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Related Experiment Videos

Intussusception nephrosis and Drash syndrome.

P A Crawshaw1, A R Watson, C H Rance

  • 1Department of Paediatric Medicine, City Hospital, Nottingham, United Kingdom.

European Journal of Pediatrics
|September 1, 1991
PubMed
Summary

A rare case of intussusception in an infant was linked to nephrotic syndrome. This association, potentially caused by an oedematous bowel wall, has not been previously documented.

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Area of Science:

  • Pediatric Nephrology
  • Gastroenterology
  • Genetics

Background:

  • Intussusception is a common surgical emergency in infants.
  • Nephrotic syndrome typically presents with edema, proteinuria, and hypoalbuminemia.
  • Drash syndrome is a rare genetic disorder characterized by nephropathy, male pseudohermaphroditism, and Wilms' tumor.

Observation:

  • A 6-month-old infant presented with intussusception.
  • Post-operatively, the infant was diagnosed with nephrotic syndrome.
  • The infant's karyotype was XY, indicating a potential association with Drash syndrome.

Findings:

  • The oedematous bowel wall in this infant may have been the cause of intussusception, a novel observation.
  • The co-occurrence of intussusception and nephrotic syndrome in this XY infant suggests a possible link to Drash syndrome.

Implications:

  • This case highlights a previously undescribed potential cause of intussusception in infants with nephrotic syndrome.
  • Further research is warranted to explore the relationship between bowel wall edema, nephrotic syndrome, and intussusception.
  • Early recognition and management of nephrotic syndrome may be crucial in preventing gastrointestinal complications like intussusception in susceptible infants.

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