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Histomorphometric analysis of liver biopsies of treated patients with Gaucher disease type 1.
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.
Afiliação
  • Starosta RT; Universidade Federal do Rio Grande do Sul, Graduate Program in Genetics and Molecular Biology, Porto Alegre, RS, Brasil.
  • Siebert M; Washington University, Department of Pediatrics, Saint Louis, MO, USA.
  • Vairo FPE; Hospital de Clínicas de Porto Alegre, Laboratorial Research Unit, Experimental Research Center, Porto Alegre, RS, Brasil.
  • Costa BLL; Universidade Federal do Rio Grande do Sul, Graduate Program in Science in Gastroenterology and Hepatology, Porto Alegre, RS, Brasil.
  • Ponzoni CT; Mayo Clinic, Center for Individualized Medicine, Rochester, MN, USA.
  • Schwartz IVD; Mayo Clinic, Department of Clinical Genomics, Rochester, MN, USA.
  • Cerski CTS; Hospital de Clínicas de Porto Alegre, Department of Surgical Pathology, Porto Alegre, RS, Brasil.
Autops Case Rep ; 11: e2021306, 2021.
Article em En | MEDLINE | ID: mdl-34458174
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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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Idioma: En Revista: Autops Case Rep Ano de publicação: 2021 Tipo de documento: Article País de afiliação: Brasil País de publicação: Brasil
Texto completo
Adicionar na Minha BVS
Imprimir
XML
PubMed Links
Buscar no Google

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Idioma: En Revista: Autops Case Rep Ano de publicação: 2021 Tipo de documento: Article País de afiliação: Brasil País de publicação: Brasil