Alglucosidase Alfa - Lumizyme-@- Enzyme - Inhibitor (May 2010)Drug Name:
Alglucosidase Alfa - Lumizyme-@- Enzyme - Inhibitor (May 2010)
List Of Brands:
Indication Type Description:
Dosages/ Overdosage Etc
Pregnancy and lactation
Pompe disease ( glycogenosis type 2 , acid maltase deficiency disease ) is an inherited
disorder of glycogen metabolism caused by the absence of marked deficiency of the lyosomal
enzyme acid glucosidase.
Cardiovascular- 62% bradycardia 21% tachycardia 23%
Dermatologic- 82% diaper dermatitis 36% rash 54% urticaria 21%
GI- 82% constipation 23% diarrhea 62% gastroenteritis 41%
gastroesophageal reflux disease 26% vomiting 49%
Haematologic/lymphatic - 44% anemia 31%
Respiratory- 97% cough 46% phraryngitis 36% pneumonia 46% respiratory distress 31%
tachypnea 23% upper respiratory tract infection 44%
Special senses- ear infection 33% nasopharyngitis 23% otitis media 44%
Miscellaneous - bronchiolitis 23% cather-related infection 28% flushing 21%
general disorders and administration site conditions 97%
infections and infestations 95% injury ,
poisioning and procedural complications 72%
oral candidiasis 31% oxygen saturation decreased 41%
post procedural pain 26% pyrexia 92% vascular disorders 36%
Cardiac effects- use caution when administering general anesthesia for placement of a central
venous catherter in an infantile- onset Pompe disease patients with cardiac hypertrophy.
Cardio-respiratory failure- acute cardio-respiratory failure requiring intubation and inotropic support has been reported after infusion with alglucerase alfa in infantile -onset Pomp disease with underlying cardiac myopathy, possibly associated with fluid overload with IV administration of alglucosidase alfa.
Infusion reactions- patients may have compromised cardiac and respiratory function, which may predispose them to a higher risk of severe complications from infusion reactions, Therefore monitor thes patients more closely during administration of alglucerase alfa.
Concomittant illness- patients with an acute underlying illness at the time of alglucosidase alfa infusion appear at greater risk for infusion reactions. Give careful consideration to the patients clinical status prior to administration of alglucosidase alfa
Hypersensitivity reactions- severe hypersensitivity reactions including anaphylactic reactions
have been reported during alglucosidase alfa infusion. Becuase of potential for severe
hypersensitivity reactions, make appropiate medical support available when administering
algucosidase alfa .
Pregnancy- there are no adequate and well controlled studies in pregnant woman. Because animal reproduction studies are not always predictive of human response, only use this drug during pregnancy if clearly required
Lactation-excercise caution when administering alglucosidae alfa to a breast feeding woman.
Children- risks and benefits of algulcosidase alfa have not been establihed in the juvenile-onset Pompe disease population
Elderly- Clinical studies did not include any subjects 65 years and older. It is not known whether thy respond differently than younger subjects.
Monitoring- evaluate liver enzymes prior to the initiation of aglucosidase alfa treatment and
periodically thereafter. Execrise care in interpreting these tests because aspartate
aminotransferase and alanine aminotransferase levels may be raised as a result of
muscle pathology with Pompe disease
Monitor vital signs at the end of each infusion increase
Dosages/ Overdosage Etc:
Recommended dosage- 20mg/kg/body weight administered every 2 weeks as an intraveous
infusion. The total volume is determined by the patients body weight and should be administrered over a period of 4 hours.
1. Inform patients and their caregivers that a registry for patients with Pompe disease has
been established in order to better understand the variability and progression of Pompe
disease and to continue to monitor and evaluate treatment
2. Encourage patients and their caregives to participate and advise them that participation
may involve long-term follow -up
Pregnancy and lactation:
There are no adequate and well controlled studies in pregnant woman. Because
animal reproduction studies are not always predictive of human response, only use this drug
during pregnancy if clearly required
Excercise caution when administering alglucosidae alfa to a breast feeding woman.
Risks and benefits of algulcosidase alfa have not been establihed in the juvenile-onset
Pompe disease population
Clinical studies did not include any subjects 65 years and older. It is not known whether
thy respond differently than younger subjects.