Title of article :
Proximal realignment surgery for unilateral chronic patella dislocation in Morquio syndrome: a case report
Author/Authors :
BAZ, Ali Bülent Antalya Training and Research Hospital - Department of Orthopedics and Traumatology, Turkey , AKALIN, Serdar Antalya Training and Research Hospital - Department of Orthopedics and Traumatology, Turkey , ARIK, Hasan Antalya Training and Research Hospital - Department of Orthopedics and Traumatology, Turkey , ERGÜN, Ali Antalya Training and Research Hospital - Department of Orthopedics and Traumatology, Turkey
Abstract :
Mucopolysaccharidosis IVA (MPS IVA: Morquio A syndrome) is a lysosomal storage disorder caused by a deficiency of N-acetylgalactosamine-6-sulfate sulfatase. Patients with MPS IVA appear healthy at birth. Morquio-specific radiographic changes can be observed prior to clinical signs and symptoms. Patients are usually affected by a severe joint degeneration from the 2nd or 3rd decade. Hyperlaxity of the joints is prominent due to the excess of intermediate metabolites. We report a patient with inherited dwarfism, in which a proximal soft tissue realignment procedure was performed to treat chronic patellar dislocation.
Keywords :
Birth defect , inherited dwarfism , Morquio syndrome , mucopolysaccharide
Journal title :
Acta Orthopaedica Et Traumatologica Turcica
Journal title :
Acta Orthopaedica Et Traumatologica Turcica