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CONGENITAL GENERALIZED LIPODYSTROPHY:

 

作者: Thorbjörn Berge,   Arne Brun,   Bengt Hansing,   Bengt Kjellman,  

 

期刊: Acta Pathologica Microbiologica Scandinavica Section A Pathology  （WILEY Available online 1976）
卷期: Volume 84A, issue 1  

页码: 47-54

 

ISSN:0365-4184

 

年代: 1976

 

DOI:10.1111/j.1699-0463.1976.tb00109.x

 

出版商: Blackwell Publishing Ltd

 

关键词: Lipodystrophy;congenital;generalized

 

数据来源: WILEY

 

摘要:

Generalized lipodystrophy (Berardinelli‐Seip's syndrome) was diagnosed in a boy at the age of 81/2 months. Ten months later he died because of aspiration of food. Necropsy with special reference to the CNS revealed hypothalamic lesions, probably of a malformative or hamartomatous nature. The findings lend additional support to the view that hypothalamic dysfunction is responsible for the signs and symptoms of generalized lipodystroph

 

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