TY - JOUR
T1 - Histomorphometric analysis of liver biopsies of treated patients with gaucher disease type 1
AU - Starosta, Rodrigo Tzovenos
AU - Siebert, Marina
AU - E Vairo, Filippo Pinto
AU - Costa, Bruno Lafaiete de Lima
AU - Ponzoni, Christiano Tomaso
AU - Schwartz, Ida Vanessa Doederlein
AU - Cerski, Carlos Thadeu Schmidt
N1 - Funding Information:
This study was supported by the Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES) and the Fundo de Apoio a Pesquisa e Eventos do Hospital de Clínicas de Porto Alegre (FIPE-HCPA). We are grateful to the statistical counseling provided by Dr. Michael Wallendorf at Washington University.
Funding Information:
for this study was provided by the Coordena??o de Aperfei?oamento de Pessoal de N?vel Superior (CAPES) and the Fundo de Apoio a Pesquisa e Eventos do Hospital de Cl?nicas de Porto Alegre (FIPE-HCPA).
Publisher Copyright:
© 2021 The Authors.
PY - 2021
Y1 - 2021
N2 - 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.
AB - 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.
KW - Bile canaliculi
KW - Biopsy, large-core needle
KW - Gaucher disease
KW - Hepatocytes
KW - Image cytometry
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U2 - 10.4322/ACR.2021.306
DO - 10.4322/ACR.2021.306
M3 - Article
AN - SCOPUS:85114830804
SN - 2236-1960
VL - 11
JO - Autopsy and Case Reports
JF - Autopsy and Case Reports
M1 - e2021306
ER -