title: Progressive pseudorheumatoid arthropathy of childhood.
abstract: Progressive pseudorheumatoid arthropathy of childhood (PPAC) described by Spranger et 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.
authors.affiliation: Department of Paediatrics, Ege University, Faculty of Medicine, Izmir, Turkey.
authors.forename: O
authors.initials: O
authors.lastname: Cogulu
authors.forename: F
authors.initials: F
authors.lastname: Ozkinay
authors.forename: C
authors.initials: C
authors.lastname: Ozkinay
authors.forename: G
authors.initials: G
authors.lastname: Sapmaz
authors.forename: O
authors.initials: O
authors.lastname: Yalman
authors.forename: H B
authors.initials: HB
authors.lastname: Deveci
mesh_terms: D000077:Acetabulum / Q000473:pathology; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D001171:Arthritis, Juvenile / Q000175:diagnosis*; D002648:Child; D003286:Contracture / Q000175:diagnosis; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008141:Lordosis / Q000175:diagnosis; D008159:Lumbar Vertebrae / Q000473:pathology; D010009:Osteochondrodysplasias / Q000175:diagnosis*; D010024:Osteoporosis / Q000175:diagnosis
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal