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Kuvan
Overview
What is Kuvan?
Kuvan (sapropterin dihydrochloride) is an orally administered Phenylalanine Hydroxylase activator (or PAH activator). Sapropterin dihydrochloride, the active pharmaceutical ingredient in Kuvan, is a synthetic preparation of the dihydrochloride salt of naturally occurring tetrahydrobiopterin (BH4). Sapropterin dihydrochloride is an off-white to light yellow crystals or crystalline powder.
The chemical name of sapropterin dihydrochloride is (6R)-2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-5,6,7,8-tetrahydro-4(1H)-pteridinone dihydrochloride and the molecular formula is CHNO·2HCl with a molecular weight of 314.17.
Sapropterin dihydrochloride has the following structural formula:
Kuvan is supplied as tablets and powder for oral solution containing 100 mg of sapropterin dihydrochloride (equivalent to 76.8 mg of sapropterin base). Kuvan is also supplied as powder for oral solution containing 500 mg of sapropterin dihydrochloride (equivalent to 384 mg of sapropterin base).
Tablets are round, off-white to light yellow, mottled, and debossed with “177”. Each tablet contains the following inactive ingredients: ascorbic acid (USP), crospovidone (NF), dibasic calcium phosphate (USP), D-mannitol (USP), riboflavin (USP), and sodium stearyl fumarate (NF).
Kuvan powder for oral solution is off-white to yellow in color. Each unit dose packet contains the following inactive ingredients: ascorbic acid (USP), D-mannitol (USP), potassium citrate (USP), and sucralose (NF).%3
What does Kuvan look like?
What are the available doses of Kuvan?
Kuvan tablets are for oral use. Each tablet contains 100 mg of sapropterin dihydrochloride (equivalent to 76.8 mg of sapropterin base). Tablets are round, off-white to light yellow, mottled, and debossed with “177”.
Kuvan powder for oral solution is available as a unit dose packet containing 100 mg of sapropterin dihydrochloride (equivalent to 76.8 mg of sapropterin base) and as a unit dose packet containing 500 mg of sapropterin dihydrochloride (equivalent to 384 mg of sapropterin base). The powder is off-white to yellow in color.
What should I talk to my health care provider before I take Kuvan?
How should I use Kuvan?
Kuvan(sapropterin dihydrochloride) is indicated to reduce blood phenylalanine (Phe) levels in patients with hyperphenylalaninemia (HPA) due to tetrahydrobiopterin- (BH4-) responsive Phenylketonuria (PKU). Kuvan is to be used in conjunction with a Phe-restricted diet.
Patients 1 month to 6 years: The recommended starting dose of Kuvan is 10 mg/kg taken once daily.
Patients 7 years and older: The recommended starting dose of Kuvan is 10 to 20 mg/kg taken once daily.
If a 10 mg/kg per day starting dose is used, then response to therapy is determined by change in blood Phe following treatment with Kuvan at 10 mg/kg per day for a period of up to 1 month. Blood Phe levels should be checked after 1 week of Kuvan treatment and periodically for up to a month. If blood Phe does not decrease from baseline at 10 mg/kg per day, the dose may be increased to 20 mg/kg per day. Patients whose blood Phe does not decrease after 1 month of treatment at 20 mg/kg per day are non-responders and treatment with Kuvan should be discontinued in these patients.
If a 20 mg/kg per day starting dose is used, then response to therapy is determined by change in blood Phe following treatment with Kuvan at 20 mg/kg per day for a period of 1 month. Blood Phe levels should be checked after 1 week of Kuvan treatment and periodically during the first month. Treatment should be discontinued in patients who do not respond to Kuvan.
Once responsiveness to Kuvan has been established, the dosage may be adjusted within the range of 5 to 20 mg/kg per day according to response to therapy. Periodic blood Phe monitoring is recommended to assess blood Phe control
What interacts with Kuvan?
Sorry No Records found
What are the warnings of Kuvan?
Sorry No Records found
What are the precautions of Kuvan?
Sorry No Records found
What are the side effects of Kuvan?
Sorry No records found
What should I look out for while using Kuvan?
None.
What might happen if I take too much Kuvan?
Two unintentional overdosages with Kuvan have been reported. One adult patient in a Kuvan clinical trial received a single Kuvan dose of 4,500 mg (36 mg/kg) instead of 2,600 mg (20 mg/kg). The patient reported mild headache and mild dizziness immediately after taking the dose; both symptoms resolved within 1 hour with no treatment intervention. There were no associated laboratory test abnormalities. The patient suspended therapy for 24 hours and then restarted Kuvan with no reports of abnormal signs or symptoms. In postmarketing, one pediatric patient received Kuvan doses of 45 mg/kg per day instead of 20 mg/kg per day. The patient reported hyperactivity that began at an unspecified time after overdose and resolved after the Kuvan dose was reduced to 20 mg/kg per day.
