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Administration of iduronate sulfatase by plasma exchange to patients with the hunter syndrome: A clinical study

✍ Scribed by Brown, Frank R. ;Hall, Clara W. ;Neufeld, Elizabeth F. ;Munoz, Louis L. ;Braine, Hayden ;Andrzejewski, Stanley ;Camargo, Edwaldo E. ;Mark, Shirley A. ;Richard, James M. ;Moser, Hugo W. ;Herrmann, Jürgen

Book ID
102699285
Publisher
John Wiley and Sons
Year
1982
Tongue
English
Weight
595 KB
Volume
13
Category
Article
ISSN
0148-7299

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✦ Synopsis

Abstract

The Hunter syndrome (MPS II) is the only mucopolysaccharidosis in which there is appreciable activity of the deficient enzyme in normal plasma. We performed enzyme‐replacement treatment by plasma exchange in five Hunter syndrome children. Carefully monitoring the cardiovascular status, we administered monthly single plasma volume exchanges for a 3 to 8 mo period. The results indicate a substantial gain of enzyme activity, persisting with a t~50%~ = 19 ± 5 hr. The maximal level and persistence of increased enzyme activity did not change after repeated exchanges, suggesting that immune responses were not elicited. Despite this, no demonstrable clinical benefit was apparent when the study group was compared with an age‐matched control group of Hunter syndrome patients.

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