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SODIUM PHENYLACETATE and SODIUM BENZOATE 100 mg/mL; 100 mg/mL Injection, Solution, Concentrate

by Ailex Pharmaceuticals, LLC · 1 VIAL, SINGLE-DOSE in 1 CARTON (70556-100-50) / 50 mL in 1 VIAL, SINGLE-DOSE

NDC 70556-0100-50

🏷️ FDA NDC (as labeled) 70556-100-50 billing pads the product segment with a zero

Rx only Generic On market Non-controlled

🗂️ Data synced Jul 24, 2026 · sources: openFDA · FDA label (DailyMed) · FDA Orange & Purple Book · First Databank · CMS NADAC, ASP, Medicare & Medicaid · RxNorm

📋 All sources & update times →

This page is curated, coded, and maintained by Dr. Brian Staiger, PharmD

Compiled from public FDA, CMS, DailyMed, NADAC, and related drug data sources. Editorial policy

🆔 Identity & classification

FDA NDC (as labeled) 70556-100-50

Product NDC 70556-100

11-digit billing NDC 70556010050

RxCUI 984118

UNII 48N6U1781G, OJ245FE5EU

Application # ANDA207096

SPL Set ID 329655ae-c3c0-46b2-a925-e49777f2076b

Established class (EPC) Nitrogen Binding Agent

Mechanism of action Ammonium Ion Binding Activity

DEA schedule Non-controlled

Marketing category ANDA

Marketing status On market

FDA listing status Listed (active directory)

Marketing start 2016-02-24

Route INTRAVENOUS

Dosage form INJECTION, SOLUTION, CONCENTRATE

Substance SODIUM PHENYLACETATE; SODIUM BENZOATE

TE code (Orange Book) AP · RLD · RS

Why two NDCs? The FDA registers this code as 70556-100-50 — a 5-3-2 layout, and that's what's printed on the package and shown on DailyMed. For insurance claims, every NDC is standardized to a uniform 11-digit 5-4-2 format by adding a zero to the product segment → 70556-0100-50. Same drug, same package — only the format differs.

Where does this data come from?

Identifiers from the FDA openFDA NDC Directory and Structured Product Labeling; RxCUI from RxNorm (NLM); GPI from Medi-Span; GCN / HIC / AHFS / legend from First Databank.

🏷️ RxNorm drug class

This medicine belongs to the Nitrogen Binding Agent class.

Pharmacologic class Nitrogen Binding Agent

Drug family (ATC) Tests for gastric secretion, Various alimentary tract and metabolism products

How it works Ammonium Ion Binding Activity

Where does this data come from?

Therapeutic classes from RxNorm RxClass (U.S. National Library of Medicine) — Established Pharmacologic Class (FDA), ATC drug family (WHO) and mechanism of action, matched by this product’s RxCUI.

🏭 Manufacturer & labeler

LabelerAilex Pharmaceuticals, LLC

Application holderAILEX PHARMACEUTICALS LLC

FDA applicationANDA207096 (ANDA)

Labeler code70556

First marketedFeb 2016

Product typeHuman Prescription Drug

🔎 All NDCs from Ailex Pharmaceuticals, LLC

The labeler markets the product; the application holder owns the FDA approval. They’re often the same company but can differ (e.g. a repackager or an authorized generic). A mailing address / phone appears here when the manufacturer includes it in the product’s FDA label (not all do).

Where does this data come from?

Labeler, application holder and registered establishment from the FDA openFDA NDC Directory and Drugs@FDA; address/contact from the product’s FDA label.

🩺 Clinical

Patient education

📘 MedlinePlus patient leaflet ↗

Supplement & herbal interactions

🌿 Drug–supplement interaction checker →

Where does this data come from?

Plain-language summary from MedlinePlus (U.S. National Library of Medicine); supplement & herbal interactions and nutrient depletion data from the Natural Medicines database; our full guide is HelloPharmacist editorial content.

🧪 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.

UNII QTT17582CB
A strong acid used to adjust and maintain the proper pH level in liquid medicines, ensuring stability and preventing breakdown of active ingredients.
UNII 55X04QC32I
A strong alkaline chemical used to adjust and maintain the pH balance of liquid medicines. It helps keep the medicine stable and ensures it stays effective during storage.
UNII 059QF0KO0R
Water is a liquid solvent that dissolves and mixes ingredients together in liquid medicines, syrups, and injections. It helps distribute the active drug evenly throughout the product.

3 inactive ingredients listed in the exact product block matched to this NDC.

Where does this data come from?

Data sourced from official FDA Structured Product Labeling (SPL) via DailyMed — 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

Are inactive ingredients the same for every manufacturer?

No. Inactive ingredients can differ by manufacturer, dosage form, strength, and package / product version.

Why might an inactive ingredient be missing?

Some SPLs do not provide a complete structured inactive-ingredient list, and older or unusual labels may only include the information in narrative text.

Can inactive ingredients matter?

Yes. They can matter for allergies, intolerances, dyes, gluten / lactose concerns, preservatives, and formulation differences — but confirm with a pharmacist or the manufacturer when it’s clinically important.

💲 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 mL	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 · quarterly	No Part D plan price is available for this NDC in our data.

ℹ️

No price is published for this exact package yet. CMS surveys NADAC per package size, so a different pack of the same drug often has one.

