Deficits in bone density and structure in children and young adults following Fontan palliation
Deficits in bone density and structure in children and young adults following Fontan palliation
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DOI：
10.1016/j.bone.2015.04.012
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发表时间：
2015-08-01
期刊：
影响因子：
4.1
通讯作者：
Leonard, Mary B.
中科院分区：
文献类型：
--
作者：
Avitabile, Catherine M.;Goldberg, David J.;Leonard, Mary B.
背景：先天性心脏病患者的存活率已经提高，现在患有这些疾病的成年人比儿童更多。需要Fontan姑息治疗的复杂单心室先天性心脏病与骨积累受损的多种危险因素相关。方法：应用胫骨外周定量计算机断层扫描(PQCT)测量43例Fontan患者(5~33岁)的骨小梁和皮质体积骨密度(VBMD)、皮质骨大小和小腿肌肉面积，中位数为10年。PQCT结果被转换为基于>；700名健康参考参与者的性别和种族相对于年龄的Z分数。结果：与参考受试者相比，丰坦组的身高Z-得分较低(平均标准差：-0.29+/-1.00vs.0.25+/-0.93，p<；0.001)；体重指数Z-得分相似(0.16+/-0.88vs.0.35+/-1.02，p=0.1)。Fontan参与者的小梁MAD Z评分较低(-0.85+/-0.96vs.0.01+/-1.02，p<；0.001)；皮质vBMDZ评分相似(-0.17+/-0.98vs.0.001.00，p=0.27)。与参考参与者相比，皮质面积(-0.59+/-0.84vs.0.00+/-0.88，p<；0.001)和骨膜周长(-0.50+/-0.82vs.0.00+/-0.84，p<；0.001)Z分数降低。在方坦受试者中，小腿肌肉面积Z分数较低(-0.45+/-0.98vs.0.00+/-0.96vs.0.00+/-0.96vs.0.00+/-0.96vs.0.00+/-0.96vs.0.00+/-0.96vs.0.00+/-0.96vs.0.00+/-0.96vs.0.00+/-0.96vs.0.00+/-0.96vs.0.00+/-0.96vs.0.00+/-0.96vs.0.00+/-0.96vs.0.00+/-0.96vs.0.00+/-0.96vs.0.00+/-0.96vs.0.00+/-0.96vs.0.00+/-0.96vs.0.00+/-0.96vs.0.00+/-0.96vs.0.00肌肉骨骼缺陷与年龄、Fontan特征、甲状旁腺激素或维生素D水平无关。结论：Fontan治疗后儿童和青壮年表现出较低的骨小梁vBMD、皮质结构和肌肉面积。肌肉缺陷与较小的骨膜尺寸有关。未来的研究应该确定这些缺陷对骨折的影响，并确定促进肌肉骨骼发育的干预措施(爱思唯尔公司发表的2015年)。
Background: Survival of patients with congenital heart disease has improved such that there are now more adults than children living with these conditions. Complex single ventricle congenital heart disease requiring Fontan palliation is associated with multiple risk factors for impaired bone accrual. Bone density and structure have not been characterized in these patients.Methods: Tibia peripheral quantitative computed tomography (pQCT) was used to assess trabecular and cortical volumetric bone mineral density (vBMD), cortical dimensions, and calf muscle area in 43 Fontan participants (5-33 years old), a median of 10 years following Fontan palliation. pQCT outcomes were converted to sex- and race-specific Z-scores relative to age based on >700 healthy reference participants. Cortical dimensions and muscle area were further adjusted for tibia length.Results: Height Z-scores were lower in Fontan compared to reference participants (mean SD: -0.29 +/- 1.00 vs. 0.25 +/- 0.93, p < 0.001); BMI Z-scores were similar (0.16 +/- 0.88 vs. 0.35 +/- 1.02, p = 0.1). Fontan participants had lower trabecular MAD Z-scores (-0.85 +/- 0.96 vs. 0.01 +/- 1.02, p < 0.001); cortical vBMD Z-scores were similar (-0.17 +/- 0.98 vs. 0.00 1.00, p = 0.27). Cortical dimensions were reduced with lower cortical area (-0.59 +/- 0.84 vs. 0.00 +/- 0.88, p < 0.001) and periosteal circumference (-0.50 +/- 0.82 vs. 0.00 +/- 0.84, p < 0.001) Z-scores, compared to reference participants. Calf muscle area Z-scores were lower in the Fontan participants (-0.45 +/- 0.98 vs. 0.00 +/- 0.96, p = 0.003) and lower calf muscle area Z-scores were associated with smaller periosteal circumference Z-scores (R = 0.62, p < 0.001). Musculoskeletal deficits were not associated with age, Fontan characteristics, parathyroid hormone or vitamin D levels.Conclusions: Children and young adults demonstrate low trabecular vBMD, cortical structure and muscle area following Fontan. Muscle deficits were associated with smaller periosteal dimensions. Future studies should determine the fracture implications of these deficits and identify interventions to promote musculoskeletal development (C) 2015 Published by Elsevier Inc.