Cirrose biliar secundária em doente com histiocitose X.

Histiocitose X or Histiocytosis of the Langerhans cells represents a complex spectrum of clinical alterations, resulting from infiltration by anomalous histiocytes of various organs, including the skin, bones, lungs, lymphatic ganglia and liver. Liver disease is rare and the mechanism by which lesio...

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Main Authors: Zita Romão, J Pontes, P Andrade, M C Leitão, A Donato, Diniz Freitas
Format: Article
Language:English
Published: Ordem dos Médicos 2002-02-01
Series:Acta Médica Portuguesa
Online Access:https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1904
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author Zita Romão
J Pontes
P Andrade
M C Leitão
A Donato
Diniz Freitas
author_facet Zita Romão
J Pontes
P Andrade
M C Leitão
A Donato
Diniz Freitas
author_sort Zita Romão
collection DOAJ
description Histiocitose X or Histiocytosis of the Langerhans cells represents a complex spectrum of clinical alterations, resulting from infiltration by anomalous histiocytes of various organs, including the skin, bones, lungs, lymphatic ganglia and liver. Liver disease is rare and the mechanism by which lesions appear is unknown. Cholestasis results from phenomena of sclerosant colangitis, which affects the intrahepatic ducts, or from proliferation of histiocytic cells in the periportal areas. Some patients develop biliar cyrrhosis. The authors present the clinical case of a 62-year-old female patient, hospitalized for chronic cholestasis, diabetes and gallstone in the main bile duct. She had metastatic lesions of the hypophysis and bones, the biopsies of which revealed infiltration by histiocytic cells. Endoscopic Retrograde Colangiopancreatography (E.R.C.P.) revealed dilatation of intrahepatic bile ducts and stenosis of left hepatic duct. A histological study of the hepatic biopsy showed chronic cholestasis and areas of fibrosis, without infiltration by histiocytic granulomas, which were observed in the medular biopsy and in the thyroid nodule cytology.
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spelling doaj.art-0533e0635f30435cbc46276dce5ad71f2022-12-22T04:13:01ZengOrdem dos MédicosActa Médica Portuguesa0870-399X1646-07582002-02-0115110.20344/amp.1904Cirrose biliar secundária em doente com histiocitose X.Zita Romão0J PontesP AndradeM C LeitãoA DonatoDiniz FreitasServiço de Gastrenterologia, Hospitais da Universidade de Coimbra, Coimbra.Histiocitose X or Histiocytosis of the Langerhans cells represents a complex spectrum of clinical alterations, resulting from infiltration by anomalous histiocytes of various organs, including the skin, bones, lungs, lymphatic ganglia and liver. Liver disease is rare and the mechanism by which lesions appear is unknown. Cholestasis results from phenomena of sclerosant colangitis, which affects the intrahepatic ducts, or from proliferation of histiocytic cells in the periportal areas. Some patients develop biliar cyrrhosis. The authors present the clinical case of a 62-year-old female patient, hospitalized for chronic cholestasis, diabetes and gallstone in the main bile duct. She had metastatic lesions of the hypophysis and bones, the biopsies of which revealed infiltration by histiocytic cells. Endoscopic Retrograde Colangiopancreatography (E.R.C.P.) revealed dilatation of intrahepatic bile ducts and stenosis of left hepatic duct. A histological study of the hepatic biopsy showed chronic cholestasis and areas of fibrosis, without infiltration by histiocytic granulomas, which were observed in the medular biopsy and in the thyroid nodule cytology.https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1904
spellingShingle Zita Romão
J Pontes
P Andrade
M C Leitão
A Donato
Diniz Freitas
Cirrose biliar secundária em doente com histiocitose X.
Acta Médica Portuguesa
title Cirrose biliar secundária em doente com histiocitose X.
title_full Cirrose biliar secundária em doente com histiocitose X.
title_fullStr Cirrose biliar secundária em doente com histiocitose X.
title_full_unstemmed Cirrose biliar secundária em doente com histiocitose X.
title_short Cirrose biliar secundária em doente com histiocitose X.
title_sort cirrose biliar secundaria em doente com histiocitose x
url https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1904
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