Progressive pseudorheumatoid arthropathy of childhood |
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Authors: | Ozgur Cogulu MD Ferda Ozkinay Cihangir Ozkinay Gül Sapmaz Osman Yalman Helda Binnur Deveci |
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Institution: | (1) Faculty of Medicine, Department of Paediatrics, Ege University, 35100 Bornova-Izmir, Turkey;(2) Department of Medical Biology, Ege University Faculty of Medicine, 35100 Bornova-Izmir, Turkey;(3) Department of Radiology, Ege University, Faculty of Medicine, 35100 Bornova-Izmir, Turkey |
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Abstract: | Progressive pseudorheumatoid arthropathy of childhood (PPAC) described by Sprangeret al is a rare autosomal recessive disorder.
An 11 year-old girl was diagnosed as having PPAC at Ege University, Faculty of Medicine, Department of Paediatrics. Her complaints
of painful joints, difficulty in walking and joint contractures began at the age of 3 years and she was treated for juvenile
rheumatoid arthritis for 8 years. Her symptoms did not respond to nonsteroid anti-inflammatory treatment. During her last
hospitalisation period, she was reinvestigated. Radiological examination showed spondyloepiphyseal dysplasia, severe acetabular
irregularity and osteoporosis. All the laboratory test results for rheumatoid arthritis were negative. The clinical and radiological
findings of the patient are illustrated. |
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Keywords: | Progressive pseudorheumatoid arthropathy Spondyloepiphyseal dysplasia |
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