Where does this data come from?

NADAC (National Average Drug Acquisition Cost) is the CMS weekly pharmacy-acquisition-cost survey — what pharmacies pay. ASP (Average Sales Price) is the CMS Medicare Part B drug-payment file, published quarterly. Medicaid pays is computed by us from CMS State Drug Utilization Data (total reimbursed ÷ units, trailing 12 months) — gross of rebates and inclusive of dispensing fees, so it reflects what Medicaid paid, not an acquisition cost. Medicare drug plans pay is the median negotiated point-of-sale unit cost across plans listing this NDC in the CMS quarterly Prescription Drug Plan pricing files, before rebates. The VA pays is the federal contract price (FSS, and the statutory Big 4 ceiling where listed) from the VA National Acquisition Center pharmaceutical price file. All are free public government data; each measures a different payer, so the figures are not directly comparable.

🔁 Therapeutic equivalents

Product	Labeler	Pack	NADAC/unit	TE	Status	Price vs. this
Sodium Phenylacetate And Sodium Benzoate 100 mg/mL; 100 mg/mL 68382-0396-01	Zydus	1 vial	—	AP	FDA listed	—
Sodium Phenylacetate and sodium benzoate 100 mg/mL; 100 mg/mL 68475-0001-50	Navinta	1 vial	—	AP	FDA listed	—
Sodium Phenylacetate And Sodium Benzoate 100 mg/mL; 100 mg/mL 70511-0101-50	MAIA	1 vial	—	AP	FDA listed	—
SODIUM PHENYLACETATE and SODIUM BENZOATE 100 mg/mL; 100 mg/mLthis 70556-0100-50	Ailex	1 vial	—	AP	FDA listed	—
SODIUM PHENYLACETATE and SODIUM BENZOATE 100 mg/mL; 100 mg/mL 81665-0203-01	Omnivium	1 vial	—	AP	FDA listed	—
SODIUM PHENYLACETATE and SODIUM BENZOATE 100 mg/mL; 100 mg/mL 81665-0204-01	Omnivium	1 vial	—	AP	FDA listed	—
Sodium Phenylacetate and Sodium Benzoate 100 mg/mL; 100 mg/mL 70511-0102-20	MAIA	1 vial	—	AP	FDA listed	—
Sodium phenylacetate and Sodium benzoate 100 mg/mL; 100 mg/mL 70710-1926-01	Zydus	1 vial	—	AP	FDA listed	—
Sodium phenylacetate and Sodium benzoate 100 mg/mL; 100 mg/mL 68475-0513-01	Navinta	1 vial	—	AP	FDA listed	—
Sodium Phenylacetate and Sodium Benzoate 100 mg/mL; 100 mg/mL 72266-0236-01	FOSUN	1 vial	—	AP	FDA listed	—

About this product: this is a generic version of the medicine. FDA equivalence ratings are shown when available, and other versions are listed above, least expensive first.

Where does this data come from?

Equivalents are other NDCs of the same ingredient, form and route from the openFDA NDC Directory, ranked least-expensive-first by NADAC. Therapeutic-equivalence (AB) ratings come from the FDA Orange Book; biologics use the FDA Purple Book for biosimilar & interchangeable status.

⏳ Availability & generic status

🏛️

2016

On the market since

Feb 2016

📍

2026

Currently FDA-listed

10 years listed

🔓

Generic on the market

this product is a generic

✅This is a generic drug

This product is an FDA-approved generic. Other versions of the same drug are listed under Therapeutic equivalents, least expensive first.

Where does this data come from?

Patents and exclusivity from the FDA Orange Book (small-molecule drugs), refreshed from public FDA data. Generic launch timing is an estimate, not a guarantee.

📦 Packaging — all sizes for this product

Package NDC	Description	Marketing start	Status
70556-0100-50 You're viewing this	1 VIAL, SINGLE-DOSE in 1 CARTON (70556-100-50) / 50 mL in 1 VIAL, SINGLE-DOSE	2016-02-26	Active

🧭 About this NDC listing & data coverage

Finished prescription product

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.

“Not published” reflects what the public FDA / CMS / NLM sources provide for this exact package code — it is a property of the data feeds, not a judgment about the product.

Questions about this listing

Why is there no price listed?

The pricing shown on our NDC pages comes from CMS NADAC, a voluntary survey of retail community pharmacy invoices. CMS does not publish a NADAC for every NDC — packages outside the retail survey (institutional and hospital products, bulk packages, discontinued items, and many OTC items) may never receive one. A missing price reflects the survey's scope, not this product's actual cost, and does not mean the product is free or unavailable.

Is the NDC printed on the package the same as the 11-digit billing NDC?

Yes, they identify this exact package in different formats. The FDA registers it as 70556-100-50, which is what is printed on the packaging and shown on DailyMed. Insurance claims use a fixed 11-digit 5-4-2 format, so the short segment is padded with a leading zero: 70556-0100-50, written without dashes as 70556010050. The Identity section at the top of this page lists every form of this code.

What do the three segments of this NDC mean?

In 70556-0100-50, the first segment (70556) is the labeler code FDA assigned to Ailex Pharmaceuticals, LLC; the middle segment (0100) identifies this specific product — its ingredient, strength, and dosage form; and the last segment (50) identifies this exact package size and type. Together they name one specific package of one specific product.

