Hemifacial Microsomia: Psychosocial and Other Sequelae

半面畸形：心理社会和其他后遗症

• 批准号: 6949173
• 负责人: MARTHA M. WERLER
• 金额: $ 48.69万
• 依托单位: BOSTON UNIVERSITY MEDICAL CAMPUS
• 依托单位国家: 美国
• 财政年份: 1997
• 资助国家: 美国
• 起止时间: 1997-07-01 至 2009-06-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/6949173
• 关键词: academic achievement; clinical research; cognition; congenital oral /facial /cranial defect; dental caries; epidemiology; functional ability; human subject; interview; learned helplessness; medical complication; medical records; middle childhood (6-11); morphology; neuropsychological tests; parent offspring interaction; psychological adaptation; psychometrics; quality of life; questionnaires; social psychology; stress management

DESCRIPTION (provided by applicant): Hemifacial microsomia is the second most common craniofacial malformation, yet there have been few studies of its impact on affected children. We propose to conduct the first large scale follow-up study of children affected with hemifacial microsomia, to evaluate psycho-social, dental, orthodontic, and medical sequelae. The study design features 280 cases and 884 age and geographically matched controls from across the U.S. and Canada who were previously enrolled in a case control study of pregnancy risk factors from 1997 to 2002. The case group includes a range of phenotypes from isolated mild facial asymmetry to severe mandibular, auricular, and orbital hypoplasia with cranial nerve paralysis and extracranial malformations. In the proposed project, data will be collected when study subjects are 6.0 to 7.5 years of age, using valid and reliable tools that were specifically developed for this age group. Information will be provided by cases and controls and their parents, teachers, and clinicians. We will measure quality of the parent-child relationship and marital, family, and child functioning with the Parenting Stress Index; pro-social behaviors, communication skills, self-control, peer popularity, and academic ability with the Child Behavior Checklist, Social Competance Scales (for both parents and teachers), Social Competance and Behavior Evaluation Short Form, and the Teacher Report Form; quality of life and adaptive functioning with PedsQL 4.0; and neurocognition with the Visual Motor Integration test, Peabody Picture Vocabulary Test-Ill, and Teacher Report Form. Composite scores will be calculated for each psycho-social domain. Scores will be compared between cases and controls, and among phenotypic case subgroups. Orthodontic and dental outcomes will include caries, hypodontia, malocclusion, periodontal status, and overall oral health. Questionnaires will be sent to case and control dentists and, if applicable, orthodontists. Copies of dental radiographs will be examined by an oral surgeon and an orthodontist and scored for hypodontia. We will obtain copies of cases' medical records from all specialists. Systematic review of medical records will be performed to tally all surgeries (e.g., orthognathic), treatments (e.g., antibiotic prophylaxis), interventions (e.g., hearing aids), and other speciality consultations. Dental, orthodontic, surgical, auditory, and other outcomes will be described for cases according to phenotype.

描述（由申请人提供）：半身微粒细胞症是第二大常见的颅面畸形，但很少有对其对受影响儿童的影响的研究。我们建议对患有半身微粒细胞感染的儿童进行首次大规模的随访研究，以评估心理社会，牙齿，正畸和医学后遗症。该研究设计的特征是来自美国和加拿大各地的280例病例和884例年龄和地理上匹配的对照，以前从1997年至2002年对怀孕危险因素进行了案例控制研究。该病例组包括一系列来自隔离的轻度面部不对称的表型，从严重下颌骨，严重的下颌骨，甲骨尿素，甲骨神经，和骨性型颅脑畸形和颅骨偏离术进行了脱颅和脱颅。在拟议的项目中，使用有效且可靠的工具专门为该年龄组开发的有效和可靠的工具，将在研究对象为6.0至7.5岁时收集数据。案件和控制及其父母，老师和临床医生将提供信息。我们将衡量父母关系的质量以及与育儿压力指数发挥作用的婚姻，家庭和孩子的质量；亲社会行为，沟通技巧，自我控制，同伴的流行和学术能力，具有儿童行为清单，社交竞争量表（对于父母和教师都），社会竞争力和行为评估简短形式以及教师报告表格； PEDSQL 4.0的生活质量和自适应功能；通过视觉运动集成测试，Peabody图片词汇测试和教师报告表格进行神经认知。将计算每个心理社会领域的综合分数。病例和对照组之间以及表型病例亚组之间的分数将进行比较。正畸和牙齿结局将包括龋齿，缩写，牙周牙周状态和整体口腔健康。问卷将被发送到案例和控制牙医，如果适用，正畸医生。牙科X光片的副本将由口腔外科医生和正畸医生检查，并为低音评分。我们将从所有专家那里获得病例病历的副本。对医疗记录的系统审查将对所有手术（例如，矫正术），治疗（例如抗生素预防），干预措施（例如助听器）和其他专业咨询进行分解。根据表型，将描述牙齿，正畸，外科，听觉和其他结果。