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.
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.

💊 Preparations

Proprietary Preparations

USA: Adagen.
Published May 08, 2019.