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

研究成果: Article査読

22 被引用数 (Scopus)

抄録

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 lifethreatening 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.

本文言語English
ページ(範囲)e426-e432
ジャーナルPediatrics
125
2
DOI
出版ステータスPublished - 2010 2 1

ASJC Scopus subject areas

  • 小児科学、周産期医学および子どもの健康

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