Histomorphometric analysis of liver biopsies of treated patients with Gaucher disease type 1

Gaucher disease (GD) is an autosomal recessive lysosomal disorder caused by a disturbance in the metabolism of glucocerebroside in the macrophages. Most of its manifestations – hepatosplenomegaly, anemia, thrombocytopenia, and bone pain – are amenable to a macrophage-target therapy such as enzyme re...

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Published in:Autopsy & case reports 2021, Vol.11, p.e2021306-e2021306
Main Authors: Starosta, Rodrigo Tzovenos, Siebert, Marina, Vairo, Filippo Pinto e, Costa, Bruno Lafaiete de Lima, Ponzoni, Christiano Tomaso, Schwartz, Ida Vanessa Doederlein, Cerski, Carlos Thadeu Schmidt
Format: Article
Language:English
Subjects:
ANATOMY & MORPHOLOGY
Bile Canaliculi
Biopsy
Gaucher Disease
Hepatocytes
Image Cytometry
Large-Core Needle
Original
PATHOLOGY
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container_title Autopsy & case reports
container_volume 11
creator Starosta, Rodrigo Tzovenos
Siebert, Marina
Vairo, Filippo Pinto e
Costa, Bruno Lafaiete de Lima
Ponzoni, Christiano Tomaso
Schwartz, Ida Vanessa Doederlein
Cerski, Carlos Thadeu Schmidt
description Gaucher disease (GD) is an autosomal recessive lysosomal disorder caused by a disturbance in the metabolism of glucocerebroside in the macrophages. Most of its manifestations – hepatosplenomegaly, anemia, thrombocytopenia, and bone pain – are amenable to a macrophage-target therapy such as enzyme replacement. However, there is increasing evidence that abnormalities of the liver persist despite the specific GD treatment. In this work, we adapted histomorphometry techniques to the study of hepatocytes in GD using liver tissue of treated patients, developing the first morphometrical method for canalicular quantification in immunohistochemistry-stained liver biopsies, and exploring histomorphometric characteristics of GD. This is the first histomorphometric technique developed for canalicular analysis on histological liver biopsy samples.
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subjects ANATOMY & MORPHOLOGY
Bile Canaliculi
Biopsy
Gaucher Disease
Hepatocytes
Image Cytometry
Large-Core Needle
Original
PATHOLOGY
title Histomorphometric analysis of liver biopsies of treated patients with Gaucher disease type 1
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