Recombinant

REBINYN, Coagulation Factor IX (Recombinant), GlycoPEGylated, is a recombinant DNA-derived coagulation Factor IX concentrate indicated for use in adults and children with hemophilia B (congenital Factor IX deficiency) for: • On-demand treatment and control of bleeding episodes • Perioperative management of bleeding • Routine prophylaxis to reduce the frequency of bleeding episodes Limitations of Use : REBINYN is not indicated for immune tolerance induction in patients with hemophilia B. …

• For intravenous infusion after reconstitution only
• For intravenous infusion after reconstitution only . • Each carton and vial label for REBINYN states the actual Factor IX potency in international units (IU)
• On-demand treatment and control of bleeding episodes: • 40 IU/kg body weight for minor and moderate bleeds, and 80 IU/kg body weight for major bleeds
• Additional doses of 40 IU/kg can be given
• Perioperative management: • Pre-operative dose of 40 IU/kg body weight for minor surgery, and 80 IU/kg body weight for major surgery
• As clinically needed for the perioperative management of bleeding, repeated doses of 40 IU/kg (in 1-3 day intervals) within the first week after major surgery may be administered. • Frequency may be extended to once weekly after the first week until bleeding stops and healing is achieved
• Routine prophylaxis : • 40 IU/kg body weight once weekly
• Dosing Guidelines • Dose and duration of treatment depend on the location and extent of bleeding, and the patient’s clinical condition. • If monitoring of Factor IX activity is performed, use a chromogenic assay or selected one-stage clotting assay validated for use with REBINYN [ see Warnings and Precautions ]. • Each carton and vial label for REBINYN states the actual Factor IX potency in IU
• On-demand Treatment and Control of Bleeding Episodes REBINYN
• Dosing for on-demand treatment and control of bleeding episodes is provided in Table 1. …

REBINYN is available as a white to off-white lyophilized powder in single-dose vials containing nominally 500, 1000, 2000, or 3000 IU per vial. Each carton and vial label for REBINYN states the actual Factor IX potency in IU. After reconstitution with 4 mL of histidine diluent, the reconstituted solution contains approximately 125, 250, 500, or 750 IU per mL of REBINYN respectively. …

• Safety and efficacy of REBINYN were evaluated in four clinical trials that included 43 PTPs and in one clinical trial that included 50 pediatric PUPs [ see Adverse Reactions and Clinical Studies ]
• Twelve of these subjects were ≤ 6 years of age; 13 subjects were 7 to 12 years of age; and 18 subjects were 13 to 17 years of age
• Pharmacokinetic parameters were evaluated for 28 of the pediatric PTPs who were treated with REBINYN 40 IU/kg [ see Clinical Pharmacology ]
• Body weight-adjusted clearance was observed to be higher for pediatric subjects than for adult subjects. …

Clinical studies of REBINYN did not include sufficient numbers of subjects age 65 and over to determine whether or not they respond differently than younger subjects. Animals administered repeat doses of REBINYN showed accumulation of PEG in the choroid plexus, pituitary, circumventricular organs, and cranial motor neurons. [ see Use in Specific Populations ( 8.4 ) and Animal Toxicology and/or Pharmacology ( 13.2 ) ]. …

• Advise patients to read the FDA-approved patient labeling (Patient Information and Instructions for Use). • Inform patients of the early signs of hypersensitivity reactions including rash, hives, itching, facial swelling, tightness of the chest and wheezing
• Advise patients to discontinue use of the product and contact their healthcare provider if these symptoms occur. • Advise patients to contact their healthcare provider for further treatment and/or assessment if they experience a lack of a clinical response to Factor IX therapy, as in some cases this may be a manifestation of an inhibitor. …

REBINYN is contraindicated in patients who have known hypersensitivity to REBINYN or its components (including hamster proteins) [ see Warnings and Precautions ( 5.1 ) and Description ( 11 ) ] Do not use in patients who have known hypersensitivity to REBINYN or its components, including hamster proteins ( 4 ).

• Hypersensitivity reactions, including anaphylaxis, have occurred. Should hypersensitivity reactions occur, discontinue REBINYN and administer appropriate treatment ( 5.1 ). • Neutralizing antibodies (inhibitors) to Factor IX have occurred following administration of REBINYN. Perform an assay that measures Factor IX inhibitor concentration if bleeding is not controlled with the recommended dose of REBINYN or if plasma Factor IX activity level fails to increase as expected (5.2 , 5.5 ). • The use of Factor IX- products has been associated with the development of thromboembolic complications ( 5.3 ). …

Common adverse reactions (incidence ≥ 1%) in PTPs reported in clinical trials for REBINYN were itching and injection site reactions. Common adverse reactions (incidence ≥ 1%) in PUPs reported in clinical trials for REBINYN were rash, FIX inhibitors, hypersensitivity, itching, injection site reaction, and anaphylactic reaction. The most frequently reported adverse reactions (≥ 1%) in previously treated patients (PTPs) and previously untreated patients (PUPs) were itching and injection site reactions ( 6 ). Additional frequently reported adverse reactions (≥ 1%) in PUPs included rash, Factor IX inhibition, hypersensitivity, and anaphylactic reaction ( 6 ). …

8.1 Pregnancy Risk Summary There are no data with REBINYN use in pregnant women to determine whether there is a drug-associated risk. Animal reproduction studies have not been conducted with REBINYN. It is unknown whether REBINYN can cause fetal harm when administered to a pregnant woman or can affect fertility. In the U.S. …

تناولها عندما تتذكر، إلا إذا اقترب موعد الجرعة التالية. عندها تخطَّ الجرعة المنسية. لا تتناول جرعتين معًا أبدًا للتعويض. راجع النشرة، فبعض الأدوية مختلفة.