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Provedor de dados:  BJMBR
País:  Brazil
Título:  Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy
Autores:  Santos-Machado,T.M.
Cristófani,L.M.
Almeida,M.T.A.
Maluf,P.T.
Costa,P.A.
Pereira,M.A.
Brito,J.L.B.C.
Odone-Filho,V.
Data:  1999-09-01
Ano:  1999
Palavras-chave:  Langerhans' cell histiocytosis
Histiocytosis X
Gastrointestinal involvement
Protein-losing enteropathy
Hypoalbuminemia
Cr51-labeled albumin test
Resumo:  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.
Tipo:  Info:eu-repo/semantics/article
Idioma:  Inglês
Identificador:  http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X1999000900007
Editor:  Associação Brasileira de Divulgação Científica
Relação:  10.1590/S0100-879X1999000900007
Formato:  text/html
Fonte:  Brazilian Journal of Medical and Biological Research v.32 n.9 1999
Direitos:  info:eu-repo/semantics/openAccess
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