HomeNDC LookupIngredientsHuman C1-Esterase Inhibitor › 63833-0825-02
Berinert HUMAN C1-ESTERASE INHIBITOR Kit — NDC 63833-0825-02 package photo

Berinert HUMAN C1-ESTERASE INHIBITOR Kit

by CSL Behring GmbH · 1 KIT in 1 CARTON (63833-825-02) * 10 mL in 1 VIAL (63833-835-01) * 10 mL in 1 VIAL (63833-765-15)
NDC 63833-0825-02
🏷️ FDA NDC (as labeled) 63833-825-02 billing pads the product segment with a zero
Brand On market Non-controlled
🗂️ Data synced Sep 17, 2026 · sources: openFDA · FDA label (DailyMed) · FDA Orange & Purple Book · First Databank · CMS NADAC, ASP, Medicare & Medicaid · RxNorm
📋 All sources & update times →

🆔 Identity & classification

FDA NDC (as labeled) 63833-825-02
Product NDC 63833-825
11-digit billing NDC 63833082502
NCPDP billing unit EA — each (per item)
Application # BLA125287
SPL Set ID 8ea0c7fe-da9d-4218-a080-0509e09bedc6
DEA schedule Non-controlled
Marketing category BLA
Marketing status On market
FDA listing status Listed (active directory)
Marketing start 2011-12-22
Dosage form KIT
GPI-14 85802022006420
GPI class Berinert
GCN Seq No 068384
GCN 31159
HICL code 018568
Ingredient (HICL) C1 Esterase Inhibitor
HIC1 code M
Therapeutic class — broad (HIC1) Blood
HIC2 code M0
Therapeutic class — intermediate (HIC2) Blood And Blood Replacement Preparations
HIC3 code M0N
Therapeutic class — specific (HIC3) C1 Esterase Inhibitors
AHFS code 92:32.00.00
AHFS class Complement Inhibitors (92:32)
FDB label name BERINERT 500 UNIT KIT
FDB brand name Berinert
Legend status F — Federal legend — prescription drug or device
Biologic (Purple Book) 351(a)
Why two NDCs? The FDA registers this code as 63833-825-02 — 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 → 63833-0825-02. 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.

🏭 Manufacturer & labeler

LabelerCSL Behring GmbH
FDA applicationBLA125287 (BLA)
Labeler code63833
First marketedDec 2011
Product typePlasma Derivative
Portfolio11 products on file
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

Label name BERINERT 500 UNIT KIT Ingredient C1 Esterase Inhibitor
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.

A current SPL was checked, but it does not contain a structured or narrative inactive-ingredient list for this product. This does not mean the product has no inactive ingredients.
Where does this data come from?
Source: official FDA Structured Product Labeling (SPL) via DailyMed and the openFDA label index. Structured IACT rows and label-wide narrative are kept separate; availability and product-level specificity depend on the submitted label.

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 systemPer eachPer package
Retail pharmacies payNADAC · weekly Not in the retail survey — common for institutional, discontinued, or low-volume packs.
Medicaid paysCMS SDUD · 12 mo $4,017.36
Medicare drug plans payPart D · Q2 2026 $4,506.38
Medicare Part B allowsASP · J0597 $79.262 / J0597 unit
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.

🧾 Billing & reimbursement

FDA NDC (as labeled)63833-825-02
11-digit billing NDC63833-0825-02
Format5-3-2 as registered → padded to 5-4-2 for billing (zero added to the product segment)
HCPCS J-codeJ0597
DescriptorInjection, c-1 esterase inhibitor (human), berinert, 10 units
Billing units / pkg50 units
Crosswalk sourceCMS ASP NDC-HCPCS Crosswalk
Where does this data come from?
The HCPCS J-code crosswalk comes from the CMS ASP NDC-HCPCS crosswalk and the DMEPDAC (DME MAC) NDC-HCPCS crosswalk — free public CMS data. Billing units are derived from the code’s descriptor and the package amount.

