Briegel Wolfgang, Hofmann Christina, Schwab K Otfried
Department of Child and Adolescent Psychiatry and Psychotherapy, Leopoldina Hospital, Schweinfurt, Germany.
J Paediatr Child Health. 2010 Apr;46(4):144-8. doi: 10.1111/j.1440-1754.2009.01652.x. Epub 2010 Jan 26.
Moebius sequence is a rare condition usually defined as congenital facial paralysis with congenital impairment of ocular abduction. At present, there is little information on behavioural problems, parental stress and possible relationships between these factors. To fill this gap, this study investigated these aspects relevant for counselling.
Parents of 4-17 year old subjects known to the German Möbius syndrome foundation were anonymously asked to fill out several questionnaires, for example, the Child Behavior Checklist (CBCL)4-18.
The primary care givers of 41/58 subjects (70.7%) sent back filled-out questionnaires. Ten subjects did not meet the inclusion criteria; 15 males and 16 females (4; 7-17; 0 years, median age: 10; 7 years) were included. Ten out of 31 subjects were rated as clinical on at least one CBCL scale; three had a total problem score in the clinical range. Social problems were the most important problems with rates of 12-17-year old subjects being about five times as high as those of younger subjects. Compared with the general population, but not with other parents of mentally and/or physically handicapped children, the primary care givers experienced higher levels of stress, which were correlated to anxious/depressed behaviour, aggressive behaviour, externalising problems and total problem score of the children. The older a child the higher the primary care giver's life satisfaction was.
Social problems seem to be frequent among 4-17-year old subjects with Moebius sequence, and primary care givers show increased strain. Therefore, families with an affected child need early and adequate support.
默比厄斯序列征是一种罕见病症，通常定义为先天性面瘫合并先天性眼球外展障碍。目前，关于行为问题、父母压力以及这些因素之间可能存在的关系的信息较少。为填补这一空白，本研究调查了这些与咨询相关的方面。
德国默比厄斯综合征基金会已知的4至17岁受试者的父母被匿名要求填写几份问卷，例如儿童行为检查表（CBCL）4 - 18。
58名受试者中的41名（70.7%）的主要照顾者寄回了填写好的问卷。10名受试者不符合纳入标准；纳入了15名男性和16名女性（4；7 - 17；0岁，中位年龄：10；7岁）。31名受试者中有10名在至少一个CBCL量表上被评定为临床问题；3名儿童的总问题得分处于临床范围。社交问题是最重要的问题，12至17岁受试者的发生率约为年幼儿童的五倍。与一般人群相比，但与其他智力和/或身体残疾儿童的父母相比，主要照顾者经历了更高水平的压力，这与儿童的焦虑/抑郁行为、攻击行为、外化问题和总问题得分相关。孩子年龄越大，主要照顾者的生活满意度越高。
默比厄斯序列征的4至17岁受试者中社交问题似乎很常见，主要照顾者表现出更大的压力。因此，有患病儿童的家庭需要早期且充分的支持。
