Partial C4 deficiency in two children with systemic lupus erythematosus

Détails

ID Serval
serval:BIB_DC7680E8B83D
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Etude de cas (case report): rapporte une observation et la commente brièvement.
Collection
Publications
Titre
Partial C4 deficiency in two children with systemic lupus erythematosus
Périodique
Turkish Journal of Pediatrics
Auteur⸱e⸱s
Bakkaloglu  A., Pascual  M., Schifferli  J. A., Ozen  S., Besbas  N., Saatci  U.
ISSN
0041-4301
Statut éditorial
Publié
Date de publication
06/1995
Peer-reviewed
Oui
Volume
37
Numéro
2
Pages
147-51
Notes
Case Reports
Journal Article
Research Support, Non-U.S. Gov't --- Old month value: Apr-Jun
Résumé
Systemic lupus erythematosus (SLE) is a rare disease in childhood. Here two cases with SLE are presented, both with C4 null alleles yielding a functional C4 deficiency. The first case, a 14-year-old girl with a C4A null allele only, had a mild disease course, whereas the second child, a seven-year-old boy with both C4A0 and C4B0, had a more relentless course leading to death in five years. We conclude that complement activation by the classical pathway might be an essential mechanism that protects against the emergence of autoimmune or immune-complex diseases, and that the deficient state in our patients predisposed them to the early development of SLE.
Mots-clé
Adolescent Child Complement C4/*deficiency Female Humans Lupus Erythematosus, Systemic/*immunology Male
Pubmed
Web of science
Création de la notice
29/01/2008 13:53
Dernière modification de la notice
20/08/2019 16:01
Données d'usage