HARLIKU nitisinone 2 mg Tablet, 60-count
🆔 Identity & classification
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🏷️ RxNorm drug class
This medicine belongs to the 4-Hydroxyphenyl-Pyruvate Dioxygenase Inhibitor class.
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🏭 Manufacturer & labeler
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🩺 Clinical
Nitisinone is used to treat hereditary tyrosinemia type 1 (HT-1; rare genetic disorder where body cannot break down amino acid tyrosine causing build-up that can harm kidneys and liver) and reduction of urine homogentisic acid (HGA) in patients with alkaptonuria (AKU; rare genetic disorder where body can't breakdown aminoacids tyrosine and phenylalanine leading to builld up of HGA causing deposits in connective tissue causing severe arthritis symptoms). Nitisinone is in a class of medications called hydroxyphenyl-pyruvate dioxygenase inhibitors. It works by by helping the body eliminate the to...
Read the full MedlinePlus article ↗- Nitisinone works by blocking an enzyme that's part of how your body breaks down an amino acid called tyrosine. In HT-1, when that breakdown goes wrong, it creates toxic byproducts...
- What exactly does nitisinone do, and why do I have to follow such a strict diet with it?
- The most common issue is a rise in blood tyrosine levels, which is why diet is so important. Some people also develop low blood cell counts, which your doctor monitors with regular...
- What side effects should I actually expect, and which ones should I call my doctor about right away?
Patient education
Supplement & herbal interactions
Some supplements/herbs that may interact with nitisinone — tap one for details:
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💊 What it looks like
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🧪 Inactive Ingredients / Excipients
Inactive ingredients, also called excipients, are components of the drug product other than the active ingredient. They may include fillers, dyes, coatings, preservatives, flavors, or other formulation ingredients.
💡 Tap an ingredient (hover on desktop) to see what it is and why it’s used.
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UNII R8WTH25YS2
Glyceryl dibehenate is a wax-like substance made from behenate fatty acids and glycerol. It functions as a binder and release-control agent in tablets and capsules, helping hold ingredients together and regulate how quickly the medicine dissolves and releases in your body.
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UNII EWQ57Q8I5X
Lactose monohydrate is a natural sugar derived from milk. It serves as a filler and binder in tablets and capsules, helping create the proper size, texture, and consistency of the medicine.
2 inactive ingredients listed in the exact product block matched to this NDC.
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ingredient classCode="IACT" elements from the exact product block matched by this NDC. Label-section narrative from DailyMed / the openFDA label index is shown separately when available.Inactive ingredient FAQ
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💲 Pricing
A drug doesn't have one price. Each row is a different public payment system, and none is what you'd pay at the counter — that depends on your insurance. The ⓘ on each row explains what it measures.
| Price system | Per each | Per package |
|---|---|---|
| Retail pharmacies payNADAC · weekly | Not in the retail survey — common for institutional, discontinued, or low-volume packs. | |
| Medicaid paysCMS SDUD · 12 mo | No recent Medicaid claims on file for this NDC — rare and low-volume NDCs are suppressed in the public data. | |
| Medicare drug plans payPart D · Q2 2026 | $1,481.43 | $88,885.81 / 60 tablets |
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🔁 Therapeutic equivalents
| Product | Labeler | Pack | NADAC/unit | TE | Status | Price vs. this |
|---|---|---|---|---|---|---|
| Nityr 2 mg 70709-0002-60 | Cycle | 60 tablets | — | — | FDA listed | — |
| Harliku 2 mgthis 70709-0112-60 | Cycle | 60 tablets | — | — | FDA listed | — |
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⏳ Availability & generic status
We did not find an FDA-approved generic match for this exact strength, form and route. Patent/protection dates below may affect future generic timing.
Why the date isn’t exact: Generic timing can change because patents may be challenged, settled, licensed, added, removed, or worked around with a narrower label — and FDA approval does not always mean a pharmacy can get the generic today.
🛈 What do these terms mean?
