Nature and Significance of Pentosuria in Neuromuscular Disease

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Abstract
Quantitative excretion of free aldopentoses in 230 24-hour urine specimens from 55 patients with and without muscle studied by p-bromaniline acetate method. All patients on fruit-free diets during study. Mean excretion of total free aldo-pe:itoses/24 hours /kg body weight were (1) by patients with no muscle disease 3.6 [mu][image] for adults and 5.5 [mu][image] for children (under 15 years) and (2) by patients with myopathies 6.6 [mu][image] for adults and 9.6 [mu][image] for children. Differences between means of two groups of adults or children were statistically significant (P< 0.001 and < 0.01 respectively). Identities of pentoses excreted established by 29 2-dimensional paper chromatograms of 24-hour urines from 18 patients. All samples de-ionized before running by descending chromatography with water-saturated phenol and butanol-acetic acid-water as solvents. Visualization of spots was by aniline hydrogen phthalate, p-anisidine HCl, and orcinol trichloracetic acid reagents. Criteria for identification were Rf values in each dimension, coincidence of urine spots with those of added standards, and appearance of spots with differential color reagents in daylight and U-V light. 8 carbohydrate compounds identified in urine specimens. Majority contained glucose, xylose, arabinose and ribose; a few contained lactose, galactose, desoxyribose and a uronide (? glucuronic acid). No ketoses detected in regular or concentrated specimens with possible exception of fructose. No differences in patterns of excretion between group with and that without muscle disease were apparent. Semiquantitation of aldopentoses excreted carried out by spot-area method on 1-dimensional chromatograms (phenol) of 14 urine specimens from 12 patients. Arabinose predominated in urines from group with no muscle disease whereas ribose appeared to be significantly higher in specimens from group with muscle disease. The findings suggest that patients with muscular dystrophy, myotonia dystrophica, myasthenia gravis and muscular atrophy excrete significantly higher amounts of free aldopentoses/24 hours, largely accounted for as ribose, than patients with no muscle disease. No correlation of findings with clinical status of muscle disease patients could be established. Good correlation (P< 0.001) between [mu][image] pentoses excreted/24 hours /kg body weight and K exchanged/kg body weight (Ke/kg.) (as measure of residual functional muscle mass) demonstrated for 22 adult patients. Finding suggests that, as residual functional muscle mass is reduced, pentosuria, mostly as ribose, increases. Implications are discussed.

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