关键词: Amyotrophia FGF23 Hypophosphatemia Limp MRI Ricket

Mesh : Humans Child Male Magnetic Resonance Imaging / methods Prospective Studies Muscle, Skeletal / diagnostic imaging Female Familial Hypophosphatemic Rickets / diagnostic imaging Adolescent Child, Preschool Imaging, Three-Dimensional Case-Control Studies

来  源:   DOI:10.1016/j.otsr.2023.103713

Abstract:
BACKGROUND: Children with X Linked Hypophosphatemia (XLH) suffer from carential ricket, bone deformities and lameness. No previous study demonstrated a morphological distinction in muscles in these patients. The aim of this prospective study was to characterize, using Magnetic Resonance Imaging (MRI), the muscle morphology of pelvis, thigh and leg in children with XLH and to compare it with typically developed (TD) children.
OBJECTIVE: We hypothesized that lower limbs muscles in children with XLH are different from TD children and could explain limp walking.
METHODS: Three-dimensional reconstructions of the muscles were performed in 11 patients with XLH and 15 TD children. Muscle lengths, sections and volumes were calculated and normalized with height and weight. Mean age was 10.
RESULTS: Lengths were all smaller in children with XLH except for the Medius/minimus gluteus muscles (p=0.64). The difference seemed higher in muscles with a long tendinous part as semitendinosus (0.139 vs 0,164; p<0.01). All volumes were significantly inferior in children with XLH. This preliminary study showed significant differences in muscle structures between patients with XLH and TD children.
CONCLUSIONS: Medius/minimus gluteus seemed to be particularly developed in children with XLH. Nevertheless it is not possible to conclude if it is related to XLH or a consequence of bone deformities.
METHODS: IV.
摘要:
背景:患有X连锁低磷血症(XLH)的儿童患有creentic病,骨畸形和跛行。以前的研究没有证明这些患者肌肉的形态学差异。这项前瞻性研究的目的是描述,使用磁共振成像(MRI),骨盆的肌肉形态,XLH儿童的大腿和腿,并将其与典型发育(TD)儿童进行比较。
目的:我们假设XLH患儿的下肢肌肉与TD患儿不同,可以解释跛行。
方法:对11例XLH患者和15例TD儿童进行了肌肉三维重建。肌肉长度,计算切片和体积,并用身高和体重进行归一化.平均年龄是10岁。
结果:除臀中肌/臀小肌外,XLH患儿的长度均较小(p=0.64)。在长肌腱部分为半腱肌的肌肉中,差异似乎更高(0.139vs0,164;p<0.01)。XLH患儿的所有体积均明显较差。这项初步研究表明,XLH和TD儿童的肌肉结构存在显着差异。
结论:在患有XLH的儿童中,臀中肌/臀小肌似乎特别发达。然而,如果它与XLH或骨骼畸形的结果有关,则无法得出结论。
方法:IV.
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