- Patent
- Legal protection listed in the Orange Book that may delay generic approval or launch. Issued by the U.S. Patent & Trademark Office.
- Substance patent
- Covers the active drug molecule itself — the hardest to design around. A generic generally can’t launch until it expires.
- Formulation (product) patent
- Covers a specific formulation or dosage form. A generic can sometimes work around it with a different formulation.
- Method-of-use patent
- A patent covering one specific approved use of the drug — not necessarily the whole molecule. A generic can sometimes launch with a “skinny label” that carves out the protected use and keeps the others.
- Skinny label
- A generic label that omits a still-patented use when the FDA allows it — letting a generic reach the market for the unprotected uses.
- Exclusivity
- FDA-granted marketing protection, separate from patents — e.g. 5-yr new chemical entity, 7-yr orphan drug, or a +6-month pediatric extension.
- Paragraph IV
- A generic applicant’s formal challenge to a listed patent. It can potentially lead to earlier generic entry, but often involves litigation or a settlement.
- RLD / RS
- Reference Listed Drug — the brand product the FDA uses as the reference for generic applications. Reference Standard — the product the FDA expects generics to compare against in bioequivalence testing.
- TE / AB rating
- FDA therapeutic-equivalence rating. An AB rating generally means the FDA considers a generic therapeutically equivalent to — and substitutable for — the brand.
- LOE (loss of exclusivity)
- The latest patent or exclusivity currently listed — the loss-of-exclusivity / latest-listed-protection date shown on this page. Paragraph-IV challenges and settlements can move the real date earlier; FDA approval and a manufacturer’s decision to market can move it later.
Built from the FDA Orange Book. The bars above are scaled to each protection’s expiry; the red LOE marker is the last one to lapse.
| Patent | Type | Use code | Expires |
|---|---|---|---|
| US 10328029 ↗ | Method of use | U-1836 | Jan 5, 2035 |
| US 10328029 ↗ | Method of use | U-1836 | Jan 5, 2035 |
| US 10328029 ↗ | Method of use | U-1836 | Jan 5, 2035 |
| Code | What it grants | Expires |
|---|---|---|
| ODE-529 | Orphan Drug Exclusivity (7-year) | Jun 10, 2032 |
Is there a generic version of HARLIKU 2 MG TABLET?
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📊 Medicare Part D spend CMS · PART D · 2026 (Q1)
📦 Packaging — all sizes for this product
| Package NDC | Description | Marketing start | Status |
|---|---|---|---|
| 70709-0112-60 You're viewing this | 1 BOTTLE, PLASTIC in 1 CARTON (70709-112-60) / 60 TABLET in 1 BOTTLE, PLASTIC | 2025-07-21 | Active |
🧭 About this NDC listing & data coverage
What data is (and isn’t) available for this NDC — tap to expand
| NDC identity (package / product / labeler codes) | ✓ Available |
| Labeler | ✓ Available |
| Product & package description | ✓ Available |
| Marketing category & status | ✓ Available |
| Active ingredient / dosage form / route | ✓ Available |
| FDA label (SPL via DailyMed) | ✓ Available |
| Package photos | ✓ Available |
| Inactive ingredients (structured) | ✓ Available |
| NADAC pharmacy acquisition price (CMS) | — Not published for this NDC CMS publishes NADAC only for NDCs reported in its retail-pharmacy survey. |
| Orange Book / therapeutic-equivalence data | ✓ Available |
| HCPCS J-code billing crosswalk | — Not published for this NDC Most self-administered / retail products have no J-code — that is normal. |
| Medicaid utilization (CMS SDUD) | — Not published for this NDC CMS reports utilization only for NDCs with Medicaid claims above its privacy threshold. |
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📄 Full prescribing information FDA SPL
🎯 Indications and Usage ▾
1 INDICATIONS AND USAGE HARLIKU TM is indicated for the reduction of urine homogentisic acid (HGA) in adult patients with alkaptonuria (AKU). HARLIKU is a hydroxyphenyl-pyruvate dioxygenase inhibitor indicated for the reduction of urine homogentisic acid (HGA) in adult patients with alkaptonuria (AKU). ( 1 )
⏱️ Dosage and Administration ▾
2 DOSAGE AND ADMINISTRATION The recommended dosage of HARLIKU is 2 mg administered orally, once daily. ( 2.1 )
2.1Recommended Dosage The recommended dosage of HARLIKU is 2 mg administered orally, once daily. Administer HARLIKU with or without food [see Clinical Pharmacology ( 12.3 )] . Missed Dose If a dose of HARLIKU is missed, do not administer two doses at once to make up for a missed dose. Take the next dose at the scheduled time.