Is this package still being marketed?

Yes, per the latest FDA NDC Directory data on this page: this package is listed as actively marketed, with no marketing end date reported by Ailex Pharmaceuticals, LLC. Listing status can change — the directory data on this page refreshes weekly.

Who lists this product with the FDA?

Ailex Pharmaceuticals, LLC is the labeler of record for this NDC — the company under whose FDA-assigned code the package is listed. The labeler may be the manufacturer itself or a distributor marketing the product under its own code.

Do I need a prescription for this product?

This NDC is listed with FDA as a prescription product, so it is dispensed under a prescriber's order. Your pharmacist can tell you whether any over-the-counter forms of the same medication exist.

This page identifies an FDA-listed package (the NDC) and reports public regulatory and pricing data about the listing. It is reference information, not a medical recommendation — talk to your pharmacist or prescriber about your own medication.

Where does this data come from?

Listing facts (marketing category, packager status, marketing dates) from the FDA openFDA NDC Directory; label availability from DailyMed; pricing coverage from CMS NADAC; equivalence scope from the FDA Orange Book.

📄 Full prescribing information FDA SPL

The complete FDA label for this product — the official prescribing information, verbatim, section by section. Jump with a chip, search within the label, or expand everything.

🎯 Indications and Usage ⏱️ Dosage and Administration 💊 Dosage Forms and Strengths ⛔ Contraindications ⚠️ Warnings and Cautions 🤒 Adverse Reactions 🔄 Drug Interactions 👥 Use in Specific Populations 🤰 Pregnancy 🧒 Pediatric Use 🧓 Geriatric Use 🆘 Overdosage 🧬 Clinical Pharmacology 🧬 Mechanism of Action 📦 How Supplied / Storage and Handling 📦 Storage and Handling 📋 Description 💬 Information for Patients

🎯 Indications and Usage 104 words ▾

1 INDICATIONS AND USAGE Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% is indicated as adjunctive therapy in pediatric and adult patients for the treatment of acute hyperammonemia and associated encephalopathy in patients with deficiencies in enzymes of the urea cycle. During acute hyperammonemic episodes, arginine supplementation, caloric supplementation, dietary protein restriction, hemodialysis, and other ammonia lowering therapies should be considered [see Warnings and Precautions (5) ]. Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% is a nitrogen binding agent indicated as adjunctive therapy for the treatment of acute hyperammonemia and associated encephalopathy in patients with deficiencies in enzymes of the urea cycle.

( 1 )

⏱️ Dosage and Administration ~3 min read ▾

2 DOSAGE AND ADMINISTRATION Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% must be diluted with sterile 10% Dextrose Injection (D10W) before administration. Administration must be through a central venous catheter. Administration through a peripheral line may cause burns.

( 2 ) Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% is administered intravenously as a loading dose infusion administered over 90 to 120 minutes, followed by an equivalent maintenance dose infusion administered over 24 hours. ( 2 )

2.1Recommended Dose Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% must be diluted with sterile 10% Dextrose Injection (D10W) before administration. The dilution and dosage of Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% are determined by weight for neonates, infants and young children, and by body surface area for larger patients, including older children, adolescents, and adults (Table 1). Table 1: Dosage and Administration Patient Population Components of Infusion Solution Sodium Phenylacetate and Sodium Benzoate Injection must be diluted with sterile 10% Dextrose Injection at ≥25 mL/kg before administration.

Dosage Provided Sodium Phenylacetate and Sodium Benzoate Injection Arginine HCl Injection, 10% Sodium Phenylacetate Sodium Benzoate Arginine HCl Patients 0 to 20 kg: CPS and OTC Deficiency Dose Loading: over 90 to 120 minutes 2.5 mL/kg 2 mL/kg 250 mg/kg 250 mg/kg 200 mg/kg Maintenance: over 24 hours ASS and ASL Deficiency Dose Loading: over 90 to 120 minutes 2.5 mL/kg 6 mL/kg 250 mg/kg 250 mg/kg 600 mg/kg Maintenance: over 24 hours Patients > 20 kg: CPS and OTC Deficiency Dose Loading: over 90 to 120 minutes 55 mL/m 2 2 mL/kg 5.5 g/m 2 5.5 g/m 2 200 mg/kg Maintenance: over 24 hours ASS and ASL Deficiency Dose Loading: over 90 to 120 minutes 55 mL/m 2 6 mL/kg 5.5 g/m 2 5.5 g/m 2 600 mg/kg Maintenance: over 24 hours

2.2Administration Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% is a concentrated solution and must be diluted before intravenous administration via a central venous catheter. Administration through a peripheral intravenous catheter may cause burns. Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% may not be administered by any other route.

Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% should be administered as a loading dose infusion over 90 to 120 minutes, followed by the same dose repeated as a maintenance infusion administered over 24 hours. Because of prolonged plasma levels achieved by phenylacetate in pharmacokinetic studies, repeat loading doses of Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% should not be administered. Maintenance infusions may be continued until elevated plasma ammonia levels have been normalized or the patient can tolerate oral nutrition and medications.

An antiemetic may be administered during Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% infusion to aid control of infusion-associated nausea and vomiting. Administration of analogous oral drugs, such as sodium phenylbutyrate, should be terminated prior to Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% infusion. Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% infusion should be started as soon as the diagnosis of hyperammonemia is made.

