Ambroszkiewicz Jadwiga, Gajewska Joanna, Sands Dorota, Chełchowska Magdalena, Ołtarzewski Mariusz, Laskowska-Klita Teresa
Zakład Badań Przesiewowych, Instytut Matki i Dziecka, ul. Kasprzaka 17a, 01-211 Warszawa, Poland.
Med Wieku Rozwoj. 2012 Apr-Jun;16(2):117-23.
The aim of this study was to assess bone formation and resorption processes and bone metabolism regulators, such as osteoprotegerin and fetuin-A in children with cystic fibrosis.
We examined 45 children with cystic fibrosis aged 5-13 years treated at the Institute of Mother and Child in Warsaw. The control group consisted of 35 healthy children in the same synage range without any diseases which may influence bone metabolism. We determined serum calcium and phosphate levels by colorimetric methods, vitamin D3 by the chemiluminiscence method and bone metabolism markers (osteocalcin, 5b isoenzyme of tartrate-resistant acid phosphatase, osteoprotegerin, fetuin-A) by immunoenzymatic methods.
Mean serum concentrations of calcium and phosphate in the studied children were within the reference ranges. However, the level of 25-hydroxyvitamin D3 was significantly lower in patients with cystic fibrosis compared to the controls (19.3±7.6 vs 25.2±8.9 ng/ml, p<0.01). In cystic fibrosis children we observed a statistically significant lower concentration of osteocalcin (81.9±28.9 vs 97.9±28.6 ng/ ml, p<0.01) and similar activity of 5b isoenzyme of tartrate-resistant acid phosphatase (12.5±2.9 vs 13.4±3.5 U/L) as compared to healthy peers. Mean serum concentration of osteoprotegerin in patients with CF was significantly lower than in the control children (4.1±0.98 vs 4.59±0.86 pmol/l, p<0.05). Serum concentration of fetuin-A was comparable in both groups of children.
In children with cystic fibrosis changes in the profile of bone metabolism markers were observed. Even patients with CF who are clinically stable and supplemented with vitamins are at risk of osteopenia and osteoporosis in their later life. Therefore, they should be under a comprehensive medical and nutritional care in order to achieve their optimal peak bone mass.
本研究旨在评估囊性纤维化患儿的骨形成与吸收过程以及骨代谢调节因子,如骨保护素和胎球蛋白-A。
我们对在华沙母婴研究所接受治疗的45名5至13岁的囊性纤维化患儿进行了检查。对照组由35名年龄相仿、无任何可能影响骨代谢疾病的健康儿童组成。我们采用比色法测定血清钙和磷水平,采用化学发光法测定维生素D3,采用免疫酶法测定骨代谢标志物(骨钙素、抗酒石酸酸性磷酸酶5b同工酶、骨保护素、胎球蛋白-A)。
所研究患儿的血清钙和磷平均浓度在参考范围内。然而,与对照组相比,囊性纤维化患者的25-羟维生素D3水平显著降低(19.3±7.6对25.2±8.9 ng/ml,p<0.01)。与健康同龄人相比,我们观察到囊性纤维化患儿的骨钙素浓度在统计学上显著降低(81.9±28.9对97.9±28.6 ng/ml,p<0.01),而抗酒石酸酸性磷酸酶5b同工酶的活性相似(12.5±2.9对13.4±3.5 U/L)。囊性纤维化患者的骨保护素平均血清浓度显著低于对照儿童(4.1±0.98对4.59±0.86 pmol/l,p<0.05)。两组儿童的胎球蛋白-A血清浓度相当。
在囊性纤维化患儿中观察到骨代谢标志物谱的变化。即使是临床稳定且补充了维生素的囊性纤维化患者,在晚年也有患骨质减少和骨质疏松症的风险。因此,他们应接受全面的医疗和营养护理,以达到最佳的峰值骨量。