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Table 1 Clinical infections and use of antibiotics in 11 Netherton syndrome patients

From: Immune cell phenotype and functional defects in Netherton syndrome

Family and patient Age (years) Antibiotic use in infancy i) Antibiotic prophylaxis ii) Bacterial skin infections iii) Conjunctivitisiv) External otitis v) Life-time infection score vi)
I.1 11 1 2 1 1 1 6
II.1 10 1 2 3 2 0 8
III.1 13 1 0 2 0 1 4
III.2 10 0 0 0 0 0 0
III.3 7 0 0 0 0 0 0
IV.1 3 1 1 0 0 1 3
V.1 7 1 1 1 0 0 3
V.2 4 1 2 1 0 1 5
VI.1 6 1 1 1 2 1 6
VI.2 3 1 2 1 0 1 5
VIII.1 17 1 1 1 0 1 4
  1. i) 0 = no; 1 = yes
  2. ii) 0 = no, 1 = yes, for 1–1.5 year period; 2 = yes, for > 1.5 years
  3. iii) 0 = not more frequently than usual in the age group; 1 = recurrent infections at 1–1.5 years of age, 2 = recurrent infections at > 1.5 years of age, 3 = yes, for > 1.5 years, additionally recurrent Staphylococcus aureus (PLV) abscesses
  4. iv) 0 = no; 1 = recurrent infections at 1–1.5 years of age; 2 = frequent conjunctivitis or constant need for antibiotic eye drops
  5. v) 0 = not; 1 = frequent external otitis or constant need for topical antibiotics; case VIII.1 had recurrent otitis media
  6. vi) Sum of the following scores: antibiotics in infancy, antibiotic prophylaxis, bacterial skin infections, conjunctivitis and external otitis. V.1 also suffered from sepsis after the neonatal period