💊 Dosage Forms and Strengths ▾
3 DOSAGE FORMS AND STRENGTHS Tablets: 2 mg white to beige, round, flat tablets, which may display light yellow to brown speckles, debossed with “2” mg on one side and “L” on the other side. Tablets: 2 mg. ( 3 )
⛔ Contraindications ▾
4 CONTRAINDICATIONS None. None. ( 4 )
⚠️ Warnings and Cautions ▾
5 WARNINGS AND PRECAUTIONS Ocular Symptoms and Hyperkeratotic Plaques Due To Elevated Plasma Tyrosine Levels : Inadequate restriction of tyrosine and phenylalanine intake can lead to elevations in plasma tyrosine and levels above 500 micromol/L may lead to ocular signs and symptoms or painful hyperkeratotic plaques on the soles and palms. ( 5.1 ) Assess plasma tyrosine levels in patients presenting with ocular signs and symptoms. ( 5.1 ) Obtain slit-lamp examination prior to treatment and regularly thereafter.
( 5.1 ) Implement diet restriction and/or treatment interruption as appropriate. ( 5.1 ) Leukopenia and Severe Thrombocytopenia: Monitor platelet and white blood cell counts. ( 5.2 )
5.1Ocular Symptoms and Hyperkeratotic Plaques Due to Elevated Plasma Tyrosine Levels Treatment with HARLIKU may cause elevated plasma tyrosine levels in patients with AKU. Tyrosine levels greater than 500 micromol/L may lead to the following: Ocular signs and symptoms including keratitis, corneal opacities, corneal irritation, corneal ulcers, conjunctivitis, eye pain, and photophobia. These ocular adverse reactions have been reported in patients treated with nitisinone [see Adverse Reactions ( 6.1 )] .
In a clinical trial in the AKU population, without dietary restriction and reported tyrosine levels > 500 micromol/L, both symptomatic and asymptomatic keratopathies have been observed. Perform a baseline ophthalmologic examination including slit-lamp examination prior to initiating HARLIKU treatment and regularly thereafter. Patients who develop photophobia, eye pain, or signs of inflammation such as redness, swelling, or burning of the eyes or tyrosine levels are > 500 micromol/L during treatment with HARLIKU should undergo slit-lamp re-examination and immediate measurement of the plasma tyrosine concentration.
Painful hyperkeratotic plaques on the soles and palms. There is no routine dietary restriction requirement for AKU patients taking HARLIKU. However, in patients who develop keratopathies, monitor plasma tyrosine levels, and implement a diet restricted in tyrosine and phenylalanine to keep the plasma tyrosine level below 500 micromol/L.
Consider temporarily interrupting HARLIKU until resolution of symptoms.
5.2Leukopenia and Severe Thrombocytopenia In clinical trials, patients with hereditary tyrosinemia type 1 (HT-1) treated with another oral formulation of nitisinone and dietary restriction developed reversible leukopenia (3%), thrombocytopenia (3%), or both (1.5%). Ten percent of patients in Trial 1 developed thrombocytopenia [see Adverse Reactions ( 6.1 )] . No patients developed infections or bleeding as a result of the episodes of leukopenia and thrombocytopenia.
Monitor platelet and white blood cell counts during HARLIKU therapy.
