Follow‐up in patients with aspartylglucosaminuria. Part I. The course of intellectual functions
作者:
M Arvio,
期刊:
Acta Pædiatrica
(WILEY Available online 1993)
卷期:
Volume 82,
issue 5
页码: 469-471
ISSN:0803-5253
年代: 1993
DOI:10.1111/j.1651-2227.1993.tb12725.x
出版商: Blackwell Publishing Ltd
关键词: Aspartylglucosaminuria;developmental profile;intellectual functions;lysosomal storage disorder;mental retardation;Portage;progressive syndrome
数据来源: WILEY
摘要:
Progressive mental retardation is the main clinical symptom in aspartylglucosaminuria. The intellectual functions of 116 patients were studied. Based on cross‐sectional test results, an average longitudinal developmental profile consisted of three parts: a period of abnormally slow but positive development in childhood, a gradual loss of reached skills after the age of 13–16 years and a rapid decline after the age of 25–28 years. Only nine young patients had a developmental age higher than six years, and two higher than seven years. The mean age of the female group was seven years higher than that of the males, but the mean developmental ages were the same for both g
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