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

Maurizio Averna, Valeria Raparelli, Michela Masotti, Licia Polimeni, Laura Napoleone, Francesco Tovoli, Dario Di Michele, Antonina Giammanco, Mario Angelico, Gino Roberto Corazza, Francesco Violi, Daniele Pastori, Antonio M. Ippolito, Lucia Parlati, Flaminia Ferri, Francesco Baratta, Marco Ciacciarelli, Francesco Santopaolo, Luigi Bolondi, Pietro Luigi PujattiTommaso Bucci, Jessica D'Amico, Giuseppe Croce, Alessandra Faedo, Monica Pellone, Luigi Bolondi, Monica Pellone, Stefano Ginanni Corradini, Giovanni Davì, Alessandra Faedo, Daniele Pastori, Daniele Pastori, Monica Pellone, Marina Colzi, Simona Francioso, Paola Andreozzi, Maria Del Ben, Giancarlo Labbadia, Giulia Tozzi, Anna Ludovica Fracanzani, Maurizio Averna, Angelo Andriulli, Silvia Fargion, Stefania Basili, Gaetano Bergamaschi, Francesco Angelico, Francesco Perticone, Luigi Iuliano, Marcello Persico, Giovanni Davì, Monica Mischitelli

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13 Citazioni (Scopus)


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.
Lingua originaleEnglish
Numero di pagine0
Stato di pubblicazionePublished - 2017

All Science Journal Classification (ASJC) codes

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