Document Detail

The renal lesion of congenital hepatic fibrosis: pathologic and morphometric analysis, with comparison to the renal lesion of infantile polycystic disease.
MedLine Citation:
PMID:  6535998     Owner:  NLM     Status:  MEDLINE    
The renal lesion of congenital hepatic fibrosis (CHF = Blyth and Ockenden's juvenile polycystic disease of liver and kidneys) was analyzed from 6 specimens from patients aged 3 3/12 to 19 3/12 years and compared with that of 5 patients with infantile polycystic disease (IPCD) aged 6 months to 14 4/12 years. Pathologic, microdissection, injection, and morphometric studies show that the predominantly medullary cystic lesion of CHF shows different distribution in medullary, cortico-medullary, and cortical zones of kidney from the lesion of IPCD, and shows a different time course, from early life to renal insufficiency, from that of IPCD. The renal cysts in CHF affect deep or central collecting tubules, in contrast to the involvement of more peripheral orders of collecting tubules in IPCD. Papillary pore counts, performed for 1 patient, gave significantly low values, in contrast to normal values reported for IPCD. The findings support the previously published conclusion, based on differences in the hepatic lesions of the two conditions, that CHF and IPCD are difference diseases, rather than different permissible manifestations of a single disease.
L Helczynski; T R Wells; B H Landing; A I Lipsey
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Pediatric pathology / affiliated with the International Paediatric Pathology Association     Volume:  2     ISSN:  0277-0938     ISO Abbreviation:  Pediatr Pathol     Publication Date:  1984  
Date Detail:
Created Date:  1985-08-22     Completed Date:  1985-08-22     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  8303527     Medline TA:  Pediatr Pathol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  441-55     Citation Subset:  IM    
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MeSH Terms
Child, Preschool
Kidney Diseases / etiology*,  pathology
Liver Diseases / complications,  congenital*,  pathology
Polycystic Kidney Diseases / pathology

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