In a clinical study to evaluate the effects of Kuvan on cardiac repolarization, a single supra-therapeutic dose of 100 mg/kg (5 times the maximum recommended dose) was administered to 54 healthy adults. No serious adverse reactions were reported during the study. The only adverse reactions reported in more than 1 subject who received the supra-therapeutic dose were upper abdominal pain (6%) and dizziness (4%). A dose-dependent shortening of the QT interval was observed
Patients should be advised to notify their physicians in cases of overdose.
How should I store and handle Kuvan?
Kuvan tablets,NDC 68135-300-02 Bottle of 120 tablets Kuvan powder for oral solution100 mg Kuvan per packet:NDC 68135-301-22 Carton of 30 unit dose packets NDC 68135-301-11 Single unit dose packet 500 mg Kuvan per packet:NDC 68135-482-11 Carton of 30 unit dose packetsNDC 68135-482-10 Single unit dose packet StorageStore Kuvan tablets at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Keep container tightly closed. Protect from moisture. Store Kuvan powder for oral solution at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Protect from moisture. Manufactured for: BioMarin Pharmaceutical Inc.Novato, CA 94949Kuvan tablets,NDC 68135-300-02 Bottle of 120 tablets Kuvan powder for oral solution100 mg Kuvan per packet:NDC 68135-301-22 Carton of 30 unit dose packets NDC 68135-301-11 Single unit dose packet 500 mg Kuvan per packet:NDC 68135-482-11 Carton of 30 unit dose packetsNDC 68135-482-10 Single unit dose packet StorageStore Kuvan tablets at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Keep container tightly closed. Protect from moisture. Store Kuvan powder for oral solution at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Protect from moisture. Manufactured for: BioMarin Pharmaceutical Inc.Novato, CA 94949Kuvan tablets,NDC 68135-300-02 Bottle of 120 tablets Kuvan powder for oral solution100 mg Kuvan per packet:NDC 68135-301-22 Carton of 30 unit dose packets NDC 68135-301-11 Single unit dose packet 500 mg Kuvan per packet:NDC 68135-482-11 Carton of 30 unit dose packetsNDC 68135-482-10 Single unit dose packet StorageStore Kuvan tablets at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Keep container tightly closed. Protect from moisture. Store Kuvan powder for oral solution at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Protect from moisture. Manufactured for: BioMarin Pharmaceutical Inc.Novato, CA 94949Kuvan tablets,NDC 68135-300-02 Bottle of 120 tablets Kuvan powder for oral solution100 mg Kuvan per packet:NDC 68135-301-22 Carton of 30 unit dose packets NDC 68135-301-11 Single unit dose packet 500 mg Kuvan per packet:NDC 68135-482-11 Carton of 30 unit dose packetsNDC 68135-482-10 Single unit dose packet StorageStore Kuvan tablets at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Keep container tightly closed. Protect from moisture. Store Kuvan powder for oral solution at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Protect from moisture. Manufactured for: BioMarin Pharmaceutical Inc.Novato, CA 94949Kuvan tablets,NDC 68135-300-02 Bottle of 120 tablets Kuvan powder for oral solution100 mg Kuvan per packet:NDC 68135-301-22 Carton of 30 unit dose packets NDC 68135-301-11 Single unit dose packet 500 mg Kuvan per packet:NDC 68135-482-11 Carton of 30 unit dose packetsNDC 68135-482-10 Single unit dose packet StorageStore Kuvan tablets at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Keep container tightly closed. Protect from moisture. Store Kuvan powder for oral solution at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Protect from moisture. Manufactured for: BioMarin Pharmaceutical Inc.Novato, CA 94949Kuvan tablets,NDC 68135-300-02 Bottle of 120 tablets Kuvan powder for oral solution100 mg Kuvan per packet:NDC 68135-301-22 Carton of 30 unit dose packets NDC 68135-301-11 Single unit dose packet 500 mg Kuvan per packet:NDC 68135-482-11 Carton of 30 unit dose packetsNDC 68135-482-10 Single unit dose packet StorageStore Kuvan tablets at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Keep container tightly closed. Protect from moisture. Store Kuvan powder for oral solution at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Protect from moisture. Manufactured for: BioMarin Pharmaceutical Inc.Novato, CA 94949Kuvan tablets,NDC 68135-300-02 Bottle of 120 tablets Kuvan powder for oral solution100 mg Kuvan per packet:NDC 68135-301-22 Carton of 30 unit