🔁 Therapeutic equivalents

ProductLabelerPackNADAC/unitTEStatusPrice vs. this
Berinertthis 63833-0825-02 CSL 1 kit FDA listed
HAEGARDA C1 Esterase Inhibitor Subcutaneous (Human) 63833-0828-02 CSL 1 kit FDA listed
HAEGARDA C1 Esterase Inhibitor Subcutaneous (Human) 63833-0829-02 CSL 1 kit FDA listed
Cinryze 42227-0083-01 Takeda 1 kit FDA listed
About this product: this is a biologic. Biologics don't have small-molecule generics — competition comes from FDA-licensed biosimilars (shown above), not generics.
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 & biosimilar status

🏛️
2009
First FDA approval
Oct 2009
📍
2026
Currently FDA-listed
17 years listed
🧬
·
Biosimilars
see Purple Book
🧬Biologic — competition comes from biosimilars

Biologics have no small-molecule generics; biosimilar competition is tracked in the FDA Purple Book.

Where does this data come from?
Patents and exclusivity from the FDA Purple Book (biologics), refreshed from public FDA data. Biosimilar launch timing is an estimate, not a guarantee.

🗺️ Medicaid utilization & spend

📍 This exact package only: Medicaid data is reported per full 11-digit NDC — labeler, product and pack size — so every number here is for 63833-0825-02, not the drug overall. Other pack sizes report separately.
💊 Pharmacy benefit only: These are Medicaid outpatient pharmacy claims, billed by NDC. They exclude the medical benefit — clinic- or hospital-administered drugs billed under HCPCS J-codes — so drugs used mostly that way (e.g. Avastin, Lucentis, Keytruda) can look low or missing here. That’s expected, not an error.
📅 Q1 2025 – Q4 2025 · 4 quarters of data
ⓘ The newest quarter is usually incomplete when first published; states restate recent quarters in later CMS releases, so the latest figures typically revise upward. State coverage-policy changes can also shift quarter-to-quarter totals.
Prescriptions last 4 qtrs
230
Units reimbursed last 4 qtrs
1.8K
Gross reimbursed last 4 qtrs
$7.04M
Avg / prescription
$30,601.83
Avg / unit
$4,017.50
Latest quarter Q4 2025
31Rx
Fee-for-service vs managed care
85% FFS 15% MCO
Fee-for-service · 195 Rx Managed care · 35 Rx
State Medicaid map
Alaska: no data reported AK Maine: no data reported ME Washington: no data reported WA Idaho: no data reported ID Montana: no data reported MT North Dakota: no data reported ND Minnesota: no data reported MN Wisconsin: no data reported WI Michigan: no data reported MI New York: 386 units · 2.0 per 100k residents NY Vermont: no data reported VT New Hampshire: no data reported NH Oregon: no data reported OR Nevada: no data reported NV Wyoming: no data reported WY South Dakota: no data reported SD Iowa: no data reported IA Illinois: no data reported IL Indiana: no data reported IN Ohio: no data reported OH Pennsylvania: no data reported PA New Jersey: no data reported NJ Massachusetts: no data reported MA California: no data reported CA Utah: no data reported UT Colorado: 310 units · 5.3 per 100k residents CO Nebraska: no data reported NE Missouri: 355 units · 5.7 per 100k residents MO Kentucky: no data reported KY West Virginia: no data reported WV Virginia: no data reported VA Maryland: no data reported MD Connecticut: no data reported CT Rhode Island: no data reported RI Arizona: no data reported AZ New Mexico: no data reported NM Kansas: no data reported KS Arkansas: no data reported AR Tennessee: no data reported TN North Carolina: no data reported NC South Carolina: no data reported SC Delaware: no data reported DE Oklahoma: no data reported OK Louisiana: no data reported LA Mississippi: no data reported MS Alabama: 142 units · 2.8 per 100k residents AL Georgia: no data reported GA D.C.: no data reported DC Hawaii: no data reported HI Texas: no data reported TX Florida: 559 units · 2.5 per 100k residents FL
Units reimbursed · per 100k residents
2.05.7
gray = no data reported
Colors are per 100,000 residents, so big states don’t automatically dominate. Tap or hover a state for its actual totals.
Tap or hover a state
…for its Medicaid breakdown
🏆 Top states by units · per 100k residents
1 Missouri 5.7 /100k
2 Colorado 5.3 /100k
3 Alabama 2.8 /100k
4 Florida 2.5 /100k
5 New York 2.0 /100k
National units — by quarter
💵 About the dollar figures: “reimbursed” is what Medicaid paid pharmacies before confidential manufacturer rebates, so the program’s real net cost is lower than these numbers. Fee-for-service and managed-care claims are combined unless split above. Source: CMS State Drug Utilization Data; per-100k rates use 2023 Census population estimates.

