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Ⅰ型和Ⅱ型黏多糖贮积症髋关节的超声特征

Ultrasonographic Features of Hip Joints in Mucopolysaccharidoses Type I and II.

作者信息

Żuber Zbigniew, Jurecka Agnieszka, Różdżyńska-Świątkowska Agnieszka, Migas-Majoch Agata, Lembas Agnieszka, Kieć-Wilk Beata, Tylki-Szymańska Anna

机构信息

Department of Pediatrics, St. Louis Regional Children's Hospital, Cracow, Poland.

Department of Pediatrics, Nutrition and Metabolic Diseases, The Children's Memorial Health Institute, Warsaw, Poland; Department of Genetics, University of Gdańsk, Gdańsk, Poland.

出版信息

PLoS One. 2015 Apr 29;10(4):e0123792. doi: 10.1371/journal.pone.0123792. eCollection 2015.

DOI:10.1371/journal.pone.0123792
PMID:25922936
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4414504/
Abstract

OBJECTIVES

The primary aim of this study was to assess the ultrasonographic features of hip joints in patients with mucopolysaccharidosis (MPS) type I and II in comparison with healthy population. The secondary aims were to correlate these features with clinical measures and to evaluate the utility of ultrasound in the diagnosis of MPS disease.

MATERIALS AND METHODS

Sixteen MPS I (n = 3) and II (n = 13) patients were enrolled in the present study and underwent clinical and radiological evaluation, and bilateral high-resolution ultrasonography (US) of hip joints. The distance from the femoral neck to joint capsule (synovial joint space, SJS), joint effusion, synovial hyperthrophy, and local pathological vascularization were evaluated. The results were compared to the healthy population and correlated with clinical and radiological measures.

RESULTS

  1. There was a difference in US SJS between children with MPS disease and the normative value for healthy population (7mm). Mean values of SJS were 15.81 ± 4.08 cm (right hip joints) and 15.69 ± 4.19 cm (left joints). 2. No inflammatory joint abnormalities were detected in MPS patients. 3. There was a clear correlation between US SJS and patients' age and height, while no clear correlation was observed between SJS and disease severity.

CONCLUSIONS

  1. Patients with MPS I and II present specific features in hip joint ultrasonography. 2. The data suggests that ultrasonography might be effective in the evaluation of hip joint involvement in patients with MPS and might present a valuable tool in facilitating the diagnosis and follow up of the disease.
摘要

目的

本研究的主要目的是评估黏多糖贮积症（MPS）I型和II型患者髋关节的超声特征，并与健康人群进行比较。次要目的是将这些特征与临床指标相关联，并评估超声在MPS疾病诊断中的效用。

材料与方法

本研究纳入了16例MPS I型（n = 3）和II型（n = 13）患者，对其进行了临床和放射学评估以及双侧髋关节高分辨率超声（US）检查。评估了从股骨颈到关节囊的距离（滑膜关节间隙，SJS）、关节积液、滑膜增生和局部病理性血管形成。将结果与健康人群进行比较，并与临床和放射学指标相关联。

结果

  1. MPS疾病患儿的US SJS与健康人群的标准值（7mm）存在差异。SJS的平均值为15.81±4.08cm（右髋关节）和15.69±4.19cm（左髋关节）。2. 在MPS患者中未检测到炎性关节异常。3. US SJS与患者的年龄和身高之间存在明显相关性，而SJS与疾病严重程度之间未观察到明显相关性。

结论

  1. MPS I型和II型患者在髋关节超声检查中呈现出特定特征。2. 数据表明，超声检查可能在评估MPS患者的髋关节受累情况方面有效，并且可能是促进该疾病诊断和随访的有价值工具。
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7886/4414504/3105ac5ca08b/pone.0123792.g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7886/4414504/7be76a5b78a6/pone.0123792.g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7886/4414504/be898ee0432e/pone.0123792.g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7886/4414504/c3081ef0518a/pone.0123792.g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7886/4414504/d00a3021957c/pone.0123792.g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7886/4414504/6cb4c92e3515/pone.0123792.g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7886/4414504/3105ac5ca08b/pone.0123792.g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7886/4414504/7be76a5b78a6/pone.0123792.g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7886/4414504/be898ee0432e/pone.0123792.g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7886/4414504/c3081ef0518a/pone.0123792.g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7886/4414504/d00a3021957c/pone.0123792.g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7886/4414504/6cb4c92e3515/pone.0123792.g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7886/4414504/3105ac5ca08b/pone.0123792.g006.jpg

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