未经批准:儿童骨累积控制峰值骨量和强度。囊性纤维化(CF)青年骨健康的纵向研究可能提供对CF相关骨病(CFBD)的见解。成人CF的普遍合并症。
UNASSIGNED:这项针对胰腺CF不足的青少年的为期一年的纵向研究,纳入一项营养干预研究[n=62(36M/26F)]1)检查了双能X线吸收测量法(DXA)定义的腰椎(LS)和全身无头(TBLH)骨累积和2),比较了它们与健康参考组的外周定量计算机断层扫描(pQCT)皮质骨密度和胫骨小梁骨几何结构的变化[n=143(68M/75F)].主要结局指标为1)DXA:腰椎面骨矿物质密度(LSaBMD)和全身少头骨矿物质含量(TBLH-BMC),性别和青春期特定的状态,身高速度(HV)-调整或HV和瘦体重速度(HV-LBMV)-调整的年化速度-Z评分和2)pQCT:年龄,性别,青春期状态和,在适当的时候,胫骨长度调整Z评分骨结构措施。将DXA速度-Z与0的预期平均值进行比较,并与临床参数(年龄,BMI-Z和FEV1%-预测)测试。在CF中进行了HV调整和LBMV-HV调整的DXA速度-Z的受试者内比较。使用纵向模型比较两组在一年内的pQCTZ评分。骨健康测量与临床参数之间的纵向关系(年龄,BMI-Z和FEV1%-预测)在CF患者中进行了检查。
UNASSIGNED:女性的DXA速度-Z高于正常水平(p<0.05),而男性的CF则不高于正常水平。对于LSaBMD或TBLH-BMC,HV调节和LBMV-HV调节的速度Z没有差异。在患有CF的男性中,HV调整和LBMV-HV调整的LSaBMD速度-Z评分均与年龄呈负相关(HVrho:-0.35;p=0.045,LBMV-HVrho:-0.47;p=0.0046).在患有CF的男性中,BMI-Z与HV调整的LSaBMD速度-Z呈正相关(rho:0.37;p=0.034),但这种关系对于LBMV-HV并不持续(rho:0.14;p=0.42).在患有CF的女性中,LSaBMD速度-Z评分与年龄和BMI-Z无相关性(均p>0.05)。在两种性别中均未发现LSaBMD速度-Z评分与FEV1%预测的相关性(均p>0.12)。TBLH-BMC速度Z评分与任一性别的临床参数均无相关性(均p>0.1)。在基线,CF的多个pQCT参数较低(p<0.05)。在CF或参考组中,pQCTZ评分在基线和1年之间没有差异。在比较CF和参考的pQCT-Z变化的纵向模型中,多次pQCT-Z结果在CF中仍然较低,但CF与参考参数的变化没有差异(所有p>0.26)。CF中较低的pQCT结果主要限于男性(CF组*女性性别相互作用β系数>0)。在这个组合纵向模型中,在CF和参考文献中,BMI-Z与pQCT-Z参数呈正相关(p<0.001)。在患有CF的男性中,多个pQCT-Z结果与BMI-Z和FEV1%呈正相关,在CF女性中预测FEV1%(p<0.05)。男性年龄与切片模量(p=0.001)呈负相关,女性年龄与皮质密度-Z呈负相关(p<0.001)。
未经评估:随着寿命的延长,CF中的骨骼健康越来越重要。平均而言,骨质积累在青年时期保留了CF,虽然发现了骨骼几何形状和强度的缺陷,在为期一年的研究中,这些缺陷并没有恶化。随着年龄的增长,LS骨累积率降低,这表明成年后是CF的脆弱时期,而LBMV调整后的累积率与BMI之间缺乏关系,突显了LBM在骨骼健康中的作用。这些发现可能有助于针对筛查实践和干预措施。
UNASSIGNED: Pediatric bone accrual governs peak bone mass and strength. Longitudinal studies of bone health in youth with cystic fibrosis (CF) may provide insight into CF-related bone disease (CFBD), a prevalent co-morbidity in adults with CF.
UNASSIGNED: This one-year longitudinal study of youth with pancreatic insufficient CF, enrolled in a nutrition intervention study [n = 62 (36 M/26F)] 1) examined dual-energy x-ray absorptiometry (DXA)-defined lumbar spine (LS) and total body less head (TBLH) bone accrual and 2) compared their changes in peripheral quantitative computed tomography (pQCT) cortical and trabecular tibial bone density and geometry to those of a healthy reference group [n = 143 (68 M/75F)].Main outcome measures were 1) DXA: lumbar spine areal bone mineral density (LSaBMD) and total body less head bone mineral content (TBLH-BMC), sex- and pubertal status-specific, height velocity (HV)-adjusted or HV and lean body mass velocity (HV-LBMV)-adjusted annualized velocity-Z scores and 2) pQCT: age, sex, pubertal status and, when appropriate, tibial length adjusted Z-scores for bone architecture measures.DXA velocity-Z were compared to expected mean of 0 and correlations with clinical parameters (age, BMI-Z and FEV1%-predicted) tested. Within-subject comparisons of HV-adjusted and LBMV-HV-adjusted DXA velocity-Z were conducted in CF.pQCT Z-scores were compared between the two groups over one year using longitudinal models. Longitudinal relationships between measures of bone health and clinical parameters (age, BMI-Z and FEV1%-predicted) were examined in individuals with CF.
UNASSIGNED: DXA velocity-Z were higher than normal in females (p < 0.05) but not males with CF. HV-adjusted and LBMV-HV-adjusted velocity-Z did not differ for LSaBMD or TBLH-BMC.In males with CF, both HV-adjusted and LBMV-HV-adjusted LSaBMD velocity-Z scores correlated negatively with age (HV rho: -0.35; p = 0.045 and LBMV-HV rho: -0.47; p = 0.0046). In males with CF BMI-Z correlated positively with HV-adjusted LSaBMD velocity-Z (rho: 0.37; p = 0.034), but this relationship did not persist for LBMV-HV (rho: 0.14; p = 0.42). In females with CF, no correlations between LSaBMD velocity-Z scores and age or BMI-Z were found (all p > 0.05). No correlations between LSaBMD velocity-Z scores and FEV1%-predicted were seen in either sex (all p > 0.12). TBLH-BMC velocity Z-scores were not correlated with clinical parameters in either sex (all p > 0.1).At baseline, multiple pQCT parameters were lower in CF (p < 0.05). pQCT Z-scores did not differ between baseline and one-year in either CF or reference group. In a longitudinal model comparing pQCT-Z changes in CF and reference, multiple pQCT-Z outcomes remained lower in CF, but the changes in parameters did not differ in CF vs reference (all p > 0.26). Lower pQCT outcomes in CF were largely restricted to males (CF group*female sex interaction beta coefficients > 0). In this combined longitudinal model, of both CF and reference, BMI-Z was positively associated with pQCT-Z parameters(p < 0.001).Multiple pQCT-Z outcomes positively correlated with both BMI-Z and FEV1%-predicted in males with CF, and with FEV1%-predicted in females with CF (p < 0.05). Age was negatively associated with section modulus (p = 0.001) in males and with cortical density-Z in females (p < 0.001).
UNASSIGNED: With improved longevity, bone health in CF is of increasing importance. On average, bone accrual was preserved in youth with CF, and while deficits in bone geometry and strength were found, these deficits did not worsen over the one-year study. Lower LS bone accrual with increasing age suggests emerging adulthood is a period of vulnerability in CF while the role of LBM in bone health is underscored by the lack of relationship between LBMV-adjusted accrual and BMI. These findings may be useful in targeting screening practices and interventions.