Oral zinc therapy in the treatment of alpha-mannosidosis.
Human alpha-mannosidosis is a lysosomal storage disorder characterized by mental retardation, dysostosis multiplex, and hepatosplenomegaly. Deficiency of the enzyme leads to accumulation of mannose-rich glycoconjugates in tissues. Zinc sulphate has been shown to stimulate alpha-mannosidase activity in vitro. Oral zinc therapy was attempted on a 4-year-old boy with alpha-mannosidosis for 3 years. After almost 10 years of follow-up on and off zinc therapy, we must conclude that oral zinc does not substantially affect the clinical course of alpha-mannosidosis.
Wong
L T
LT
Department of Pediatrics, B.C.'s Children's Hospital, Vancouver, Canada.
Vallance
H
H
Savage
A
A
Davidson
A G
AG
Applegarth
D
D
D000284:Administration, Oral; D002675:Child, Preschool; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D009844:Oligosaccharides / Q000652:urine; D013997:Time Factors; D015032:Zinc / Q000008:administration & dosage*; D008363:alpha-Mannosidosis / Q000188:drug therapy* / Q000652:urine
D009844:Oligosaccharides; D015032:Zinc