Thornton L, Dawson K P
PWS Association (NZ), Wellington.
N Z Med J. 1990 Mar 14;103(885):97-8.
The Prader-Willi syndrome consists of infantile hypotonia, failure to thrive, hypogonadism and developmental delay. It was first described in 1956. Later in life hypotonia improves. Between the age of two and four obesity becomes noticeable and between six and ten there is uncontrollable behaviour. A survey has been carried out of the 36 known cases in New Zealand and the pattern of the disease is similar to that elsewhere. In most cases the diagnosis was not established until between the ages of six and 10 when the obesity was marked and uncontrolled behaviour a problem. Early control of the obesity is helpful.
普拉德-威利综合征包括婴儿期肌张力减退、生长发育迟缓、性腺功能减退和发育迟缓。该病于1956年首次被描述。在生命后期,肌张力减退会有所改善。在2至4岁之间,肥胖开始明显,在6至10岁之间会出现无法控制的行为。对新西兰已知的36例病例进行了一项调查,该病的模式与其他地方相似。在大多数情况下,直到6至10岁时肥胖明显且出现行为失控问题时才确诊。早期控制肥胖是有帮助的。