📊 Medicare Part D spend CMS · PART D · 2026 (Q1)

Medicare Part D (outpatient prescription) spending for Berinert — the program that covers self-administered drugs. 1 manufacturer.
⚠️ Drug-level data: CMS publishes Part D spending by drug, not by NDC — these figures combine every manufacturer, strength and package size sold under the name Berinert. That’s a different level of aggregation than the Medicaid card above, which is specific to this exact 11-digit NDC (pack size included), so the two aren’t directly comparable.
Period
Total Part D spend
$8.24M
Claims incl. refills
107
Beneficiaries
33
Spend / beneficiary
$249,564.93
Spend / claim
$76,968.62
Trend by period
💵 About the dollar figures: spending is what Part D plans paid before confidential manufacturer rebates, so the program’s real net cost is lower. A blank patient count means fewer than 11 people — CMS hides counts that small to protect privacy. Source: CMS Medicare Quarterly Part D Spending by Drug (data.cms.gov), updated quarterly.

📦 Packaging — all sizes for this product

Package NDCDescription Marketing startStatus
63833-0825-02 You're viewing this 1 KIT in 1 CARTON (63833-825-02) * 10 mL in 1 VIAL (63833-835-01) * 10 mL in 1 VIAL (63833-765-15) 2011-12-22 Active

🧭 About this NDC listing & data coverage

Kit / multi-component package

Kit / multi-component package

This NDC identifies a kit — a package containing more than one component. Structured data (pricing, ingredients, equivalents) is often reported per component rather than for the kit NDC itself, which can make this page look thinner than the components' own pages.

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) — Not published for this NDC The labeler did not submit a structured excipient list, or no SPL is 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 — Not published for this NDC Applies only to products approved under an NDA/ANDA; many listings are out of scope.
HCPCS J-code billing crosswalk ✓ Available
Medicaid utilization (CMS SDUD) ✓ Available
“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 63833-825-02, 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: 63833-0825-02, written without dashes as 63833082502. 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 63833-0825-02, the first segment (63833) is the labeler code FDA assigned to CSL Behring GmbH; the middle segment (0825) identifies this specific product — its ingredient, strength, and dosage form; and the last segment (02) 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 CSL Behring GmbH. Listing status can change — the directory data on this page refreshes weekly.
Who lists this product with the FDA?
CSL Behring GmbH 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.
Does this product have a billing J-code?
Yes — this NDC cross-references HCPCS code J0597 for medical-claim billing (typically used when a product is administered in a clinical setting rather than dispensed at a retail pharmacy). See the Billing section on this page.
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 FDA label FDA SPL

The complete FDA label for this product, verbatim, section by section. Jump with a chip, search within the label, or expand everything.
🎯 Indications and Usage 92 words

1 INDICATIONS AND USAGE Berinert is a plasma-derived concentrate of C1 Esterase Inhibitor (Human) indicated for the treatment of acute abdominal or facial attacks of hereditary angioedema (HAE) in adult and adolescent patients. The safety and efficacy of Berinert for prophylactic therapy have not been established. Berinert is a plasma-derived C1 Esterase Inhibitor (Human) indicated for the treatment of acute abdominal or facial attacks of hereditary angioedema (HAE) in adult and adolescent patients ( 1 ).

The safety and efficacy of Berinert for prophylactic therapy have not been established ( 1 ).

⏱️ Dosage and Administration ~3 min read

2 DOSAGE AND ADMINISTRATION For Intravenous Use Only. Administer Berinert at a dose of 20 units per kg body weight by intravenous injection. Berinert is provided as a freeze-dried powder for reconstitution with the diluent (sterile water) provided.

Store the vial in the original carton in order to protect from light. Do not freeze. For intravenous use only.

