Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Symptoms of gastrointestinal disease are variable in Langerhans' cell histiocytosis (LCH). The incidence of gastrointestinal involvement is estimated to be approximately 5% in disseminated LCH. This report focuses on a 10.5 months old female infant who suffered from relapsing diarrhea and intermittent anal blood loss since the second week of life and from weight loss later in the course of disease. Definite diagnosis of LCH was not established before the patient developed additional characteristic symptoms. Analysis of large series of patients and a retrospective histopathological study reveals that an involvement of the gastrointestinal tract must be anticipated in about 50% of all cases with disseminated LCH.
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PMID:[Gastrointestinal symptoms in disseminated Langerhans cell histiocytosis. Case report and review of the literature]. 207 67

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.
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PMID:Protein-losing enteropathy caused by gastrointestinal tract-involved Langerhans cell histiocytosis. 2008 19