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Bilateral Charnley Low-Friction Arthroplasty with Cement in a Patient with PyknodysostosisA Case Report
Farokh Wadia, MRCS(Edin), MS(Orth), DNB(Orth)1; Nikhil Shah, FRCS(Orth), FRCS Glasgow, MS(Orth), DNB(Orth)1; Martyn Porter, MB ChB, FRCS(Ed), FRCS Ed(Orth)1
1 Centre for Hip Surgery, Wrightington Hospital, Hall Lane, Appley Bridge, Wigan WN6 9EP, United Kingdom. E-mail address for F. Wadia: farokh_w@yahoo.co.in
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The authors did not receive grants or outside funding in support of their research for or preparation of this manuscript. They did not receive payments or other benefits or a commitment or agreement to provide such benefits from a commercial entity. No commercial entity paid or directed, or agreed to pay or direct, any benefits to any research fund, foundation, educational institution, or other charitable or nonprofit organization with which the authors are affiliated or associated.
Investigation performed at the Centre for Hip Surgery, Wrightington Hospital, Wigan, United Kingdom

The Journal of Bone and Joint Surgery, Incorporated
J Bone Joint Surg Am, 2006 Aug 01;88(8):1846-1848. doi: 10.2106/JBJS.E.00915
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Extract

Pyknodysostosis, also known as Maroteaux-Lamy syndrome, is a rare, autosomal recessive disorder, which was first described by Maroteaux and Lamy1 and Andren et al.2 in 1962. It has been reported to have a prevalence of 1.7 per one million individuals3. The disorder is characterized by short stature with short limbs, large skull vault, small chin and face, high arched palate, retained deciduous teeth, apparent clubbed fingers due to acro-osteolysis, pectus excavatum, and kyphoscoliosis3,4. Fractures of long bones are common with minor trauma, and they usually heal with deformity. End-stage arthritis of the weight-bearing joints may occur. The clinical and radiographic features are similar to those of osteopetrosis and other osteosclerotic conditions. The most distinguishing feature in pyknodysostosis is short stature with short limbs. Radiographically, the osseous sclerosis is more uniform and a medullary canal is still identifiable in the long bones, which is not the case with osteopetrosis.
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    These activities have been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint sponsorship of the American Academy of Orthopaedic Surgeons and The Journal of Bone and Joint Surgery, Inc. The American Academy of Orthopaedic Surgeons is accredited by the ACCME to provide continuing medical education for physicians.
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