ISSN:
1432-1076
Keywords:
Leucocyte
;
Adhesion
;
Complement receptor
;
Interferon-γ
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract We describe a patient with leucocyte adhesion deficiency (LAD). Clinically, the patient had delayed umbilical cord detachment, omphalitis, impaired wound healing and persistent leucocytosis. The patient had the severe form of LAD, with a total absence of leucocyte cell adhesion molecules (LeuCAMs) and undetectable mRNA for the β chain, the common subunit of the LeuCAMs. In vitro neutrophil chemotaxis, aggregation and oxygen consumption were severely impaired. In vitro incubation of neutrophils with recombinant human interferon-γ (rIFN-γ) showed an increase in oxygen consumption, but no effect on the expression of the LeuCAMs, or the β chain mRNA. In vivo treatment with IFN-γ was started. The FcγRI receptor appeared on the neutrophils, the LeuCAMs remained undetectable, while the neutrophil functions remained disturbed. The patient died of surgical complications after 10 weeks of rIFN-γ treatment. No new infections or side-effects due to rIFN-γ were observed.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF01958952
