Neutral lipid storage disease---response to dietary intervention

Editor,—Neutral lipid storage disease (NLSD) is an autosomal recessive metabolic disorder characterised by a multisystem accumulation of neutral lipids (triglycerides). The patients with NLSD have congenital ichthyosis and variable systemic manifestations.1 The storage of cytoplasmic triglycerides in NLSD patients results either from a severe defect in the degradation of cytoplasmic triacyglycerols containing long chain fatty acids2 or rapid triacyglycerol resynthesis.3Based on these biochemical data, one might expect that a low fat diet poor in long chain fatty acids …