Treatment of hyperammonemia also requires caloric supplementation and restriction of dietary protein. Non-protein calories should be supplied principally as glucose (8–10 mg/kg/min) with an intravenous fat emulsion added. Attempts should be made to maintain a caloric intake of greater than 80 kcal/kg/day.

During and after infusion of Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10%, ongoing monitoring of the following clinical laboratory values is crucial: plasma ammonia, glutamine, quantitative plasma amino acids, blood glucose, electrolytes, venous or arterial blood gases, AST and ALT. On-going monitoring of the following clinical responses is a…

💊 Dosage Forms and Strengths 41 words ▾

3 DOSAGE FORMS AND STRENGTHS Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% is a sterile, concentrated, aqueous solution of sodium phenylacetate and sodium benzoate. Injection: 10% per 10% sterile, concentrated, aqueous solution of sodium phenylacetate and sodium benzoate. ( 3 )

⛔ Contraindications 7 words ▾

4 CONTRAINDICATIONS None. None ( 4 )

⚠️ Warnings and Cautions ~3 min read ▾

5 WARNINGS AND PRECAUTIONS Management of Acute Hyperammonemia : Monitor plasma ammonia levels during treatment. Prolonged exposure to elevated plasma ammonia levels can rapidly result in injury to the brain or death. Prompt use of all therapies necessary to reduce plasma ammonia levels is essential.

( 5.1 ) Decreased Potassium Levels : Plasma potassium levels should be carefully monitored and appropriate treatment given when necessary. ( 5.2 ) Conditions Associated with Fluid Overload : Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% contains 30.5 mg of sodium per mL of undiluted product. Caution should be used if Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% is administered to patients with congestive heart failure, severe renal insufficiency, or with conditions in which there is sodium retention with edema.

( 5.3 ) Extravasation : Extravasation of Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% into the perivenous tissues during high flow bolus infusion may lead to skin necrosis, especially in infants. The infusion site must be monitored closely for possible tissue infiltration during drug administration. ( 5.4 ) Neurotoxicity of Phenylacetate: Because of prolonged plasma levels achieved by phenylacetate in pharmacokinetic studies, repeat loading doses should not be administered.

Additionally, neurotoxicity related to phenylacetate has been reported in cancer patients. Monitor patients for symptoms of acute neurotoxicity. ( 5.5 ) Hyperventilation and Metabolic Acidosis: Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% may cause side effects typically associated with salicylate overdose, such as hyperventilation and metabolic acidosis.

Monitor patient blood chemistry profiles and perform frequent blood pH and pCO 2 measurements. ( 5.6 )

5.1Management of Acute Hyperammonemia Any episode of acute symptomatic hyperammonemia should be treated as a life-threatening emergency. Uncontrolled hyperammonemia can rapidly result in brain damage or death, and prompt use of all therapies necessary, including hemodialysis, to reduce ammonia levels is essential. Hyperammonemic coma (regardless of cause) in the newborn infant should be aggressively treated while the specific diagnosis is pursued.

Hemodialysis should be promptly initiated in all newborn patients. A blood flow rate of 150 mL/min/m 2 should be targeted (ammonia clearance [mL/min] is similar to the blood flow rate [mL/min] through the dialyzer). Clearance of ammonia is approximately ten times greater by hemodialysis than by peritoneal dialysis or hemofiltration.

Exchange transfusion is ineffective in the management of hyperammonemia. Hemodialysis may be repeated until the plasma ammonia level is stable at normal or near normal levels. Hyperammonemia due to urea cycle disorders should be managed in coordination with medical personnel experienced in metabolic disorders.

Ongoing monitoring of plasma ammonia levels, neurological status, laboratory tests, and clinical response in patients receiving Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% is crucial to assess patient response to treatment.

5.2Decreased Potassium Levels Because urine potassium loss is enhanced by the excretion of the non-reabsorbable anions, phenylacetylglutamine and hippurate, plasma potassium levels should be carefully monitored and appropriate treatment given when necessary.

5.3Conditions Associated with Fluid Overload Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% contains 30.5 mg of sodium per mL of undiluted product. Thus, Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% should be used with great care, if at all, in patients with congestive heart failure or severe renal insufficiency, and in clinical states in which there is sodium retention with edema. Discontinue administration of Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10%, evaluate the patient, and institute appropriate therapeutic countermeasures if…

🤒 Adverse Reactions ~3 min read ▾

6 ADVERSE REACTIONS The most frequently reported adverse reactions (incidence ≥6%) are vomiting, hyperglycemia, hypokalemia, convulsions, and mental impairment. ( 6 ) To report SUSPECTED ADVERSE REACTIONS, contact Ailex Pharmaceuticals, LLC at 1-888-514-4727 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.

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 clinical practice. The safety data were obtained from 316 patients who received Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% as emergency (rescue) or prospective treatment for hyperammonemia as part of an uncontrolled, open-label study.

