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Neutral lipid storage disease—response to dietary intervention
  1. TALIA KAKOUROU,
  2. EURIDIKI DROGARI,
  3. HELEN CHRISTOMANOU

    *

    ,
  4. AGLAEA GIANNOULIA,
  5. CATHERINE DACOU-VOUTETAKIS
  1. First Department of Paediatrics
  2. Athens University
  3. Aghia Sophia Children’s Hospital
  4. Athens 11527, Greece and the
  5. *Neurochemistry and Molecular Biology Laboratory, Athens

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    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 …

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