INTRAUTERINE GROWTH ACCELERATION IN THE CASE OF A SEVERE FORM OF MUCOPOLYSACCHARIDOSIS TYPE-VII

We describe a Japanese male infant with mucopoly-saccharidosis type VI I (MPS VII) who was born at 32 weeks of gestation presenting as a non- immune hydrops fetalis. His birth weight was 2900 g (+ 4.1 SD), his bi rth length was 48 cm (+ 2.2 SD), and thoracic spine length was 9.5 cm (+ 2.7 SD) at birth. Ossification center was already discernible bilat erally in coracoid process and distal femur at birth. In eight newborn infants with fetal hydrops of various other etiologies born in our in stitution, birth length ranged from -0.2 to -1.5 SD and thoracic spine length ranged from +0.5 to -1.7 SD, and neither ossification center o f coracoid process nor distal femur were visible. The above data indic ate that hydrops fetalis form of MPS VII is associated with intrauteri ne growth acceleration accompanied by early bone maturation. This feat ure may be useful in differentiating MPS VII associated hydrops fetali s from those of other etiologies.