🤒 Adverse Reactions ▾
6 ADVERSE REACTIONS Most common adverse reactions (>1%) are elevated tyrosine levels, keratitis and thrombocytopenia. ( 6.1 ) To report SUSPECTED ADVERSE REACTIONS, contact Cycle Pharmaceuticals Ltd at 1-855-831-5413 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch. Serious and/or clinically significant adverse reactions described elsewhere in labeling include: Ocular Symptoms and Hyperkeratotic Plaques Due to Elevated Plasma Tyrosine Levels [see Warnings and Precautions ( 5.1 )] Leukopenia and Severe Thrombocytopenia [see Warnings and Precautions ( 5.2 )]
6.1Clinical Trials Experience Because clinical trials are conducted under widely varying conditions, adverse reaction rates observed in the clinical trials of a drug cannot be directly compared to rates in the clinical trials of another drug and may not reflect the rates observed in practice. The safety of HARLIKU was evaluated in Trial 1, a three-year, open-label, randomized, no-treatment controlled trial in 40 patients with AKU. Patients were between 38 and 68 years of age (27 male, 13 female) [see Clinical Studies ( 14 )] .
Patients received either HARLIKU 2 mg orally once daily or no treatment [see Dosage and Administration ( 2 )]. The serious adverse reactions reported with HARLIKU were ocular/visual complaints associated with elevated tyrosine levels (keratitis) [see Warnings and Precautions ( 5.1 )] . Keratitis led to permanent treatment discontinuation in 1 (5%) treated patient.
The most common adverse reactions (>1%) reported in Trial 1 are summarized in TABLE 1. TABLE 1. Most Common Adverse Reactions* in Patients with AKU Treated with Nitisinone** Adverse Reactions Nitisinone (N=20) n (%) No Treatment (N=20) n (%) Elevated tyrosine levels 19 (95) 0 (0) Keratitis*** 3 (15) 0 (0) Thrombocytopenia 2 (10) 0 (0) * reported in at least 1% of patients; ** another oral formulation of nitisinone; *** keratitis also includes eye irritation, eye pain and photophobia.
🔄 Drug Interactions ▾
7 DRUG INTERACTIONS Sensitive CYP2C9 Substrates: Reduce dosage of co-administered drug metabolized by CYP2C9 by half. ( 7.1 ) OAT1/OAT3 Substrates : Avoid concomitant use of HARLIKU with OAT1/OAT3 substrates. Concomitant use with OAT1/OAT3 substrates may increase the risk of adverse reactions related to the co-administered drug. ( 7.1 )
7.1Effects of HARLIKU on Other Drugs Sensitive CYP2C9 Substrates Reduce the dosage of the co-administered drug metabolized by CYP2C9 by half. Additional dosage adjustments may be needed to maintain therapeutic drug concentrations where minimal concentration changes may lead to serious adverse reactions. See prescribing information for those drugs.
Nitisinone is a moderate CYP2C9 inhibitor. Nitisinone may increase exposure of co-administered drugs metabolized by CYP2C9 [see Clinical Pharmacology ( 12.3 )] . OAT1/OAT3 Substrates The concomitant use of HARLIKU with OAT1/OAT3 substrates may increase the risk of adverse reactions related to the co-administered drug.
See prescribing information for those drugs. Nitisinone is an OAT1/OAT3 inhibitor which can lead to increased exposure of the co-administered drug. [ see Clinical Pharmacology ( 12.3 )] .
👥 Use in Specific Populations ▾
8 USE IN SPECIFIC POPULATIONS
8.1Pregnancy Risk Summary Available data from published case reports with nitisinone use in pregnant women are insufficient to identify a drug-associated risk of major birth defects, miscarriage or other adverse maternal or fetal outcomes. Animal reproduction studies have been conducted for nitisinone. In these studies, nitisinone was administered to mice and rabbits during organogenesis with oral doses of nitisinone up to 600 and 240 times respectively, the maximum recommended human daily dose (MRHDD) of 2 mg/day.
