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Atherosclerosis. 2017 Jul;262:179-184. doi: 10.1016/j.atherosclerosis.2017.03.038. Epub 2017 Mar 31.

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

Author information

1
I Clinica Medica, Atherothrombosis Center, Department of Internal Medicine and Medical Specialties, Sapienza University of Rome, Italy; Department of Public Health and Infectious Diseases, Sapienza University of Rome, Italy. Electronic address: francesco.angelico@uniroma1.it.
2
Gastroenterology Division, Department of Clinical Medicine, Sapienza University of Rome, Italy.
3
I Clinica Medica, Atherothrombosis Center, Department of Internal Medicine and Medical Specialties, Sapienza University of Rome, Italy; Department of Anatomical, Histological, Forensic Medicine and Orthopedics Sciences - Sapienza University of Rome, Italy.
4
Department of Pathophysiology and Transplantation, Ca' Granda Foundation IRCCS Maggiore Policlinico Hospital, University of Milan, Milan, Italy.
5
Hepatology Unit, Tor Vergata University, Rome, Italy.
6
Department of Medical and Surgical Sciences, University of Bologna, Italy.
7
Unit for Neuromuscular and Neurodegenerative Diseases, Children's Hospital and Research Institute "Bambino Gesù", Rome, Italy.
8
Department of Internal Medicine, Ospedale di Arzignano, ULSS n.5 "Ovest Vicentino", Italy.
9
I Clinica Medica, Atherothrombosis Center, Department of Internal Medicine and Medical Specialties, Sapienza University of Rome, Italy.
10
First Department of Internal Medicine, Fondazione IRCCS Policlinico San Matteo, University of Pavia, Italy.
11
Department of Internal Medicine and Medical Specialties - DIBIMIS, School of Medicine, University of Palermo, Palermo, Italy.
12
Department of Medical and Surgical Sciences, University Magna Græcia of Catanzaro, Italy.
13
Internal Medicine Unit, Giuseppe Mazzini Hospital, Teramo, Italy.
14
Internal Medicine and Hepatology Unit, Salerno University of Medicine, Salerno, Italy.
15
Sapienza - University of Rome, Department of Internal Medicine and Medical Specialties, Italy.
16
Department of Emergency, Chest Pain Unit, Sapienza University of Rome, Italy.
17
Department of Cardiovascular, Respiratory, Nephrologic, Anesthesiologic and Geriatric Sciences, Sapienza University of Rome, Italy.
18
Department of Medical and Surgical Sciences, University Magna Graecia of Catanzaro, Catanzaro, Italy.
19
Department of Medicine (DIMED), University of Padova, Padua, Italy.
20
Division of Gastroenterology, Casa Sollievo Sofferenza, Hospital, IRCCS, San Giovanni Rotondo, Italy.
21
Department of Medicine and Aging, University of Chieti "G. d'Annunzio", School of Medicine, Chieti, Italy.
22
Department of Medico-Surgical Sciences and Biotechnology, Vascular Biology and Mass Spectrometry Laboratory, Sapienza University of Rome, Latina, Italy.

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.

KEYWORDS:

Cryptogenic cirrhosis; Liver disease; Lysosomal acid lipase; Pathogenesis

[Indexed for MEDLINE]

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