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. 2014 Feb;55(2):338–343. doi: 10.1194/jlr.D044024

TABLE 3.

Plasma 7-KC mean values, range, and P value in healthy subjects, ASMase-deficient NPD, ASMase-deficient NPD heterozygotes, and other LSD patients

NC NPA/B NPA/B-h NPC GD MPSII GSDII MLD/KD
n 314 38 8 7 34 38 19 21
Mean and range (ng/ml) 5.2 (ND–23) 245 (101–976) 3.7 (ND–6.1) 279 (32–603) 6.7 (1.5–19) 6.4 (ND–20) 6.3 (ND–20) 3.7 (1–7.9)
P <0.001a NA <0.001a 0.849a <0.001a <0.001a <0.001a <0.001a

NC, healthy subjects; NPA/B, ASMase-deficient NPD; NPA/B-h, ASMase-deficient NPD heterozygote; NA, not applicable; ND, not detectable.

a

Comparison versus NPA/B.