Comparison of biochemical data, ectopic ossifications, and bone deformity between 4 groups
| . | Female . | Male . | . | ||
|---|---|---|---|---|---|
| . | NMD-sensitive (group 1) . | NMD-insensitive (group 2) . | NMD-insensitive (group 3) . | NMD-sensitive (group 4) . | Pa . |
| N | 3 | 11 | 5 | 5 | |
| Height, SD | −3.1 (−3.2 to −2.6) | −2.0 (−4.7 to 0.7) | −2.5 (−4.2 to −0.3) | −2.5 (−4.2 to −0.3) | .18 |
| Serum FGF23, pg/mLb | 62.4 (48.9-113.8) | 87.1 (71.9-1317.8) | 100.5 (54.0-158.0) | 678.2 (63.5-945.6) | .13 |
| Plasma PPi, nM | 2433 (2366-2780) | 2492 (1476-4000) | 1602 (1185-3596) | 1757 (1473-2941) | .51 |
| OALL, n (%) | 2 (67) | 6 (55) | 3 (60) | 3 (60) | ≥.999 |
| OPLL, n (%) | 1 (33) | 4 (36) | 1 (20) | 1 (20) | ≥.999 |
| OYL, n (%) | 3 (100) | 9 (82) | 3 (60) | 3 (60) | .46 |
| Osteophytes around hip joints, n (%) | 3 (100) | 11 (100) | 5 (100) | 5 (100) | NA |
| Osteophytes around knee joints, n (%) | 3 (100) | 6 (55) | 4 (80) | 4 (80) | ≥.999 |
| OA index | 6 (0-15) | 1 (0-9) | 2 (0-9) | 1 (0-22) | .65 |
| OP index | 0 (0-14) | 0 (0-15) | 0 (0-2) | 0 (0-9) | .56 |
| OY index | 6 (2-9) | 8 (0-13) | 7 (0-9) | 5 (0-10) | .76 |
| OS index | 12 (2-38) | 14 (0-25) | 7 (0-16) | 6 (0-41) | .65 |
| Hip KL grade | 3 (2-3) | 3 (2-4) | 3 (2-3) | 3 (3-4) | .17 |
| Knee KL grade | 3 (2-3) | 3 (2-4) | 3 (2-3) | 3 (3-4) | .64 |
| Varus deformity, n (%) | 2 (68) | 4 (36) | 3 (60) | 3 (60) | ≥.999 |
| Valgus deformity, n (%) | 0 (0) | 3 (27) | 1 (20) | 1 (20) | ≥.999 |
| . | Female . | Male . | . | ||
|---|---|---|---|---|---|
| . | NMD-sensitive (group 1) . | NMD-insensitive (group 2) . | NMD-insensitive (group 3) . | NMD-sensitive (group 4) . | Pa . |
| N | 3 | 11 | 5 | 5 | |
| Height, SD | −3.1 (−3.2 to −2.6) | −2.0 (−4.7 to 0.7) | −2.5 (−4.2 to −0.3) | −2.5 (−4.2 to −0.3) | .18 |
| Serum FGF23, pg/mLb | 62.4 (48.9-113.8) | 87.1 (71.9-1317.8) | 100.5 (54.0-158.0) | 678.2 (63.5-945.6) | .13 |
| Plasma PPi, nM | 2433 (2366-2780) | 2492 (1476-4000) | 1602 (1185-3596) | 1757 (1473-2941) | .51 |
| OALL, n (%) | 2 (67) | 6 (55) | 3 (60) | 3 (60) | ≥.999 |
| OPLL, n (%) | 1 (33) | 4 (36) | 1 (20) | 1 (20) | ≥.999 |
| OYL, n (%) | 3 (100) | 9 (82) | 3 (60) | 3 (60) | .46 |
| Osteophytes around hip joints, n (%) | 3 (100) | 11 (100) | 5 (100) | 5 (100) | NA |
| Osteophytes around knee joints, n (%) | 3 (100) | 6 (55) | 4 (80) | 4 (80) | ≥.999 |
| OA index | 6 (0-15) | 1 (0-9) | 2 (0-9) | 1 (0-22) | .65 |
| OP index | 0 (0-14) | 0 (0-15) | 0 (0-2) | 0 (0-9) | .56 |
| OY index | 6 (2-9) | 8 (0-13) | 7 (0-9) | 5 (0-10) | .76 |
| OS index | 12 (2-38) | 14 (0-25) | 7 (0-16) | 6 (0-41) | .65 |
| Hip KL grade | 3 (2-3) | 3 (2-4) | 3 (2-3) | 3 (3-4) | .17 |
| Knee KL grade | 3 (2-3) | 3 (2-4) | 3 (2-3) | 3 (3-4) | .64 |
| Varus deformity, n (%) | 2 (68) | 4 (36) | 3 (60) | 3 (60) | ≥.999 |
| Valgus deformity, n (%) | 0 (0) | 3 (27) | 1 (20) | 1 (20) | ≥.999 |
Values are reported as median (range).
Abbreviations: FGF, fibroblast growth factor; KL; Kellgren-Lawrence; NA, not available; NMD, nonsense-mediated messenger RNA decay; OA index, ossification of the anterior longitudinal ligament index; OALL, ossification of the anterior longitudinal ligament; OP, ossification of the posterior longitudinal ligament index; OPLL, ossification of the posterior longitudinal ligament; OS index, ossification index; OY index, ossification of the yellow ligament index; OYL, ossification of the yellow ligament.
aP value was evaluated between group 1 and group 4.
bSerum FGF23 levels were compared after excluding patients with estimated glomerular filtration rate lower than 60 mL/min/1.73 m2.
