Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy
Autor(a) principal: | |
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Data de Publicação: | 1999 |
Outros Autores: | , , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Brazilian Journal of Medical and Biological Research |
Texto Completo: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X1999000900007 |
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. |
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Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathyLangerhans' cell histiocytosishistiocytosis Xgastrointestinal involvementprotein-losing enteropathyhypoalbuminemiaCr51-labeled albumin testSymptomatic 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.Associação Brasileira de Divulgação Científica1999-09-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X1999000900007Brazilian Journal of Medical and Biological Research v.32 n.9 1999reponame:Brazilian Journal of Medical and Biological Researchinstname:Associação Brasileira de Divulgação Científica (ABDC)instacron:ABDC10.1590/S0100-879X1999000900007info:eu-repo/semantics/openAccessSantos-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.eng1999-08-27T00:00:00Zoai:scielo:S0100-879X1999000900007Revistahttps://www.bjournal.org/https://old.scielo.br/oai/scielo-oai.phpbjournal@terra.com.br||bjournal@terra.com.br1414-431X0100-879Xopendoar:1999-08-27T00:00Brazilian Journal of Medical and Biological Research - Associação Brasileira de Divulgação Científica (ABDC)false |
dc.title.none.fl_str_mv |
Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy |
title |
Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy |
spellingShingle |
Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy Santos-Machado,T.M. Langerhans' cell histiocytosis histiocytosis X gastrointestinal involvement protein-losing enteropathy hypoalbuminemia Cr51-labeled albumin test |
title_short |
Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy |
title_full |
Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy |
title_fullStr |
Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy |
title_full_unstemmed |
Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy |
title_sort |
Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy |
author |
Santos-Machado,T.M. |
author_facet |
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. |
author_role |
author |
author2 |
Cristófani,L.M. Almeida,M.T.A. Maluf,P.T. Costa,P.A. Pereira,M.A. Brito,J.L.B.C. Odone-Filho,V. |
author2_role |
author author author author author author author |
dc.contributor.author.fl_str_mv |
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. |
dc.subject.por.fl_str_mv |
Langerhans' cell histiocytosis histiocytosis X gastrointestinal involvement protein-losing enteropathy hypoalbuminemia Cr51-labeled albumin test |
topic |
Langerhans' cell histiocytosis histiocytosis X gastrointestinal involvement protein-losing enteropathy hypoalbuminemia Cr51-labeled albumin test |
description |
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. |
publishDate |
1999 |
dc.date.none.fl_str_mv |
1999-09-01 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X1999000900007 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X1999000900007 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.1590/S0100-879X1999000900007 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
text/html |
dc.publisher.none.fl_str_mv |
Associação Brasileira de Divulgação Científica |
publisher.none.fl_str_mv |
Associação Brasileira de Divulgação Científica |
dc.source.none.fl_str_mv |
Brazilian Journal of Medical and Biological Research v.32 n.9 1999 reponame:Brazilian Journal of Medical and Biological Research instname:Associação Brasileira de Divulgação Científica (ABDC) instacron:ABDC |
instname_str |
Associação Brasileira de Divulgação Científica (ABDC) |
instacron_str |
ABDC |
institution |
ABDC |
reponame_str |
Brazilian Journal of Medical and Biological Research |
collection |
Brazilian Journal of Medical and Biological Research |
repository.name.fl_str_mv |
Brazilian Journal of Medical and Biological Research - Associação Brasileira de Divulgação Científica (ABDC) |
repository.mail.fl_str_mv |
bjournal@terra.com.br||bjournal@terra.com.br |
_version_ |
1754302930053234688 |