Department of Pediatric Orthopaedic Surgery, Hôpital Universitaire Necker Enfants Malades, 149, rue de Sèvres, 75015 Paris, France.
Arts et Métiers ParisTech, CNRS, Laboratoire de Biomécanique (LBM), 151, boulevard de l'Hôpital, 75013 Paris, France.
Orthop Traumatol Surg Res. 2024 May;110(3):103713. doi: 10.1016/j.otsr.2023.103713. Epub 2023 Oct 18.
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.
We hypothesized that lower limbs muscles in children with XLH are different from TD children and could explain limp walking.
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.
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.
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.
IV.
患有 X 连锁低磷血症（XLH）的儿童患有佝偻病、骨骼畸形和跛行。以前没有研究表明这些患者的肌肉存在形态学差异。本前瞻性研究的目的是使用磁共振成像（MRI）来描述 XLH 患儿骨盆、大腿和小腿的肌肉形态，并将其与典型发育（TD）儿童进行比较。
我们假设 XLH 患儿的下肢肌肉与 TD 儿童不同，这可以解释其跛行。
对 11 名 XLH 患儿和 15 名 TD 儿童进行了三维肌肉重建。计算并分别用身高和体重对肌肉长度、横截面积和体积进行了归一化。平均年龄为 10 岁。
除臀中肌/小肌（p=0.64）外，XLH 患儿的所有肌肉长度均较小。在具有长肌腱部分的肌肉中，如半腱肌（0.139 与 0.164；p<0.01），差异似乎更高。XLH 患儿的所有肌肉体积均显著较小。这项初步研究表明，XLH 患儿与 TD 儿童的肌肉结构存在显著差异。
臀中肌/小肌在 XLH 患儿中似乎特别发达。然而，尚不能确定这是与 XLH 相关，还是骨骼畸形的结果。
IV。
