Histomorphometric analysis of liver biopsies of treated patients with Gaucher disease type 1
Autops. Case Rep
;
11: e2021306, 2021. tab, graf
Article
in English
| LILACS
| ID: biblio-1285400
ABSTRACT
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.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Image Cytometry
/
Gaucher Disease
Limits:
Humans
Language:
English
Journal:
Autops. Case Rep
Journal subject:
Anatomia
/
Patologia Cl¡nica
/
Patologia Legal
Year:
2021
Type:
Article
Affiliation country:
Brazil
/
United States
Institution/Affiliation country:
Hospital de Clínicas de Porto Alegre/BR
/
Mayo Clinic/US
/
Universidade Federal do Rio Grande do Sul/BR
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