Related Experiment Video

Updated: Sep 15, 2026

Evaluation of Zebrafish Kidney Function Using a Fluorescent Clearance Assay
08:13

Evaluation of Zebrafish Kidney Function Using a Fluorescent Clearance Assay

Published on: February 20, 2015

Infantile polycystic disease of the liver and kidneys

D L Gang, J T Herrin

    Clinical Nephrology
    |January 1, 1986
    PubMed

    Insights

    Infantile polycystic kidney disease (IPKD) shows varied severity. Early and extensive kidney cysts indicate poor survival, while less severe cases allow long-term survival, highlighting the need for individual patient evaluation.

    Area of Science:

    • Pediatric Nephrology
    • Medical Genetics
    • Hepatology

    Background:

    • Recessively inherited infantile polycystic disease (IPCD) presents a significant challenge in pediatric healthcare.
    • Understanding the phenotypic spectrum is crucial for accurate diagnosis and prognosis.

    Purpose of the Study:

    • To document renal tubular cyst formation and hepatic fibrosis in infantile polycystic disease (IPCD).
    • To assess survival rates in relation to disease severity.
    • To evaluate the utility of current IPCD classification systems.

    Main Methods:

    • Histopathological examination of kidney and liver tissue from eleven IPCD patients.
    • Correlation of pathological findings with clinical outcomes and survival data.

    Main Results:

    • Infants with >90% cystic renal change did not survive past 20 days.
    • Patients with 20-75% cystic renal change showed survival rates of 6-21 years.
    • All patients exhibited hamartomatous bile duct proliferation; portal fibrosis was most severe in the oldest patient.
    • Sibling cases displayed dissimilar clinical courses and pathological expressions of renal disease.

    Conclusions:

    • Infantile polycystic disease (IPCD) exhibits a spectrum of phenotypic expression.
    • Tissue diagnosis in individual patients has prognostic implications.
    • Current classifications may not adequately represent the diverse genetic and clinical presentations of IPCD.
    • Each child with IPCD requires separate evaluation due to variable disease progression.

    More Related Videos

    Use of Ultra-high Field MRI in Small Rodent Models of Polycystic Kidney Disease for In Vivo Phenotyping and Drug Monitoring07:35

    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

    Implementing Patch Clamp and Live Fluorescence Microscopy to Monitor Functional Properties of Freshly Isolated PKD Epithelium08:46

    Implementing Patch Clamp and Live Fluorescence Microscopy to Monitor Functional Properties of Freshly Isolated PKD Epithelium

    Published on: September 1, 2015

    Related Concept Videos

    Cystic Fibrosis: Pathogenesis01:23

    Cystic Fibrosis: Pathogenesis

    Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
    CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation, but...
    Diseases of the Liver and Gallbladder01:26

    Diseases of the Liver and Gallbladder

    Liver and gallbladder diseases are a significant health concern, with prominent conditions including cirrhosis, hepatitis, non-alcoholic fatty liver disease (NAFLD), and gallstones. Jaundice is a common manifestation of liver and biliary disease.
    Cirrhosis is characterized by the scarring of hepatic lobules in the liver, which are replaced by fibrous tissue, affecting the liver's normal functioning. NAFLD, on the other hand, is caused by an excessive build-up of fat in the liver, not related to...
    Nephrons01:10

    Nephrons

    The kidneys are intricate organs with millions of working units known as nephrons. Each nephron features two major structures: the renal corpuscle, which facilitates blood plasma filtration, and the renal tubule, which handles the glomerular filtrate. Blood supply is directly linked to the nephrons. The renal corpuscle consists of the glomerulus, a capillary network, and the Bowman's capsule, a double-walled epithelial structure that encases the glomerulus. The filtering of blood plasma happens...
    Renal Corpuscle01:20

    Renal Corpuscle

    The glomerulus and Bowman's capsule are two essential components of the nephron, which is the functional unit of the kidney. These microscopic structures play a critical role in the process of blood filtration to produce urine.
    Glomerulus: Structure and Function
    The glomerulus is a tiny, intricate network of capillaries located at the beginning of the nephron. It's enveloped by the Bowman's capsule and receives its blood supply from an afferent arteriole, which divides into numerous capillaries...
    Pharmacokinetics in Pediatric Patients: Drug Metabolism01:24

    Pharmacokinetics in Pediatric Patients: Drug Metabolism

    In pediatric care, understanding the nuances of hepatic drug metabolism is crucial, as it significantly differs from that of adults. This divergence is primarily due to the developmental stage of drug-metabolizing enzymes, which affects how medications are processed in the body. In neonates, for instance, the activity of Phase I enzymes—critical for the initial breakdown of drugs—is markedly reduced, functioning at just 20–40% of the levels seen in adults. This reduction poses a challenge in...
    Nephrotic Syndrome I : Introduction01:24

    Nephrotic Syndrome I : Introduction

    Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of fluid...