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Bone Mineral Density in Cystic Fibrosis Patients – Results of a 3-years Folow-up and Intervention


Authors: E. Ondrušová-Očenášková 1;  Hubert Vaníček 1;  A. Jebavá 2
Authors‘ workplace: Dětská klinika LF a FN, Hradec Králové přednosta prof. MUDr. M. Bayer, CSc. 1;  Ústav klinické biochemie a diagnostiky FN, Hradec Králové přednosta prof. MUDr. V. Palička, CSc. 2
Published in: Čes-slov Pediat 2008; 63 (12): 660-667.
Category: Original Papers

Overview

Objectives:
Low bone mineral density (BMD) in cystic fibrosis (CF) patients is a problem of multifactorial origin. The aim of this analysis was to assess prospectively BMD in CF patients and the effect of vitamin D (vit. D) and calcium (Ca) supplementation adjustment.

Patients:
45 CF patients (aged 4.3–45 years, 27 female, 27 % >18 years).

Methods:
Lumbar spine BMD (L1–L4) was measured by DXA (dual-energy X-ray absorptiometry), correction of BMD Z-score was conducted according to height and weight. Other parameters: anthropometry, FEV1, serum levels of Ca, 25-(OH)-vit. D and 1,25-(OH)₂-vit. D, alkaline phosphatase (ALP), calciuria. Measurements were repeated yearly, conducted altogether 4x. Ergocalciferol and Ca supplementation were adjusted according to calciuria every 3 months.

Results:
Weight, height and BMI were lower than normal (mean Z-score (SD) = -1.2 (1.14), -1.01 (1.16) a -0.48 (0.90)). Weight and height Z-score did not change during follow up, BMI Z-score decreased. BMD was lower than in normal population in all measurements (mean Z-score (SD) = -1.7 (1.0), -1.2 (0.9), -1.3 (1.1), -1.4 (1.0), p <0.001), even after correction according to weight and height. Between the 1st and 2nd measurement BMD Z-score increased in patients aged <18 years (p = 6.6 x 10-7), then stayed stabilized. BMD Z-score in patients aged >18 years did not change significantly. Vit. D and Ca supplementation was increased between the 1st and 2nd measurement. 25-(OH)-vit. D a 1,25-(OH)₂-vit. D levels were normal and did not change significantly. However, 70% values of 25-(OH)-vit. D levels were <75 nmol/l (the lower limit of levels recommended for CF patients by the international consensus). Calciuria was in normal range and decreased by the time.

Conclusions:
Low BMD may be present already in prepubertal CF patients. Vitamin D and Ca supplementation adjustment according to calciuria may help to improve lumbar spine BMD in CF patients.

Key words:
bone mineral density, calcium, cystic fibrosis, DXA, osteoporosis,

vitamin D


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