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一例新生儿的肢根型点状软骨发育不良。

A case of rhizomelic chondrodysplasia punctata in newborn.

作者信息

Karabayır Nalan, Keskindemirci Gonca, Adal Erdal, Korkmaz Orhan

机构信息

Pediatrics Department, Bakırköy Maternity and Children Education and Research Hospital, Kartaltepe mah Aksoy sok. Petrol Sitesi 6/11 Bakırköy, Istanbul, Turkey.

Radiology Department, Bakırköy Maternity and Children Education and Research Hospital, Turkey.

出版信息

Case Rep Med. 2014;2014:879679. doi: 10.1155/2014/879679. Epub 2014 Mar 9.

Abstract

Rhizomelic chondrodysplasia punctate (RCDP) is a rare autosomal recessive peroxisomal disease. The main features of the disease are shortening of the proximal long bones, punctate calcifications located in the epiphyses of long bones and in soft tissues around joints and vertebral column, vertebral clefting, dysmorphic face, and severe growth retardation, whereas cervical spinal stenosis may also rarely be present. Imaging of the brain and spinal cord in patients with this disorder may aid prognosis and guide management decisions. We report the newborn diagnosed as CDP with cervical stenosis. Our aim is to discuss current knowledge on etiopathogenesis as well as radiological and clinical symptoms of diseases associated with CDP.

摘要

肢根型点状软骨发育不良(RCDP)是一种罕见的常染色体隐性遗传性过氧化物酶体病。该病的主要特征是近端长骨缩短、位于长骨骺端以及关节和脊柱周围软组织中的点状钙化、椎体裂、面部畸形和严重生长发育迟缓,而颈椎管狭窄也可能很少出现。对患有这种疾病的患者进行脑和脊髓成像可能有助于预后评估并指导治疗决策。我们报告了一名被诊断为患有颈椎管狭窄的CDP新生儿。我们的目的是讨论关于病因发病机制以及与CDP相关疾病的放射学和临床症状的现有知识。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/aeb4/3970257/c28a34ac0415/CRIM2014-879679.001.jpg

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