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Ultrasonographic Features of Hip Joints in Mucopolysaccharidoses Type I and II.
Zuber, Zbigniew; Jurecka, Agnieszka; Rózdzynska-Swiatkowska, Agnieszka; Migas-Majoch, Agata; Lembas, Agnieszka; Kiec-Wilk, Beata; Tylki-Szymanska, Anna.
Afiliación
  • Zuber Z; Department of Pediatrics, St. Louis Regional Children's Hospital, Cracow, Poland.
  • Jurecka A; Department of Pediatrics, Nutrition and Metabolic Diseases, The Children's Memorial Health Institute, Warsaw, Poland; Department of Genetics, University of Gdansk, Gdansk, Poland.
  • Rózdzynska-Swiatkowska A; Anthropology Laboratory, The Children's Memorial Health Institute, Warsaw, Poland.
  • Migas-Majoch A; Department of Pediatrics, St. Louis Regional Children's Hospital, Cracow, Poland.
  • Lembas A; Department of Radiology, The Children's Memorial Health Institute, Warsaw, Poland.
  • Kiec-Wilk B; Department of Metabolic Diseases, Medical College Jagiellonian University, Krakow, Poland.
  • Tylki-Szymanska A; Department of Pediatrics, Nutrition and Metabolic Diseases, The Children's Memorial Health Institute, Warsaw, Poland.
PLoS One ; 10(4): e0123792, 2015.
Article en En | MEDLINE | ID: mdl-25922936
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.
Asunto(s)

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Mucopolisacaridosis II / Mucopolisacaridosis I / Articulación de la Cadera Tipo de estudio: Diagnostic_studies / Observational_studies / Prevalence_studies / Risk_factors_studies Límite: Adolescent / Adult / Child / Female / Humans / Male Idioma: En Revista: PLoS One Asunto de la revista: CIENCIA / MEDICINA Año: 2015 Tipo del documento: Article País de afiliación: Polonia

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Mucopolisacaridosis II / Mucopolisacaridosis I / Articulación de la Cadera Tipo de estudio: Diagnostic_studies / Observational_studies / Prevalence_studies / Risk_factors_studies Límite: Adolescent / Adult / Child / Female / Humans / Male Idioma: En Revista: PLoS One Asunto de la revista: CIENCIA / MEDICINA Año: 2015 Tipo del documento: Article País de afiliación: Polonia
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