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Primary Immunodeficiency Diseases: Definition, Diagnosis, ...
autoimmune diseases, in particular of the gut and endocrine organs, should therefore raise the suspicion of IPEX. 5.7.4 Diagnosis The various autoimmune features in IPEX often appear sequentially rather than simultaneously, rendering the ...
Nima Rezaei, Asghar Aghamohammadi, Luigi D. Notarangelo, 2008
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Nutrition Support for Infants and Children at Risk - Page 87
However IPEX boys often have several thousands, up to 70,000kU, and if you perform RAST testing they are all positive. We did not go further to test if these IgE levels are really functional. It is not very surprising when you have 10,000 times ...
Richard J. Cooke, Yvan Vandenplas, Ulrich Wahn, 2007
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Textbook of Clinical Pediatrics - Page 1308
Eczema is the common dermatitis in IPEX but erythroderma, psoriasiform dermatitis, and pemphigoid nodularis have also been observed. In addition to the ''IPEX-triad,'' most patients with IPEX also have other associated autoimmune ...
A. Y. Elzouki, H. A. Harfi, F. B. Stapleton, 2012
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Primary Immunodeficiency Diseases : A Molecular & Cellular ...
Updated pedigree of the IPEX kindred originally described. In 1982, Powell and colleagues reported on a large family with multiple affected males in a five-generation pedigree (Fig. 26.1). Affected males presented early in life with multiple ...
Department of Pediatrics University of Washington School of Medicine Hans D. Ochs Professor, Seattle, Center for Biotechnology C. I. Edward Smith Division of Clinical Immunology at Huddinge Hospital, Immunologic Genetics Section Jennifer M. Puck Director, Genetics and Molecular Biology Branch National Human Genome Research Institute National Inst, 2006
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Pediatric Allergy: Principles and Practice - Page 125
IPEX syndrome (OMIM #304930) is the prototype of defective peripheral immune tolerance. The basic clinical triad of IPEX includes autoimmune enteropathy, early onset endocrinopathy and dermatitis (Box 13-2).16–18 The enteropathy ...
Donald Y. M. Leung, Hugh Sampson, Cezmi A. Akdis, M.D., 2015
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Textbook of Pediatric Gastroenterology, Hepatology and ... - Page 18
IPEX is a rare disease and represents a systemic form of AIE characterized by immune dysfunction, polyendocrinopathy, enteropathy and X-linked inheritance. The prevalence of the syndrome remains unknown. Even though the severity of ...
Stefano Guandalini, Anil Dhawan, David Branski, 2015
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Standards and Global Trade: A Voice for Africa - Page 83
Export Promotion The Export Promotion Institute (IPEX). The Export Promotion Institute (IPEX) has administrative and financial autonomy. It was set up in November 1990 to spearhead the export promotion efforts of the Government of the ...
John Sullivan Wilson, Victor O. Abiola, 2003
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Stiehm's Immune Deficiencies - Page 502
Box 23.2 Clinical Manifestations of IPEX G Autoimmune enteropathy G Eczema G Type 1 diabetes mellitus G Allergic disease G Autoimmune thyroiditis G Autoimmune cytopenias G Autoimmune hepatitis G Autoimmune renal disease G ...
Kathleen E Sullivan, E. Richard Stiehm, 2014
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Immunoendocrinology: Scientific and Clinical Aspects - Page 137
We previously demonstrated that CD4+CD25+ T cells are not required for the normal differentiation of Tr1 cells (49) and we recently observed that Tr1 cells can also develop in IPEX patients even when FOXP3 expression is completely ...
George S. Eisenbarth, 2010
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Mount Sinai Expert Guides: Allergy and Clinical Immunology - Page 382
ChApter 46 X‐linked Immune Dysregulation with Polyendocrinopathy (IPEX) Syndrome Elena S. Resnick Icahn School of Medicine at Mount Sinai, New York, NY, USA OvErall BOttOm lInE • Immunodysregulation, polyendocrinopathy, ...
