Dataset Information

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

ABSTRACT: 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.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.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.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.

SUBMITTER: Zuber Z

PROVIDER: S-EPMC4414504 | biostudies-literature | 2015

REPOSITORIES: biostudies-literature

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

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

PloS one 20150429 4

<h4>Objectives</h4>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.<h4>Materials and methods</h4>Sixteen MPS I (n = 3) and II (n = 13) patients were enrolled in the present study and underwent clinical and radiolo ...[more]

PMID: 25922936

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Project description:BackgroundTransbronchial lung biopsy (TBLB) of peripheral pulmonary lesions (PPLs) is usually performed for a definite diagnosis. Radial probe endobronchial ultrasonography is often acknowledged as a good guidance method for TBLB as it can help physicians confirm the lesions' position. It is also a non-invasive imaging diagnostic method. This clinical study was designed to evaluate the ability of radial endobronchial ultrasonography (R-EBUS) to differentiate benign from malignant predominant solid PPLs based on imaging features.MethodsPatients with predominant solid PPLs were enrolled in this study retrospectively. TBLB was performed using R-EBUS with or without other guidance techniques. One typical sonographic image and one video of each lesion were recorded for analysis. Six radial probe endobronchial ultrasonographic image features (size, shape, echogenicity, margin, blood vessel, and linear-discrete air bronchogram) were studied by ultrasonography specialists and physicians who were blinded to the final diagnosis. The sum score model of the combined predictive factors indicated the best diagnostic accuracies for predicting malignant PPLs. The model group results were used to establish the diagnostic standard for a verification group.ResultsA total of 303 patients were enrolled in the model group from July 2018 to July 2019 at the Shanghai Chest Hospital (214 with malignant and 89 with benign lesions). The mean lesion long axis on computed tomographic images was 34.39±13.79 mm. There were significant statistical differences between benign and malignant lesions in the long axis, short axis, shape, margin, blood vessel, and linear-discrete air bronchogram assessed by radial endobronchial ultrasound. Long axis, lobulation, distinct but not sharp margin, absence of blood vessel, and absence of linear-discrete air bronchogram were good predictive factors of malignant lesions. A sum score model value of 79.54% of these combined factors indicated the best diagnostic accuracy for predicting malignant lesions. Eighty-seven patients were enrolled in the verification group from August to October 2019. The sum score model showed a diagnostic accuracy of 82.76%.ConclusionsRadial endobronchial ultrasonographic features can differentiate malignant from benign lesions and thus have potential diagnosis value in predominant solid PPLs.

Dataset Information

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

Publications

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

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