Pegademase
(rINN)
Synonyms: PEG-ADA; Pegademasa; Pégadémase; Pegademasum; PEG-Adenosine Deaminase.
Cyrillic synonym: Пэгадемаза.
💊 Chemical information
ATC — L03AX04.
ATC Vet — QL03AX04.
NOTE. Pegademase Bovine is USAN.
💊 Profile
Pegademase is a conjugate of adenosine deaminase, an endogenous enzyme that converts adenosine to inosine, with a macrogol (polyethylene glycol). Pegademase bovine is used in the treatment of severe combined immunodeficiency disease (SCID) associated with a deficiency of adenosine deaminase, in patients who are not suitable for bone marrow transplantation or in whom the transplantation has failed. It is given by intramuscular injection once every 7 days, in an initial dose of 10 units/kg; increments of 5 units/kg are then given weekly up to a usual weekly maintenance dose of 20 units/kg. A single dose of 30 units/kg should not be exceeded. Pegademase should be given with caution to patients with thrombocytopenia and avoided if the latter is severe.
◊ References.
1. Hershfield MS, et al. Treatment of adenosine deaminase deficiency with polyethylene glycol-modified adenosine deaminase. N Engl J Med 1987; 316: 589–96
2. Anonymous. Pegademase. Med Lett Drugs Ther 1990; 32: 87–8
3. Lee CR, et al. Pegademase bovine: replacement therapy for severe combined immunodeficiency disease. DICP Ann Pharmacother 1991; 25: 1092–5
4. Shovlin CL, et al. Adult presentation of adenosine deaminase deficiency. Lancet 1993; 341: 1471
5. Hershfield MS. Adenosine deaminase deficiency: clinical expression, molecular basis, and therapy. Semin Hematol 1998; 35: 291–8
6. Husain M, et al. Burkitt’s lymphoma in a patient with adenosine deaminase deficiency-severe combined immunodeficiency treated with polyethylene glycol-adenosine deaminase. J Pediatr 2007; 151: 93–5.
2. Anonymous. Pegademase. Med Lett Drugs Ther 1990; 32: 87–8
3. Lee CR, et al. Pegademase bovine: replacement therapy for severe combined immunodeficiency disease. DICP Ann Pharmacother 1991; 25: 1092–5
4. Shovlin CL, et al. Adult presentation of adenosine deaminase deficiency. Lancet 1993; 341: 1471
5. Hershfield MS. Adenosine deaminase deficiency: clinical expression, molecular basis, and therapy. Semin Hematol 1998; 35: 291–8
6. Husain M, et al. Burkitt’s lymphoma in a patient with adenosine deaminase deficiency-severe combined immunodeficiency treated with polyethylene glycol-adenosine deaminase. J Pediatr 2007; 151: 93–5.
💊 Preparations
Proprietary Preparations
USA: Adagen.
Published May 08, 2019.