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Increased Prevalence of Alpha-1-Antitrypsin Deficiency in Patients with Biliary Tract Cancer and Its Associated Clinicopathological Features.
Cornillet, Martin; Zemack, Helen; Jansson, Hannes; Sparrelid, Ernesto; Ellis, Ewa; Björkström, Niklas K.
Afiliação
  • Cornillet M; Center for Infectious Medicine, Department of Medicine Huddinge, Karolinska Institutet, Karolinska University Hospital, SE-14186 Stockholm, Sweden.
  • Zemack H; Division of Transplantation Surgery, Department of Clinical Science, Intervention and Technology, Karolinska Institutet, Karolinska University Hospital, SE-14186 Stockholm, Sweden.
  • Jansson H; Division of Surgery and Oncology, Department of Clinical Science, Intervention and Technology, Karolinska Institutet, Karolinska University Hospital, SE-14186 Stockholm, Sweden.
  • Sparrelid E; Division of Surgery and Oncology, Department of Clinical Science, Intervention and Technology, Karolinska Institutet, Karolinska University Hospital, SE-14186 Stockholm, Sweden.
  • Ellis E; Division of Transplantation Surgery, Department of Clinical Science, Intervention and Technology, Karolinska Institutet, Karolinska University Hospital, SE-14186 Stockholm, Sweden.
  • Björkström NK; Center for Infectious Medicine, Department of Medicine Huddinge, Karolinska Institutet, Karolinska University Hospital, SE-14186 Stockholm, Sweden.
Cells ; 12(12)2023 06 19.
Article em En | MEDLINE | ID: mdl-37371133
Alpha-1 antitrypsin deficiency (A1ATD) is underdiagnosed and associated with liver diseases. Here, we genotyped 130 patients with biliary tract cancer (BTC) scheduled for liver resection and found A1ATD in 10.8% of the patients. A1ATD was found in all BTC subtypes, and patients had similar clinical features as non-A1ATD BTC, not permitting their identification using clinical routine liver tests. In intrahepatic cholangiocarcinoma (iCCA), the abundance of A1AT protein was increased in the tumor and appeared to be influenced by the genomic alterations. On the one hand, BTC with A1ATD had lower perineural invasion at histopathology and displayed a longer survival, suggesting that a deficiency in this protein is associated with a less aggressive phenotype. On the other hand, iCCA with high A1AT expression had more advanced tumor staging and enriched pathways for complement system and extracellular matrix interactions, indicating that A1AT protein might contribute to a more aggressive phenotype. With increased awareness, screening, and basic studies, A1ATD could represent one more layer of stratification for future targeted therapies in BTC.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Neoplasias do Sistema Biliar / Alfa 1-Antitripsina / Deficiência de alfa 1-Antitripsina Tipo de estudo: Diagnostic_studies / Etiology_studies / Prevalence_studies / Prognostic_studies / Risk_factors_studies Limite: Humans Idioma: En Revista: Cells Ano de publicação: 2023 Tipo de documento: Article

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Neoplasias do Sistema Biliar / Alfa 1-Antitripsina / Deficiência de alfa 1-Antitripsina Tipo de estudo: Diagnostic_studies / Etiology_studies / Prevalence_studies / Prognostic_studies / Risk_factors_studies Limite: Humans Idioma: En Revista: Cells Ano de publicação: 2023 Tipo de documento: Article