벨마나제알파

is indicated for the treatment of non-central nervous system manifestations of alpha-mannosidosis in adult and pediatric patients. LAMZEDE is recombinant human lysosomal alpha-mannosidase indicated for the treatment of non-central nervous system manifestations of alpha-mannosidosis in adult and pediatric patients. ( 1 )

None. None. ( 4 )

Infusion -Associ ated Reactions (IARs) : If severe IARs occur, discontinue LAMZEDE and initiate appropriate medical treatment. ( 5.2 ) Embryo - F etal Toxicity : May cause fetal harm. Advise females of reproductive potential to use effective contraception during treatment and for 14 days after the last dose if LAMZEDE is discontinued. ( 5.3 , 8.1 , 8.3 ) 5.1 Hypersensitivity Reactions Including Anaphylaxis Hypersensitivity reactions including anaphylaxis have been reported in LAMZEDE-treated patients. In clinical trials, 19 (50%) LAMZEDE-treated patients (5 adult patients and 14 pediatric patients) experienced hypersensitivity reactions, including 2 (5%) patients (1 adult patient and 1 pediatric patient) who experienced anaphylaxis and an additional 3 (8%) pediatric patients who experienced severe hypersensitivity reactions that required medical treatment [see Clinical Studies ( 14 )] . In the 5 patients who experienced anaphylaxis or severe hypersensitivity requiring medical treatment, 4 (80%) were anti-drug antibody (ADA) positive [see Clinical Pharmacology ( 12.6 ) ] . Anaphylaxis and severe hypersensitivity signs and symptoms included cyanosis, hypotension, emesis, urticaria, erythema, facial swelling, pyrexia, and tremor. Prior to LAMZEDE administration, consider pretreating with antihistamines, antipyretics, and/or corticosteroids. Appropriate medical support measures, including cardiopulmonary resuscitation equipment, should be readily available during LAMZEDE …

8.1 Pregnancy Risk Summary Based on findings from animal reproduction studies, LAMZEDE may cause embryo-fetal harm when administered to a pregnant female. In animal reproduction studies, major visceral malformations were observed in rats and rabbits when velmanase alfa-tycv was administered in pregnant rats and rabbits during the period of organogenesis. These malformations were observed in rats at the highest dose level, at exposures that were approximately 7-fold the recommended dose in patients of 1 mg/kg. Malformations occurred at all dose levels in rabbits with the highest dose exposures approximately 2.5-fold the recommended patient dose of 1 mg/kg ( see Data ) . There are no …