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Braz. j. med. biol. res ; 32(9): 1095-9, Sept. 1999. tab
Article in English | LILACS | ID: lil-241603

ABSTRACT

Symptomatic involvement of the gastrointestinal (GI) tract as a prominent symptom in Langerhans' cell histiocytosis (LCH) is uncommon, occurring in less than 1 to 5 percent of all cases, even when the disease is in its disseminated form. Up to now, there have been reports of 18 cases of LCH with GI manifestations, including our 2 cases, with diarrhea (77.7 percent), protein-losing enteropathy (33.3 percent) and bloody stool being the most frequent findings. The authors present two patients with severe diarrhea and refractory hypoalbuminemia, and with the protein-losing enteropathy documented by Cr51-labeled albumin studies. A review of the literature indicated that the presence of GI symptoms is often associated with systemic disease as well as with poor prognosis, mainly under 2 years of age. Radioisotopes are useful for documenting protein loss in several diseases with high specificity and sensitivity, and their utilization in the cases reviewed here permitted diagnoses in 6 children, as well as improved therapeutic management


Subject(s)
Female , Humans , Child, Preschool , Digestive System/pathology , Histiocytosis, Langerhans-Cell/pathology , Protein-Losing Enteropathies/pathology , Biopsy , Fatal Outcome , Hypoaldosteronism/complications , Protein-Losing Enteropathies/diagnosis
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