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首页> 外文期刊>Ultrasound in Medicine and Biology >Phalangeal quantitative ultrasound in children with phenylketonuria: a pilot study.
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Phalangeal quantitative ultrasound in children with phenylketonuria: a pilot study.

机译：苯丙酮尿症患儿的对角膜定量超声：一项初步研究。

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摘要

Bone alterations in phenylketonuria (PKU) have been detected, especially with increasing age, in several studies by using different radiologic techniques. Quantitative ultrasound (QUS) assesses skeletal status by measuring the amplitude-dependent speed of sound (AD-SoS) and the bone transmission time (BTT), mainly dependent on mineral density and cortical thickness. Bone condition in 30 children and adolescents (mean age 15.1 +/- 6.4 y) affected by PKU was evaluated by phalangeal QUS, considering its relationship with their clinical, biochemical and therapeutic features. Measured AD-SoS Z-Score and BTT Z-Score were 0.27 +/- 1.42 and -0.26 +/- 1.21, respectively. In patients with previous fractures, the two QUS parameters were lower than in patients without history of fracture (p < 0.001 and p = 0.006, respectively). AD-SoS Z-Score and BTT Z-Score were negatively correlated with plasma phenylalanine (Phe) concentration in the year before QUS (p = 0.005 and p < 0.001, respectively) and with age (p < 0.001 for both parameters). These results parallel the previous findings obtained by different radiologic tools and suggest phalangeal QUS as an attractive option for the regular evaluation and longitudinal monitoring of bone condition in children and adolescents affected by PKU.
机译：在几项研究中，使用不同的放射学技术已发现苯丙酮酸尿症（PKU）的骨改变，尤其是随着年龄的增长。定量超声（QUS）通过测量幅度相关的声音速度（AD-SoS）和骨骼传输时间（BTT）来评估骨骼状态，骨骼的传播时间主要取决于矿物质密度和皮质厚度。通过指骨QUS评估30例受PKU影响的儿童和青少年（平均年龄15.1 +/- 6.4岁）的骨质状况，考虑到其与临床，生化和治疗特征之间的关系。测得的AD-SoS Z分数和BTT Z分数分别为0.27 +/- 1.42和-0.26 +/- 1.21。在先前有骨折的患者中，两个QUS参数均低于没有骨折史的患者（分别为p <0.001和p = 0.006）。 AD-SoS Z评分和BTT Z评分与QUS前一年的血浆苯丙氨酸（Phe）浓度呈负相关（分别为p = 0.005和p <0.001），并且与年龄（两个参数均p <0.001）呈负相关。这些结果与以前通过不同的放射学工具获得的发现相吻合，并建议指骨QUS作为定期评估和纵向监测受PKU影响的儿童和青少年骨骼状况的有吸引力的选择。

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