In mice, nitisinone caused incomplete skeletal ossification of fetal bones and decreased pup survival at doses 12 times the MRHDD, and increased gestational length at doses 120 times the MRHDD. In rabbits, nitisinone caused maternal toxicity and incomplete skeletal ossification of fetal bones at doses 48 times the MRHDD ( see Data ) . The background risk of major birth defects and miscarriage for the indicated population is unknown.
All pregnancies have a background risk of birth defect, loss, or other adverse outcomes. In the U.S. general population, the estimated background risk of major birth defects and miscarriage in clinically recognized pregnancies is 2 to 4% and 15 to 20%, respectively. Data Animal Data In an embryo-fetal development study in mice, nitisinone was administered via oral gavage to pregnant mice at dose levels of 12, 120 and 600 times the maximum recommended human daily dose (MRHDD) of 2 mg/day.
Nitisinone caused incomplete skeletal ossification of fetal bones and decreased pup survival at doses 12 times the MRHDD and increased gestational length at doses 120 times the MRHDD. In an embryo-fetal development study in rabbits, nitisinone was administered via oral gavage to pregnant rabbits at dose levels of 48, 120 and 240 times the MRHDD of 2 mg/day. Nitisinone caused maternal toxicity and incomplete skeletal ossification of fetal bones at doses 48 times the MRHDD.
In a single dose-group study in rats given 100 mg/kg (486 times the MRHDD of 2 mg/day), reduced litter size, decreased pup weight at birth, and decreased survival of pups after birth was demonstrated.
8.2Lactation Risk Summary There are no data on the presence of nitisinone in human milk, the effects on the breastfed infant, or the effects on milk production. Data suggest that nitisinone is present in rat milk due to findings of ocular toxicity and lower body weight seen in drug naive nursing rat pups. The developmental and health benefits of breastfeeding should be considered along with the mother’s clinical need for HARLIKU and any potential adverse effects on the breastfed infant from HARLIKU or from the underlying maternal condition.
8.4Pediatric Use The safety and effectiveness of HARLIKU have not been established in pediatric patients with AKU.
8.5Geriatric Use There was 1 patient 65 years of age and older in the clinical studies for AKU [see Clinical Studies ( 14 )] . Of the total number of HARLIKU-treated patients in these studies, 1 (2.5 %) were 65 years of age and older, while 0 (0%) were 75 years of age and older. Clinical studies of HARLIKU did not include sufficient numbers of patients 65 years of age and older to determine whether they respond differently from younger adult patients.
🤰 Pregnancy ▾
8.1Pregnancy Risk Summary Available data from published case reports with nitisinone use in pregnant women are insufficient to identify a drug-associated risk of major birth defects, miscarriage or other adverse maternal or fetal outcomes. Animal reproduction studies have been conducted for nitisinone. In these studies, nitisinone was administered to mice and rabbits during organogenesis with oral doses of nitisinone up to 600 and 240 times respectively, the maximum recommended human daily dose (MRHDD) of 2 mg/day.
In mice, nitisinone caused incomplete skeletal ossification of fetal bones and decreased pup survival at doses 12 times the MRHDD, and increased gestational length at doses 120 times the MRHDD. In rabbits, nitisinone caused maternal toxicity and incomplete skeletal ossification of fetal bones at doses 48 times the MRHDD ( see Data ) . The background risk of major birth defects and miscarriage for the indicated population is unknown.
All pregnancies have a background risk of birth defect, loss, or other adverse outcomes. In the U.S. general population, the estimated background risk of major birth defects and miscarriage in clinically recognized pregnancies is 2 to 4% and 15 to 20%, respectively. Data Animal Data In an embryo-fetal development study in mice, nitisinone was administered via oral gavage to pregnant mice at dose levels of 12, 120 and 600 times the maximum recommended human daily dose (MRHDD) of 2 mg/day.
