TABLE 5.

Features of LAD

Clinical and laboratory feature	LAD I	LAD I variant	LAD II
Diagnosis	Diminished CD11b/CD18 expression	Functional analyses	Bombay blood type, absent Lewis sialyl X
Typical white cell count (μl−1)	20,000-100,000	35,000-96,000	20,000-70,000
T-cell function	Diminished delayed-type hypersensitivity	Normal except diminished proliferation to CD2	Absent cutaneous lymphocyte antigen, and diminished delayed-type hypersensitivity
Binding defect	Fails to bind ICAMs and complement opsonized particles	Normal binding, failure to signal	Fails to bind endothelial selectins
Types of infections	Necrotic skin infections, cellulitis, periodontal disease, pneumonia, spontaneous peritonitis, frequent sepsis	Recurrent skin infections, periodontitis, otitis media, pneumonia	Pneumonia early in life, periodontitis in later childhood, severe recurrent sepsis early in life in one patient Pneumonia early in life periodontitis in later childhood, severe recurrent sepsis early in life in patient
Types of bacteria	S. aureus, Psuedomonas spp., Enterococcus spp., E. coli, Klebsiella spp. many mixed bacterial infections	S. aureus, Psuedomonas spp., Streptococcus spp., Enterococcus spp., E. coli, Bacteroides spp.	Not reported
Other infections	Candida spp., Aspergillus spp., two deaths from viral infections	P. carinii	Not reported
Other features	Abnormal NK cell function; colitis seen in 50% of severely affected individuals; 87% of severely affected individuals have delayed separation of the umbilical cord	Myelodysplasia in one case, hypogammaglobulinemia in one case, and diminished platelet activation in two cases	Developmental delay (in 5 of 5 patients), overlapping toes (in 2 of 5), response to oral fucose (in 1 of 5), no response to oral fucose (in 2 of 5)