Store the vial in the original carton in order to protect from light. Store at 2-25°C (36-77°F). Do not freeze ( 2 ).

Administer 20 units per kg body weight ( 2 ). Reconstitute Berinert prior to use using the diluent (sterile water) provided ( 2.1 ). Administer at room temperature within 8 hours of reconstitution ( 2.1 ).

Inject at a rate of approximately 4 mL per minute ( 2.2 ). Do not mix Berinert with other medicinal products or solutions ( 2.2 ).

2.1Preparation and Handling Check the expiration date on the product vial label. Do not use beyond the expiration date. Use aseptic technique when preparing and administering Berinert (see Reconstitution and Administration [2.2] ).

After reconstitution and prior to administration, inspect Berinert visually for particulate matter and discoloration. The reconstituted solution should be colorless, clear, and free from visible particles. Do not use if the solution is cloudy, discolored, or contains particulates.

The Berinert vial is for single use only. Berinert contains no preservative. Any product that has been reconstituted should be used promptly.

The reconstituted solution must be used within 8 hours. Discard partially used vials. Do not freeze the reconstituted solution.

2.2Reconstitution and Administration Each Berinert kit consists of one carton containing one single-use vial of Berinert, one 10 mL vial of diluent (sterile water), one Mix2Vial ™ transfer set, and one alcohol swab. Use either the Mix2Vial transfer set provided with Berinert ( see How Supplied [16.1] ) or a commercially available double-ended needle and vented filter spike. Reconstitution The procedures below are provided as general guidelines for the reconstitution and administration of Berinert.

1. Ensure that the Berinert vial and diluent vial are at room temperature. Use aseptic technique during the reconstitution procedure.

2. Place the Berinert vial, diluent vial and Mix2Vial transfer set on a flat surface. 3.

Remove the flip caps from the Berinert and diluent vials. Treat the vial stoppers with the alcohol swab provided and allow to dry prior to opening the Mix2Vial transfer set package. 4.

Open the Mix2Vial transfer set package by peeling away the lid (Fig. 1). Leave the Mix2Vial transfer set in the clear package.

Fig. 1 5. Place the diluent vial on a flat surface and hold the vial tightly.

Grip the Mix2Vial transfer set together with the clear package and snap the blue end of the Mix2Vial transfer set onto the diluent vial stopper at a 90° angle (Fig. 2). Fig.

2 6. Carefully remove the clear package from the Mix2Vial transfer set. Make sure that you pull up only the clear package, and not the Mix2Vial transfer set (Fig.

3). Fig. 3 7.

With the Berinert vial placed firmly on a flat surface, invert the diluent vial with the Mix2Vial transfer set attached and snap the transparent adapter onto the Berinert vial stopper at a 90° angle (Fig. 4). The diluent will automatically transfer into the Berinert vial.

Fig. 4 8. With the diluent and Berinert vial still attached to the Mix2Vial transfer set, gently swirl the Berinert vial to ensure that the Berinert is fully dissolved (Fig.

5). Do not shake the vial. Fig.

5 9. With one hand, grasp the Berinert-side of the Mix2Vial transfer set and with the other hand grasp the blue diluent-side of the Mix2Vial transfer set and unscrew the set into two pieces. (Fig.

6). Fig. 6 10.

Draw air into an empty, sterile syringe. While the Berinert vial is upright, screw the syringe to the Mix2Vial transfer set. Inject air into the Berinert vial.

While keeping the syringe plunger pressed, invert the system upside down and draw…

💊 Dosage Forms and Strengths 57 words

3 DOSAGE FORMS AND STRENGTHS Berinert is available in a single-use vial that contains 500 units of C1 esterase inhibitor as a lyophilized concentrate. Each vial must be reconstituted with 10 mL of diluent (sterile water) provided. 500 units lyophilized concentrate in a single-use vial for reconstitution with 10 mL of diluent (sterile water) ( 3 ).

Contraindications 43 words

4 CONTRAINDICATIONS Berinert is contraindicated in individuals who have experienced life-threatening hypersensitivity reactions, including anaphylaxis, to C1 esterase inhibitor preparations. Do not use in patients with a history of life-threatening immediate hypersensitivity reactions, including anaphylaxis, to C1 esterase inhibitor preparations ( 4 ).

