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An Unusual Case of Hypoproteinemia in Childhood: Keep in Mind Trichobezoar

Mariangela Stinco1, Alessandra Montemaggi1, Bruno Noccioli2

  • 1Pediatric Section, Department of Health Sciences, Meyer Children's University Hospital, University of Florence, Florence, Italy.

Frontiers in Pediatrics
|March 21, 2020
PubMed

Insights

Protein-losing enteropathy (PLE), a rare condition, was caused by Rapunzel Syndrome (RS) in a young girl. Surgical removal of the trichobezoar resolved her symptoms and normalized protein levels.

Area of Science:

  • Gastroenterology
  • Pediatric Surgery
  • Medical Case Reports

Background:

  • Protein-losing enteropathy (PLE) is characterized by excessive gastrointestinal protein loss, leading to hypoproteinemia and potential complications like edema and effusions.
  • Rapunzel Syndrome (RS), a rare condition involving a gastric trichobezoar extending into the small intestine, can cause gastrointestinal obstruction and malabsorption.

Observation:

  • A young girl with a history of jejunal atresia and intestinal malrotation presented with generalized edema, hypoproteinemia, and microcytic anemia.
  • Endoscopic examination revealed a large trichobezoar (Rapunzel Syndrome) obstructing the small intestine and impairing lymphatic drainage, leading to PLE.

Findings:

  • Surgical laparotomy successfully removed the trichobezoar, resulting in complete resolution of PLE symptoms and normalization of serum protein levels.
  • The case highlights the rare association between Rapunzel Syndrome and protein-losing enteropathy, likely exacerbated by prior intestinal surgery influencing motility.

Implications:

  • Rapunzel Syndrome should be considered in the differential diagnosis of pediatric patients presenting with unexplained hypoproteinemia and gastrointestinal symptoms, especially those with behavioral disorders like trichophagia.
  • Prompt surgical intervention for large trichobezoars causing intestinal obstruction is crucial for managing associated conditions like PLE and ensuring favorable patient outcomes.

Keywords:
Rapunzel Syndromehypoproteinemiaoedemaprotein-losing enteropathytrichobezoar

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