Document Detail

Non-syndromic association of congenital hepatic fibrosis and bilateral cystic renal dysplasia.
MedLine Citation:
PMID:  11155779     Owner:  NLM     Status:  MEDLINE    
Congenital hepatic fibrosis (CHF) is associated with autosomal recessive polycystic kidney disease (ARPKD). Although cystic renal dysplasia (CRD) is the most common form of newborn cystic renal disease, this disorder of anomalous metanephric differentiation is only rarely found concurrent with CHF. Our literature review found only 13 sporadic and 12 familial non-syndromic cases of combined bilateral CRD and CHF reported outside Taiwan. We report the first domestic case, occurring in a fetus of 18 weeks' gestational age, which was the second pregnancy of a 24-year-old mother with a previous history of spontaneous abortion at 10 weeks' gestational age. Postmortem autopsy confirmed the concurrence of bilateral CRD and CHF without associated anomalies of other visceral organs and external appearance. This particular association must be differentiated from ARPKD and liver disease, in regard to ultrasonographic examination and genetic counseling.
H Y Huang; H Y Huang; W J Chen
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of the Formosan Medical Association = Taiwan yi zhi     Volume:  99     ISSN:  0929-6646     ISO Abbreviation:  J. Formos. Med. Assoc.     Publication Date:  2000 Nov 
Date Detail:
Created Date:  2001-01-11     Completed Date:  2001-01-25     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9214933     Medline TA:  J Formos Med Assoc     Country:  China (Republic: 1949- )    
Other Details:
Languages:  eng     Pagination:  863-5     Citation Subset:  IM    
Department of Pathology, Chang Gung Memorial Hospital, 123 Ta-Pei Road, Niao-Sung, Kaohsiung, Taiwan.
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MeSH Terms
Fetal Diseases / pathology*
Kidney / pathology
Liver Cirrhosis / complications*,  congenital
Polycystic Kidney Diseases / complications*,  pathology

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