Alkane Storage Disease (Very Long Chain N-Alkanes): An Original Type of Lipid Storage of Dietary Origin from Plant Wax Hydrocarbons
We have recently reported a unique case of a new type of lipid storage disorder (Duboucher et al., 1986; Rocchiccioli et al., 1987) which we have named Alkane Storage Disease. This novel human morbid entity is characterized by the visceral accumulation of odd-numbered long chain n-alkanes. These alkanes are characteristic of vegetal cuticular waxes and differ from those present in mineral oil paraffin (Lester, 1979; Levade et al., 1987). The nature of the stored compounds, their visceral distribution and the clinical anamnesis strongly suggest a dietary origin. We also discuss the hypothetical metabolic defect which may cause the n-alkane accumulation and the pathophysiological mechanism explaining their tissular distribution.
KeywordsNile Blue Abdominal Lymph Node Dietary Origin Lipoid Pneumonia Exogenous Lipoid Pneumonia
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