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| Content Provider | World Health Organization (WHO)-Global Index Medicus |
|---|---|
| Author | Randhawa, Shahid R. Chahine, Bassem G. Lowery-Nordberg, Mary Cotelingam, James D. Casillas, Adrian M. |
| Description | Country affiliation: United States Author Affiliation: Randhawa SR ( Department of Pediatrics, Section of Allergy and Immunology, LSU Health Sciences Center, Shreveport, Louisiana 71130, USA.) |
| Abstract | OBJECTIVE: To compare autoimmune lymphoproliferative syndrome (ALPS) and Stevens-Johnson syndrome (SJS) with respect to the defects in Fas- and Fas ligand (FasL)-mediated apoptosis. DATA SOURCES: Selected reviews, case reports, and original studies were searched in PubMed and MEDLINE for the keywords ALPS, SJS, Fas, FasL, and apoptosis. STUDY SELECTION: Case reports of ALPS and SJS were selected as examples of Fas- and FasL-mediated diseases. In addition, we selected articles that examined the pathophysiology of apoptosis in the context of Fas-FasL interaction. RESULTS: Failure to initiate apoptosis of abnormal T lymphocytes occurs in such diseases as ALPS, leading to the accumulation of double negative T cells with an increase in autoimmunity. In contrast to apoptotic failure, SJS is associated with a pathological increase in programmed keratinocyte cell death. CONCLUSION: The consequences of dysregulated Fas- and FasL-mediated apoptosis leads to self-reactivity, malignant transformation, and immune dysfunction. An understanding of underlying mechanisms and qualitative assessment of Fas and FasL may have clinical benefits when control of these homeostatic mechanisms is in question. |
| File Format | HTM / HTML |
| ISSN | 10811206 |
| Issue Number | 4 |
| Volume Number | 104 |
| e-ISSN | 15344436 |
| Journal | Annals of Allergy, Asthma & Immunology |
| Language | English |
| Publisher | Elsevier (on behalf of the American College of Allergy, Asthma and Immunology) |
| Publisher Date | 2010-04-01 |
| Publisher Place | United States |
| Access Restriction | One Nation One Subscription (ONOS) |
| Subject Keyword | Discipline Immunology Antigens, Cd95 Metabolism Autoimmune Lymphoproliferative Syndrome Physiopathology Fas Ligand Protein Stevens-johnson Syndrome Adult Genetics Apoptosis Physiology Drug Therapy Immunology Pathology Female Humans Infant Case Reports Journal Article Meta-analysis Review |
| Content Type | Text |
| Resource Type | Case study Article |
| Subject | Pulmonary and Respiratory Medicine Immunology and Allergy Immunology |
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