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Protein-losing enteropathy caused by gastrointestinal tract-involved Langerhans cell histiocytosis.

Abstract
Protein-losing enteropathy (PLE) is frequently complicated in patients with gastrointestinal tract-involved Langerhans cell histiocytosis (LCH); however, LCH per se is not generally included in the list of diseases that cause PLE. We report here a case of infantile PLE that presented with continuous diarrhea at the onset of LCH. She was initially diagnosed as having allergic gastroenteropathy and, thus, received intravenous prednisolone, which was thought to have induced immunodeficiency and consequently resulted in life-threatening cytomegalovirus-associated hemophagocytic syndrome and disseminated intravascular coagulation. Because chemotherapy for hemophagocytic syndrome was transiently effective for underlying LCH as well, the diagnosis of LCH was delayed until its recurrence. Gastrointestinal tract-involved LCH, a rare but highly fatal disease, should be considered for infants with refractory gastrointestinal symptoms, especially for those with PLE; endoscopic biopsy is strongly recommended for immediate diagnosis.
AuthorsHaruko Shima, Takao Takahashi, Hiroyuki Shimada
JournalPediatrics (Pediatrics) Vol. 125 Issue 2 Pg. e426-32 (Feb 2010) ISSN: 1098-4275 [Electronic] United States
PMID20083519 (Publication Type: Case Reports, Journal Article, Review)
Chemical References
  • Glucocorticoids
  • Prednisolone
Topics
  • Colonoscopy
  • Disseminated Intravascular Coagulation (chemically induced)
  • Female
  • Gastrointestinal Diseases (complications, diagnosis, drug therapy)
  • Glucocorticoids (adverse effects)
  • Histiocytosis, Langerhans-Cell (complications, diagnosis, drug therapy)
  • Humans
  • Infant
  • Prednisolone (adverse effects)
  • Protein-Losing Enteropathies (etiology)

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