TY - JOUR
T1 - Recombinant human interferon-gamma treatment in severe leucocyte adhesion deficiency
AU - Weening, R. S.
AU - Bredius, R. G.
AU - Vomberg, P. P.
AU - van der Schoot, C. E.
AU - Hoogerwerf, M.
AU - Roos, D.
PY - 1992
Y1 - 1992
N2 - 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 beta 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-gamma (rIFN-gamma) showed an increase in oxygen consumption, but no effect on the expression of the LeuCAMs, or the beta chain mRNA. In vivo treatment with IFN-gamma was started. The Fc gamma RI 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-gamma treatment. No new infections or side-effects due to rIFN-gamma were observed
AB - 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 beta 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-gamma (rIFN-gamma) showed an increase in oxygen consumption, but no effect on the expression of the LeuCAMs, or the beta chain mRNA. In vivo treatment with IFN-gamma was started. The Fc gamma RI 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-gamma treatment. No new infections or side-effects due to rIFN-gamma were observed
U2 - https://doi.org/10.1007/BF01958952
DO - https://doi.org/10.1007/BF01958952
M3 - Article
C2 - 1537350
SN - 0340-6199
VL - 151
SP - 103
EP - 107
JO - European journal of pediatrics
JF - European journal of pediatrics
IS - 2
ER -