[Clinical, biochemical and ultrasonic changes in the liver in small intestine enzymopathy in children]

Insights

Children with small intestine enzymopathy variations (ESI) show distinct liver and hepatobiliary disorders. Fat hepatosis was more frequent in celiac disease than celiac syndrome, informing pediatric therapy.

Area of Science:

  • Pediatric Gastroenterology
  • Hepatology
  • Clinical Nutrition

Background:

  • Small intestine enzymopathy variations (ESI) encompass primary celiac enzymopathy (CE), celiac syndrome, and disaccharidase insufficiency syndrome (DIS).
  • Children with ESI often present with overlapping clinical signs, complicating diagnosis and management.
  • Liver and hepatobiliary system involvement can occur in pediatric ESI.

Purpose of the Study:

  • To investigate and compare liver and hepatobiliary system disorders in children with different types of ESI.
  • To identify specific ultrasound findings differentiating celiac disease from celiac syndrome.
  • To inform rational diet and drug therapy for pediatric ESI.

Main Methods:

  • Prospective follow-up of 70 pediatric patients (aged 1-11 years).
  • Categorization into primary celiac enzymopathy (CE, n=30), celiac syndrome (n=20), and disaccharidase insufficiency syndrome (DIS, n=30).
  • Clinical sign assessment and ultrasound examination for liver and hepatobiliary evaluation, specifically noting fat hepatosis.

Main Results:

  • Distinct liver and hepatobiliary disorders were observed in children across the ESI spectrum.
  • Fat hepatosis was identified more frequently in children with celiac disease compared to those with celiac syndrome.
  • While clinical signs were generally similar, ultrasound findings showed differences in the prevalence of fat hepatosis.

Conclusions:

  • Children with ESI exhibit varied liver and hepatobiliary system pathologies.
  • Ultrasound detection of fat hepatosis can help differentiate celiac disease from celiac syndrome in pediatric patients.
  • These findings support tailored diet and drug therapy approaches for children diagnosed with ESI.

Related Concept Videos

Inborn Errors of Metabolism01:20

Inborn Errors of Metabolism

Phenylketonuria (PKU) is a protein metabolism disorder characterized by high blood levels of the amino acid phenylalanine. This results from a mutation in the gene responsible for phenylalanine hydroxylase, an enzyme that converts phenylalanine into tyrosine. When this enzyme is deficient, phenylalanine builds up in the blood, leading to symptoms such as vomiting, rashes, seizures, growth deficiency, and severe mental retardation. An early diagnosis and a diet restricting phenylalanine intake...
Ultrasound II: Endoscopic Ultrasound and FibroScan01:25

Ultrasound II: Endoscopic Ultrasound and FibroScan

Endoscopic Ultrasound (EUS) and FibroScan are valuable diagnostic tools in gastroenterology and hepatology, each with specific applications and techniques.
Endoscopic Ultrasound (EUS):
Pharmacokinetics in Pediatric Patients: Overview and Drug Absorption01:23

Pharmacokinetics in Pediatric Patients: Overview and Drug Absorption

Understanding the physiological differences in the pediatric population is crucial for effective pharmacotherapy. Neonates, infants, and children exhibit significant variations in gastric pH, gastric emptying time, intestinal transit time, and biliary function. These variations profoundly affect oral drug absorption, necessitating a nuanced approach to pediatric dosing.Neonates present with a unique physiological profile, having a gastric pH greater than 4 and faster and more irregular gastric...
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...
Effect of Hepatic Disease on Pharmacokinetics: Drug Dosing and Hepatic Blood Flow01:26

Effect of Hepatic Disease on Pharmacokinetics: Drug Dosing and Hepatic Blood Flow

Chronic liver disease significantly impacts drug metabolism due to alterations in hepatic blood flow and enzyme accessibility. This disruption affects the body's pharmacokinetics—the movement and processing of drugs within the system. Key enzymes crucial for metabolizing medications become less accessible, changing how drugs are processed and utilized. Furthermore, liver disease influences the synthesis of plasma proteins, such as albumin and globulins, which play critical roles in drug binding...
Effect of Hepatic Disease on Pharmacokinetics: Pathophysiologic Assessment and Liver Function Test01:22

Effect of Hepatic Disease on Pharmacokinetics: Pathophysiologic Assessment and Liver Function Test

In clinical practice, the direct measurement of hepatic blood flow to evaluate liver function presents significant challenges due to the intricate and specialized nature of the necessary techniques. Consequently, healthcare professionals often rely on empirical estimates derived from thorough patient examinations and liver function tests to gauge liver health. Among the tools at their disposal, the Child–Pugh and MELD scoring systems stand out for their ability to categorize and assess the...