NSTL国家科技图书文献中心

1. The protective role of transcription factor Nrf2 in murine macrophage activation syndrome NSTL国家科技图书文献中心

Gallo, Paul M. | Elliott, Em ... - 《Journal of Leukocyte Biology》 - 2025,117(3) - qiae225～qiae225 - 共10页

摘要：Objective: Macrophage activation syndrome (MAS) is characterized by multilineage cytopenias, hypercytokinemia, and tissue hemophagocytosis. Transcription factor Nrf2 is a master regulator of redox hom...

关键词： hemophagocytosis | inflammation | macrophage activation syndrome

2. The Liver in Hemophagocytic Lymphohistiocytosis: Not an Innocent Bystander NSTL国家科技图书文献中心

Diamond, Tamir | Bennett, Aaron D.... - 《Journal of pediatric gastroenterology and nutrition》 - 2023,77(2) - 153～159 - 共7页 - 被引量：1

摘要：Hemophagocytic lymphohistiocytosis (HLH) is a rare multisystemic hyperinflammatory disease commonly associated with hepatic dysfunction. Liver injury is mediated by unchecked antigen presentation, hyp...

关键词： hemophagocytic lymphohistiocytosis | hemopoietic stem cell transplant | interferon gamma | liver transplant | macrophage activation syndrome | pediatric acute liver failure | T-CELLS | FAILURE | GAMMA | ETOPOSIDE...

3. Adenovirus is Not Detected in Liver Tissue From a Historical Multicenter Cohort of Children With Acute Liver Failure NSTL国家科技图书文献中心

Chapin, Catherine A. | Diamond, Tamir ... - 《Journal of pediatric gastroenterology and nutrition》 - 2023,77(3) - 393～395 - 共3页

摘要：There has been a recent surge in cases of pediatric acute hepatitis and pediatric acute liver failure (PALF) of unknown cause. Several reports have described clusters of these children who were positi...

关键词： hepatitis | immune dysregulation | inflammation | HEPATITIS

4. Improvement of Refractory Systemic Juvenile Idiopathic Arthritis-Associated Lung Disease with Single-Agent Blockade of IL-1 beta and IL-18 NSTL国家科技图书文献中心

Rood, Julia E. | Rezk, Ayman ... - 《Journal of Clinical Immunology》 - 2023,43(1) - 101～108 - 共8页 - 被引量：22

摘要：Systemic juvenile idiopathic arthritis associated with interstitial lung disease (SJIA-LD) represents a highly morbid subset of SJIA for which effective therapies are lacking. We report the case of a ...

关键词： Systemic juvenile idiopathic arthritis | interleukin-18 | interleukin-1 beta | interstitial lung disease | immunotherapy | MACROPHAGE ACTIVATION SYNDROME | INTERLEUKIN-18

5. Lymphocytes Utilize Somatic Mutations, Epigenetic Silencing, and the Proteasome to Escape Truncated WASP Expression SCIE Web of Science核心 NSTL国家科技图书文献中心

Khanna, Caroline | Le Coz, Carole ... - 《Journal of Clinical Immunology》 - 2022,42(4) - 753～759 - 共7页

摘要：Wiskott-Aldrich Syndrome Protein (WASP) deficiency causes Wiskott-Aldrich Syndrome (WAS), a sex-linked disorder characterized by combined immunodeficiency, microthrombocytopenia, and eczema. Like WASP...

关键词： Wiskott-Aldrich Syndrome | WAS | WASP | Proteosome | WISKOTT-ALDRICH-SYNDROME

6. American College of Rheumatology Clinical Guidance for Multisystem Inflammatory Syndrome in Children Associated With SARS-CoV-2 and Hyperinflammation in Pediatric COVID-19: Version 3 SCIE Web of Science核心 NSTL国家科技图书文献中心

Henderson, Lauren A. | Canna, Scott W.... - 《Arthritis & rheumatology.》 - 2022,74(4) - E1～E20 - 共20页 - 被引量：86

摘要：Objective To provide guidance on the management of Multisystem Inflammatory Syndrome in Children (MIS-C), a condition characterized by fever, inflammation, and multiorgan dysfunction that manifests la...

关键词： JUVENILE IDIOPATHIC ARTHRITIS | REFRACTORY KAWASAKI-DISEASE | CONGENITAL HEART-DISEASE | LONG-TERM MANAGEMENT | RANDOMIZED-TRIAL | SCIENTIFIC STATEMENT | HEALTH-PROFESSIONALS | OPEN-LABEL | THERAPY | MULTICENTER

7. Severe Acute Respiratory Syndrome-Coronavirus-2 (SARS-CoV-2) Antibody Responses in Children With Multisystem Inflammatory Syndrome in Children (MIS-C) and Mild and Severe Coronavirus Disease 2019 (COVID-19) SCIE Web of Science核心 NSTL国家科技图书文献中心

Anderson, Elizabeth ... | Diorio, Caroline ... - 《Journal of the Pediatric Infectious Diseases Society.》 - 2021,10(5) - 669～673 - 共5页 - 被引量：21

摘要：Severe acute respiratory syndrome-coronavirus-2 (SARSCoV-2) antibody responses in children remain poorly characterized. Here, we show that pediatric patients with multisystem inflammatory syndrome in ...

关键词： antibodies | COVID-19 | multisystem inflammatory syndrome in children (MIS-C) | pediatric | SARS-COV-2

8. Colonoids From Patients With Pediatric Inflammatory Bowel Disease Exhibit Decreased Growth Associated With Inflammation Severity and Durable Upregulation of Antigen Presentation Genes SCOPUS Scopus数据库 SCIE Web of Science核心 NSTL国家科技图书文献中心

Kelsen, Judith R. | Dawany, Noor ... - 《Inflammatory bowel diseases》 - 2021,27(2) - 256～267 - 共12页 - 被引量：6

摘要：Background: Defining epithelial cell contributions to inflammatory bowel disease (IBD) is essential for the development of much needed therapies for barrier repair. Children with very early onset (VEO...

关键词： epithelial cells | inflammatory bowel disease | VEO-IBD | enteroids | colonoids

9. Variants in STXBP3 are Associated with Very Early Onset Inflammatory Bowel Disease, Bilateral Sensorineural Hearing Loss and Immune Dysregulation SCIE Web of Science核心 SCOPUS Scopus数据库 NSTL国家科技图书文献中心

Ouahed, Jodie | Kelsen, Judith R.... - 《Journal of Crohn’s & colitis》 - 2021,15(11) - 1908～1919 - 共12页 - 被引量：8

摘要：Background and Aims: Very early onset inflammatory bowel disease [VEOIBD] is characterized by intestinal inflammation affecting infants and children less than 6 years of age. To date, over 60 monogeni...

关键词： VEOIBD | sensorineural hearing loss | STXBP3 | FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS | CLINICAL PRESENTATIONS | CELL-SURFACE | HUMAN COLON | MUTATIONS | DEFICIENCY | MECHANISMS...

10. Multisystem Inflammatory Syndrome in Children During the Coronavirus 2019 Pandemic: A Case Series SCOPUS Scopus数据库 SCIE Web of Science核心 NSTL国家科技图书文献中心

Chiotos, Kathleen | Bassiri, Hamid ... - 《Journal of the Pediatric Infectious Diseases Society.》 - 2020,9(3) - 393～398 - 共6页 - 被引量：170

摘要：We present a series of 6 critically ill children with multisystem inflammatory syndrome in children. Key findings of this syndrome include fever, diarrhea, shock, and variable presence of rash, conjun...