⚠️ Warnings and Cautions ~2 min read

5 WARNINGS AND PRECAUTIONS Hypersensitivity reactions may occur. Epinephrine should be immediately available to treat any acute severe hypersensitivity reactions following discontinuation of administration ( 5.1 ). Thrombotic events have occurred in patients receiving off-label high doses of Berinert.

Monitor patients with known risk factors for thrombotic events ( 5.2 ). Berinert is made from human plasma and may contain infectious agents, eg, viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent ( 5.3 ).

5.1Hypersensitivity Severe hypersensitivity reactions may occur. Epinephrine should be immediately available for treatment of acute severe hypersensitivity reaction (see Patient Counseling Information [17] ). The signs and symptoms of hypersensitivity reactions may include hives, generalized urticaria, tightness of the chest, wheezing, hypotension, and/or anaphylaxis during or after injection of Berinert.

Because hypersensitivity reactions may have symptoms similar to HAE attacks, treatment methods should be carefully considered. In case of suspected hypersensitivity, immediately discontinue administration of Berinert and institute appropriate treatment.

5.2Thrombotic Events Thrombotic events have been reported in association with Berinert when used off-label and at higher than labeled doses. 1 Animal studies have confirmed the risk of thrombosis from intravenous administration of C1 esterase inhibitor products 2 ( see Overdosage [10] and Animal Toxicology and/or Pharmacology [13.2] ).

5.3Transmission of Infectious Agents Because Berinert is made from human blood, it may contain infectious agents (eg, viruses and, theoretically, the Creutzfeldt-Jakob disease [CJD] agent) that can cause disease. The risk that such products will transmit an infectious agent has been reduced by screening plasma donors for prior exposure to certain viruses, by testing for the presence of certain current virus infections, and by processes demonstrated to inactivate and/or remove certain viruses during manufacturing ( see Description [11] and Patient Counseling Information [17] ).

Despite these measures, such products may still potentially transmit disease. There is also the possibility that unknown infectious agents may be present in such products. Since 1979, a few suspected cases of viral transmission have been reported with the use of Berinert outside the US, including cases of acute hepatitis C.

From the incomplete information available from these cases, it was not possible to determine with certainty if the infections were or were not related to prior administration of Berinert. The physician should discuss the risks and benefits of this product with the patient before prescribing or administering it to the patient. (See Patient Counseling Information [17.1] ).

All infections thought by a physician possibly to have been transmitted by Berinert should be reported by lot number, by the physician, or other healthcare provider to the CSL Behring Pharmacovigilance Department at 1-866-915-6958.

🤒 Adverse Reactions ~3 min read

6 ADVERSE REACTIONS The most serious adverse reaction reported in subjects enrolled in clinical studies who received Berinert was an increase in the severity of pain associated with HAE. The most common adverse reactions that have been reported in greater than 4% of the subjects who received Berinert in clinical studies were subsequent HAE attack, headache, abdominal pain, nausea, muscle spasm, pain, diarrhea and vomiting. The most serious adverse reaction reported in subjects who received Berinert was an increase in the severity of pain associated with HAE ( 6.1 ).

The most common adverse reactions observed by ≥4% of subjects after Berinert treatment were subsequent HAE attack, headache, abdominal pain, nausea, muscle spasm, pain, diarrhea and vomiting ( 6.1 ). To report SUSPECTED ADVERSE REACTIONS, contact the CSL Behring Pharmacovigilance Department at 1-866-915-6958 or to the FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.

6.1Clinical Trials Experience Because clinical studies 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. Placebo-controlled Clinical Study In the placebo-controlled clinical study, referred to as the randomized clinical trial (RCT) ( see Clinical Studies [14] ), 124 subjects experiencing an acute moderate to severe abdominal or facial HAE attack were treated with Berinert (either a 10 unit per kg body weight or a 20 unit per kg body weight dose), or placebo (physiological saline solution).

