Mucopolysaccharidosis

Cardiovascular -       dependent edema  9%      hypotension  9%     vein disorder 14%

CNS-                           hyperereflexia  14%     paresthesia  14%

Local -                        injection site pain  9%  

Miscellaneous-          abscess   9%    bilirubnemia  9%   chest pain  9%  corneal opacity 9%
                                    facial edema  9%        rash   9%     thrombocytopenia   9%
                                    upper respiratory tract infection  32%

Not known

Special Pecaution-

Pretreatment-  ensure that patients receive antipyretics and/or antihistamines prior to infusion
If an infusion reaction occurs regardless of pretreatment , decreasing the infusion rate, temporarily
stopping the infusion and/ or additional antipyretics and or antihistamines may amelorate
symptoms .

If severe hypersensitivity or anaphylactic reactions occur immediately discontinue infusion
of lardoniase and initiate appropriate treatment

Immunogenicity- the clinical significane of antibodies to laronidase including the potential  for
product neutralization is not known.

Hypersensitivity- life threatening anaphylactic reactions have been observed in some patients
during or upto 3 hours after lardoniase infusions. Recations included  respiratory failure ,
respiratory distress, stridor, tachypnea, bronchospasm, airway obstruction, hypotension,
bradycardia, urticaria

Consider the risks and benefits of readministrering laronidase following severe
hypersensitivity reactions

Pregnancy- use lardonidase during pregnancy only if needed

Lactation- excercise caution when laronodase is administred to a breast feeding woman

Children- not known if children younger than 5 years of age respond differently from older children.

Indication-
Mucopolysaccharidosis

Dosage-
0.58mg/kg once weekly as an intervenous infusion

Pretreatment with antipyretic is recommended and or antihistamine is recommended 60 minutes
prior to start of the infusion.

1. Inform patients that a registry for mucopolysaccharidosis I patients has been established in
   order to better understand the variability and progression of mucopolysaccharodosis I disease
   and to monitor and evaluate treatments.

2. Encourage patients to participate andadvice them of their participation invloves long-term
    follow up .  

Pregnancy-
Use lardonidase during pregnancy only if needed

Lactation-
Excercise caution when laronodase is administred to a breast feeding woman

Children-
Not known if children younger than 5 years of age respond differently from older children.