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猫叫综合征——一例罕见病例报告

Cri-Du-Chat Syndrome - A Rare Case Report.

作者信息

Dhanasekaran Balaji, Srinivasan Rajasekaran, Kanagamuthu Priya, Somu Prabakaran, R B Namasivaya Navin, Gowthame K, Kumar Sarath, Shree C Ramya, Karthika S R, Gopan Greeshma U

机构信息

Dept. of ENT, Chettinad Hospital and Research Institute, Kelambakkam, India.

出版信息

Indian J Otolaryngol Head Neck Surg. 2023 Dec;75(4):3993-3998. doi: 10.1007/s12070-023-04039-y. Epub 2023 Jun 30.

Abstract

The Cri-du-chat Syndrome (CdCs) is a rare genetic syndrome first described by Jerome Lejeune in 1963, characterized mainly by the high pitched cat like cry. The prevalence of CdCs was varied in between 1:15,000 to 1:50,000 in live birth and more common in female gender with a ratio of 4:3 [1, 2] .The condition may be accompanied by developmental and cognitive delays, poor spatial awareness, impaired ambulation, and poor sensori-motor skills. Other associated problems described include cardiovascular, renal, gastrointestinal, neurological abnormalities, preauricular tags, syndactyly, hypospadias, and cryptorchidism.1 Recent literatures show that autistic behaviours are common in various genetic disorders [3].Fatigue level of children with cri du chat syndrome was associated with the expression of autistic features [4]. Cri-du-chat syndrome is a rare genetic disorder resulting in various physical and psychological abnormalities due the deletion of chromosome 5P-. We encountered a case of cri-du-chat syndrome having external auditory canal atresia, hearing loss with speech delay. A multidisciplinary approach is required for diagnosis and management of such patients. Otological management is early identification of hearing loss and speech rehabilitation. Awareness about antenatal screening for congenital anomalies and genetic counselling is necessary among the general population.

摘要

猫叫综合征(CdCs)是一种罕见的基因综合征,1963年由杰罗姆·勒热纳首次描述,主要特征为高音调的猫叫样哭声。猫叫综合征在活产儿中的患病率在1:15000至1:50000之间,女性更为常见,男女比例为4:3[1,2]。该病症可能伴有发育和认知迟缓、空间意识差、行走障碍以及感觉运动技能差。描述的其他相关问题包括心血管、肾脏、胃肠道、神经异常、耳前赘生物、并指(趾)畸形、尿道下裂和隐睾症。1近期文献表明,自闭症行为在各种基因疾病中很常见[3]。猫叫综合征患儿的疲劳程度与自闭症特征的表现有关[4]。猫叫综合征是一种罕见的基因疾病,由于5号染色体短臂缺失导致各种身体和心理异常。我们遇到一例猫叫综合征患者,伴有外耳道闭锁、听力损失和言语迟缓。此类患者的诊断和管理需要多学科方法。耳科管理是早期识别听力损失和言语康复。普通人群有必要了解先天性异常的产前筛查和遗传咨询。

相似文献

1
Cri-Du-Chat Syndrome - A Rare Case Report.猫叫综合征——一例罕见病例报告
Indian J Otolaryngol Head Neck Surg. 2023 Dec;75(4):3993-3998. doi: 10.1007/s12070-023-04039-y. Epub 2023 Jun 30.
2
Cri du Chat syndrome.猫叫综合征。
Orphanet J Rare Dis. 2006 Sep 5;1:33. doi: 10.1186/1750-1172-1-33.
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Cri du Chat syndrome: Characteristics of 73 Brazilian patients.猫哭综合征:73 例巴西患者的特征。
J Intellect Disabil Res. 2018 Jun;62(6):467-473. doi: 10.1111/jir.12476. Epub 2018 Feb 20.
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Perioperative Care of a Child With Cri Du Chat Syndrome.猫叫综合征患儿的围手术期护理
J Med Cases. 2020 Sep;11(9):279-282. doi: 10.14740/jmc3494. Epub 2020 Aug 6.

本文引用的文献

1
When Cri du chat syndrome meets Edwards syndrome.当猫叫综合征遇上爱德华兹综合征。
Mol Med Rep. 2015 Mar;11(3):1933-8. doi: 10.3892/mmr.2014.2920. Epub 2014 Nov 10.
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Cri du Chat syndrome.猫叫综合征。
Orphanet J Rare Dis. 2006 Sep 5;1:33. doi: 10.1186/1750-1172-1-33.
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Auditory neuropathy.听觉神经病
Brain. 1996 Jun;119 ( Pt 3):741-53. doi: 10.1093/brain/119.3.741.
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Terminal deletion of the short arm of chromosome 5.5号染色体短臂的末端缺失。
Clin Genet. 1988 Oct;34(4):219-23. doi: 10.1111/j.1399-0004.1988.tb02868.x.

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