title: CINCA syndrome with surgical intervention for valgus deformity and flexion contracture of the knee joint: A case report.
abstract: Chronic infantile neurological, cutaneous, and articular (CINCA) syndrome is a systemic autoinflammatory disease caused by increased production of interleukin (IL)-1β. We present a case of CINCA syndrome followed up to skeletal maturity. Joint contracture and valgus deformity of the knee had developed before diagnosis. Surgical interventions by soft tissue release and hemiepiphysiodesis improved the contracture and the deformity, and IL-1 receptor antagonist dramatically controlled systemic inflammation, and the patient lives without any disabilities.
authors: a Department of Pediatric Orthopaedics , Shiga Medical Center for Children , Shiga , Japan.
authors: Yuki
authors: Y
authors: Harada
authors: a Department of Pediatric Orthopaedics , Shiga Medical Center for Children , Shiga , Japan.
authors: Kenichi
authors: K
authors: Fukiage
authors: b Department of Pediatrics , Kyoto University Hospital , Shiga , Japan.
authors: Ryuta
authors: R
authors: Nishikomori
authors: c Department of Pediatric Orthopaedics , Mizuno Memorial Hospital , Shiga , Japan.
authors: Shigeo
authors: S
authors: Suzuki
authors: b Department of Pediatrics , Kyoto University Hospital , Shiga , Japan.
authors: Tohru
authors: T
authors: Futami
mesh_terms: D018501:Antirheumatic Agents / Q000627:therapeutic use; D003286:Contracture / Q000175:diagnosis* / Q000188:drug therapy / Q000601:surgery; D056587:Cryopyrin-Associated Periodic Syndromes / Q000175:diagnosis* / Q000188:drug therapy / Q000601:surgery; D006801:Humans; D007223:Infant; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use; D007719:Knee Joint / Q000473:pathology / Q000601:surgery; D008297:Male
chemical_list: D018501:Antirheumatic Agents; D053590:Interleukin 1 Receptor Antagonist Protein
keywords: CINCA syndrome; Flexion contracture; Hemiepiphysiodesis; IL-1 beta inhibitor