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.
Highlights
Fatty liver has become the most common liver disorder [1] and is recognized as a major health burden in the Western world
Seventy-four patients diagnosed with cirrhosis according to the International Classification of Diseases 9 (ICD9) classification, and having an available liver biopsy were identified
The 22 patients in the study were analyzed as one group and as two groups, designated as higher-risk for lysosomal acid lipase (LAL)-d (13 patients, nine with microvesicular steatosis and four with cryptogenic cirrhosis) and lower-risk for lysosomal acid lipase deficiency (LAL-d)
Summary
Fatty liver has become the most common liver disorder [1] and is recognized as a major health burden in the Western world. The spectrum of histological abnormalities includes simple steatosis (steatosis without other liver injuries) and nonalcoholic steatohepatitis in its more extreme forms [2]. Over 30% of adults in developed countries suffer from hepatic fat accumulation [3]. Among these patients, 60% are diabetic, obese or morbidly obese [3,4,5]. The earliest stage of nonalcoholic fatty liver disease (NAFLD) consists of hepatic steatosis or lipid deposition in the cytoplasm of hepatocytes [6,7]. Hepatic steatosis may progress to the more aggressive necro-inflammatory form of NAFLD, nonalcoholic steatohepatitis (NASH) [2]. It is still unclear what leads to the progression from simple steatosis to advanced liver disease. In some cases hepatic steatosis is merely a marker for other diseases, such as microvesicular steatosis in metabolic diseases [10] and in viral hepatitis [11]
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