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Int. J. Mol. Sci. 2016, 17(3), 312; doi:10.3390/ijms17030312

Low Serum Lysosomal Acid Lipase Activity Correlates with Advanced Liver Disease

1
The Liver Unit, Gastroenterology Institute, Hadassah Medical Center, Hadassah Medical School, The Hebrew University, Jerusalem 9112001, Israel
2
Pediatric Gastroenterology Institute, Shaare Zedek Medical Center, Hadassah Medical School, The Hebrew University, Jerusalem 9103102, Israel
3
Liver Unit, Gastroenterology Institute, Shaare Zedek Medical Center, Hadassah Medical School, The Hebrew University, Jerusalem 9112001, Israel
4
Liver Unit, Holy Family Hospital; Safed Medical School, Bar Ilan University, Nazareth 1641110, Israel
These authors contributed equally to this work.
*
Author to whom correspondence should be addressed.
Academic Editors: Amedeo Lonardo and Giovanni Targher
Received: 12 December 2015 / Revised: 18 February 2016 / Accepted: 18 February 2016 / Published: 27 February 2016
(This article belongs to the Special Issue Non-Alcoholic Fatty Liver Disease Research 2016)
View Full-Text   |   Download PDF [211 KB, uploaded 27 February 2016]

Abstract

Fatty liver has become the most common liver disorder and is recognized as a major health burden in the Western world. The causes for disease progression are not fully elucidated but lysosomal impairment is suggested. Here we evaluate a possible role for lysosomal acid lipase (LAL) activity in liver disease. To study LAL levels in patients with microvesicular, idiopathic cirrhosis and nonalcoholic fatty liver disease (NAFLD). Medical records of patients with microvesicular steatosis, cryptogenic cirrhosis and NAFLD, diagnosed on the basis of liver biopsies, were included in the study. Measured serum LAL activity was correlated to clinical, laboratory, imaging and pathological data. No patient exhibited LAL activity compatible with genetic LAL deficiency. However, serum LAL activity inversely predicted liver disease severity. A LAL level of 0.5 was the most sensitive for detecting both histologic and noninvasive markers for disease severity, including lower white blood cell count and calcium, and elevated γ-glutamyltransferase, creatinine, glucose, glycated hemoglobin, uric acid and coagulation function. Serum LAL activity <0.5 indicates severe liver injury in patients with fatty liver and cirrhosis. Further studies should define the direct role of LAL in liver disease severity and consider the possibility of replacement therapy. View Full-Text
Keywords: lysosomal acid lipase; cholesteryl ester storage disease; non-alcoholic liver disease; non-alcoholic steatohepatitis; cirrhosis lysosomal acid lipase; cholesteryl ester storage disease; non-alcoholic liver disease; non-alcoholic steatohepatitis; cirrhosis
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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MDPI and ACS Style

Shteyer, E.; Villenchik, R.; Mahamid, M.; Nator, N.; Safadi, R. Low Serum Lysosomal Acid Lipase Activity Correlates with Advanced Liver Disease. Int. J. Mol. Sci. 2016, 17, 312.

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