TABLE 2.

When to consider testing for less common causes of hepatic steatosis and steatohepatitis

Condition	Clinical scenario	Diagnostic test	Treatment
Hypobetalipoproteinemia	Low LDL, low triglycerides, fat malabsorption	ApoB level, genetic testing (MTTP, PCSK-9)	Low-fat diet, fat-soluble vitamin supplementation
LAL deficiency	Markedly elevated LDL-C and low HDL-C, elevated triglycerides, xanthelasma, hypersplenism, advanced fibrosis in young age, predominately microvesicular steatosis on liver biopsy	Enzyme assay, genetic testing	LAL replacement
Nutrient deficiency (eg, carnitine, choline)	Anorexia, short bowel, bypass surgeries	Nutrient levels	Supplementation
Wilson disease	Younger age, neuropsychiatric symptoms, low alkaline phosphatase, low ceruloplasmin	24-h urine copper; quantitative copper on liver biopsy	Chelation
Celiac disease	Iron deficiency, abdominal pain, bloating, vitamin D deficiency, bone loss, diarrhea, dermatitis herpetiformis	Tissue transglutaminase IgA, duodenal biopsy	Gluten-free diet

Abbreviations: ApoB, apolipoprotein B; HDL-C, high-density lipoprotein cholesterol; IgA, immunoglobulin A; LAL, lysosomal acid lipase; LDL-C, LDL cholesterol.