CONGENITAL FIBER TYPE DISPROPORTION MYOPATHY - HISTOLOGICAL DIAGNOSIS WITH AN UNCERTAIN CLINICAL OUTLOOK

被引:30
作者
CAVANAGH, NPC
LAKE, BD
MCMENIMAN, P
机构
[1] HOSP SICK CHILDREN, DEPT HISTOPATHOL, LONDON WC1N 3JH, ENGLAND
[2] HOSP SICK CHILDREN, DEPT ORTHOPAED, LONDON WC1N 3JH, ENGLAND
[3] INST CHILD HLTH, LONDON WC1N 1EH, ENGLAND
关键词
D O I
10.1136/adc.54.10.735
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Nine children with congenital fibre type disproportion (CFTD) are described. Their muscle biopsies contained type 1 fibres which were smaller than the largest type 2 fibres by at least 13.5%. Attention is drawn to the variable natural history of this disorder which generally carries a good prognosis but may sometimes be associated with fatal respiratory problems. For important therapeutic, genetic, and prognostic reasons CFTD must be distinguished from other conditions with similar histochemieal or clinical features.
引用
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页码:735 / 743
页数:9
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