Cobanoglu Nazan, Atasoy Halil, Ozcelik Ugur, Yalcin Ebru, Dogru Deniz, Kiper Nural, Gocmen Ayhan
Hacettepe University, Paediatric Pulmonary Diseases Unit, Ankara, Turkey.
Pediatr Pulmonol. 2009 Jul;44(7):706-12. doi: 10.1002/ppul.21050.
To study bone mineral density (BMD) of pre-pubertal cystic fibrosis (CF) children, and its relation with clinical and laboratory parameters, we enrolled 16 CF (8 girls) (4-8 years), and 16 control children (8 girls) (4-8 years). After anthropometric measurements, BMD, serum calcium, phosphorus, total alkaline phosphatase (ALP), 25-hydroxy vitamin D (25-OHD), parathyroid hormone, osteocalcin, tumor necrosis factor (TNF)-alpha, soluble TNF-alpha receptor 2 (sTNFR2), and soluble IL-2 receptor (sIL-2R) levels, and urinary calcium and hydroxyproline excretions were assessed. Disease severity of CF patients was determined with Shwachman-Kulczycki clinical and Brasfield radiological scoring systems.The mean Shwachman-Kulczycki and Brasfield scores of CF patients were indicating well-controlled disease. The anthropometric measurements, mean BMD values, and serum calcium, phosphorus and parathyroid hormone levels were within normal range and similar in both groups. Serum osteocalcin levels were lower, and ALP and 25-OHD levels were higher in CF. Although 24-hr urinary calcium excretions was higher in CF patients, hydroxyproline excretions were similar in both groups. There was no difference between two groups for the serum levels of sIL-2R, TNF-alpha and sTNFR2. Children with low vertebral z-scores had higher serum sIL-2R levels in both groups, but the same relation could not be shown for TNF-alpha and sTNFR2.We may speculate that younger, healthier and well-nourished patients with CF may have normal BMD, but the bone disease develop as patients get older because of the other contributing factors. Future well-designed longitudinal studies with large cohorts might show a relation with BMD and cytokines in CF.
为研究青春期前囊性纤维化（CF）儿童的骨密度（BMD）及其与临床和实验室参数的关系，我们纳入了16名CF儿童（8名女孩）（4 - 8岁）和16名对照儿童（8名女孩）（4 - 8岁）。在进行人体测量后，评估了骨密度、血清钙、磷、总碱性磷酸酶（ALP）、25 - 羟基维生素D（25 - OHD）、甲状旁腺激素、骨钙素、肿瘤坏死因子（TNF）-α、可溶性TNF - α受体2（sTNFR2）和可溶性白细胞介素 - 2受体（sIL - 2R）水平，以及尿钙和羟脯氨酸排泄量。采用Shwachman - Kulczycki临床评分系统和Brasfield放射学评分系统确定CF患者的疾病严重程度。CF患者的平均Shwachman - Kulczycki评分和Brasfield评分表明疾病得到良好控制。两组的人体测量、平均骨密度值以及血清钙、磷和甲状旁腺激素水平均在正常范围内且相似。CF患者的血清骨钙素水平较低，而ALP和25 - OHD水平较高。虽然CF患者的24小时尿钙排泄量较高，但两组的羟脯氨酸排泄量相似。两组的血清sIL - 2R、TNF - α和sTNFR2水平无差异。两组中椎体z评分较低的儿童血清sIL - 2R水平较高，但TNF - α和sTNFR2未显示出相同的关系。我们可以推测，年龄较小、健康状况较好且营养良好的CF患者可能骨密度正常，但随着患者年龄增长，由于其他因素的影响会发生骨病。未来设计良好的大型队列纵向研究可能会揭示CF中骨密度与细胞因子之间的关系。
