UNSUCCESSFUL DIETARY-TREATMENT OF SJOGREN-LARSSON SYNDROME

被引:19
作者
MAASWINKELMOOIJ, PD
BROUWER, OF
RIZZO, WB
机构
[1] LEIDEN UNIV HOSP, DEPT NEUROL, 2333 AA LEIDEN, NETHERLANDS
[2] VIRGINIA COMMONWEALTH UNIV MED COLL VIRGINIA, DEPT PEDIAT, RICHMOND, VA USA
[3] VIRGINIA COMMONWEALTH UNIV MED COLL VIRGINIA, DEPT HUMAN GENET, RICHMOND, VA USA
关键词
D O I
10.1016/S0022-3476(05)81369-1
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
We treated five children with Sjogren-Larsson syndrome. The patients, 5 months to 8 years of age, were given a low fat diet supplemented with medium-chain fatty acids. Plasma octadecanol levels remained unchanged, and skin lesions and neurologic symptoms did not abate. Two patients also failed to respond to dietary supplementation with polyunsaturated fatty acids. We conclude that dietary therapy is usually unsuccessful in patients with Sjogren-Larsson syndrome even when started in early infancy.
引用
收藏
页码:748 / 750
页数:3
相关论文
共 7 条
[1]   SJOGREN-LARSSON SYNDROME - REPORT OF 2 CASES IN TWINS [J].
GUILLEMINAULT, C ;
HARPEY, JP ;
LAFOURCADE, J .
NEUROLOGY, 1973, 23 (04) :367-373
[2]   DIGESTION OF HUMAN-MILK LIPIDS - PHYSIOLOGIC SIGNIFICANCE OF SN-2 MONOACYLGLYCEROL HYDROLYSIS BY BILE SALT-STIMULATED LIPASE [J].
HERNELL, O ;
BLACKBERG, L .
PEDIATRIC RESEARCH, 1982, 16 (10) :882-885
[3]  
HOOFT C, 1967, HELV PAEDIATR ACTA, V5, P447
[4]   SJOGREN-LARSSON SYNDROME - INHERITED DEFECT IN THE FATTY ALCOHOL CYCLE [J].
RIZZO, WB ;
DAMMANN, AL ;
CRAFT, DA ;
BLACK, SH ;
TILTON, AH ;
AFRICK, D ;
CHAVESCARBALLO, E ;
HOLMGREN, G ;
JAGELL, S .
JOURNAL OF PEDIATRICS, 1989, 115 (02) :228-234
[5]   SJOGREN-LARSSON SYNDROME - IMPAIRED FATTY ALCOHOL OXIDATION IN CULTURED FIBROBLASTS DUE TO DEFICIENT FATTY ALCOHOL - NICOTINAMIDE ADENINE-DINUCLEOTIDE OXIDOREDUCTASE ACTIVITY [J].
RIZZO, WB ;
DAMMANN, AL ;
CRAFT, DA .
JOURNAL OF CLINICAL INVESTIGATION, 1988, 81 (03) :738-744
[6]   SJOGREN-LARSSON SYNDROME - DEFICIENT ACTIVITY OF THE FATTY ALDEHYDE DEHYDROGENASE COMPONENT OF FATTY ALCOHOL NAD+ OXIDOREDUCTASE IN CULTURED FIBROBLASTS [J].
RIZZO, WB ;
CRAFT, DA .
JOURNAL OF CLINICAL INVESTIGATION, 1991, 88 (05) :1643-1648
[7]  
WILLIAMS ML, 1991, ADV LIPID RES, V24, P211