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Updated: May 8, 2026

Use of Ultra-high Field MRI in Small Rodent Models of Polycystic Kidney Disease for In Vivo Phenotyping and Drug Monitoring
07:35

Use of Ultra-high Field MRI in Small Rodent Models of Polycystic Kidney Disease for In Vivo Phenotyping and Drug Monitoring

Published on: June 23, 2015

[Infantile polycystic kidney disease: a case report and literature review]

Fang Luo1, Wei-Zhong Gu, Zheng Chen

  • 1Intensive Care Unit, The Children's Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China.

Zhonghua Er Ke Za Zhi = Chinese Journal of Pediatrics
|August 15, 2013
PubMed

Insights

Perinatal autosomal recessive polycystic kidney disease in infants often leads to severe respiratory and renal failure. Early aggressive respiratory support and renal replacement therapy can improve outcomes for affected newborns.

Area of Science:

  • Pediatric Nephrology
  • Medical Genetics
  • Neonatology

Background:

  • Autosomal recessive polycystic kidney disease (ARPKD) is a severe genetic disorder affecting newborns.
  • Perinatal ARPKD presents significant diagnostic and management challenges.

Observation:

  • Clinical data from 12 perinatal ARPKD cases were analyzed.
  • Infants exhibited enlarged kidneys, severe respiratory distress, and renal compromise.
  • Prenatal ultrasound often detected enlarged kidneys and oligohydramnios.

Findings:

  • Histological analysis revealed pulmonary hypoplasia and dilated renal and intrahepatic bile ducts.
  • Neonatal mortality was high due to respiratory and renal failure.
  • Survivors required renal replacement therapy, including dialysis, nephrectomy, or transplantation.

Implications:

  • Aggressive respiratory support is crucial for immediate survival.
  • Renal replacement therapies offer a potential pathway to improved long-term outcomes.
  • Early diagnosis and intervention are critical for managing perinatal ARPKD.
Abstract

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