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约旦唇腭裂患者样本中的先天性相关畸形

Congenital associated malformations in a sample of Jordanian patients with cleft lip and palate.

作者信息

Rawashdeh Ma'amon A, Jawdat Abu-Hawas Basim

机构信息

Jordan University of Science and Technology and King Abdullah University Hospital/Cleft lip and Palate Centre, Ar Ramtha, Jordan.

出版信息

J Oral Maxillofac Surg. 2008 Oct;66(10):2035-41. doi: 10.1016/j.joms.2008.01.009.

Abstract

PURPOSE

To report the prevalence of associated congenital malformations in Jordanian subjects with oral clefts.

PATIENTS AND METHODS

A retrospective study of 196 cleft patients was carried out at the Cleft Centre at King Abdullah University Hospital and Maxillofacial Unit at Jordan University of Science and Technology during the period December 2005 to September 2006. Data were collected by interviewing the parents or patients and reviewing the patient's medical records.

RESULTS

Twenty-eight cleft subjects had 44 associated malformations other than cleft. Prevalence of associated congenital malformations in Jordanian cleft patients was 14.3%. Of 44 associated malformations, 20 (45.5%) affected the cardiovascular system (CVS), 7 (15.9%) affected the skeletal system, 6 (13.6%) affected the central nervous system, 4 (9.1%) affected the urogenital system, 4 (9.1%) affected the eyes, 2 (4.5%) affected the ears, and 1 (2.3%) affected the digestive system. Approximately 35.3% of these anomalies affected cleft palate subjects, while 17.4% occurred in bilateral cleft lip with or without cleft palate subjects, and 6.4% affected unilateral cleft lip with or without cleft palate subjects. The most commonly recorded anomaly was atrial septal defect (15.9%).

CONCLUSIONS

Increased risk of associated malformations in children with clefts must to be highlighted and disseminated to health professionals involved in the management of children with clefts. Routine screening for other malformation, especially cardiac, skeletal, and central nervous system malformations, may need to be considered in infants with clefts, and genetic counseling seems warranted in most of these complicated cases. There should be a close liaison between the cleft team members to comprehensively cover all aspects of the cleft patient's management.

摘要

目的

报告约旦唇腭裂患者相关先天性畸形的患病率。

患者与方法

2005年12月至2006年9月期间,在阿卜杜拉国王大学医院腭裂中心及约旦科技大学颌面外科对196例唇腭裂患者进行了一项回顾性研究。通过与父母或患者面谈并查阅患者病历收集数据。

结果

28例唇腭裂患者有44种除唇腭裂外的相关畸形。约旦唇腭裂患者相关先天性畸形的患病率为14.3%。在44种相关畸形中,20种(45.5%)影响心血管系统(CVS),7种(15.9%)影响骨骼系统,6种(13.6%)影响中枢神经系统,4种(9.1%)影响泌尿生殖系统,4种(9.1%)影响眼睛,2种(4.5%)影响耳朵,1种(2.3%)影响消化系统。这些畸形中约35.3%影响腭裂患者,17.4%发生在双侧唇裂伴或不伴腭裂患者中,6.4%影响单侧唇裂伴或不伴腭裂患者。最常记录的畸形是房间隔缺损(15.9%)。

结论

必须强调并向参与唇腭裂患儿管理的卫生专业人员传播唇腭裂患儿相关畸形风险增加的信息。对于唇腭裂婴儿,可能需要考虑对其他畸形进行常规筛查,尤其是心脏、骨骼和中枢神经系统畸形,并且在大多数这些复杂病例中似乎有必要进行遗传咨询。唇腭裂治疗团队成员之间应密切联络,以全面涵盖唇腭裂患者管理的各个方面。

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