The study population included patients between the ages of 0 to 53 years with a mean (SD) of 6.2 (8.54) years; 51% were male and 49% were female who had the following diagnoses: OTC (46%), ASS (22%), CPS (12%), ASL (2%), ARG (<1%), THN (<1%), and other (18%). Table 2: Adverse Reactions Occurring in ≥3% of Patients Treated with Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% Patients N=316 Number of patients with any adverse event 163 (52%) Blood and lymphatic system disorders 35 (11%) Anemia 12 (4%) Disseminated intravascular coagulation 11 (3%) Cardiac disorders 28 (9%) Gastrointestinal disorders 42 (13%) Diarrhea 10 (3%) Nausea 9 (3%) Vomiting 29 (9%) General disorders and administration-site conditions 45 (14%) Injection-site reaction 11 (3%) Pyrexia 17 (5%) Infections 39 (12%) Urinary tract infection 9 (3%) Injury, poisoning and procedural complications 12 (4%) Investigations 32 (10%) Metabolism and nutrition disorders 67 (21%) Acidosis 8 (3%) Hyperammonemia 17 (5%) Hyperglycemia 22 (7%) Hypocalcemia 8 (3%) Hypokalemia 23 (7%) Metabolic acidosis 13 (4%) Nervous system disorders 71 (22%) Brain edema 17 (5%) Coma 10 (3%) Convulsions 19 (6%) Mental impairment 18 (6%) Psychiatric disorders 16 (5%) Agitation 8 (3%) Renal and urinary disorders 14 (4%) Respiratory, thoracic and mediastinal disorders 47 (15%) Respiratory distress 9 (3%) Skin and subcutaneous tissue disorders 19 (6%) Vascular disorders 19 (6%) Hypotension 14 (4%) Adverse reactions were reported with similar frequency in patients with OTC, ASS, CPS, and diagnoses categorized as "other." Nervous system disorders were more frequent in patients with OTC and CPS, compared with patients with ASS and patients with "other" diagnoses.

Convulsions and mental impairment were reported in patients with OTC and CPS. These observations are consistent with literature reports that patients with enzyme deficiencies occurring earlier in the urea cycle (i.e., OTC and CPS) tend to be more severely affected. Adverse reactions profiles differed by age group.

Patients ≤30 days of age had more blood and lymphatic system disorders and vascular disorders (specifically hypotension), while patients >30 days of age had more gastrointestinal disorders (specifically nausea, vomiting and diarrhea). Less common adverse reactions (<3% of patients) that are characterized as severe are listed below by body system. BLOOD AND LYMPHATIC SYSTEM DISORDERS: coagulopathy, pancytopenia, thrombocytopenia CARDIAC DISORDERS: atrial rupture, bradycardia, cardiac or cardiopulmonary arrest/failure, cardiogenic shock, cardiomyopathy, pericardial effusion EYE DISORDERS: blindness GASTROINTESTINAL DISORDERS: abdominal distension, gastrointestinal hemorrhage GENERAL DISORDERS AND ADMINISTRATION-SITE CONDITIONS: asthenia, brain death, chest pain, multiorgan failure, edema HEPATOBILIARY DISORDERS: cholestasis, hepatic artery stenosis, hepatic failure/hepatotoxicity, jaundice INFECTIONS AND INFESTATIONS: sepsis/septic shock INJURY, POISONING AND PROCEDURAL COMPLICATIONS: brain herniation, subdural hematoma, overdose INVESTIGATIONS: blood carbon dioxide changes, blood glucose chang…

🔄 Drug Interactions ~1 min read ▾

7 DRUG INTERACTIONS Formal drug interaction studies have not been performed with Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10%. Some antibiotics such as penicillin may compete with phenylacetylglutamine and hippurate for active secretion by renal tubules, which may affect the overall disposition of the infused drug. Probenecid is known to inhibit the renal transport of many organic compounds, including aminohippuric acid, and may affect renal excretion of phenylacetylglutamine and hippurate.

There have been reports that valproic acid can induce hyperammonemia through inhibition of the synthesis of N-acetylglutamate, a co-factor for carbamyl phosphate synthetase. Therefore, administration of valproic acid to patients with urea cycle disorders may exacerbate their condition and antagonize the efficacy of Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10%. Use of corticosteroids may cause a protein catabolic state and, thereby, potentially increase plasma ammonia levels in patients with impaired ability to form urea.

Some antibiotics such as penicillin may affect the overall disposition of the infused drug. ( 7 ) Probenecid may affect renal excretion of phenylacetylglutamine and hippurate. ( 7 ) Valproic acid given to patients with urea cycle disorders may exacerbate their condition and antagonize the efficacy of Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% through inhibition of the synthesis of N-acetylglutamate, a co-factor for carbamyl phosphate synthetase.

( 7 ) Use of corticosteroids may cause the breakdown of body protein and potentially increase plasma ammonia levels in patients with impaired ability to form urea. ( 7 )

👥 Use in Specific Populations ~2 min read ▾

8 USE IN SPECIFIC POPULATIONS

8.1Pregnancy Pregnancy Category C. Animal reproduction studies have not been conducted with Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10%. It is not known whether Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% can cause fetal harm when administered to a pregnant woman or can affect reproduction capacity. Thus, Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% should be given to a pregnant woman only if clearly needed.

8.3Nursing Mothers It is not known whether sodium phenylacetate, sodium benzoate, or their conjugation products are excreted in human milk. Because many drugs are excreted in human milk, caution should be exercised when Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% is administered to a nursing woman.

8.4Pediatric Use Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% has been used as a treatment for acute hyperammonemia in pediatric patients including patients in the early neonatal period [see Dosage and Administration (2) ].