dose packets NDC 68135-301-11 Single unit dose packet 500 mg Kuvan per packet:NDC 68135-482-11 Carton of 30 unit dose packetsNDC 68135-482-10 Single unit dose packet StorageStore Kuvan tablets at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Keep container tightly closed. Protect from moisture. Store Kuvan powder for oral solution at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Protect from moisture. Manufactured for: BioMarin Pharmaceutical Inc.Novato, CA 94949Kuvan tablets,NDC 68135-300-02 Bottle of 120 tablets Kuvan powder for oral solution100 mg Kuvan per packet:NDC 68135-301-22 Carton of 30 unit dose packets NDC 68135-301-11 Single unit dose packet 500 mg Kuvan per packet:NDC 68135-482-11 Carton of 30 unit dose packetsNDC 68135-482-10 Single unit dose packet StorageStore Kuvan tablets at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Keep container tightly closed. Protect from moisture. Store Kuvan powder for oral solution at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Protect from moisture. Manufactured for: BioMarin Pharmaceutical Inc.Novato, CA 94949Kuvan tablets,NDC 68135-300-02 Bottle of 120 tablets Kuvan powder for oral solution100 mg Kuvan per packet:NDC 68135-301-22 Carton of 30 unit dose packets NDC 68135-301-11 Single unit dose packet 500 mg Kuvan per packet:NDC 68135-482-11 Carton of 30 unit dose packetsNDC 68135-482-10 Single unit dose packet StorageStore Kuvan tablets at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Keep container tightly closed. Protect from moisture. Store Kuvan powder for oral solution at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Protect from moisture. Manufactured for: BioMarin Pharmaceutical Inc.Novato, CA 94949Kuvan tablets,NDC 68135-300-02 Bottle of 120 tablets Kuvan powder for oral solution100 mg Kuvan per packet:NDC 68135-301-22 Carton of 30 unit dose packets NDC 68135-301-11 Single unit dose packet 500 mg Kuvan per packet:NDC 68135-482-11 Carton of 30 unit dose packetsNDC 68135-482-10 Single unit dose packet StorageStore Kuvan tablets at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Keep container tightly closed. Protect from moisture. Store Kuvan powder for oral solution at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Protect from moisture. Manufactured for: BioMarin Pharmaceutical Inc.Novato, CA 94949Kuvan tablets,NDC 68135-300-02 Bottle of 120 tablets Kuvan powder for oral solution100 mg Kuvan per packet:NDC 68135-301-22 Carton of 30 unit dose packets NDC 68135-301-11 Single unit dose packet 500 mg Kuvan per packet:NDC 68135-482-11 Carton of 30 unit dose packetsNDC 68135-482-10 Single unit dose packet StorageStore Kuvan tablets at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Keep container tightly closed. Protect from moisture. Store Kuvan powder for oral solution at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Protect from moisture. Manufactured for: BioMarin Pharmaceutical Inc.Novato, CA 94949Kuvan tablets,NDC 68135-300-02 Bottle of 120 tablets Kuvan powder for oral solution100 mg Kuvan per packet:NDC 68135-301-22 Carton of 30 unit dose packets NDC 68135-301-11 Single unit dose packet 500 mg Kuvan per packet:NDC 68135-482-11 Carton of 30 unit dose packetsNDC 68135-482-10 Single unit dose packet StorageStore Kuvan tablets at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Keep container tightly closed. Protect from moisture. Store Kuvan powder for oral solution at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Protect from moisture. Manufactured for: BioMarin Pharmaceutical Inc.Novato, CA 94949Kuvan tablets,NDC 68135-300-02 Bottle of 120 tablets Kuvan powder for oral solution100 mg Kuvan per packet:NDC 68135-301-22 Carton of 30 unit dose packets NDC 68135-301-11 Single unit dose packet 500 mg Kuvan per packet:NDC 68135-482-11 Carton of 30 unit dose packetsNDC 68135-482-10 Single unit dose packet StorageStore Kuvan tablets at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Keep container tightly closed. Protect from moisture. Store Kuvan powder for oral solution at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Protect from moisture. Manufactured for: BioMarin Pharmaceutical Inc.Novato, CA 94949Kuvan tablets,NDC 68135-300-02 Bottle of 120 tablets Kuvan powder for oral solution100 mg Kuvan per packet:NDC 68135-301-22 Carton of 30 unit dose packets NDC 68135-301-11 Single unit dose packet 500 mg Kuvan per packet:NDC 68135-482-11 Carton of 30 unit