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


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.
Department of Pediatrics, St. Louis Regional Children's Hospital, Cracow, Poland.
Zbigniew
Z
Żuber
Department of Pediatrics, Nutrition and Metabolic Diseases, The Children's Memorial Health Institute, Warsaw, Poland; Department of Genetics, University of Gdańsk, Gdańsk, Poland.
Agnieszka
A
Jurecka
Anthropology Laboratory, The Children's Memorial Health Institute, Warsaw, Poland.
Agnieszka
A
Różdżyńska-Świątkowska
Department of Pediatrics, St. Louis Regional Children's Hospital, Cracow, Poland.
Agata
A
Migas-Majoch
Department of Radiology, The Children's Memorial Health Institute, Warsaw, Poland.
Agnieszka
A
Lembas
Department of Metabolic Diseases, Medical College Jagiellonian University, Krakow, Poland.
Beata
B
Kieć-Wilk
Department of Pediatrics, Nutrition and Metabolic Diseases, The Children's Memorial Health Institute, Warsaw, Poland.
Anna
A
Tylki-Szymańska
D000293:Adolescent; D000328:Adult; D002648:Child; D003430:Cross-Sectional Studies; D005260:Female; D005272:Femur Neck / Q000000981:diagnostic imaging; D006621:Hip Joint / Q000000981:diagnostic imaging* / Q000473:pathology; D006801:Humans; D008297:Male; D008059:Mucopolysaccharidosis I / Q000175:diagnosis / Q000000981:diagnostic imaging* / Q000473:pathology; D016532:Mucopolysaccharidosis II / Q000175:diagnosis / Q000000981:diagnostic imaging* / Q000473:pathology; D012720:Severity of Illness Index; D014463:Ultrasonography