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

Żuber Z, Jurecka A, Różdżyńska-Świątkowska A, Migas-Majoch A, Lembas A, Kieć-Wilk B, Tylki-Szymańska A - PLoS ONE (2015)

Bottom Line: The results were compared to the healthy population and correlated with clinical and radiological measures. 1.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. 1.

View Article: PubMed Central - PubMed

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

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.

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Related in: MedlinePlus

Ultrasound images of hip joints.(Left) Longitudinal scan of hip joint in a 14-year-old patient with a severe phenotype of MPS II. (Right) Longitudinal scan of hip joint in a 14-year-old healthy child. Arrows shows differences in the distance from the femoral neck to joint capsule (synovial joint space, SJS).
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pone.0123792.g001: Ultrasound images of hip joints.(Left) Longitudinal scan of hip joint in a 14-year-old patient with a severe phenotype of MPS II. (Right) Longitudinal scan of hip joint in a 14-year-old healthy child. Arrows shows differences in the distance from the femoral neck to joint capsule (synovial joint space, SJS).

Mentions: The protocol was approved by the human-subjects institutional review board at St. Louis Hospital (Ethics Committee, St. Louis Hospital, Cracow, Poland). Written informed consent had to be provided by the parents or legal guardians.


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

Żuber Z, Jurecka A, Różdżyńska-Świątkowska A, Migas-Majoch A, Lembas A, Kieć-Wilk B, Tylki-Szymańska A - PLoS ONE (2015)

Ultrasound images of hip joints.(Left) Longitudinal scan of hip joint in a 14-year-old patient with a severe phenotype of MPS II. (Right) Longitudinal scan of hip joint in a 14-year-old healthy child. Arrows shows differences in the distance from the femoral neck to joint capsule (synovial joint space, SJS).
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4414504&req=5

pone.0123792.g001: Ultrasound images of hip joints.(Left) Longitudinal scan of hip joint in a 14-year-old patient with a severe phenotype of MPS II. (Right) Longitudinal scan of hip joint in a 14-year-old healthy child. Arrows shows differences in the distance from the femoral neck to joint capsule (synovial joint space, SJS).
Mentions: The protocol was approved by the human-subjects institutional review board at St. Louis Hospital (Ethics Committee, St. Louis Hospital, Cracow, Poland). Written informed consent had to be provided by the parents or legal guardians.

Bottom Line: The results were compared to the healthy population and correlated with clinical and radiological measures. 1.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. 1.

View Article: PubMed Central - PubMed

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

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.

Show MeSH
Related in: MedlinePlus