Nitisinone caused incomplete skeletal ossification of fetal bones and decreased pup survival at doses 12 times the MRHDD and increased gestational length at doses 120 times the MRHDD. In an embryo-fetal development study in rabbits, nitisinone was administered via oral gavage to pregnant rabbits at dose levels of 48, 120 and 240 times the MRHDD of 2 mg/day. Nitisinone caused maternal toxicity and incomplete skeletal ossification of fetal bones at doses 48 times the MRHDD.
In a single dose-group study in rats given 100 mg/kg (486 times the MRHDD of 2 mg/day), reduced litter size, decreased pup weight at birth, and decreased survival of pups after birth was demonstrated.
🧒 Pediatric Use ▾
8.4Pediatric Use The safety and effectiveness of HARLIKU have not been established in pediatric patients with AKU.
🧓 Geriatric Use ▾
8.5Geriatric Use There was 1 patient 65 years of age and older in the clinical studies for AKU [see Clinical Studies ( 14 )] . Of the total number of HARLIKU-treated patients in these studies, 1 (2.5 %) were 65 years of age and older, while 0 (0%) were 75 years of age and older. Clinical studies of HARLIKU did not include sufficient numbers of patients 65 years of age and older to determine whether they respond differently from younger adult patients.
🧬 Clinical Pharmacology ▾
12 CLINICAL PHARMACOLOGY
12.1Mechanism of Action Nitisinone is a competitive inhibitor of hydroxyphenyl-pyruvate dioxygenase, an enzyme upstream of homogentisate 1,2-dioxygenase (HGD) in the tyrosine catabolic pathway.
12.2Pharmacodynamics In patients with AKU, HGA accumulates in various tissues and urine. In an open-label, single center, randomized, no-treatment controlled trial nitisinone treatment resulted in reduction of urinary HGA concentrations in patients with AKU [see Clinical Studies ( 14 )]. Nitisinone exposure-response relationship and time course of pharmacodynamic response for the effectiveness have not been fully characterized.
Nitisinone inhibits catabolism of the amino acid tyrosine and can result in elevated plasma levels of tyrosine in patients with AKU. Treatment with nitisinone does not require routine dietary restriction in patients with AKU; however, patients who develop keratopathies should be monitored and dietary restriction of tyrosine and phenylalanine should be implemented [see Warnings and Precautions ( 5.1 )].
12.3Pharmacokinetics The single-dose pharmacokinetics of nitisinone tablets have been studied in healthy adult subjects. Nitisinone pharmacokinetic parameters are presented as geometric mean [range] unless otherwise specified. Nitisinone maximum concentration (Cmax) and area under the curve from time 0 to 120 hours (AUC0-120h) were 1278 [780 to 1649] ng/mL and 77874 [42335 to 104211] ng•h/mL following oral administration of 10 mg (5 times the recommended dosage) nitisinone under fasting conditions.
Absorption Nitisinone time to C max (T max ) is 3.5 hours (median, ranging from 1 to 4 hours) following single oral administration of 10 mg (5 times the recommended dosage) nitisinone under fasting conditions. Effect of Food : In a food effect study, a high-fat and high-calorie breakfast (973.6 cal distributed in carbohydrate 250.1 cal, proteins 157 cal, fat 566.5 cal) did not significantly affect the total exposure (AUC 0-120h ) and C max of nitisinone following single oral administration of 10 mg nitisinone (5 times the recommended dosage).
The median T max was delayed to 6 hours under fed conditions [see Dosage and Administration ( 2 )] . Distribution The arithmetic mean (SD) apparent volume of distribution of nitisinone is 8.2 (1.6) L in healthy subjects (n=23). In vitro binding of nitisinone to human plasma proteins is greater than 95% at 50 micromolar concentration.
Elimination The arithmetic mean (SD) terminal half-life of nitisinone is 59.3 (8.9) hours in healthy subjects (n=23). The mean (CV%) apparent plasma clearance in 18 healthy adults following multiple once daily doses of nitisinone 80 mg (40 times the recommended dosage) is 113 (16) mL/hr. The mean of the fraction of dose excreted renally as unchanged nitisinone in the urine (fe(%)) was 3% (n=3) following multiple oral doses of 80 mg (40 times the recommended dosage) daily in healthy subjects.
