Bone Mineral Density in Cystic Fibrosis Patients with the CFTR I1234V Mutation in a Large Kindred Family Is Associated with Pancreatic Sufficiency

Table 1

Baseline demographic, anthropometric, nutritional, and other clinical characteristics.

Characteristics	Mean ± SD [median (min–max)]; 𝑁 (%)
Age (years)	17.3±4.9 [16.9 (10–33)]
Height (cm)	159.2±11.85 [160 (133–178)]
Weight (kg)	56.8±19.42 [53 (26–95)]
Body mass index (BMI)	22.1±6.2 [19.7 (14.9–36.7)]
Phosphorus (mmol/L)	1.3±0.20 [1.4 (1–1.7)]
Calcium level (mmol/L)	2.3±0.08 [2.3 (2.2–2.5)]
ALP (U/L)	113.2±50.9 [92.5 (58–225)]
25[OH]D levels (ng/mL)	21.6±9.6 [22 (6–42)]
Vitamin K levels (ng/L)	222.5±136.8 [181 (107–585)]
FEV1	82.9±14.7 [86 (39–103)]
L1–L4 BMD 𝑍 score	−0.69±0.96 [−0.4 (−3.3–0.5)]
Total hip 𝑍 score	−0.48±0.92 [−0.2 (−2.3–0.9)]
Total body 𝑍 score	−0.38±0.86 [−0.2 (−2.5–1)]
L1–L4 BMD 𝑇 score	0.14±1.13 [0.7 (−1.8–0.9)]
Total hip 𝑇 score	0.38±1.0 [0.6 (−1.3–1.4)]
Total body 𝑇 score	0.52±1.03 [0.6 (−1.1–1.7)]
Gender
Male	16 (61.5%)
Female	10 (38.5%)
Chronic P. aeruginosa
Yes	16 (61.5%)
No	10 (38.5%)
Multivitamin
Yes	6 (23.1%)
No	20 (76.9%)
Time of exposure to the sun
>30 min per day	7 (26.9%)
≤30 min per day	19 (73.1%)
25[OH]D levels
≥30 (ng/mL)	6 (23.1%)
<30 (ng/mL)	20 (76.9%)

𝑇 score was calculated for a patients’ age more than 21 years.