Clinical aspects and adrenal functions in eleven Japanese children with X-linked adrenoleukodystrophy.
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X-linked adrenoleukodystrophy (X-ALD) is a genetic disease associated with demyelination of the central nervous system, adrenocortical insufficiency and accumulation of very long chain fatty acids. It is a clinically heterogeneous disorder ranging from a severe childhood cerebral form to an asymptomatic form. The incidence in Japan is estimated to be between 1:30,000 and 1:50,000 boys as determined by a nationwide retrospective survey between 1990 and 1999, which found no cases with Addison's form. We reviewed the medical records of eleven Japanese boys with X-ALD from 1990 to 2010 in our institute. Eight patients were detected by neuropsychological abnormalities, whereas a higher prevalence of unrecognized adrenocortical insufficiency (5/11: 45%) was observed than previously recognized. While no neurological abnormalities were demonstrated in two brothers, the elder brother had moderate Addison's disease at diagnosis and the presymptomatic younger brother progressed to Addison's disease six months after the diagnosis of X-ALD. Early detection of impaired adrenal function as well as early identification of neurologically presymptomatic patients by genetic analysis is essential for better prognosis. Addison's form might be overlooked in Japan; therefore, X-ALD should be suspected in patients with adrenocortical insufficiency. (+info)
Are the n-3 fatty acids from dietary fish oil deposited in the triglyceride stores of adipose tissue?
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Adipose tissue is the chief reservoir of the essential fatty acids (n-3 and n-6). To study the incorporation of the dietary n-3 fatty acids eicosapentaenoic acid (EPA) (20:5) and docosahexaenoic acid (DHA) (22:6), and a unique monounsaturated fatty acid, cetoleic acid (22:1n-11), into adipose tissue, rabbits were fed two different processed fish oils: MaxEPA (high in EPA and DHA; Seven Sea Ltd, Hull, UK) and herring oil (high in cetoleic acid). EPA and DHA increased from 0% of total adipose tissue fatty acid, in the adipose tissue of control rabbits to 2.2% and 4.9%, respectively, in MaxEPA-fed rabbits. The DHA-to-EPA ratio in the adipose tissue was higher than that in the diet, indicating alternative metabolic pathways for EPA. In the adipose tissue of herring-oil-fed rabbits, cetoleic acid increased from 0% to 7.9% of total fatty acids. The deposition of EPA and DHA was 1.8% and 2.8%, respectively. Our data indicated that these unique long-chain unsaturated fatty acids from dietary fish oils were readily incorporated into the fat stores from whence they could be mobilized. (+info)
Treatment of an adrenomyeloneuropathy patient with Lorenzo's oil and supplementation with docosahexaenoic acid--a case report.
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A mixture of oleic, erucic and conjugated linoleic acids modulates cerebrospinal fluid inflammatory markers and improve somatosensorial evoked potential in X-linked adrenoleukodystrophy female carriers.
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Gas chromatographic separation of docosenoic acid positional isomers on an SLB-IL100 ionic liquid column.
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Gas chromatography (GC) of docosenoic acid (22:1) has been performed for the separation of positional isomers on the novel SLB-IL100 column with a highly polar ionic liquid stationary phase. A test mixture of 22:1 methyl esters prepared from total lipids of flounder was subjected to GC on a 60 mx0.32 mm i.d. column at an isothermal temperature of 150 degrees C-180 degrees C. On this column, all five positional isomers separated in the elution order of 22:1n-15, 22:1n-13, 22:1n-11, 22:1n-9, and 22:1n-7. The positional isomers, 22:1n-15, 22:1n-13, and 22:1n-11, unresolvable on conventional polar polymer phase columns, were almost completely separated from each other within 24 min at 170 degrees C. The equivalent chain length values of 22:1n-11 to 22:1n-7 were parallel to those on polyethylene glycol and cyanopropyl polysiloxane columns, whereas 22:1n-15 and 22:1n-13 were relatively lower and closer to saturated 22:0 acid. Similar findings were also obtained for co-injected 20:1n-15 to 20:1n-11 isomers. Analysis of fish 22:1 revealed that 22:1n-13 is not always a minor isomer, as previously reported for several samples. The results of this study confirm the view that SLB-IL100 is a powerful tool for GC analysis of monounsaturated fatty acids. (+info)