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What is Sapropterin
Sapropterin is indicated in conjunction with a phenylalanine-restricted diet to reduce blood phenylalanine concentrations in patients with hyperphenylalaninemia due to tetrahydrobiopterin- (BH4-) responsive phenylketonuria (PKU).
Sapropterin is a synthetic form, 6R-BH4, of tetrahydrobiopterin and was the first FDA-approved drug to treat PKU.
Brand Name
KUVAN
Indications
- phenylketonuria (PKU)
Side Effects
- abdominal pain
- agitation
- anaphylactoid reactions
- arthralgia
- bleeding
- cough
- diarrhea
- dizziness
- dyspepsia
- esophagitis
- gastritis
- GI bleeding
- headache
- hyperactivity
- irritability
- myocardial infarction
- nasal congestion
- nausea
- peripheral edema
- pharyngitis
- polyuria
- rash
- rhinitis
- rhinorrhea
- seizures
- vomiting
Monitoring Parameters
- serum phenylalanine (Phe) concentrations
Contraindications
- anorexia nervosa
- breast-feeding
- bulimia nervosa
- children
- fever
- GI disease
- hepatic disease
- infants
- infection
- neonates
- pregnancy
- renal impairment
Interactions
- Abacavir; Dolutegravir; Lamivudine
- Afatinib
- Aliskiren
- Aliskiren; Amlodipine
- Aliskiren; Amlodipine; Hydrochlorothiazide, HCTZ
- Aliskiren; Hydrochlorothiazide, HCTZ
- Aliskiren; Valsartan
- Alpelisib
- Amlodipine; Atorvastatin
- Apixaban
- Armodafinil
- Atorvastatin
- Atorvastatin; Ezetimibe
- Atropine; Hyoscyamine; Phenobarbital; Scopolamine
- Avanafil
- Belladonna Alkaloids; Ergotamine; Phenobarbital
- Betrixaban
- Bictegravir; Emtricitabine; Tenofovir Alafenamide
- Budesonide
- Budesonide; Formoterol
- Carbidopa; Levodopa
- Carbidopa; Levodopa; Entacapone
- Carvedilol
- Cobimetinib
- Colchicine
- Colchicine; Probenecid
- Cyclosporine
- Dabigatran
- Dapagliflozin; Saxagliptin
- Darunavir
- Darunavir; Cobicistat
- Darunavir; Cobicistat; Emtricitabine; Tenofovir alafenamide
- Dasabuvir; Ombitasvir; Paritaprevir; Ritonavir
- Dexamethasone
- Dextromethorphan; Quinidine
- Digoxin
- Docetaxel
- Dolutegravir
- Dolutegravir; Lamivudine
- Dolutegravir; Rilpivirine
- Doravirine; Lamivudine; Tenofovir disoproxil fumarate
- Doxorubicin
- Drospirenone; Ethinyl Estradiol
- Drospirenone; Ethinyl Estradiol; Levomefolate
- Efavirenz; Emtricitabine; Tenofovir
- Efavirenz; Lamivudine; Tenofovir Disoproxil Fumarate
- Eletriptan
- Elvitegravir; Cobicistat; Emtricitabine; Tenofovir Alafenamide
- Elvitegravir; Cobicistat; Emtricitabine; Tenofovir Disoproxil Fumarate
- Emtricitabine; Rilpivirine; Tenofovir alafenamide
- Emtricitabine; Rilpivirine; Tenofovir disoproxil fumarate
- Emtricitabine; Tenofovir alafenamide
- Emtricitabine; Tenofovir disoproxil fumarate
- Erythromycin
- Erythromycin; Sulfisoxazole
- Ethinyl Estradiol
- Ethinyl Estradiol; Desogestrel
- Ethinyl Estradiol; Ethynodiol Diacetate
- Ethinyl Estradiol; Etonogestrel
- Ethinyl Estradiol; Levonorgestrel
- Ethinyl Estradiol; Levonorgestrel; Ferrous bisglycinate
- Ethinyl Estradiol; Levonorgestrel; Folic Acid; Levomefolate
- Ethinyl Estradiol; Norelgestromin
- Ethinyl Estradiol; Norethindrone
- Ethinyl Estradiol; Norethindrone Acetate
- Ethinyl Estradiol; Norethindrone Acetate; Ferrous fumarate
- Ethinyl Estradiol; Norethindrone; Ferrous fumarate
- Ethinyl Estradiol; Norgestimate
- Ethinyl Estradiol; Norgestrel
- Everolimus
- Fexofenadine
- Fexofenadine; Pseudoephedrine
- Fluticasone; Umeclidinium; Vilanterol
- Fluticasone; Vilanterol
- Folate analogs
- Fosamprenavir
- Glecaprevir; Pibrentasvir
- Glyburide
- Glyburide; Metformin
- Imatinib
- Indinavir
- Isoniazid, INH; Pyrazinamide, PZA; Rifampin
- Isoniazid, INH; Rifampin
- Itraconazole
- Lamivudine; Tenofovir Disoproxil Fumarate
- Lapatinib
- Ledipasvir; Sofosbuvir
- Lefamulin
- Levodopa
- Loperamide
- Loperamide; Simethicone
- Lopinavir; Ritonavir
- Maraviroc
- Mefloquine
- Metformin; Saxagliptin
- Methadone
- Mitomycin
- Morphine
- Morphine; Naltrexone
- Naldemedine
- Nelfinavir
- Ombitasvir; Paritaprevir; Ritonavir
- Ombitasvir; Paritaprevir; Ritonavir
- Ondansetron
- Ozanimod
- Paclitaxel
- Panobinostat
- Pazopanib
- Phenobarbital
- Phosphodiesterase inhibitors
- Posaconazole
- Prednisone
- Pyrimethamine
- Pyrimethamine; Sulfadoxine
- Quinidine
- Quinine
- Ranolazine
- Rifampin
- Rifaximin
- Rimegepant
- Riociguat
- Risperidone
- Ritonavir
- Rivaroxaban
- Romidepsin
- Saquinavir
- Saxagliptin
- Segesterone Acetate; Ethinyl Estradiol
- Selexipag
- Sildenafil
- Silodosin
- Simeprevir
- Sirolimus
- Sofosbuvir
- Sofosbuvir; Velpatasvir
- Sofosbuvir; Velpatasvir; Voxilaprevir
- Sulfamethoxazole; Trimethoprim, SMX-TMP, Cotrimoxazole
- Tadalafil
- Talazoparib
- Temsirolimus
- Teniposide
- Tenofovir Alafenamide
- Tenofovir Alafenamide
- Tenofovir, PMPA
- Testosterone
- Ticagrelor
- Tipranavir
- Tolvaptan
- Topotecan
- Trandolapril; Verapamil
- Trimethoprim
- Ubrogepant
- Umeclidinium
- Umeclidinium; Vilanterol
- Valproic Acid, Divalproex Sodium
- Vardenafil
- Vemurafenib
- Venetoclax
- Verapamil
