A new human pathology with visceral accumulation of long-chain n-alkanes; tissue distribution of the stored compounds and pathophysiological hypotheses.
This report deals with a new human disorder characterized by the accumulation of plant long-chain n-alkanes in viscera of a human patient. Lipid analysis of tissues from an adult male after sudden death (affected with diffuse visceral granuloma containing lipophilic crystallized material) showed the presence of abnormal compounds identified as long-chain n-alkanes with 29 (n-nonacosane), 31 (n-hentriacontane) and 33 carbons (n-tritriacontane). Study of n-alkane distribution in patient tissues showed a major accumulation in lumbo-aortic lymph nodes, adrenal glands, lung (the highest levels were found in lung granulomas) and liver; significantly lower amounts were detected in myocardium and kidney, whereas no detectable level was found in brain. On the basis of the structural composition and of the tissue distribution of the accumulated n-alkanes, their dietary (plant) origin and the pathophysiological mechanism of the storage are discussed.
Salvayre
R
R
Laboratoire de Biochimie Médicale et INSERM 101 faculté de Médecine, Toulouse, France.
Nègre
A
A
Rocchiccioli
F
F
Duboucher
C
C
Maret
A
A
Vieu
C
C
Lageron
A
A
Polonovski
J
J
Douste-Blazy
L
L
D000311:Adrenal Glands / Q000378:metabolism; D000473:Alkanes / Q000032:analysis / Q000378:metabolism*; D002849:Chromatography, Gas; D002855:Chromatography, Thin Layer; D004032:Diet; D006099:Granuloma / Q000209:etiology* / Q000378:metabolism / Q000473:pathology; D006801:Humans; D008055:Lipids / Q000032:analysis; D008099:Liver / Q000378:metabolism; D008168:Lung / Q000378:metabolism; D008171:Lung Diseases / Q000209:etiology / Q000378:metabolism / Q000473:pathology; D008198:Lymph Nodes / Q000378:metabolism; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D010945:Plants, Edible; D014018:Tissue Distribution
D000473:Alkanes; D008055:Lipids