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[进行性骨化性纤维发育不良一例的影像学表现]

[Imaging aspect of a case of fibrodysplasia ossificans progressiva].

作者信息

Haddad S, Menassa L, Nasnas R, Abadjian G, Slaba S, Atallah N

机构信息

Service de Radiologie, Hôtel-Dieu de France, Beyrouth, Liban.

出版信息

J Radiol. 1997 Jun;78(6):449-52.

PMID:9239351
Abstract

Fibrodysplasia ossificans progressiva is a rare congenital disease that affects children under the age of five years. Soft tissue swelling of the cervical and dorsal regions with local pain, warmth and low grade fever are the early clinical manifestations, usually associated with hallux valgus and microdactily of the fingers and toes. Calcifications of the fascias and muscles cause muscular contractures leading to progressive disability and restrictive lung disease. In the early stage, CT shows edema of the soft tissues and later on, calcifications of muscular fascia. The association of these radiographic and CT findings is specific and should avoid muscular biopsies which are known to be an aggravating factor in this disease.

摘要

进行性骨化性纤维发育不良是一种罕见的先天性疾病,影响五岁以下儿童。颈部和背部软组织肿胀伴局部疼痛、发热和低热是早期临床表现,通常伴有拇外翻以及手指和脚趾短小。筋膜和肌肉钙化导致肌肉挛缩,进而导致进行性残疾和限制性肺病。早期,CT显示软组织水肿,后期显示肌肉筋膜钙化。这些影像学和CT表现具有特异性,应避免进行肌肉活检,因为已知肌肉活检是该疾病的一个加重因素。

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