The treatment-emergent serious adverse reactions/events that occurred in 5 subjects in the RCT were laryngeal edema, facial attack with laryngeal edema, swelling (shoulder and chest), exacerbation of hereditary angioedema, and laryngospasm. Table 1: Adverse Reactions The study protocol specified that adverse events that began within 72 hours of blinded study medication administration were to be classified as at least possibly related to study medication (ie, adverse reactions). Occurring up to 4 hours After Initial Infusion in More Than 4% of Subjects, Irrespective of Causality The following abdominal symptoms were identified in the protocol as associated with HAE abdominal attacks: abdominal pain, bloating, cramps, nausea, vomiting, and diarrhea.

Adverse Reactions Number (%) of Subjects Reporting Adverse Reactions Berinert 20 units/kg (n = 43) Number (%) of Subjects Reporting Adverse Reactions Placebo Group (n = 42) Nausea 3 (7%) 5 (11.9%) Dysgeusia 2 (4.7%) 0 (0) Abdominal Pain 2 (4.7%) 3 (7.1%) Vomiting 1 (2.3%) 3 (7.1%) Diarrhea 0 (0) 4 (9.5%) Headache 0 (0) 2 (4.8%) Table 2: Adverse Reactions The study protocol specified that adverse events that began within 72 hours of blinded study medication administration were to be classified as at least possibly related to study medication (ie, adverse reactions).

Occurring in More Than 4% of Subjects up to 72 hours After Infusion of Initial or Rescue Medication If a subject experienced no relief or insufficient relief of symptoms within 4 hours after infusion, investigators had the option to administer a blinded second infusion (“rescue” treatment) of Berinert (20 units/kg for the placebo group or 10 units/kg for the 10 units/kg group), or placebo (for the 20 units/kg group). by Intent-to-Treat, Irrespective of Causality Adverse Reactions Number (%) of Subjects Reporting Adverse Reactions Adverse reactions following either initial treatment and/or blinded "rescue" treatment.

Because more subjects in the placebo randomization group than in the Berinert randomization group received rescue treatment, the median observation period in this analysis for subjects randomized to placebo was slightly longer than for subjects randomized to receive Berinert. Berinert 20 units/kg (n = 43) Number (%) of Subjects Reporting Adverse Reactions Placebo Group (n = 42) Nausea 3 (7%) 11 (26.2%) Headache 3 (7%…

🔄 Drug Interactions 20 words

7 DRUG INTERACTIONS No drug interaction studies have been conducted. No drug interaction studies have been conducted ( 7 ).

👥 Use in Specific Populations ~2 min read

8 USE IN SPECIFIC POPULATIONS Pregnancy: No animal data. Limited human data. Use only if clearly needed ( 8.1 ).

Children: Safety and effectiveness in children ages 0 through 12 have not been established. Berinert was evaluated in 5 children (ages 3 through 12) and in 8 adolescent subjects (ages 13 through 16) [ 8.4 ]. Compared to adults, the half-life of Berinert was shorter and clearance was faster in children.

The clinical implication of this difference is not known ( 12.3 ).

8.1Pregnancy Pregnancy Category C. Animal reproduction studies have not been conducted with Berinert. It is not known whether Berinert can cause fetal harm when administered to a pregnant woman or can affect reproduction capacity.

Berinert should be given to a pregnant woman only if clearly needed. In a retrospective case collection study, 20 pregnant women ranging in age from 20 to 35 years received Berinert with repeated doses up to 3,500 units per attack; these women reported no complications during delivery and no harmful effects on their 34 neonates.

8.2Labor and Delivery The safety and effectiveness of Berinert administration prior to or during labor and delivery have not been established. Use only if clearly needed.

8.3Nursing Mothers It is not known whether Berinert is excreted in human milk. Because many drugs are excreted in human milk, use only if clearly needed when treating a nursing woman.

8.4Pediatric Use Safety and efficacy of Berinert in children (ages 0 through 12) have not been established. The clinical studies included an insufficient number of subjects in this age group to determine whether they respond differently from older subjects. The safety and efficacy of Berinert were evaluated in 5 children (ages 3 through 12) and in 8 adolescent subjects (ages 13 through 16) ( see Pharmacokinetics [12.3] ).

8.5Geriatric Use Safety and efficacy of Berinert in the geriatric population have not been established. Clinical studies with Berinert included four subjects older than 65 years. The clinical studies included an insufficient number of subjects in this age group to determine whether they respond differently from younger subjects.

