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拉森综合征:文献综述及38例病例分析

Larsen's syndrome: review of the literature and analysis of thirty-eight cases.

作者信息

Laville J M, Lakermance P, Limouzy F

机构信息

Service de Chirurgie Infantile, C.H.D. Félix Guyon, Ile de la Réunion.

出版信息

J Pediatr Orthop. 1994 Jan-Feb;14(1):63-73. doi: 10.1097/01241398-199401000-00014.

DOI:10.1097/01241398-199401000-00014
PMID:8113375
Abstract

Thirty-eight cases of Larsen's syndrome in patients living on Reunion Island were analyzed, with an average follow-up of 13 years. Fourteen of these patients died of early complications secondary to dislocation of the cervical spine, tracheomalacias, heart disease, and severe respiratory infection. These children pose a problem of planning early treatment. Clubfeet must be treated conservatively early, and operation should be postponed until after knee deformities are corrected by plaster cast and/or early operation. Unilateral hip dislocations should be treated surgically in the second year of life. Treatment of bilateral hip dislocations is best neglected. Finally, the spinal status of these children must be monitored throughout their life.

摘要

对留尼汪岛38例拉森综合征患者进行了分析,平均随访13年。其中14例患者死于颈椎脱位、气管软化、心脏病和严重呼吸道感染等早期并发症。这些儿童带来了早期治疗规划的问题。马蹄内翻足必须尽早进行保守治疗,手术应推迟到通过石膏固定和/或早期手术矫正膝关节畸形之后。单侧髋关节脱位应在患儿1岁时进行手术治疗。双侧髋关节脱位最好不予治疗。最后,必须对这些儿童的脊柱状况进行终身监测。

相似文献

1
Larsen's syndrome: review of the literature and analysis of thirty-eight cases.拉森综合征:文献综述及38例病例分析
J Pediatr Orthop. 1994 Jan-Feb;14(1):63-73. doi: 10.1097/01241398-199401000-00014.
2
Clinical features and treatment of joint dislocations in Larsen's syndrome. Report of three cases in one family.
Clin Orthop Relat Res. 1976 Sep(119):206-10.
3
Ossicular abnormality in Larsen's syndrome: a case report.拉森综合征中的听小骨异常:病例报告
Am J Otolaryngol. 1991 Jan-Feb;12(1):51-3. doi: 10.1016/0196-0709(91)90073-o.
4
Early operation of the dislocated knee in Larsen's syndrome. A report of 2 cases.
Acta Orthop Scand. 1988 Oct;59(5):582-4. doi: 10.3109/17453678809148790.
5
Prenatal diagnosis and obstetric management of Larsen's syndrome in a patient with an unrecognized family history of the disease.一名患有未被识别的Larsen综合征家族病史患者的产前诊断及产科管理
Gynecol Obstet Invest. 1998;46(4):274-8. doi: 10.1159/000010050.
6
Spinal deformities in Larsen's syndrome.
Clin Orthop Relat Res. 1985 Jul-Aug(197):159-63.
7
Larsen's syndrome: anaesthetic implications. Six case reports.拉森综合征:麻醉相关问题。六例病例报告。
Paediatr Anaesth. 1995;5(2):133-8. doi: 10.1111/j.1460-9592.1995.tb00261.x.
8
Larsen's syndrome: four patients belonging to three generations of a Saudi Arabian family.拉森综合征:沙特阿拉伯一个家族三代中的四名患者。
Rev Rhum Engl Ed. 1997 Oct;64(10):538-41.
9
[Larsen's syndrome: congenital dislocation of the knees and other joints, distinctive facies, and, frequently, cleft palate].
Ann Radiol (Paris). 1972 Mar-Apr;15(3):297-328.
10
Larsen's syndrome: a heritable disorder.拉森综合征:一种遗传性疾病。
J Bone Joint Surg Am. 1976 Jun;58(4):558-61.

引用本文的文献

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FLNB haploinsufficiency-related short stature: a new syndrome or an expanded spectrum of Larsen syndrome.与FLNB单倍剂量不足相关的身材矮小:一种新综合征还是 Larsen 综合征的扩展谱系?
World J Pediatr. 2024 Sep;20(9):976-980. doi: 10.1007/s12519-024-00832-z. Epub 2024 Jul 30.
2
Craniovertebral junction instability in Larsen syndrome: An institutional series and review of literature.拉森综合征中的颅颈交界区不稳定:一项机构病例系列研究及文献综述
J Craniovertebr Junction Spine. 2020 Oct-Dec;11(4):276-286. doi: 10.4103/jcvjs.JCVJS_164_20. Epub 2020 Nov 26.
3
An orthodontic perspective on Larsen syndrome.
Larsen 综合征的正畸观点。
BMC Oral Health. 2021 Mar 10;21(1):111. doi: 10.1186/s12903-021-01454-x.
4
Knee arthrodesis for a congenital luxation with Larsen syndrome.膝关节融合术治疗先天性脱位合并 Larsen 综合征。
BMJ Case Rep. 2020 Jun 1;13(6):e232109. doi: 10.1136/bcr-2019-232109.
5
One Stage Bilateral Total Hip Arthroplasty in Siblings with Larsen Syndrome.患有拉森综合征的兄弟姐妹的一期双侧全髋关节置换术。
Open Orthop J. 2016 Nov 30;10:569-576. doi: 10.2174/1874325001610010569. eCollection 2016.
6
Spine malformation complex in 3 diverse syndromic entities: Case reports.3种不同综合征实体中的脊柱畸形复合体:病例报告
Medicine (Baltimore). 2016 Dec;95(50):e5505. doi: 10.1097/MD.0000000000005505.
7
Dwarfism with joint laxity in Friesian horses is associated with a splice site mutation in B4GALT7.弗里斯兰马的侏儒症伴有关节松弛与B4GALT7基因的剪接位点突变有关。
BMC Genomics. 2016 Oct 28;17(1):839. doi: 10.1186/s12864-016-3186-0.
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Hip Dysplasia in Children With Osteogenesis Imperfecta: Association With Collagen Type I C-Propeptide Mutations.成骨不全患儿的髋关节发育不良:与I型胶原C-前肽突变的关联
J Pediatr Orthop. 2017 Oct/Nov;37(7):479-483. doi: 10.1097/BPO.0000000000000644.
9
Expanding the clinical spectrum of B4GALT7 deficiency: homozygous p.R270C mutation with founder effect causes Larsen of Reunion Island syndrome.扩展B4GALT7缺乏症的临床谱:具有奠基者效应的纯合p.R270C突变导致留尼汪岛拉森综合征。
Eur J Hum Genet. 2015 Jan;23(1):49-53. doi: 10.1038/ejhg.2014.60. Epub 2014 Apr 23.
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[Congenital dislocation of the knee (CDK)].[先天性膝关节脱位(CDK)]
Orthopade. 2012 Jan;41(1):75-82; quiz 83. doi: 10.1007/s00132-011-1874-7.