| Autor: |
María Adela Aguirre, Marcelina Carretero, Eugenia Villanueva, Elsa Mercedes Nucifora, María Soledad Saez, Erika Bárbara Brulc, Diego Pérez De Arenaza, Sebastián Marciano, María Agustina Marco, Gisela Bendelman, Patricia Beatriz Sorroche, María Lourdes Posadas Martínez |
| Jazyk: |
angličtina |
| Rok vydání: |
2023 |
| Předmět: |
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| Zdroj: |
Annals of Hepatology, Vol 28, Iss , Pp 100931- (2023) |
| Druh dokumentu: |
article |
| ISSN: |
1665-2681 |
| DOI: |
10.1016/j.aohep.2023.100931 |
| Popis: |
Introduction and Objectives: The liver can be either compromised by infiltrative damage of amyloid, as it happens in AL and AA amyloidosis, or its cause, as it occurs in transthyretin TTR-related amyloidosis. In the latter, the liver synthesizes a defective variant TTR which has the capacity for cardiac, neurological, and renal damage, but the liver function is preserved. This study aimed to describe the clinical characteristics and prognosis of patients with liver involvement of amyloidosis (AL and AA) Materials and Methods: Retrospective cohort of patients with hepatic involvement included in the Institutional Amyloidosis Registry (ClinicalTrials.gov NCT01347047) between June 2010 and January 2022. Clinical characteristics and complementary studies were analyzed, as well as their evolution. Results: 359 patients with amyloidosis were included in the registry, of whom 16 (5% (CI 2.7-7.3)) had liver involvement. The most frequent types of amyloidosis were: AL 88% (14), AA 6% (1) and non-typed 6% (1). The median age at diagnosis was 64 years (IR 63-74), male 44% (7). The median albumin value was 3.0 gr/dL (IR 2.5-3.8), alkaline phosphatase 705 IU (IR 395-114), total bilirubin mg/dL 1.1 (IR 0.5-14.8), and more than 25% had jaundice. Thirty-one percent presented a cardiac compromise. The mortality rate in the study period was 56% (CI 30%-80%). When comparing patients with amyloidosis with and without liver involvement, mortality was higher in the liver involvement group (29% vs. 56%, p 0.02). Conclusions: We present the first report in our region with adequate sampling that allows us to approximate the burden of this disease in relation to the liver. Hepatic infiltrative involvement has a high mortality rate in amyloidosis compared to those without liver involvement. |
| Databáze: |
Directory of Open Access Journals |
| Externí odkaz: |
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