No

Enzyme

Enzyme replacement therapy for adenosine deaminase (ADA) deficiency in patients with severe combined immunodeficiency disease (SCID) who can not benefit from bone marrow transplant; not a cure for SCID, unlike bone marrow transplants, injections must be used the rest of the child's life, therefore is not really an alternative

C

Hypersensitivity to pegademase bovine; not to be used as preparatory or support therapy for bone marrow transplantation

Use with caution in patients with thrombocytopenia

<1%: Headache, pain at injection site

Decreased effect: Vidarabine

Refrigerate at 2°C to 8°C (36°F to 46°F); do not freeze

Adenosine deaminase is an enzyme that catalyzes the deamination of both adenosine and deoxyadenosine. Hereditary lack of adenosine deaminase activity results in severe combined immunodeficiency disease, a fatal disorder of infancy characterized by profound defects of both cellular and humoral immunity. It is estimated that 25% of patients with the autosomal recessive form of severe combined immunodeficiency lack adenosine deaminase.

Plasma adenosine deaminase activity generally normalizes after 2-3 weeks of weekly I.M. injections

Absorption: Rapid

Half-life: 48-72 hours

Children: I.M.: Dose given every 7 days, 10 units/kg the first dose, 15 units/kg the second dose, and 20 units/kg the third dose; maintenance dose: 20 units/kg/week is recommended depending on patient's ADA level; maximum single dose: 30 units/kg

No information available to require special precautions

No effects or complications reported

Not a cure for SCID; unlike bone marrow transplants, injections must be used the rest of the child's life; frequent blood tests are necessary to monitor effect and adjust the dose as needed

Not a cure for SCID; unlike bone marrow transplants, injections must be used the rest of the child's life; frequent blood tests are necessary to monitor effect and adjust the dose as needed

Injection: 250 units/mL (1.5 mL)