The emergence of hepatic fibrosis and portal hypertension in infants and children with autosomal recessive polycystic kidney disease. Initial and follow-up sonographic and radiographic findings.

Abstract:

:Long-term imaging and clinical findings are reported in six children whose polycystic kidney disease was detected in infancy or early childhood. Over time (2 years to 20 years) all patients developed portal hypertension from hepatic fibrosis, a problem primarily noted in recessive pattern polycystic kidney disease. Mild renal failure (two patients) was accompanied by serious systemic hypertension in the same patients. In one family, one of the babies also showed dilated right hepatic ducts. Imaging studies included urography and CT although recently ultrasonography was the method of choice. The relative renal and hepatic manifestations in these patients so changed with time that it would seem fallacious to attempt to use rigid classifications based on findings at initial diagnosis.

journal_name

Pediatr Radiol

journal_title

Pediatric radiology

authors

Premkumar A,Berdon WE,Levy J,Amodio J,Abramson SJ,Newhouse JH

doi

10.1007/BF02387555

subject

Has Abstract

pub_date

1988-01-01 00:00:00

pages

123-9

issue

2

eissn

0301-0449

issn

1432-1998

journal_volume

18

pub_type

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