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BIBTEX RIS

Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy

1999; Associação Brasileira de Divulgação Científica; Volume: 32; Issue: 9 Linguagem: Inglês

10.1590/s0100-879x1999000900007

1414-431X

Telma M. Santos-Machado, Lílian Maria Cristófani, Maria T. A. Almeida, Paulo T. Maluf, Paulo Aguirre Costa, Marco Aurélio Amador Pereira, J. L. B. C. Brito, Vicente Odone Filho,

Tumors and Oncological Cases

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% 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%), protein-losing enteropathy (33.3%) 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.