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儿童遗传性玻璃样纤维瘤病综合征患者的股骨骨折。

Femur fracture in a paediatric patient with hereditary hyaline fibromatosis syndrome.

机构信息

Trauma and Orthopaedics, Bradford Royal Infirmary, Bradford, UK

Trauma and Orthopaedics, Bradford Royal Infirmary, Bradford, UK.

出版信息

BMJ Case Rep. 2022 Apr 5;15(4):e248447. doi: 10.1136/bcr-2021-248447.

Abstract

An 18-month-old girl with hereditary hyaline fibromatosis syndrome (HHFS) and fixed flexion contractures presented with an oblique femur fracture, following a fall out of her mother's arms. The fracture was abutting intramedullary hyaline lesions. Due to her condition, balanced traction was impossible to apply. The authors report effective treatment of her injury using a non-operative approach in an early hip spica, over a 4-week period. There was no evidence of delayed osseous healing. Early spica application could be used as a definitive management option in children with femur fractures and fixed flexion contractures in future. This case emphasises the need for preventative measures to support bone health in patients with HHFS.

摘要

一位 18 个月大的女孩患有遗传性玻璃样纤维瘤病综合征(HHFS)和固定性屈曲挛缩症,从母亲怀中摔下后发生股骨斜形骨折。骨折紧贴着髓内玻璃样病变。由于她的病情,无法应用平衡牵引。作者报告了在 4 周的早期髋人字石膏中,使用非手术方法有效治疗她的损伤。没有证据表明骨愈合延迟。早期人字石膏固定可作为未来股骨骨折伴固定性屈曲挛缩患儿的确定性治疗选择。该病例强调了需要采取预防措施来支持 HHFS 患者的骨骼健康。

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本文引用的文献

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Infantile systemic hyalinosis: Variable grades of severity.婴儿全身玻璃样变性:严重程度不一。
Afr J Paediatr Surg. 2021 Oct-Dec;18(4):224-230. doi: 10.4103/ajps.AJPS_162_20.
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Juvenile Hyaline Fibromatosis.青少年透明纤维瘤病
Contemp Clin Dent. 2018 Jul-Sep;9(3):484-487. doi: 10.4103/ccd.ccd_117_18.
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The management of paediatric diaphyseal femoral fractures: a modern approach.小儿股骨干骨折的治疗:现代方法
Strategies Trauma Limb Reconstr. 2016 Aug;11(2):87-97. doi: 10.1007/s11751-016-0258-2. Epub 2016 Jul 11.
9
Use of flexible intramedullary nails in pediatric femur fractures.弹性髓内钉在儿童股骨骨折中的应用。
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