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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

Journal Article:

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.
Publication Date:
Feb 01, 1988
Product Type:
Journal Article
Reference Number:
DEN-88-002872; EDB-88-068517
Resource Relation:
Journal Name: Pediatr. Radiol.; (Germany, Federal Republic of); Journal Volume: 18:2
Subject:
62 RADIOLOGY AND NUCLEAR MEDICINE; ABDOMEN; BIOMEDICAL RADIOGRAPHY; COMPUTERIZED TOMOGRAPHY; CHILDREN; DIGESTIVE SYSTEM DISEASES; UROGENITAL SYSTEM DISEASES; DIAGNOSIS; FIBROSIS; HEREDITARY DISEASES; HYPERTENSION; INFANTS; KIDNEYS; LIVER; ULTRASONOGRAPHY; X-RAY RADIOGRAPHY; AGE GROUPS; BODY; BODY AREAS; CARDIOVASCULAR DISEASES; DIAGNOSTIC TECHNIQUES; DIGESTIVE SYSTEM; DISEASES; GLANDS; INDUSTRIAL RADIOGRAPHY; MEDICINE; NUCLEAR MEDICINE; ORGANS; PATHOLOGICAL CHANGES; RADIOLOGY; SYMPTOMS; TOMOGRAPHY; VASCULAR DISEASES; 550602* - Medicine- External Radiation in Diagnostics- (1980-)
OSTI ID:
5480683
Research Organizations:
Columbia-Presbyterian Medical Center, New York (USA). Dept. of Radiology; Columbia-Presbyterian Medical Center, New York (USA). Dept. of Pediatrics
Country of Origin:
Germany
Language:
English
Other Identifying Numbers:
Journal ID: CODEN: PDRYA
Submitting Site:
DEN
Size:
Pages: 123-129
Announcement Date:
Apr 01, 1988

Journal Article:

Citation Formats

Premkumar, A, Berdon, W E, Abramson, S J, Newhouse, J H, and Levy, J. 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. Germany: N. p., 1988. Web.
Premkumar, A, Berdon, W E, Abramson, S J, Newhouse, J H, & Levy, J. 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. Germany.
Premkumar, A, Berdon, W E, Abramson, S J, Newhouse, J H, and Levy, J. 1988. "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." Germany.
@misc{etde_5480683,
title = {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}
author = {Premkumar, A, Berdon, W E, Abramson, S J, Newhouse, J H, and Levy, J}
abstractNote = {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 = {Pediatr. Radiol.; (Germany, Federal Republic of)}
volume = {18:2}
journal type = {AC}
place = {Germany}
year = {1988}
month = {Feb}
}