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, Mario Angelico, Gino Roberto Corazza, Francesco Violi, Daniele Pastori, Antonio M. Ippolito, Lucia Parlati, Flaminia Ferri, Francesco Baratta, Marco Ciacciarelli, Francesco Santopaolo, Pietro Luigi Pujatti, Tommaso Bucci, Jessica D'AmicoGiuseppe Croce, Alessandra Faedo, Monica Pellone, Luigi Bolondi, Monica Pellone, Stefano Ginanni Corradini, Giovanni Davì, Marina Colzi, Simona Francioso, Paola Andreozzi, Maria Del Ben, Giancarlo Labbadia, Giulia Tozzi, Anna Ludovica Fracanzani, Angelo Andriulli, Silvia Fargion, Stefania Basili, Gaetano Bergamaschi, Francesco Angelico, Francesco Perticone, Luigi Iuliano, Marcello Persico, Giovanni Davì, Monica Mischitelli

Risultato della ricerca: Article

8 Citazioni (Scopus)

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.
Lingua originaleEnglish
Numero di pagine0
RivistaATHEROSCLEROSIS
Stato di pubblicazionePublished - 2017

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Sterol Esterase
Multicenter Studies
Cohort Studies
Cytidine Triphosphate
Fibrosis
Gastroenterology
Liver Cirrhosis
Cryptogenic Cirrhosis
Fatty Liver
Internal Medicine
Platelet Count
Leukocyte Count
Italy
Liver Diseases
Linear Models
Chronic Disease
Regression Analysis
Prospective Studies

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine

Cita questo

Averna, M., Raparelli, V., Masotti, M., Polimeni, L., Napoleone, L., Tovoli, F., ... Mischitelli, M. (2017). Severe reduction of blood lysosomal acid lipase activity in cryptogenic cirrhosis: A nationwide multicentre cohort study. ATHEROSCLEROSIS.

Severe reduction of blood lysosomal acid lipase activity in cryptogenic cirrhosis: A nationwide multicentre cohort study. / Averna, Maurizio; Raparelli, Valeria; Masotti, Michela; Polimeni, Licia; Napoleone, Laura; Tovoli, Francesco; Di Michele, Dario; Angelico, Mario; Corazza, Gino Roberto; Violi, Francesco; Pastori, Daniele; Ippolito, Antonio M.; Parlati, Lucia; Ferri, Flaminia; Baratta, Francesco; Ciacciarelli, Marco; Santopaolo, Francesco; Pujatti, Pietro Luigi; Bucci, Tommaso; D'Amico, Jessica; Croce, Giuseppe; Faedo, Alessandra; Pellone, Monica; Bolondi, Luigi; Pellone, Monica; Corradini, Stefano Ginanni; Davì, Giovanni; Colzi, Marina; Francioso, Simona; Andreozzi, Paola; Del Ben, Maria; Labbadia, Giancarlo; Tozzi, Giulia; Fracanzani, Anna Ludovica; Andriulli, Angelo; Fargion, Silvia; Basili, Stefania; Bergamaschi, Gaetano; Angelico, Francesco; Perticone, Francesco; Iuliano, Luigi; Persico, Marcello; Davì, Giovanni; Mischitelli, Monica.

In: ATHEROSCLEROSIS, 2017.

