Increased levels of chitotriosidase in a patient with Alagille syndrome: association or coincidence?

Increased levels of chitotriosidase in a patient with Alagille syndrome: association or coincidence?

ABSTRACT We describe a case of a patient with Alagille syndrome (AS) presenting an increased level of the enzyme chitotriosidase (ChT), evaluating factors that could justify the relationship between AS and ChT. He was a male patient with cholestatic jaundice, facial dysmorphia and congenital heart disease who presented a brief septicemia. He underwent liver biopsy and analyses for inborn errors of metabolism that respectively showed ductopenia and increased levels of ChT. This increase could be potentially explained by inflammatory and infectious processes, or even by AS itself.

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Bibliographic Details
Main Authors: Diniz,Bruna L., Floriani,Maiara A., Ferreira,Maria Angélica T., Gonzales,João Francisco O., Lisboa,Nathan H., Jakimiu,André Ricardo, Ferreira,Janaina Yacy H., Rosa,Rafael Fabiano M., Zen,Paulo Ricardo G.
Format: Digital revista
Language:English
Published: Sociedade Brasileira de Patologia Clínica 2018
Online Access:http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1676-24442018000100037
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http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1676-24442018000100037

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