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Pronunciation |
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(peg
A de mase BOE
vine) |
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U.S. Brand
Names |
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Adagen™ |
|
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Generic
Available |
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No |
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Pharmacological Index |
|
Enzyme |
|
|
Use |
|
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 |
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Pregnancy Risk
Factor |
|
C |
|
|
Contraindications |
|
Hypersensitivity to pegademase bovine; not to be used as preparatory or
support therapy for bone marrow transplantation |
|
|
Warnings/Precautions |
|
Use with caution in patients with thrombocytopenia |
|
|
Adverse
Reactions |
|
<1%: Headache, pain at injection site |
|
|
Drug
Interactions |
|
Decreased effect: Vidarabine |
|
|
Stability |
|
Refrigerate at 2°C to 8°C
(36°F to 46°F); do not
freeze |
|
|
Mechanism of
Action |
|
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. |
|
|
Pharmacodynamics/Kinetics |
|
Plasma adenosine deaminase activity generally normalizes after 2-3 weeks of
weekly I.M. injections
Absorption: Rapid
Half-life: 48-72 hours |
|
|
Usual Dosage |
|
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 |
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|
Dental Health: Local
Anesthetic/Vasoconstrictor
Precautions |
|
No information available to require special precautions |
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|
Dental Health:
Effects on Dental Treatment |
|
No effects or complications reported |
|
|
Patient
Information |
|
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 |
|
|
Nursing
Implications |
|
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 |
|
|
Dosage Forms |
|
Injection: 250 units/mL (1.5
mL) |
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