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Cytomegalovirus infection in infants with autoimmune lymphoproliferative syndrome (ALPS)

Lookup NU author(s): Dr Brian Angus, Professor Andrew Cant


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Fas-mediated apoptosis may be one of the effector pathways leading to the elimination of virus-infected cells. Cytomegalovirus (CMV) infection in two brothers with Fas deficiency associated with autoimmunity and benign lymphoproliferation (ALPS) provided a unique opportunity to study the clinical course of CMV infection in children with defective apoptosis. The clinical courses of two brothers with autosomal dominant ALPS who were infected with CMV in the neonatal period are described. CMV was detected from throat and urine culture from the brothers. ALPS was confirmed by in vitro anti-CD95 MoAb-induced T lymphocyte apoptosis assay and subsequent sequencing and identification of mutations in the Fas gene. A de novo mutation in the Fas gene, leading to a truncated cytoplasmic Fas product, was associated with autosomal dominant ALPS in a mother and her two sons. Both boys had evidence of CMV infection acquired early in infancy which cleared by the age of 2-3 years. There were no neurodevelopmental sequelae. The natural history of CMV infection in two infants with ALPS was similar to that described in normal children.

Publication metadata

Author(s): Arkwright PD, Rieux-Laucat F, Le Deist F, Stevens RF, Angus B, Cant AJ

Publication type: Article

Publication status: Published

Journal: Clinical and Experimental Immunology

Year: 2000

Volume: 121

Issue: 2

Pages: 353-357

ISSN (print): 0009-9104

ISSN (electronic): 1365-2249

Publisher: Blackwell Science


DOI: 10.1046/j.1365-2249.2000.01304.x


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