Novel Association of Cholesterol Ester Storage Disease Due to Lysosomal Acid Lipase Deficiency and Non-Alcoholic Fatty Liver Disease: A Prospective Clinical Study

Non-alcoholic Fatty Liver Disease Clinical Trial

Clinical Trial Details — Status: Not yet recruiting

Administrative data

• NCT number: NCT01791452
• Other study ID #: 0042-12-ZIV
• Status: Not yet recruiting
• Phase: N/A
• First received: February 10, 2013
• Last updated: February 13, 2013

Study information

• Verified date: February 2013
• Source: Ziv Hospital
• Contact: Nimer Assy, M
• Phone: +972-4-6828442
• Email: assy.n[@]ziv.health.gov.il
• Is FDA regulated: No
• Health authority: Israel: Ministry of Health
• Study type: Observational

Clinical Trial Summary

Non-alcoholic fatty liver disease (NAFLD) is a world-wide problem with a global prevalence estimated at 1.5 billion people. It is characterised by significant diversity and phenotypic heterogeneity. Morbidity rates are estimated at 20% to 30% in Western adults, increasing to 90% in patients who are morbidly obese or diabetic. Risk factors in non-obese NAFLD patients are of especial practical and theoretical importance. Cholesterol Ester Storage Disease (CESD) is an autosomal recessive chronic disease of variable phenotype, caused by a deficiency in lysosomal acid lipase (LAL) and characterized by accumulation of fat in tissues and organs. Hepatic accumulation of fat in this disorder can cause hepatomegaly with varying degrees of damage varying from steatosis to fibrosis, elevated aminotransaminases, and isolated splenomegaly. Since the contribution of LAL deficiency to non-obese NAFLD is poorly understood, the investigators propose to evaluating the association between NAFLD and LAL deficiency in a prospective study in our population.

Recruitment information / eligibility

• Status: Not yet recruiting
• Enrollment: 0
• Est. primary completion date: February 2014
• Accepts healthy volunteers: No
• Gender: Both
• Age group: 18 Years to 80 Years

Eligibility

Inclusion Criteria:

• Age between 18-80 years;

• BMI 25-40;

• Fatty liver disease (bright liver, hepatomegaly by ultrasound (Liver span > 15 cm mid clavicle line), splenomegaly (>13 cm) or both.

Exclusion Criteria:

• Alcohol abuse>30 gm/day, or > 70 gram per week;

• Soft drink abuse;

• Drugs known to cause fatty liver;

• Chronic hepatitis (B and C);

• Biliary liver disease;

• Autoimmune hepatitis;

• HIV;

• Genetic/Metabolic liver disease (Wilson, alpha-1 antitrypsin, CF);

• Failure to give informed consent

Study Design

Time Perspective: Prospective

Related Conditions & MeSH terms

• Cholesterol Ester Storage Disease
• Fatty Liver
• Liver Diseases
• Non-alcoholic Fatty Liver Disease
• Wolman Disease

Locations

Country	Name	City	State
Israel	Assy Nimer	Safed

Sponsors (1)

Lead Sponsor	Collaborator
Assy Nimer

Country where clinical trial is conducted

Israel, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	liver ultrasound, ultrasound Doppler of the common carotid artery, hepatic Fibroscan evaluation (transient elastography) for assessment of steatosis and fibrosis		year