Ujhelyi Rita, Treszl András, Vásárhelyi Barna, Holics Klára, Tóth Miklós, Arató András, Tulassay Tivadar, Tulassay Zsolt, Szathmári Miklós
Heim Pál Children's Hospital, Budapest, Hungary.
J Pediatr Gastroenterol Nutr. 2004 Apr;38(4):401-6. doi: 10.1097/00005176-200404000-00007.
To investigate bone mineral density and bone homeostasis in cystic fibrosis (CF) and to assess changes in a 2-year period.
Thirty-eight patients with clinically stable CF (11 children, 16 adolescents, 11 young adults) were enrolled. No patient was treated with corticosteroids before or during the study. Weight and height Z scores and bone mineral density (BMD) Z-score at the femoral neck and the lumbar spine were recorded at the beginning of the study and after 2 years. Osteocalcin and cross-link excretion, both measurements of bone turnover were also measured. Correlations between BMD, bone turnover parameters, disease severity, pubertal stage, and nutritional state were calculated. The maternal BMD was also determined and related to that of the child.
Height and weight Z scores were normal in children and below normal in adolescents. Puberty was delayed in most patients. Bone age was lower than chronological age in adolescents. Lumbar spine and femoral neck BMD Z scores were below normal in each age group. Disease severity determined by Schwachman score correlated with lumbar BMD (r = 0.45, P < 0.02). BMD Z scores did not change during 2 year follow-up. Maternal and patient lumbar and femoral BMD correlated significantly (r = 0.51, P < 0.01, and r = 0.54, P < 0.01, respectively).
Bone deficit is present in patients with CF who have never received steroid treatment. Delay of puberty, chronic inflammation, or genetic susceptibility might be responsible for this phenomenon which was found in patients who had never received steroids and who were in relatively good clinical state.
研究囊性纤维化（CF）患者的骨矿物质密度和骨稳态，并评估2年内的变化情况。
招募了38例临床病情稳定的CF患者（11名儿童、16名青少年、11名青年）。研究前及研究期间所有患者均未接受皮质类固醇治疗。在研究开始时及2年后记录体重和身高Z评分以及股骨颈和腰椎的骨矿物质密度（BMD）Z评分。还测量了骨钙素和交联物排泄，这两者都是骨转换的指标。计算BMD、骨转换参数、疾病严重程度、青春期阶段和营养状态之间的相关性。同时测定母亲的BMD并与孩子的BMD进行关联分析。
儿童的身高和体重Z评分正常，青少年的身高和体重Z评分低于正常水平。大多数患者青春期延迟。青少年的骨龄低于实际年龄。各年龄组的腰椎和股骨颈BMD Z评分均低于正常水平。由Schwachman评分确定的疾病严重程度与腰椎BMD相关（r = 0.45，P < 0.02）。在2年的随访期间，BMD Z评分没有变化。母亲与患者的腰椎和股骨BMD显著相关（分别为r = 0.51，P < 0.01和r = 0.54，P < 0.01）。
从未接受过类固醇治疗的CF患者存在骨量不足的情况。青春期延迟、慢性炎症或遗传易感性可能是导致这一现象的原因，该现象在从未接受过类固醇治疗且临床状态相对良好的患者中被发现。
