Ultrasound's role in differentiating camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome from inflammatory arthritis in children. A narrative review.

Mihaela Sparchez, Daniela Fodor
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Abstract

Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome is a rare genetic disorder characterized by a combination of congenital flexion contractures of the fingers (camptodactyly), non-inflammatory joint swelling (arthropathy), hip deformities (coxa vara), and recurrent pericarditis. In early childhood, the clinical presentation is dominated by the articular manifestations that can easily mimic juvenile idiopathic arthritis, often leading to delayed diagnosis and inappropriate treatments. Although not pathognomonic, ultrasound may provide specific ultrasound characteristics of joint involvement in CACP syndrome that help differentiate it from inflammatory arthropathies. This report aims to highlight the role of ultrasound in the initial assessment, differential diagnosis, and ongoing monitoring of CACP syndrome, drawing on both our patient series and a review of the literature.

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超声在鉴别儿童喜树趾-关节病-髋外翻-心包炎(CACP)综合征与炎性关节炎中的作用。叙述性评论
喜树指-关节病-髋内翻-心包炎(CACP)综合征是一种罕见的遗传性疾病,其特征是先天性手指屈曲挛缩(喜树指)、非炎症性关节肿胀(关节病)、髋关节畸形(髋内翻)和复发性心包炎的结合。在儿童早期,临床表现以关节表现为主,很容易模仿青少年特发性关节炎,经常导致诊断延误和治疗不当。虽然不是典型的,但超声可以提供CACP综合征关节受累的特定超声特征,有助于将其与炎性关节病区分开来。本报告旨在强调超声在CACP综合征的初步评估,鉴别诊断和持续监测中的作用,借鉴我们的患者系列和文献综述。
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