ArticleClinical immunology (Orlando, Fla.)2009
Persistent systemic inflammation and atypical enterocolitis in patients with NEMO syndrome.
Article in Clinical immunology (Orlando, Fla.), 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers.
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Who cites it
31 citing papers in PubMed.
- Interface between inborn errors of immunity and rheumatological disorders in children: A pediatrician's conundrum.World journal of clinical pediatrics · 2026Review
- Pediatric ectodermal dysplasia with immunodeficiency caused by a hemizygousFrontiers in immunology · 2026Review
- Clinical relevance of loss-of-function mutations ofGenes & diseases · 2025Article
- Clinical Features and Genetic Analysis of Taiwanese Primary Immunodeficiency Patients with Prolonged Diarrhea and Monogenetic Inflammatory Bowel Disease.Journal of clinical immunology · 2023Article
- Article
- Low Density Granulocytes and Dysregulated Neutrophils Driving Autoinflammatory Manifestations in NEMO Deficiency.Journal of clinical immunology · 2022Article
- Very Early Onset Inflammatory Bowel Disease: A Clinical Approach With a Focus on the Role of Genetics and Underlying Immune Deficiencies.Inflammatory bowel diseases · 2020Review
- T Cell Impairment Is Predictive for a Severe Clinical Course in NEMO Deficiency.Journal of clinical immunology · 2020Article
- Gastrointestinal Disorders Associated with Primary Immunodeficiency Diseases.Clinical reviews in allergy & immunology · 2019Review
- Missense variants in NOX1 and p22phox in a case of very-early-onset inflammatory bowel disease are functionally linked to NOD2.Cold Spring Harbor molecular case studies · 2019Article
- Recent Advance in Very Early Onset Inflammatory Bowel Disease.Pediatric gastroenterology, hepatology & nutrition · 2019Review
- Recent advance in very early-onset inflammatory bowel disease.Intestinal research · 2019Article
- Monogenic Intestinal Epithelium Defects and the Development of Inflammatory Bowel Disease.Physiology (Bethesda, Md.) · 2018Review
- NOX1 loss-of-function genetic variants in patients with inflammatory bowel disease.Mucosal immunology · 2018Article
- A Nationwide Study of Severe and Protracted Diarrhoea in Patients with Primary Immunodeficiency Diseases.Scientific reports · 2017Article
- Novel Mutations of theFrontiers in immunology · 2017Article
- Transplantation from a symptomatic carrier sister restores host defenses but does not prevent colitis in NEMO deficiency.Clinical immunology (Orlando, Fla.) · 2016Article
- Article
- Recruitment of A20 by the C-terminal domain of NEMO suppresses NF-κB activation and autoinflammatory disease.Proceedings of the National Academy of Sciences of the United States of America · 2016Article
- Primary Immunodeficiencies and Inflammatory Disease: A Growing Genetic Intersection.Trends in immunology · 2016Review
Corrections and comments
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Authors and funding
8 authors.
Funding
Abstract
The NEMO syndrome is a primary immunodeficiency with immune and non-immune manifestations. The immune deficiency is heterogeneous showing defects in humoral, innate, and cell-mediated immunity. While the clinical aspects of the immunodeficiency are increasingly well understood, little is known about autoimmune manifestations in NEMO patients. We therefore sought to examine serologic markers of systemic inflammation and intestinal pathology in a kindred of patients with the NEMO syndrome. We observed persistent elevation of erythrocyte sedimentation rates in five patients, and two were symptomatic, with a chronic but atypical enterocolitis. Though pathologic lesions in these two patients were consistent with acute inflammation, sustained clinical improvement was only achieved with systemic and/or topical glucocorticoid therapy. Our data suggest that some patients with the NEMO syndrome exhibit persistent elevation of inflammatory markers similar to systemic autoimmune diseases and may subsequently develop an atypical enterocolitis.
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