Document Detail


Protein-losing enteropathy caused by gastrointestinal tract-involved Langerhans cell histiocytosis.
MedLine Citation:
PMID:  20083519     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Haruko Shima; Takao Takahashi; Hiroyuki Shimada
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Publication Detail:
Type:  Case Reports; Journal Article; Review     Date:  2010-01-18
Journal Detail:
Title:  Pediatrics     Volume:  125     ISSN:  1098-4275     ISO Abbreviation:  Pediatrics     Publication Date:  2010 Feb 
Date Detail:
Created Date:  2010-02-03     Completed Date:  2010-03-04     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0376422     Medline TA:  Pediatrics     Country:  United States    
Other Details:
Languages:  eng     Pagination:  e426-32     Citation Subset:  AIM; IM    
Affiliation:
Keio University School of Medicine, Department of Pediatrics, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan.
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MeSH Terms
Descriptor/Qualifier:
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*
Chemical
Reg. No./Substance:
0/Glucocorticoids; 50-24-8/Prednisolone

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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