Immune cell phenotype and functional defects in Netherton syndrome

Table 2 Specific lymphocyte populations associating with infection frequency in 11 Netherton syndrome patients as analyzed with Spearman’s rank-order correlation

Immunological parameter (%) ⁱ⁾	Change in NS patients (n = 11) ⁱⁱ⁾	Antibiotic prophylaxis (r_s) ⁱⁱⁱ⁾	Antibiotic prophylaxis (p value)	Skin infection score ^iv) (r_s)	Skin infections (p)	Total infection score ^iv) (r_s)	Total infection score (p)
Positive correlation
CD62L expression in CD4 T cells	increased	0.764	0.010	0.822	0.007	0.712	0.031
CD62L expression in CD8 T cells	increased	0.745	0.013	0.798	0.006	0.678	0.045
CD27 expression in CD8 T cells	decreased		> 0.05	0.71	0.014		> 0.05
Naïve CD4 T cells	decreased	0.742	0.009	0.798	0.006		> 0.05
Activated B cells	decreased		> 0.05	0.692	0.006	0.613	0.020
Negative correlation
Memory B cells	decreased	−0.671	0.009		> 0.05		> 0.05
Non-switched B cells	decreased	−0.723	0.004	−0.698	0.006	−0.623	0.017
CD27 expression in NK cells	decreased	−0.788	0.004	−0.775	0.005		> 0.05
Central memory CD8 T cells	increased	−0.834	0.001	−0.71	0.014		> 0.05
CD57 expression in CD4 T cells	increased	−0.822	0.004	−0.798	0.006	−0.678	0.045
CD57 expression in CD8 T cells	increased	−0.564	0.070	−0.775	0.005		> 0.05

i) see precise definitions in Additional file 1
ii) compared with age-matched healthy persons
iii) Spearman’s rank correlation coefficient,
iv) Sum of the following scores as specified in Table 1: antibiotics in infancy (0–1), antibiotic prophylaxis (0–2), bacterial skin infections (0–3), conjunctivitis (0–2) and external otitis (0–1)

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