Indication:
Hunter syndrome
Adverse Reaction:
Cardiovascular- atrial abnormality 13% hypertension 25%
CNS- anxirty , irritability 13% headache 59%
Dermatologic- abcess 16% pruritic rash 13% pruritus 28% skin disorder 13%
urticaria 16%
GI- dyspesia 13%
Musculoskeletal - arthalgia 31% chest wall musculoskeletal pain 16%
musculoskeletal dysfunction 16%
Respiratory- wheezing 19%
Special senses- visual disturbance 22%
Miscellaneous- adverse reactions resulting from injury 13% infusion site edema 13%
limb pain 28% malaise 22% pyrexia 63%
superficial injury 13%
Contra-Indications:
Special Precautions-
Hypersensitivity reactions- anaphylactoid reactions which may be life-threatening have been
observed in some patients during idrursulfase infusions. patients with compromised respiratory
functions are at great risks. Therefore make appropiate medical support readily available during
the infusion.
Reactions included distress, hypoxia, hypotension, angioedema, or seizures,
Hunter Outcome survey- A Hunter Outcome Survey has been established to better understand
the variability and progression of Hunter syndrome in population a whole and monitor and
evaluate long-term treatment effects of Idursulfase
Pregnancy- give Irandurase to pregnant women only if required.
Lactation- excercise caution when idursulfase is administered to a breast feeding woman.
Children- safety and efficacy hav not been established in children younge than 5 years of age,
Dosages/ Overdosage Etc:
Indication-
Hunter syndrome
Dosage-
0.5mg/kg of body weight administered once weekly an an intraveonus IV infusion
The total volume of infusion should be administred over a period of 1 to 3 hours.
Patients may require longer infusion times because of infusion reactions , however
infusion should not exceed 8 hours.
Patient Information:
1. A Hunter Outcome Survey has been established to better understand the variablity and
progression of Hunter syndrome in the population and monitor and evaluate long term treatment
effects of Idursulfase
2. Patients should be encouraged to participate in this survey programme,
Pharmacology/ Pharmacokinetics:
Ref -Drug Facts and comparisons (2010)
Pharmacology-
Hunter syndrome is an X-linked recessive disease caused by insufficient levels of the lysosomal enzyme iduronate- 2-sulfatase. This cleaves the terminal 2-O- sulfate moieties from the glycosoaminoglycans GAG dermatan sulfate and heparin sulfate
Hunter syndrome GAG progressively accumulate in the lyosomes of a variety of cells , leading to cellular engorement . organomegaly, tissue destruction and organ dysfunction
Pharmacokinetics-
The pharmacokinetic parameters at the recommended dosage regimen
(indursulfase 0.5mg/kg) administered weekly as a 3 hour infusion) were determined
at weeks 1 and 27 in 10 patients 7.7 to 27 years of age. There were no apparent
differences in pharmacokinetics values between weeks 1 and 27
The details are-
Indursulfase Pharmacokinetics Parameters (Mean standard Deviation)
Pharmacokinetic Week 1 Week 27
parameters
Cmax (mcg/mL) 1.5(0.6) 1.1(0.3)
(max plasma conc)
AUC 9min*mcg/mL) 206(87) 169(55)
t 1/2 (min) 44(19) 48(21)
(terminal half life)
Cl (mL/min/kg) 3(1.2) 3.4(1)
(clearance)
V -vol of distrib 21(8) 25(9)
at steady state
Pregnancy and lactation:
Pregnancy-
Give Irandurase to pregnant women only if required.
Lactation-
Excercise caution when idursulfase is administered to a breast feeding woman.
Children-
Safety and efficacy hav not been established in children younge than 5 years of age,
Manufacturer Details