HUNTER’S SYNDROME

HUNTER’S SYNDROME

Inherited as x-linked, it resembles type l but is less severe. Clouding of cornea does not occur but deafness is frequently present.

CLINICAL FEATURES

Normal intelligence. The neck is short. The sternum is bulging forward and protuberant. Kyphosis, long arms, genu valgum, flat feet, waddling gait, dwarfism are the other features. Face and skull changes produce characteristic appearance. Hypoplasia of odontoid process leads to atlantoaxial sub-luxation and inability to hold the head. Keratan sulphate is excreted in urine.