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15岁之后的普拉德-威利综合征。

Prader-Willi Syndrome after age 15 years.

作者信息

Laurance B M, Brito A, Wilkinson J

出版信息

Arch Dis Child. 1981 Mar;56(3):181-6. doi: 10.1136/adc.56.3.181.

Abstract

Twenty-four patients, all of them over 15 years, with the Prader-Willi syndrome are described. Obesity, often extreme, associated with an insatiable appetite, was their principal handicap and this was made worse by educational subnormality and hypogonadism. Three of the them developed diabetes. Each attended a special school or an adult training centre. Although most of them were of short stature and had scoliosis, 2 were tall but they even more severely mentally retarded than is usually the case. Nine other patients died aged between 3 and 23 years. The most common cause of death was cor pulmonale.

摘要

本文描述了24例年龄均超过15岁的普拉德-威利综合征患者。肥胖(常极为严重)伴有无休止的食欲是他们的主要障碍,而教育水平低下和性腺功能减退使情况更加恶化。其中3例患了糖尿病。每位患者都就读于特殊学校或成人培训中心。虽然他们大多数身材矮小且患有脊柱侧弯,但有2例身材高大,不过其智力发育迟缓程度比通常情况更为严重。另外9例患者在3至23岁之间死亡。最常见的死因是肺心病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f54b/1627148/49cdd24274f7/archdisch00771-0029-a.jpg

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