Lysosomal acid lipase activity in children with dyslipidemia and hepatic dysfunction

Abstract

Background: Lysosomal acid lipase (LAL) enzyme, is responsible for the hydrolysis of intracellular triacylglycerol and cholesterol esters. We investigate the LAL activity (LAL-A) in patients with hepatic dysfunction and/or dyslipidemia and determine the associated clinical and biochemical parameters. Methods: This prospective, cross-sectional study included 360 children (3 months -18 years; 40 control, and 320 screening patients). Demographic data, major clinical and laboratory findings, LAL-A and possible biomarkers were evaluated. Screening group was divided into two: LAL-A<0.6 nmol/ml/h (Group1); LAL-A ≥ 0.6 nmol/ml/h (Group 2). LAL-A predictive model was evaluated using logistic regression. Results: The mean LAL-A in the screening group (1.43 ± 2.05 (0.03-16.8) nmol/ml/h) was significantly reduced compare to controls (p < 0.001). No LAL deficiency was detected. There was a negative correlation between LAL-A and low-density lipoprotein cholesterol, triglyceride, and alanine aminotransferase (ALT) levels. LAL-A in patients with chronic fatigue (p = 0.002), hepatomegaly (p = 0.013) and splenomegaly (p = 0.001) were significantly lower compare to those without. The median thiobarbituric acid reactive substances, myeloperoxidase, chitotriosidase, hs-CRP, Citokeratin levels in Group 1 were higher compare to the controls (p < 0.005). Conclusions: LAL-A was reduced in paediatric patients with dyslipidemia and/or elevated transaminase. Our final multivariable predictive model for reduced LAL-A included: ALT, triglyceride, and hepatomegaly. *Correspondence to: Sema Kalkan Uçar, Department of Pediatrics, Division of Metabolism and Nutrition, Ege University Medical Faculty, Bornova, Izmir, Turkey, Tel: 90.232.390.1037, E-mail: semakalkan@hotmail.com