Hip disease in Mucopolysaccharidoses and Mucolipidoses: A review of mechanisms, interventions and future perspectives
نویسندگان
چکیده
The hips are frequently involved in inheritable diseases which affect the bones. clinical and radiological presentation of these may be very similar to common hip disorders as developmental dysplasia hip, osteoarthritis avascular necrosis, so diagnosis easily overlooked treatment suboptimal. Mucopolysaccharidosis (MPS) Mucolipidosis (ML II III) lysosomal storage with multisystemic involvement. Characteristic skeletal abnormalities, known dysostosis multiplex, MPS ML originate from intra-lysosomal glycosaminoglycans cells cartilage, bones ligaments. joint is severely affected ML. Hip pathology results limitations mobility pain young age, negatively affects quality life. In order better understand underlying process that causes disease ML, this review first describes normal physiological (embryonic) development, including interplay between acetabulum femoral head. second part factors contributing altered morphology function discussed, such abnormal development pelvic- (which biomechanical forces) inflammation. last therapeutic options future perspectives addressed.
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ژورنال
عنوان ژورنال: Bone
سال: 2021
ISSN: ['8756-3282', '1873-2763']
DOI: https://doi.org/10.1016/j.bone.2020.115729