Schneider M B, Davis J G, Boxer R A, Fisher M, Friedman S B
Department of Pediatrics, North Shore University Hospital, Manhasset, NY 11030.
J Dev Behav Pediatr. 1990 Jun;11(3):122-7.
Twenty-two subjects with Marfan syndrome (age range 11-24 years, mean 15.7 years) were studied to evaluate the impact of Marfan syndrome on their lives, level of psychosocial adaptation, concerns about their disorder, compliance with medical regimens, and knowledge of Marfan syndrome. Subjects were interviewed and then completed the Offer Self-Image Questionnaire and two questionnaires designed for the study. Parents also were interviewed. Subjects demonstrated normal psychosocial adaptation on the Offer Self-Image Questionnaire. Nevertheless, the subjects perceived that their lives would be significantly better without Marfan syndrome, especially in the areas of physical activities and self-image. They expressed concerns about their illness most often to their parents, less frequently to their doctors. Their compliance with medical regimens were suboptimal and similar to the published description of compliance among teenagers with other chronic illnesses. Their knowledge base was weakest in the areas pertaining to future childbearing. Thus, although these subjects showed normal psychosocial adaptation, doctors caring for them may foster better coping with Marfan syndrome by discussing self-image issues and Marfan syndrome-related concerns, and by encouraging compliance and imparting knowledge.
对22名患有马凡氏综合征的受试者（年龄范围为11至24岁，平均15.7岁）进行了研究，以评估马凡氏综合征对他们生活的影响、心理社会适应水平、对自身疾病的担忧、对医疗方案的依从性以及对马凡氏综合征的了解程度。对受试者进行了访谈，然后让他们完成了奥弗自我形象问卷以及两份为本研究设计的问卷。还对受试者的父母进行了访谈。在奥弗自我形象问卷中，受试者表现出正常的心理社会适应能力。然而，受试者认为如果没有马凡氏综合征，他们的生活将会明显更好，尤其是在体育活动和自我形象方面。他们最常向父母表达对自己疾病的担忧，向医生表达的频率较低。他们对医疗方案的依从性欠佳，与已发表的关于其他慢性病青少年依从性的描述相似。他们在与未来生育相关的领域知识最薄弱。因此，尽管这些受试者表现出正常的心理社会适应能力，但照顾他们的医生可以通过讨论自我形象问题和与马凡氏综合征相关的担忧，鼓励依从性并传授知识，来促进他们更好地应对马凡氏综合征。
