title: Low Serum Lysosomal Acid Lipase Activity Correlates with Advanced Liver Disease.
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.
authors.affiliation: The Liver Unit, Gastroenterology Institute, Hadassah Medical Center, Hadassah Medical School, The Hebrew University, Jerusalem 9112001, Israel. eyals@szmc.org.il.|Pediatric Gastroenterology Institute, Shaare Zedek Medical Center, Hadassah Medical School, The Hebrew University, Jerusalem 9103102, Israel. eyals@szmc.org.il.
authors.forename: Eyal
authors.initials: E
authors.lastname: Shteyer
authors.affiliation: The Liver Unit, Gastroenterology Institute, Hadassah Medical Center, Hadassah Medical School, The Hebrew University, Jerusalem 9112001, Israel. eshteyer@hotmail.com.
authors.forename: Rivka
authors.initials: R
authors.lastname: Villenchik
authors.affiliation: Liver Unit, Gastroenterology Institute, Shaare Zedek Medical Center, Hadassah Medical School, The Hebrew University, Jerusalem 9112001, Israel. mmahamid@szmc.org.il.|Liver Unit, Holy Family Hospital; Safed Medical School, Bar Ilan University, Nazareth 1641110, Israel. mmahamid@szmc.org.il.
authors.forename: Mahmud
authors.initials: M
authors.lastname: Mahamid
authors.affiliation: The Liver Unit, Gastroenterology Institute, Hadassah Medical Center, Hadassah Medical School, The Hebrew University, Jerusalem 9112001, Israel. nidaa@hadassah.org.il.
authors.forename: Nidaa
authors.initials: N
authors.lastname: Nator
authors.affiliation: The Liver Unit, Gastroenterology Institute, Hadassah Medical Center, Hadassah Medical School, The Hebrew University, Jerusalem 9112001, Israel. safadi@hadassah.org.il.|Liver Unit, Holy Family Hospital; Safed Medical School, Bar Ilan University, Nazareth 1641110, Israel. safadi@hadassah.org.il.
authors.forename: Rifaat
authors.initials: R
authors.lastname: Safadi
mesh_terms: D000293:Adolescent; D000328:Adult; D015415:Biomarkers / Q000097:blood; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D065626:Non-alcoholic Fatty Liver Disease / Q000097:blood*; D002787:Sterol Esterase / Q000097:blood*
chemical_list: D015415:Biomarkers; D002787:Sterol Esterase
keywords: cholesteryl ester storage disease; cirrhosis; lysosomal acid lipase; non-alcoholic liver disease; non-alcoholic steatohepatitis