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The Robin anomalad (Pierre Robin syndrome)--a follow up study.

机译:罗宾异常(皮埃尔·罗宾综合症)-一项随访研究。

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摘要

During a 10-year period 55 patients with the Robin anomalad were admitted to the Liverpool Regional Cleft Palate Units. Fourteen (25%) children died. All deaths were within 3 months of birth. Congenital abnormalities other than mandibular retrognathia and cleft palate were present in 14 (26%) children. Peripheral limb defects were particularly common. Thirty children were recalled and reviewed to assess speech, hearing, growth, and educational achievement. There was a clear association between severe nasal escape of air in speech and atypical articulatory patterns. Almost half the children tested had abnormal articulation. Only 4 (13%) of 30 children showed delayed language development. Half the children tested audiometrically showed a binaural handicap but in only one patient was this sufficiently severe to warrant amplification. There was no trend towards abnormalities of growth and only 2 children could be firmly classified as educationally subnormal.
机译:在10年的时间里,有55名Robin异常患者进入利物浦地区Regional裂病区。 14名(25%)儿童死亡。所有死亡均在出生后三个月内。 14名(26%)儿童中存在除下颌后突和and裂以外的先天性异常。周围肢体缺损尤为常见。召回并审查了30名儿童,以评估其言语,听力,成长和教育成就。言语中严重的鼻部空气逸出与非典型的发音模式之间存在明显的关联。接受测试的孩子中几乎有一半的发音异常。 30名儿童中只有4名(13%)的语言发育迟缓。一半接受听力检查的儿童表现出双耳残障,但只有一名患者如此严重以至于需要扩大。没有增长异常的趋势,只有2个孩子可以被牢牢地归为教育程度低于正常的孩子。

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