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

• Autor(a) principal: Starosta, Rodrigo Tzovenos
• Data de Publicação: 2021
• Outros Autores: Siebert, Marina, Vairo, Filippo Pinto e, Costa, Bruno Lafaiete de Lima, Ponzoni, Christiano Tomasso Silveira, Schwartz, Ida Vanessa Doederlein, Cerski, Carlos Thadeu Schmidt
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Repositório Institucional da UFRGS
• Texto Completo: http://hdl.handle.net/10183/232170
• topic: Doença de Gaucher; Citometria por imagem; Biópsia; Hepatócitos; Gaucher disease; Image cytometry; Hepatocytes; Bile canaliculi; Biopsy; Large-core needle

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.