PMID: 6535998Jan 1, 1984Paper

The renal lesion of congenital hepatic fibrosis: pathologic and morphometric analysis, with comparison to the renal lesion of infantile polycystic disease

Pediatric Pathology
L HelczynskiA I Lipsey

Abstract

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.

References

Apr 1, 1978·Annals of Internal Medicine·J L DupondR LeConte-des-Floris
Sep 1, 1971·Journal of Medical Genetics·H Blyth, B G Ockenden
Mar 1, 1967·American Journal of Clinical Pathology·T R Wells
Mar 1, 1982·Clinical Pediatrics·A FiorilloR Vecchione

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Citations

Jan 1, 1990·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·J M Kissane
Jan 1, 1989·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·B S KaplanT M Barratt

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