8.5Geriatric Use Clinical studies of Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% did not include any patients aged 65 and over to determine whether they respond differently from younger patients. Urea cycle disorders are presently diseases of the pediatric and younger adult populations. No pharmacokinetic studies of Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% have been performed in geriatric patients.

In general, dose selection for an elderly patient should be cautious, usually starting at the low end of the dosing range, reflecting the greater frequency of decreased hepatic, renal, or cardiac function, and concomitant disease or other drug therapy in this patient population.

8.6Gender Pharmacokinetic parameters of Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% were compared in healthy males and females. Bioavailability of both benzoate and phenylacetate was slightly higher in females than in males. However, conclusions cannot be drawn due to the limited number of subjects in this study.

8.7Hepatic Insufficiency Limited information is available on the metabolism and excretion of sodium phenylacetate and sodium benzoate in patients with impaired hepatic function. However, metabolic conjugation of sodium phenylacetate and sodium benzoate is known to take place in the liver and kidney. Therefore, caution should be used in administering Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% to patients with hepatic insufficiency.

8.8Renal Impairment The drug metabolites of Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% (phenylacetylglutamine and hippurate) and subsequently ammonia are primarily excreted by the kidney. Therefore, use caution and closely monitor patients with impaired renal function who receive Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10%.

🤰 Pregnancy 66 words ▾

🧒 Pediatric Use 35 words ▾

🧓 Geriatric Use 104 words ▾

🆘 Overdosage 200 words ▾

10 OVERDOSAGE Overdosage has been reported during Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% treatment in urea cycle-deficient patients. All patients in the uncontrolled open-label study were to be treated with the same dose of Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10%. However, some patients received more than the dose level specified in the protocol.

In 16 of the 64 deaths, the patient received a known overdose of Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10%. Causes of death in these patients included cardiorespiratory failure/arrest (6 patients), hyperammonemia (3 patients), increased intracranial pressure (2 patients), pneumonitis with septic shock and coagulopathy (1 patient), error in dialysis procedure (1 patient), respiratory failure (1 patient), intractable hypotension and probable sepsis (1 patient), and unknown (1 patient). Additionally, other signs of intoxication may include obtundation (in the absence of hyperammonemia), hyperventilation, a severe compensated metabolic acidosis, perhaps with a respiratory component, large anion gap, hypernatremia and hyperosmolarity, progressive encephalopathy, cardiovascular collapse, and death.

In case of overdose of Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10%, discontinue the drug and institute appropriate emergency medical monitoring and procedures. In severe cases, the latter may include hemodialysis (procedure of choice) or peritoneal dialysis (when hemodialysis is unavailable).

🧬 Clinical Pharmacology ~3 min read ▾

12 CLINICAL PHARMACOLOGY

12.1Mechanism of Action Urea cycle disorders can result from decreased activity of any of the following enzymes: N -acetylglutamate synthetase (NAGS), carbamyl phosphate synthetase (CPS), argininosuccinate synthetase (ASS), ornithine transcarbamylase (OTC), argininosuccinate lyase (ASL), or arginase (ARG). Sodium phenylacetate and sodium benzoate are metabolically active compounds that can serve as alternatives to urea for the excretion of waste nitrogen. Figure 2 is a schematic illustrating how the components of Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10%, phenylacetate and benzoate, provide an alternative pathway for nitrogen disposal in patients without a fully functioning urea cycle.

Phenylacetate conjugates with glutamine in the liver and kidneys to form phenylacetylglutamine, via acetylation. Phenylacetylglutamine is excreted by the kidneys via glomerular filtration and tubular secretion. The nitrogen content of phenylacetylglutamine per mole is identical to that of urea (both contain two moles of nitrogen).

Two moles of nitrogen are removed per mole of phenylacetate when it is conjugated with glutamine. Similarly, preceded by acylation, benzoate conjugates with glycine to form hippuric acid, which is rapidly excreted by the kidneys by glomerular filtration and tubular secretion. One mole of hippuric acid contains one mole of waste nitrogen.

Thus, one mole of nitrogen is removed per mole of benzoate when it is conjugated with glycine. Figure 2 CPS = carbamyl phosphate synthetase; OTC = ornithine transcarbamylase; ASS = argininosuccinate synthetase; ASL = argininosuccinate lyase; ARG = arginase; NAGS = N -acetylglutamate synthetase Figure 2

12.2Pharmacodynamics In patients with hyperammonemia due to deficiencies in enzymes of the urea cycle, Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% has been shown to decrease elevated plasma ammonia levels. These effects are considered to be the result of reduction in nitrogen overload through glutamine and glycine scavenging by Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% in combination with appropriate dietary and other supportive measures.

12.3Pharmacokinetics The pharmacokinetics of intravenously administered Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% was characterized in healthy adult volunteers. Both benzoate and phenylacetate exhibited nonlinear kinetics. Following 90 minute intravenous infusion mean AUC last for benzoate was 20.3, 114.9, 564.6, 562.8, and 1599.1 mcg/mL following doses of 1, 2, 3.75, 4, and 5.5 g/m 2 , respectively.

The total clearance decreased from 5.19 to

3.62L/h/m 2 at the 3.75 and 5.5 g/m 2 doses, respectively. Similarly, phenylacetate exhibited nonlinear kinetics following the priming dose regimens. AUC last was 175.6, 713.8, 2040.6, 2181.6, and 3829.2 mcg∙h/mL following doses of 1, 2, 3.75, 4, and 5.5 g/m 2 , respectively.

