Severe reduction of blood lysosomal acid lipase activity in cryptogenic cirrhosis: A nationwide multicentre cohort study

Francesco Angelico, Stefano Ginanni Corradini, Daniele Pastori, Silvia Fargion, Anna Ludovica Fracanzani, Mario Angelico, Luigi Bolondi, Giulia Tozzi, Pietro Luigi Pujatti, Giancarlo Labbadia, Gino Roberto Corazza, Maurizio Averna, Francesco Perticone, Giuseppe Croce, Marcello Persico, Tommaso Bucci, Francesco Baratta, Licia Polimeni, Maria Del Ben, Francesco Violi

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Abstract

Background and aims Blood lysosomal acid lipase (LAL) is reduced in non-alcoholic steatohepatitis, which is the major cause of cryptogenic cirrhosis (CC); few data on LAL activity in CC do exist. We investigated LAL activity in a cohort of patients with liver cirrhosis. Methods This is a multicentre cohort study including 274 patients with liver cirrhosis of different aetiology from 19 centres of Internal Medicine, Gastroenterology and Hepatology distributed throughout Italy. Blood LAL activity (nmol/spot/h) was measured with dried blood spot extracts using Lalistat 2. Results Overall, 133 patients had CC, and 141 patients had cirrhosis by other causes (61 viral, 53 alcoholic, 20 alcoholic + viral, 7 autoimmune). Mean age was 64.2 ± 13.4 years, and 28.5% were women. Patients with CC were older compared to other aetiology-cirrhosis, with a lower Child-Turcotte-Pugh (CTP, p=0.003) and MELD (p=0.009) score, and a higher prevalence of cardio-metabolic risk factors and previous ischemic events. In the whole cohort, median LAL activity value was 0.58 nmol/spot/h, 0.49 and 0.65 in the groups of CC and known-aetiology cirrhosis, respectively (p=0.002). The difference remained significant after adjustment for white blood cells count (p=0.001). Multivariable linear regression analysis showed that CC (vs. known aetiology, Beta = −0.144, p=0.018), platelet count (Beta = 0.398, p < 0.001) and CTP score (Beta = −0.133, p=0.022) were associated with log-LAL activity. Similar results were found using MELD as covariate. Conclusions We found a marked reduction of LAL activity in patients with cryptogenic cirrhosis compared to the other known aetiologies. A prospective study will clarify the role of LAL in chronic liver diseases.

Original languageEnglish
Pages (from-to)179-184
Number of pages6
JournalAtherosclerosis
Volume262
DOIs
Publication statusPublished - Jul 1 2017

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Keywords

  • Cryptogenic cirrhosis
  • Liver disease
  • Lysosomal acid lipase
  • Pathogenesis

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Angelico, F., Corradini, S. G., Pastori, D., Fargion, S., Fracanzani, A. L., Angelico, M., Bolondi, L., Tozzi, G., Pujatti, P. L., Labbadia, G., Corazza, G. R., Averna, M., Perticone, F., Croce, G., Persico, M., Bucci, T., Baratta, F., Polimeni, L., Del Ben, M., & Violi, F. (2017). Severe reduction of blood lysosomal acid lipase activity in cryptogenic cirrhosis: A nationwide multicentre cohort study. Atherosclerosis, 262, 179-184. https://doi.org/10.1016/j.atherosclerosis.2017.03.038