{'fraction_non_alphanumeric': 0.03221649484536082, 'fraction_numerical': 0.04252577319587629, 'mean_word_length': 5.090196078431372, 'pattern_counts': {'":': 0, '<': 0, '<?xml version=': 0, '>': 0, 'https://': 0, 'lorem ipsum': 0, 'www.': 0, 'xml': 0}, 'pii_count': 0, 'substrings_counts': 0, 'word_list_counts': {'cursed_substrings.json': 0, 'profanity_word_list.json': 0, 'sexual_word_list.json': 0, 'zh_pornsignals.json': 0}}
1589 NEUROLOGIC STABILITY AND OLIGOSACCHARIDE EXCRETION IN MANNOSIDOSIS

Mannosidosis is noteworthy among lysosomal storage diseases affecting the nervous system for having a low mortality and a stable clinical course. Comparative studies of oligosaccharides were carried out on serum and urine of a 31-year-old man with mannosidosis (Arch Neurol 34:45, 1977) whose neurologic status has been stable since the age of 8 years. A mannose-containing trisaccharide with two mannose and one N-acetyl glucosamine residues was predominant in urine (304 mg/1), in agreement with other reported cases. By use of a sensitive new high pressure chromatographic procedure, a very low level of this trisaccharide was detected in serum (0.1-0.4 nmol/ml), with respective urinary values from 161 to 558 nmol/ml. The urinary clearance of trisaccharide was on average 16.1 times the glomerular filtration rate of creatinine, suggesting that the renal tubular cell is the immediate source of urinary oligosaccharides. The substrate burden in brain is much less in mannosidosis (0.11 μmol/g; J Pediatr 75:360, 1969) than it is in fucosidosis (19.9 μmol/g; J Neurochem 27:733, 1976), despite the fact that both diseases reflect a failure to catabolize glycoproteins. Our data suggest that upon maturation of the kidney a mannose receptor is elaborated which, by removing mannose-rich oligosaccharides from the circulation, favors the elimination of excess oligosaccharides and lowers the substrate burden for brain. (Supported in part by USPHS grants HD 05515 and HD 04147)