🤰 Pregnancy 90 words

8.1Pregnancy Pregnancy Category C. Animal reproduction studies have not been conducted with Berinert. It is not known whether Berinert can cause fetal harm when administered to a pregnant woman or can affect reproduction capacity.

Berinert should be given to a pregnant woman only if clearly needed. In a retrospective case collection study, 20 pregnant women ranging in age from 20 to 35 years received Berinert with repeated doses up to 3,500 units per attack; these women reported no complications during delivery and no harmful effects on their 34 neonates.

🧒 Pediatric Use 69 words

8.4Pediatric Use Safety and efficacy of Berinert in children (ages 0 through 12) have not been established. The clinical studies included an insufficient number of subjects in this age group to determine whether they respond differently from older subjects. The safety and efficacy of Berinert were evaluated in 5 children (ages 3 through 12) and in 8 adolescent subjects (ages 13 through 16) ( see Pharmacokinetics [12.3] ).

🧓 Geriatric Use 49 words

8.5Geriatric Use Safety and efficacy of Berinert in the geriatric population have not been established. Clinical studies with Berinert included four subjects older than 65 years. The clinical studies included an insufficient number of subjects in this age group to determine whether they respond differently from younger subjects.

🆘 Overdosage 64 words

10 OVERDOSAGE The development of thrombosis has been reported after doses exceeding 20 units/kg body weight of Berinert when used off-label 1 in newborns and young children with congenital heart anomalies during or after cardiac surgery under extracorporeal circulation. The maximum dose administered in clinical studies in hereditary angioedema was 20 units/kg body weight. Overdosage did not occur in connection with treatment of HAE.

🧬 Clinical Pharmacology ~3 min read

12 CLINICAL PHARMACOLOGY

12.1Mechanism of Action C1 esterase inhibitor is a normal constituent of human plasma and belongs to the group of serine protease inhibitors (serpins) that includes antithrombin III, alpha 1 -protease inhibitor, alpha 2 -antiplasmin, and heparin cofactor II. As with the other inhibitors in this group, C1 esterase inhibitor has an important inhibiting potential on several of the major cascade systems of the human body, including the complement system, the intrinsic coagulation (contact) system, the fibrinolytic system, and the coagulation cascade.

Regulation of these systems is performed through the formation of complexes between the proteinase and the inhibitor, resulting in inactivation of both and consumption of the C1 esterase inhibitor. C1 esterase inhibitor, which is usually activated during the inflammatory process, inactivates its substrate by covalently binding to the reactive site. C1 esterase inhibitor is the only known inhibitor for the subcomponent of the complement component 1 (C1r), C1s, coagulation factor XIIa, and kallikrein.

Additionally, C1 esterase inhibitor is the main inhibitor for coagulation factor XIa of the intrinsic coagulation cascade. HAE patients have low levels of endogenous or functional C1 esterase inhibitor. Although the events that induce attacks of angioedema in HAE patients are not well defined, it has been postulated that increased vascular permeability and the clinical manifestation of HAE attacks may be primarily mediated through contact system activation.

Suppression of contact system activation by C1 esterase inhibitor through the inactivation of plasma kallikrein and factor XIIa is thought to modulate this vascular permeability by preventing the generation of bradykinin. 5 Administration of Berinert to patients with C1 esterase inhibitor deficiency replaces the missing or malfunctioning protein in patients. The plasma concentration of C1 esterase inhibitor in healthy volunteers is approximately 270 mg/L.

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12.3Pharmacokinetics The pharmacokinetics of Berinert were evaluated in an open-label, uncontrolled, single-center study in 40 subjects (35 adults and 5 children under 16 years of age) with either mild or severe HAE. All subjects received a single intravenous injection of Berinert ranging from 500 units to 1500 units. Blood samples were taken during an attack-free period at baseline and for up to 72 hours after drug administration.

Pharmacokinetic parameters were estimated using non-compartmental analysis (with or without baseline adjustment). Table 6 summarizes the pharmacokinetic parameters in 35 adult subjects with HAE. Table 6: Pharmacokinetic Parameters of Berinert in Adult Subjects with HAE by Non-compartmental Analysis (n=35) Parameters Unadjusted for baseline Adjusted for baseline AUC: Area under the curve CL: Clearance V ss: Volume steady state MRT: Mean residence time AUC (0-t) (hr × IU/mL) Based on a 15 unit/kg dose.

