PRETREATMENT WITH ANTIPYRETICS AND/OR ANTIHISTAMINES IS RECOMMENDED 60 MINUTES PRIOR TO THE START OF THE INFUSION.
FOR PATIENTS 5 YEARS OF AGE AND OLDER, IT IS GIVEN BY INTRAVENOUS INFUSION IN A DOSE OF 100 UNITS/KG EACH WEEK. THE INITIAL INFUSION RATE IT IMPROVE PULMONARY FUNCTION AND WALKING CAPACITY BUT HAS NOT BEEN EVALUATED FOR EFFECTS ON THE CNS MANIFESTATIONS OF THE DISORDER. SHOULD BE STARTED AT 2 UNITS/KG PER HOUR, INCREASED EVERY 15 MINUTES DURING THE FIRST HOUR, AS TOLERATED, TO A MAXIMUM OF 43 UNITS/KG PER HOUR SUCH THAT THE INFUSION IS COMPLETED IN ABOUT 3 TO 4 HOURS (BUT SEE ALSO UNDER ADVERSE EFFECTS, TREATMENT, AND PRECAUTIONS ). IN SOME COUNTRIES, THE DOSE IS EXPRESSED AS MG/KG: 100 UNITS IS EQUIVALENT TO ABOUT 0.58 MG OF LARONIDASE.
HAEMATOPOIETIC STEM-CELL TRANSPLANTATION USING BONE MARROW OR UMBILICAL CORD BLOOD IS OF BENEFIT IN SYSTEMIC DISEASE AND CAN PREVENT (BUT NOT USUALLY REVERSE) CNS DETERIORATION.