The total clearance decreased from 1.82 to 0.89 mcg∙h/mL with increasing dose (3.75 and 4 g/m 2 , respectively). During the sequence of 90 minute priming infusion followed by a 24 hour maintenance infusion, phenylacetate was detected in the plasma at the end of infusion (T max of 2 hr at 3.75 g/m 2 ) whereas, benzoate concentrations declined rapidly (T max of 1.5 hr at 3.75 g/m 2 ) and were undetectable at 14 and 26 hours following the 3.75 and 4 g/m 2 dose, respectively. A difference in the metabolic rates for phenylacetate and benzoate was noted.

The formation of hippurate from benzoate occurred more rapidly than that of phenylacetylglutamine from phenylacetate, and the rate of elimination for hippurate appeared to be more rapid than that for phenylacetylglutamine. Pharmacokinetic observations have also been reported from twelve episodes of hyperammonemic encephalopathy in seven children diagnosed (age 3 to 26 months) with urea cycle disorders who had been administered Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% intravenously. T…

🧬 Mechanism of Action ~1 min read ▾

📦 How Supplied / Storage and Handling 46 words ▾

16 HOW SUPPLIED/STORAGE AND HANDLING Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% is supplied in a single-dose glass vial. NDC 70556-100-50 single-dose vial containing 50 mL of sodium phenylacetate and sodium benzoate injection 10% per 10%. Storage: Store at 25°C (77°F), excursions permitted to 15°–30°C (59°–86°F).

📦 Storage and Handling 10 words ▾

Storage: Store at 25°C (77°F), excursions permitted to 15°–30°C (59°–86°F).

📋 Description 179 words ▾

11 DESCRIPTION Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% (a nitrogen binding agent), is a sterile, concentrated, aqueous solution of sodium phenylacetate and sodium benzoate. The pH of the solution is between 6 and 8. Sodium phenylacetate is a crystalline, white to off-white powder with a strong, offensive odor.

It is soluble in water. Sodium benzoate is a white and odorless, crystalline powder that is readily soluble in water. Figure 1 Sodium phenylacetate has a molecular weight of 158.13 and the molecular formula C 8 H 7 NaO 2 .

Sodium benzoate has a molecular weight of 144.11 and the molecular formula C 7 H 5 NaO 2 . Each mL of Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% contains 100 mg of sodium phenylacetate and 100 mg of sodium benzoate, and Water for Injection. Sodium hydroxide and/or hydrochloric acid may have been used for pH adjustment.

Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% injection is a sterile, concentrated solution intended for intravenous administration via a central line only after dilution [see Dosage and Administration (2) ]. Chemical Structure

💬 Information for Patients 91 words ▾

17 PATIENT COUNSELING INFORMATION Physicians should advise patients and caregivers about the following for safe use of Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10%: When plasma ammonia levels have normalized, dietary protein intake can usually be increased with the goal of unrestricted protein intake. Caution should be exercised when Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% is administered to a nursing woman. The most common adverse reactions are vomiting, hyperglycemia, hypokalemia, convulsions, and mental impairment.

Generally BUPHENYL is stopped during the time Sodium Phenylacetate and Sodium Benzoate Injection, 10%/10% is used.

Glossary — plain-language definitions of medical terms used in this label:

abdominal distension: A swollen or stretched feeling in the belly, often from gas or fluid.

adjunctive therapy: A medicine added alongside a main treatment to help it work better.

adverse event: Any unwanted medical problem that happens during treatment, whether or not the medicine actually caused it.

adverse reaction: An unwanted or harmful effect that a medicine causes in the body.

Adverse Reactions: Unwanted or harmful effects that people experienced while taking a medicine, listed on its official label.

agitation: A state of feeling restless and upset and being unable to relax, sometimes with pacing or fidgeting.

ALT: A liver enzyme (alanine aminotransferase) checked by a blood test; raised levels can signal that the liver is irritated or damaged.

anemia: A low level of red blood cells or the oxygen-carrying protein inside them, which can cause tiredness, weakness, and paleness.

animal reproduction studies: Tests in pregnant animals used to check whether a medicine might harm a developing baby.

AST: A liver enzyme (aspartate aminotransferase) measured in a blood test; higher levels may point to liver or sometimes muscle injury.

asthenia: Unusual weakness or lack of physical strength and energy.

AUC: A measure of total drug exposure over time, showing how much medicine the blood is exposed to overall.

Bioavailability: The share of a dose that actually reaches the bloodstream and is able to have an effect.

bolus: A single dose given all at once, often as a quick push into a vein.

bradycardia: A slower-than-normal heart rate, usually under 60 beats per minute.

cardiomyopathy: A disease of the heart muscle that makes it harder for the heart to pump blood.

cholestasis: A slowing of bile flow out of the liver; bile is the fluid that helps digest fat, and this backup can cause itching and yellow skin.

clearance: How quickly the body removes a medicine from the blood, usually through the liver and kidneys.

Clinical Pharmacology: The label section explaining how a medicine works in the body and how the body processes it.

Clinical Studies: The label section summarizing the research trials used to show that a medicine works and is acceptably safe.

coagulopathy: A problem with the blood's ability to clot properly, which can cause too much bleeding or unwanted clotting.

