lung disease: classification and definitions.

Authors:  Megan K Dishop
Journal:  Paediatr Respir Rev       Date:  2011-03-05       Impact factor: 2.726

Review 3.  IPEX is a unique X-linked syndrome characterized by immune dysfunction, polyendocrinopathy, enteropathy, and a variety of autoimmune phenomena.

Authors:  C L Bennett; H D Ochs
Journal:  Curr Opin Pediatr       Date:  2001-12       Impact factor: 2.856

4.  FOXP3 forkhead domain mutation and regulatory T cells in the IPEX syndrome.

Authors:  Eva d'Hennezel; Moshe Ben-Shoshan; Hans D Ochs; Troy R Torgerson; Laura J Russell; Christine Lejtenyi; Francisco J Noya; Nada Jabado; Bruce Mazer; Ciriaco A Piccirillo
Journal:  N Engl J Med       Date:  2009-10-22       Impact factor: 91.245

Review 5.  Immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome (IPEX) associated with pemphigoid nodularis: a case report and review of the literature.

Authors:  Jamie L McGinness; Mary-Margaret C Bivens; Kenneth E Greer; James W Patterson; Frank T Saulsbury
Journal:  J Am Acad Dermatol       Date:  2006-07       Impact factor: 11.527

6.  Diagnostic Pathology of Diffuse Lung Disease in Children.

Authors:  Megan K Dishop
Journal:  Pediatr Allergy Immunol Pulmonol       Date:  2010-03       Impact factor: 1.349

Review 7.  Dermatologic and immunologic findings in the immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome.

Authors:  David S Nieves; Richard P Phipps; Stephen J Pollock; Hans D Ochs; Qili Zhu; Glynis A Scott; Charlotte K Ryan; Ichiro Kobayashi; Thomas M Rossi; Lowell A Goldsmith
Journal:  Arch Dermatol       Date:  2004-04

Review 8.  Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) and IPEX-related disorders: an evolving web of heritable autoimmune diseases.

Authors:  James W Verbsky; Talal A Chatila
Journal:  Curr Opin Pediatr       Date:  2013-12       Impact factor: 2.856

9.  Clinical, Immunological, and Molecular Heterogeneity of 173 Patients With the Phenotype of Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked (IPEX) Syndrome.

Authors:  Eleonora Gambineri; Sara Ciullini Mannurita; David Hagin; Marina Vignoli; Stephanie Anover-Sombke; Stacey DeBoer; Gesmar R S Segundo; Eric J Allenspach; Claudio Favre; Hans D Ochs; Troy R Torgerson
Journal:  Front Immunol       Date:  2018-11-01       Impact factor: 7.561

10.  Bullous pemphigoid in infants: characteristics, diagnosis and treatment.

Authors:  Agnes Schwieger-Briel; Cornelia Moellmann; Birgit Mattulat; Franziska Schauer; Dimitra Kiritsi; Enno Schmidt; Cassian Sitaru; Hagen Ott; Johannes S Kern
Journal:  Orphanet J Rare Dis       Date:  2014-12-10       Impact factor: 4.123
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  1 in total

1.  Effects of pulmonary rehabilitation therapy combined with conventional drugs on BODE and pulmonary function indexes in elderly patients with interstitial pneumonia.

Authors:  Zhen Zhang; Yumei Tian; Jing Yang
Journal:  Pak J Med Sci       Date:  2022 Jul-Aug       Impact factor: 2.340
  1 in total

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