dose packetsNDC 68135-482-10 Single unit dose packet StorageStore Kuvan tablets at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Keep container tightly closed. Protect from moisture. Store Kuvan powder for oral solution at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Protect from moisture. Manufactured for: BioMarin Pharmaceutical Inc.Novato, CA 94949Kuvan tablets,NDC 68135-300-02 Bottle of 120 tablets Kuvan powder for oral solution100 mg Kuvan per packet:NDC 68135-301-22 Carton of 30 unit dose packets NDC 68135-301-11 Single unit dose packet 500 mg Kuvan per packet:NDC 68135-482-11 Carton of 30 unit dose packetsNDC 68135-482-10 Single unit dose packet StorageStore Kuvan tablets at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Keep container tightly closed. Protect from moisture. Store Kuvan powder for oral solution at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Protect from moisture. Manufactured for: BioMarin Pharmaceutical Inc.Novato, CA 94949Kuvan tablets,NDC 68135-300-02 Bottle of 120 tablets Kuvan powder for oral solution100 mg Kuvan per packet:NDC 68135-301-22 Carton of 30 unit dose packets NDC 68135-301-11 Single unit dose packet 500 mg Kuvan per packet:NDC 68135-482-11 Carton of 30 unit dose packetsNDC 68135-482-10 Single unit dose packet StorageStore Kuvan tablets at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Keep container tightly closed. Protect from moisture. Store Kuvan powder for oral solution at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Protect from moisture. Manufactured for: BioMarin Pharmaceutical Inc.Novato, CA 94949Kuvan tablets,NDC 68135-300-02 Bottle of 120 tablets Kuvan powder for oral solution100 mg Kuvan per packet:NDC 68135-301-22 Carton of 30 unit dose packets NDC 68135-301-11 Single unit dose packet 500 mg Kuvan per packet:NDC 68135-482-11 Carton of 30 unit dose packetsNDC 68135-482-10 Single unit dose packet StorageStore Kuvan tablets at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Keep container tightly closed. Protect from moisture. Store Kuvan powder for oral solution at 20ºC to 25ºC (68ºF to 77ºF); excursions allowed between 15ºC to 30ºC (59ºF to 86ºF) [see USP Controlled Room Temperature]. Protect from moisture. Manufactured for: BioMarin Pharmaceutical Inc.Novato, CA 94949
Clinical Information
Chemical Structure
No Image foundClinical Pharmacology
Kuvan is a synthetic form of BH4, the cofactor for the enzyme phenylalanine hydroxylase (PAH). PAH hydroxylates Phe through an oxidative reaction to form tyrosine. In patients with PKU, PAH activity is absent or deficient. Treatment with BH4 can activate residual PAH enzyme activity, improve the normal oxidative metabolism of Phe, and decrease Phe levels in some patients.
Non-Clinical Toxicology
None.Nephrotoxicity has been reported following concomitant administration of cephalosporins with aminoglycoside antibacterial drugs or potent diuretics such as furosemide. Renal function should be carefully monitored, especially if higher dosages of the aminoglycosides are to be administered or if therapy is prolonged, because of the potential nephrotoxicity and ototoxicity of aminoglycoside antibacterial drugs. Nephrotoxicity and ototoxicity were not noted when ceftazidime was given alone in clinical trials.
Chloramphenicol has been shown to be antagonistic to beta-lactam antibacterial drugs, including ceftazidime, based on studies and time kill curves with enteric gram-negative bacilli. Due to the possibility of antagonism , particularly when bactericidal activity is desired, this drug combination should be avoided.
In common with other antibacterial drugs, ceftazidime may affect the gut flora, leading to lower estrogen reabsorption and reduced efficacy of combined oral estrogen/progesterone contraceptives.
Kuvan is not recommended in patients with a history of anaphylaxis to Kuvan. Hypersensitivity reactions, including anaphylaxis and rash, have occurred Signs of anaphylaxis include wheezing, dyspnea, coughing, hypotension, flushing, nausea, and rash. Discontinue treatment with Kuvan in patients who experience anaphylaxis and initiate appropriate medical treatment. Continue dietary Phe restrictions in patients who experience anaphylaxis.
Reference
This information is obtained from the National Institute of Health's Standard Packaging Label drug database.
"https://dailymed.nlm.nih.gov/dailymed/"
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