TY - JOUR
T1 - Severe reduction of blood lysosomal acid lipase activity in cryptogenic cirrhosis
T2 - A nationwide multicentre cohort study
AU - Angelico, Francesco
AU - Corradini, Stefano Ginanni
AU - Pastori, Daniele
AU - Fargion, Silvia
AU - Fracanzani, Anna Ludovica
AU - Angelico, Mario
AU - Bolondi, Luigi
AU - Tozzi, Giulia
AU - Pujatti, Pietro Luigi
AU - Labbadia, Giancarlo
AU - Corazza, Gino Roberto
AU - Averna, Maurizio
AU - Perticone, Francesco
AU - Croce, Giuseppe
AU - Persico, Marcello
AU - Bucci, Tommaso
AU - Baratta, Francesco
AU - Polimeni, Licia
AU - Del Ben, Maria
AU - Violi, Francesco
PY - 2017/7/1
Y1 - 2017/7/1
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.
KW - Cryptogenic cirrhosis
KW - Liver disease
KW - Lysosomal acid lipase
KW - Pathogenesis
UR - http://www.scopus.com/inward/record.url?scp=85017165741&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85017165741&partnerID=8YFLogxK
U2 - 10.1016/j.atherosclerosis.2017.03.038
DO - 10.1016/j.atherosclerosis.2017.03.038
M3 - Article
VL - 262
SP - 179
EP - 184
JO - Atherosclerosis
JF - Atherosclerosis
SN - 0021-9150
ER -