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October 1977


Author Affiliations

Eunice Kennedy Shriver Center for Mental Retardation, Inc 200 Tropelo Rd Waltham, MA 02154

Arch Neurol. 1977;34(10):650-651. doi:10.1001/archneur.1977.00500220084023

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In Reply.—  The reports by Booth et al (J Pediatr 88:821-824, 1976) and Aylsworth et al (J Pediatr 88:814-818, 1976) were published after our manuscript had been submitted. The siblings reported by Loeb et al (Acta Paediatr Scand 58:220-228, 1969) were originally judged to be an example of atypical mucopolysaccharidosis (mucolipidosis I), and the diagnosis was subsequently changed to mannosidosis (Acta Paediatr Scand 64:865-867, 1975) after deficient acid α-mannosidase in cultured skin fibroblasts was found, along with mannose-rich oligosaccharides in urine.We agree with Drs Loeb and Vamos-Hurwitz that mannosidosis is likely to be more common than previously thought, and it was this notion that prompted the publication of our two unrelated cases. Particular attention was called to the nonprogressive course of case 1, necessitating the consideration of mannosidosis in all mentally retarded individuals with idiopathic gingival hyperplasia.We have completed a trial of dietary zinc supplement based on our published observation showing

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