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Hepatic amyloidosis: a prevalence study and clinical characterization of a rare and severe disease

Revista Espanola de Enfermedades Digestivas, ISSN: 1130-0108, Vol: 115, Issue: 1, Page: 16-21
2023
  • 5
    Citations
  • 0
    Usage
  • 14
    Captures
  • 1
    Mentions
  • 0
    Social Media
Metric Options:   Counts1 Year3 Year

Metrics Details

  • Citations
    5
  • Captures
    14
  • Mentions
    1
    • News Mentions
      1
      • News
        1

Most Recent News

Findings from University Hospital Center Reveals New Findings on Amyloidosis (Hepatic Amyloidosis: a Prevalence Study and Clinical Characterization of a Rare and Severe Disease)

2023 JUN 30 (NewsRx) -- By a News Reporter-Staff News Editor at Health & Medicine Daily -- Current study results on Nutritional and Metabolic Diseases

Article Description

Background and aim: amyloidosis is a systemic disease characterized by extracellular deposition of amyloid protein, most commonly in the heart and kidney. Hepatic amyloidosis is a rare form of presentation that ranges from mild hepatomegaly and altered liver biochemical tests to acute liver failure. The aims of this study were to evaluate the prevalence of amyloidosis in patients undergoing liver biopsy and describe its main clinical characteristics and prognostic impact. Methods: a retrospective analysis of all patients with a histological diagnosis of hepatic amyloidosis between January 2010 and December 2019 was performed. Results: seven patients were identified from a total of 1,773 liver biopsy procedures (0.4 %), with a female predominance (6/7) and median age of diagnosis of 62 years. The most common clinical manifestations included hepatomegaly (4/7), jaundice (2/7) and peripheral edema (2/7), whereas 3/7 patients were asymptomatic. Every patient presented abnormalities in liver biochemical tests, more commonly cholestasis (6/7), but also cytolysis (4/7) or hyperbilirubinemia (2/7). Abnormal imaging findings included hepatomegaly, steatosis or parenchymal heterogeneity. In most patients (5/7), other organs were involved, most commonly with nephrotic syndrome (3/7) and infiltrative cardiomyopathy (3/7). The most common type was AA amyloidosis (3/7) followed by AL amyloidosis (2/7). The one-year mortality rate was 43 % and the median survival was 24 months. Conclusions: we report a low prevalence (0.4 %) of amyloidosis among patients undergoing liver biopsy. Although rare, hepatic amyloidosis is associated with a dismal prognosis and a high index of suspicion is crucial to achieve an early diagnosis.

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