Document Detail

Three autopsied cases of cystic fibrosis in Japan.
MedLine Citation:
PMID:  11422809     Owner:  NLM     Status:  MEDLINE    
The incidence of cystic fibrosis (CF) is very rare in Japanese, while it is frequent in Caucasians. Here we report on three Japanese cases of CF. One of the patients had consanguineous parents. All three patients initially developed meconium ileus, and hepatobiliary and pancreatic changes became more severe as age increased. The DeltaF508 mutation, the most frequent mutation associated with CF in Caucasians, was not found in these patients. To evaluate the relationship between the severity of hepatic lesions and a history of meconium ileus, we examined hepatic lesions in the present three cases, and we reviewed 22 Japanese autopsied cases of CF in the literature. No correlation was found between the incidence of biliary cirrhosis and a history of meconium ileus, because the cases with meconium ileus showed a high mortality during the neonatal period, before biliary cirrhosis developed. The high incidence of meconium ileus in Japanese CF patients may relate to a clinically severe phenotype and reflect a different genetic background between Caucasians and Japanese.
S Iwasa; M Fujiwara; M Nagata; T Watanabe
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Publication Detail:
Type:  Case Reports; Comparative Study; Journal Article    
Journal Detail:
Title:  Pathology international     Volume:  51     ISSN:  1320-5463     ISO Abbreviation:  Pathol. Int.     Publication Date:  2001 Jun 
Date Detail:
Created Date:  2001-06-25     Completed Date:  2001-09-06     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  9431380     Medline TA:  Pathol Int     Country:  Australia    
Other Details:
Languages:  eng     Pagination:  467-72     Citation Subset:  IM    
Department of Pathology, Institutes of Basic Medical Sciences, University of Tsukuba, Tsukuba, Japan.
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MeSH Terms
Child, Preschool
Cystic Fibrosis / complications,  epidemiology,  pathology*
Fatal Outcome
Intestinal Obstruction / complications,  pathology
Japan / epidemiology
Liver / pathology
Liver Cirrhosis, Biliary / complications,  pathology
Pancreas / pathology
United States / epidemiology

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