Document Detail

Abnormal production of tumor necrosis factor (TNF) -- alpha and clinical efficacy of the TNF inhibitor etanercept in a patient with PAPA syndrome [corrected]
MedLine Citation:
PMID:  15580218     Owner:  NLM     Status:  MEDLINE    
We report a family with pyogenic sterile arthritis, pyoderna and acne syndrome (PAPA). The proband presented several episodes of sterile pyogenic arthritis and became unresponsive to glucocorticoids. After treatment with the tumor necrosis factor inhibitor etanercept, the disease underwent rapid and sustained clinical remission. Production of tumor necrosis factor-alpha by mononuclear cells of the proband and of the affected relatives was abnormally elevated.
Elisabetta Cortis; Fabrizio De Benedetti; Antonella Insalaco; Stefania Cioschi; Flaminia Muratori; Leila E D'Urbano; Alberto G Ugazio
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The Journal of pediatrics     Volume:  145     ISSN:  0022-3476     ISO Abbreviation:  J. Pediatr.     Publication Date:  2004 Dec 
Date Detail:
Created Date:  2004-12-06     Completed Date:  2005-01-06     Revised Date:  2005-02-18    
Medline Journal Info:
Nlm Unique ID:  0375410     Medline TA:  J Pediatr     Country:  United States    
Other Details:
Languages:  eng     Pagination:  851-5     Citation Subset:  AIM; IM    
Department of Medicine, Division of Rheumathology and Laboratory of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy.
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MeSH Terms
Acne Vulgaris / complications*,  drug therapy,  genetics
Adaptor Proteins, Signal Transducing / genetics
Anti-Inflammatory Agents, Non-Steroidal / therapeutic use*
Arthritis / complications*,  drug therapy,  genetics
Child, Preschool
Cytoskeletal Proteins / genetics
Immunoglobulin G / therapeutic use*
Pyoderma / complications*,  drug therapy,  genetics
Receptors, Tumor Necrosis Factor / therapeutic use*
Tumor Necrosis Factor-alpha / antagonists & inhibitors*,  biosynthesis
Reg. No./Substance:
0/Adaptor Proteins, Signal Transducing; 0/Anti-Inflammatory Agents, Non-Steroidal; 0/Cytoskeletal Proteins; 0/Immunoglobulin G; 0/PSTPIP1 protein, human; 0/Receptors, Tumor Necrosis Factor; 0/Tumor Necrosis Factor-alpha; 185243-69-0/TNFR-Fc fusion protein
Erratum In:
J Pediatr. 2005 Feb;146(2):193

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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