1B,C. Unlike the distinct description by Youssefian et al., (2019) of biallelic ABHD5 mutations causing autosomal recessive CDS and monoallelic ABHD5 mutations causing autosomal dominant NAFLD, we report the first cases of autosomal recessive NASH caused by biallelic ABHD5 missense mutations. This finding was not reported before in CDS, and should lead to the consideration of a lipid malabsorption or lipid storage disorder.[6] Mild CDS (homozygous mutations in ABHD5) should be excluded in unexplained NASH. [Extracted from the article]