Risultato della ricerca: Article

Averna, M, Raparelli, V, Masotti, M, Polimeni, L, Napoleone, L, Tovoli, F, Di Michele, D, Angelico, M, Corazza, GR, Violi, F, Pastori, D, Ippolito, AM, Parlati, L, Ferri, F, Baratta, F, Ciacciarelli, M, Santopaolo, F, Pujatti, PL, Bucci, T, D'Amico, J, Croce, G, Faedo, A, Pellone, M, Bolondi, L, Pellone, M, Corradini, SG, Davì, G, Colzi, M, Francioso, S, Andreozzi, P, Del Ben, M, Labbadia, G, Tozzi, G, Fracanzani, AL, Andriulli, A, Fargion, S, Basili, S, Bergamaschi, G, Angelico, F, Perticone, F, Iuliano, L, Persico, M, Davì, G & Mischitelli, M 2017, 'Severe reduction of blood lysosomal acid lipase activity in cryptogenic cirrhosis: A nationwide multicentre cohort study', ATHEROSCLEROSIS.
Averna, Maurizio ; Raparelli, Valeria ; Masotti, Michela ; Polimeni, Licia ; Napoleone, Laura ; Tovoli, Francesco ; Di Michele, Dario ; Angelico, Mario ; Corazza, Gino Roberto ; Violi, Francesco ; Pastori, Daniele ; Ippolito, Antonio M. ; Parlati, Lucia ; Ferri, Flaminia ; Baratta, Francesco ; Ciacciarelli, Marco ; Santopaolo, Francesco ; Pujatti, Pietro Luigi ; Bucci, Tommaso ; D'Amico, Jessica ; Croce, Giuseppe ; Faedo, Alessandra ; Pellone, Monica ; Bolondi, Luigi ; Pellone, Monica ; Corradini, Stefano Ginanni ; Davì, Giovanni ; Colzi, Marina ; Francioso, Simona ; Andreozzi, Paola ; Del Ben, Maria ; Labbadia, Giancarlo ; Tozzi, Giulia ; Fracanzani, Anna Ludovica ; Andriulli, Angelo ; Fargion, Silvia ; Basili, Stefania ; Bergamaschi, Gaetano ; Angelico, Francesco ; Perticone, Francesco ; Iuliano, Luigi ; Persico, Marcello ; Davì, Giovanni ; Mischitelli, Monica. / Severe reduction of blood lysosomal acid lipase activity in cryptogenic cirrhosis: A nationwide multicentre cohort study. In: ATHEROSCLEROSIS. 2017.
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title = "Severe reduction of blood lysosomal acid lipase activity in cryptogenic cirrhosis: A nationwide multicentre cohort study",
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.",
author = "Maurizio Averna and Valeria Raparelli and Michela Masotti and Licia Polimeni and Laura Napoleone and Francesco Tovoli and {Di Michele}, Dario and Mario Angelico and Corazza, {Gino Roberto} and Francesco Violi and Daniele Pastori and Ippolito, {Antonio M.} and Lucia Parlati and Flaminia Ferri and Francesco Baratta and Marco Ciacciarelli and Francesco Santopaolo and Pujatti, {Pietro Luigi} and Tommaso Bucci and Jessica D'Amico and Giuseppe Croce and Alessandra Faedo and Monica Pellone and Luigi Bolondi and Monica Pellone and Corradini, {Stefano Ginanni} and Giovanni Dav{\`i} and Marina Colzi and Simona Francioso and Paola Andreozzi and {Del Ben}, Maria and Giancarlo Labbadia and Giulia Tozzi and Fracanzani, {Anna Ludovica} and Angelo Andriulli and Silvia Fargion and Stefania Basili and Gaetano Bergamaschi and Francesco Angelico and Francesco Perticone and Luigi Iuliano and Marcello Persico and Giovanni Dav{\`i} and Monica Mischitelli",
year = "2017",
language = "English",
journal = "ATHEROSCLEROSIS",
issn = "0021-9150",

}

TY - JOUR

T1 - Severe reduction of blood lysosomal acid lipase activity in cryptogenic cirrhosis: A nationwide multicentre cohort study

AU - Averna, Maurizio

AU - Raparelli, Valeria

AU - Masotti, Michela

AU - Polimeni, Licia

AU - Napoleone, Laura

AU - Tovoli, Francesco

AU - Di Michele, Dario

AU - Angelico, Mario

AU - Corazza, Gino Roberto

AU - Violi, Francesco

AU - Pastori, Daniele

AU - Ippolito, Antonio M.

AU - Parlati, Lucia

AU - Ferri, Flaminia

AU - Baratta, Francesco

AU - Ciacciarelli, Marco

AU - Santopaolo, Francesco

AU - Pujatti, Pietro Luigi

AU - Bucci, Tommaso

AU - D'Amico, Jessica

AU - Croce, Giuseppe

AU - Faedo, Alessandra

AU - Pellone, Monica

AU - Bolondi, Luigi

AU - Pellone, Monica

AU - Corradini, Stefano Ginanni

AU - Davì, Giovanni

AU - Colzi, Marina

AU - Francioso, Simona

AU - Andreozzi, Paola

AU - Del Ben, Maria

AU - Labbadia, Giancarlo

AU - Tozzi, Giulia

AU - Fracanzani, Anna Ludovica

AU - Andriulli, Angelo

AU - Fargion, Silvia

AU - Basili, Stefania

AU - Bergamaschi, Gaetano

AU - Angelico, Francesco

AU - Perticone, Francesco

AU - Iuliano, Luigi

AU - Persico, Marcello

AU - Davì, Giovanni

AU - Mischitelli, Monica

PY - 2017

Y1 - 2017

N2 - 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.

AB - 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.

UR - http://hdl.handle.net/10447/232637

UR - http://www.elsevier.com/locate/atherosclerosis

M3 - Article

JO - ATHEROSCLEROSIS

JF - ATHEROSCLEROSIS

SN - 0021-9150

ER -