Contraindications: A list of situations or health conditions in which a medicine should not be used because the chance of harm is too high.

diarrhea: Loose, watery bowel movements that happen more often than normal.

disseminated intravascular coagulation: A serious condition where many small clots form throughout the body, using up the proteins needed to clot and causing bleeding.

Dosage and Administration: The label section explaining how much of a medicine to use and how to take or give it.

Drug Interactions: The label section listing other medicines, foods, or substances that can change how a medicine works or raise the chance of side effects.

edema: Swelling caused by extra fluid trapped in the body's tissues.

efficacy: How well a treatment works under the controlled conditions of a clinical study.

Elimination: The overall removal of a medicine from the body by breaking it down and passing it out.

encephalopathy: Any condition that affects how the brain works, causing confusion, memory problems, or changes in alertness.

Excretion: How the body removes a medicine or its leftover pieces, mainly through the urine or stool.

extravasation: The accidental leaking of an injected medicine out of the vein into the surrounding tissue, which can cause pain, swelling, or tissue damage.

fetal harm: Injury or damage to a developing baby while it is growing inside the womb.

heart failure: A condition in which the heart cannot pump blood as well as it should, causing tiredness, breathlessness, and swelling.

hematoma: A pocket of blood that collects outside a blood vessel, often causing swelling and a bruise.

hemodialysis: A treatment that uses a machine to filter waste and extra fluid from the blood when the kidneys cannot.

hemorrhage: Bleeding, especially heavy or uncontrolled bleeding, either inside the body or from an outside wound.

hepatotoxicity: Damage to the liver caused by a medicine or other substance.

How Supplied/Storage and Handling: The label section describing the forms and strengths a medicine comes in and how to store it properly.

hyperglycemia: High blood sugar, meaning there is more glucose (sugar) in the blood than normal.

hypernatremia: A higher-than-normal level of sodium (salt) in the blood.

hypocalcemia: A lower-than-normal level of calcium, a mineral, in the blood.

hypokalemia: A lower-than-normal level of potassium, an important mineral, in the blood.

hypotension: Blood pressure that is lower than normal, which can cause dizziness or fainting.

incidence: How often a new event, such as a side effect, happens in a group of people over a set period of time.

Indications and Usage: The label section stating which medical conditions the FDA has officially approved the medicine to treat, prevent, or manage.

infusion: Slowly delivering fluid or medicine into a vein over a period of time, usually through a drip.

intravenous: Given directly into a vein through a needle or thin tube; often written as IV.

jaundice: Yellowing of the skin and the whites of the eyes caused by too much bilirubin in the blood.

loading dose: A larger dose given at the start to quickly build the medicine up to an effective level in the body.

maintenance dose: The regular ongoing dose taken to keep a steady, effective amount of medicine in the body over time.

Mechanism of Action: How a medicine produces its effect in the body, described at the level of cells or body chemistry.

metabolic acidosis: A condition where too much acid builds up in the body's blood and fluids.

Metabolism: How the body chemically changes a medicine, usually in the liver, into other substances.

NDC: A unique number that identifies a specific medicine, its maker, strength, and package size.

neonatal: Relating to a newborn baby during its first four weeks of life.

open-label: A study in which both the patients and their doctors know which treatment each person is receiving.

Overdosage: The label section describing what can happen if too much of a medicine is taken and how it may be treated.

pancytopenia: A low level of all three main blood cell types at once: red cells, white cells, and platelets.

Patient Counseling Information: The label section telling health professionals what to explain to patients about using a medicine safely.

Pharmacokinetics: The study of how the body takes in a medicine, spreads it around, breaks it down, and removes it over time.

pneumonitis: Inflammation of the lung tissue that is not caused by an infection and can lead to cough and breathlessness.

pregnancy category: An older letter rating (A, B, C, D, or X) once used to describe a medicine's possible risk during pregnancy.

pyrexia: A raised body temperature; a fever.

renal excretion: Removal of a medicine from the body through the kidneys into the urine.

renal impairment: Reduced kidney function, meaning the kidneys do not filter waste and fluid from the blood as well as normal.

respiratory failure: A serious condition in which the lungs cannot get enough oxygen into the blood or remove enough carbon dioxide.

sepsis: A serious, body-wide reaction to an infection in which the body's own response can start to injure its tissues and organs.

single-dose vial: A small container holding one dose of medicine, meant to be used once and then thrown away.

subcutaneous: An injection placed into the fatty layer just under the skin; often written as subcut or SC.

thrombocytopenia: A low level of platelets, the blood cells that help form clots, which can lead to easier bruising or bleeding.

Use in Specific Populations: The label section describing how a medicine affects particular groups, such as pregnant people, children, or older adults.

Warnings and Precautions: The label section describing serious risks to know about and safety steps to follow while using a medicine.

Source: FDA Structured Product Labeling, mirrored from DailyMed / openFDA. Prefer the government’s original formatting? View this label on DailyMed ↗

← 70554-0339-00 · THE 70558-0004-01 · Pro →

For educational and professional reference only — not medical advice. Pricing reflects published NADAC and CMS ASP (free public data) and may differ from your acquisition cost; always verify before billing or dispensing.

SODIUM PHENYLACETATE and SODIUM BENZOATE 100 mg/mL; 100 mg/mL Injection, Solution, Concentrate

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12 CLINICAL PHARMACOLOGY

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