Document Detail


Congenital generalized lipodystrophy, a case report.
MedLine Citation:
PMID:  8629139     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Generalized lipodystrophy is a rare condition which can be divided into congenital and acquired types, based on the age at presentation and pattern of inheritance. The congenital type of generalized lipodystrophy or Lawrence-Seip syndrome presents in first two years of life and is inherited in an autosomal recessive pattern. The diagnosis is made on the basis of loss of body fat, muscular hypertrophy, acanthosis nigricans, hirsutism, hepatomegaly with fatty liver, hyperlipidemia and hyperglycemia with insulin resistance. A 2 1/2-year-old Thai girl with the clinical features of Lawrence-Seip syndrome is reported. Abnormal platelet function was detected in this girl.
Authors:
S Wacharasindhu; K Veerakul; S Likitmaskul; L Punnakanta; K Angsusingha; C Tuchinda; P Wasant
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The Southeast Asian journal of tropical medicine and public health     Volume:  26 Suppl 1     ISSN:  0125-1562     ISO Abbreviation:  Southeast Asian J. Trop. Med. Public Health     Publication Date:  1995  
Date Detail:
Created Date:  1996-06-26     Completed Date:  1996-06-26     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0266303     Medline TA:  Southeast Asian J Trop Med Public Health     Country:  THAILAND    
Other Details:
Languages:  eng     Pagination:  44-6     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, Siriraj Hospital, Mahidol University, Bangkok, Thailand.
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MeSH Terms
Descriptor/Qualifier:
Blood Platelets / physiology
Child, Preschool
Fatty Liver / pathology
Female
Genes, Recessive
Glucagon / blood
Humans
Lipodystrophy / genetics*,  pathology,  physiopathology
Liver / pathology
Muscle, Skeletal / pathology
Nuclear Family
Platelet Aggregation
Reference Values
Syndrome
Chemical
Reg. No./Substance:
9007-92-5/Glucagon

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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