Metabolism In vitro studies have shown that nitisinone is relatively stable in human liver microsomes with minor metabolism possibly mediated by CYP3A4 enzyme. Excretion The estimated mean (CV%) renal clearance of nitisinone was 0.003 L/h (25%). Drug Interaction Studies Clinical Studies Nitisinone does not inhibit CYP2D6.
Nitisinone is a moderate inhibitor of CYP2C9, and a weak inducer of CYP2E1 (TABLE 2). Nitisinone is an inhibitor of OAT1/3 (TABLE 2). TABLE 2.
Percent Change in AUC 0-inf and Cmax for Co-administered Drugs in the Presence of Nitisinone in 18 Healthy Subjects Co-administered Drug a Dose of Co-administered Drug (Route of Administration) Effect of Nitisinone on the Pharmacokinetics of Co-administered Drug b AUC 0-inf C max CYP2C9 Substrate Tolbutamide c 500 mg (oral) 131%↑ 16%↑ CYP2E1 Substrate Chlorzoxazone 250 mg (oral) 27%↓ 18%↓ OAT1/3 Substrate Furosemide 20 mg (intravenous) 72%↑ 12%↑ ↑= Increased; ↓= Decreased a The interacting drug was administered alone on Day 1 and together with nitisinone on Day 17. b Multiple dose…
🧬 Mechanism of Action ▾
12.1Mechanism of Action Nitisinone is a competitive inhibitor of hydroxyphenyl-pyruvate dioxygenase, an enzyme upstream of homogentisate 1,2-dioxygenase (HGD) in the tyrosine catabolic pathway.
📦 How Supplied / Storage and Handling ▾
16 HOW SUPPLIED/STORAGE AND HANDLING How Supplied HARLIKU (nitisinone) tablet is white to beige, round, flat which may display light yellow to brown speckles, debossed with the “strength” in mg on one side and “L” on the other side. Each tablet contains 2 mg nitisinone. HARLIKU is supplied in a high-density polyethylene (HDPE) square bottle with a child-resistant tamper-evident polypropylene (PP) screw cap.
Each bottle contains 60 tablets. 2 mg tablets: NDC 70709-112-60 Storage and Handling Store HARLIKU tablets at room temperature between 20°C to 25°C (68°F to 77°F); excursions permitted between 15°C and 30°C (59°F and 86°F) [see USP Controlled Room Temperature]. Pharmacist: Dispense in tight and light resistant container as defined in USP.
📋 Description ▾
11 DESCRIPTION HARLIKU (nitisinone) is a hydroxyphenyl-pyruvate dioxygenase inhibitor. Nitisinone occurs as a white to yellowish-white, crystalline powder. It is practically insoluble in water, soluble in 2M sodium hydroxide and in methanol, and sparingly soluble in alcohol.
The chemical name of nitisinone is 2-(2-nitro-4-trifluoromethylbenzoyl) cyclohexane-1,3-dione. The molecular formula is C 14 H 10 F 3 NO 5 and the molecular weight is 329.23. The structural formula is: Each HARLIKU (nitisinone) tablet contains 2 mg of nitisinone to be administered orally.
The inactive ingredients are glyceryl dibehenate and lactose monohydrate. Figure 1 Structural Formula
💬 Information for Patients ▾
17 PATIENT COUNSELING INFORMATION Ocular Symptoms and Hyperkeratotic Plaques Due to Elevated Plasma Tyrosine Levels Advise the patient or caregiver to report any unexplained ocular or other symptoms promptly to their healthcare provider [see Warnings and Precautions ( 5.1 )] . How Supplied/Storage and Handling Advise the patient or caregiver to store HARLIKU in the container that it is dispensed in and keep the container tightly closed [see How Supplied/Storage and Handling ( 16 )].