Comparison of biochemical data, ectopic ossifications, and bone deformity between 4 groups
| . | Female . | Male . | . | ||
|---|---|---|---|---|---|
| . | NMD-sensitive (group 1) . | NMD-insensitive (group 2) . | NMD-insensitive (group 3) . | NMD-sensitive (group 4) . | Pa . |
| N | 3 | 11 | 5 | 5 | |
| Height, SD | −3.1 (−3.2 to −2.6) | −2.0 (−4.7 to 0.7) | −2.5 (−4.2 to −0.3) | −2.5 (−4.2 to −0.3) | .18 |
| Serum FGF23, pg/mLb | 62.4 (48.9-113.8) | 87.1 (71.9-1317.8) | 100.5 (54.0-158.0) | 678.2 (63.5-945.6) | .13 |
| Plasma PPi, nM | 2433 (2366-2780) | 2492 (1476-4000) | 1602 (1185-3596) | 1757 (1473-2941) | .51 |
| OALL, n (%) | 2 (67) | 6 (55) | 3 (60) | 3 (60) | ≥.999 |
| OPLL, n (%) | 1 (33) | 4 (36) | 1 (20) | 1 (20) | ≥.999 |
| OYL, n (%) | 3 (100) | 9 (82) | 3 (60) | 3 (60) | .46 |
| Osteophytes around hip joints, n (%) | 3 (100) | 11 (100) | 5 (100) | 5 (100) | NA |
| Osteophytes around knee joints, n (%) | 3 (100) | 6 (55) | 4 (80) | 4 (80) | ≥.999 |
| OA index | 6 (0-15) | 1 (0-9) | 2 (0-9) | 1 (0-22) | .65 |
| OP index | 0 (0-14) | 0 (0-15) | 0 (0-2) | 0 (0-9) | .56 |
| OY index | 6 (2-9) | 8 (0-13) | 7 (0-9) | 5 (0-10) | .76 |
| OS index | 12 (2-38) | 14 (0-25) | 7 (0-16) | 6 (0-41) | .65 |
| Hip KL grade | 3 (2-3) | 3 (2-4) | 3 (2-3) | 3 (3-4) | .17 |
| Knee KL grade | 3 (2-3) | 3 (2-4) | 3 (2-3) | 3 (3-4) | .64 |
| Varus deformity, n (%) | 2 (68) | 4 (36) | 3 (60) | 3 (60) | ≥.999 |
| Valgus deformity, n (%) | 0 (0) | 3 (27) | 1 (20) | 1 (20) | ≥.999 |
| . | Female . | Male . | . | ||
|---|---|---|---|---|---|
| . | NMD-sensitive (group 1) . | NMD-insensitive (group 2) . | NMD-insensitive (group 3) . | NMD-sensitive (group 4) . | Pa . |
| N | 3 | 11 | 5 | 5 | |
| Height, SD | −3.1 (−3.2 to −2.6) | −2.0 (−4.7 to 0.7) | −2.5 (−4.2 to −0.3) | −2.5 (−4.2 to −0.3) | .18 |
| Serum FGF23, pg/mLb | 62.4 (48.9-113.8) | 87.1 (71.9-1317.8) | 100.5 (54.0-158.0) | 678.2 (63.5-945.6) | .13 |
| Plasma PPi, nM | 2433 (2366-2780) | 2492 (1476-4000) | 1602 (1185-3596) | 1757 (1473-2941) | .51 |
| OALL, n (%) | 2 (67) | 6 (55) | 3 (60) | 3 (60) | ≥.999 |
| OPLL, n (%) | 1 (33) | 4 (36) | 1 (20) | 1 (20) | ≥.999 |
| OYL, n (%) | 3 (100) | 9 (82) | 3 (60) | 3 (60) | .46 |
| Osteophytes around hip joints, n (%) | 3 (100) | 11 (100) | 5 (100) | 5 (100) | NA |
| Osteophytes around knee joints, n (%) | 3 (100) | 6 (55) | 4 (80) | 4 (80) | ≥.999 |
| OA index | 6 (0-15) | 1 (0-9) | 2 (0-9) | 1 (0-22) | .65 |
| OP index | 0 (0-14) | 0 (0-15) | 0 (0-2) | 0 (0-9) | .56 |
| OY index | 6 (2-9) | 8 (0-13) | 7 (0-9) | 5 (0-10) | .76 |
| OS index | 12 (2-38) | 14 (0-25) | 7 (0-16) | 6 (0-41) | .65 |
| Hip KL grade | 3 (2-3) | 3 (2-4) | 3 (2-3) | 3 (3-4) | .17 |
| Knee KL grade | 3 (2-3) | 3 (2-4) | 3 (2-3) | 3 (3-4) | .64 |
| Varus deformity, n (%) | 2 (68) | 4 (36) | 3 (60) | 3 (60) | ≥.999 |
| Valgus deformity, n (%) | 0 (0) | 3 (27) | 1 (20) | 1 (20) | ≥.999 |
Values are reported as median (range).
Abbreviations: FGF, fibroblast growth factor; KL; Kellgren-Lawrence; NA, not available; NMD, nonsense-mediated messenger RNA decay; OA index, ossification of the anterior longitudinal ligament index; OALL, ossification of the anterior longitudinal ligament; OP, ossification of the posterior longitudinal ligament index; OPLL, ossification of the posterior longitudinal ligament; OS index, ossification index; OY index, ossification of the yellow ligament index; OYL, ossification of the yellow ligament.
aP value was evaluated between group 1 and group 4.
bSerum FGF23 levels were compared after excluding patients with estimated glomerular filtration rate lower than 60 mL/min/1.73 m2.
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