Numbers in parenthesis are the range. 27.5 ± 8.5 (15.7-44.7) 12.8 ± 6.7 (3.9-34.7) CL (mL/hr/kg) 0.60 ± 0.17 (0.34-0.96) 1.44 ± 0.67 (0.43-3.85) V ss (mL/kg) 18.6 ± 4.9 (11.1-27.6) 35.4 ± 10.5 (14.1-56.1) Half-life (hrs) 21.9 ± 1.7 (16.5-24.4) 18.4 ± 3.5 (7.4-22.8) MRT (hrs) 31.5 ± 2.4 (23.7-35.2) 26.4 ± 5.0 (10.7-33.0) Table 7 summarizes the pharmacokinetic parameters in 5 pediatric subjects (ages 6 through 13) with HAE. Based on adjusted baseline, compared to adults, the half-life of Berinert was shorter and clearance was faster in this limited cohort of children.

However, the clinical implication of this difference is not known. Table 7: Pharmacokinetic Parameters of Berinert in Pediatric Subjects with HAE by Non-compartmental Analysis (n=5) Parameters Unadjusted for baseline Adjusted for baseline AUC: Area under the curve CL: Clearance V ss: Volume steady state MRT: Mean residence time AUC (0-t) (hr × IU/mL) Based on a 15 unit/kg dose. Numbers in parenthesis are the range.

25.45± 5.8 (16.8-31.7) 9.78 ± 4.37 (4.1-15.2) CL (mL/hr/…

🧬 Mechanism of Action ~1 min read

12.1Mechanism of Action C1 esterase inhibitor is a normal constituent of human plasma and belongs to the group of serine protease inhibitors (serpins) that includes antithrombin III, alpha 1 -protease inhibitor, alpha 2 -antiplasmin, and heparin cofactor II. As with the other inhibitors in this group, C1 esterase inhibitor has an important inhibiting potential on several of the major cascade systems of the human body, including the complement system, the intrinsic coagulation (contact) system, the fibrinolytic system, and the coagulation cascade.

Regulation of these systems is performed through the formation of complexes between the proteinase and the inhibitor, resulting in inactivation of both and consumption of the C1 esterase inhibitor. C1 esterase inhibitor, which is usually activated during the inflammatory process, inactivates its substrate by covalently binding to the reactive site. C1 esterase inhibitor is the only known inhibitor for the subcomponent of the complement component 1 (C1r), C1s, coagulation factor XIIa, and kallikrein.

Additionally, C1 esterase inhibitor is the main inhibitor for coagulation factor XIa of the intrinsic coagulation cascade. HAE patients have low levels of endogenous or functional C1 esterase inhibitor. Although the events that induce attacks of angioedema in HAE patients are not well defined, it has been postulated that increased vascular permeability and the clinical manifestation of HAE attacks may be primarily mediated through contact system activation.

Suppression of contact system activation by C1 esterase inhibitor through the inactivation of plasma kallikrein and factor XIIa is thought to modulate this vascular permeability by preventing the generation of bradykinin. 5 Administration of Berinert to patients with C1 esterase inhibitor deficiency replaces the missing or malfunctioning protein in patients. The plasma concentration of C1 esterase inhibitor in healthy volunteers is approximately 270 mg/L.

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📦 How Supplied / Storage and Handling 142 words

16 HOW SUPPLIED/STORAGE AND HANDLING

16.1How Supplied Berinert is supplied in a single-use vial. Each carton contains a 500 unit vial of Berinert for reconstitution with 10 mL of diluent containing sterile water (meets USP chemistry requirements except for pH; pH 4.5-8.5). The components used in the packaging for Berinert are latex-free.

Each product package consists of the following: NDC Number Component 63833-825-02 Carton (kit) containing one 500 unit vial of Berinert [NDC 63833-835-01], one 10 mL vial of diluent (sterile water) [NDC 63833-765-15], one Mix2Vial filter transfer set, and one alcohol swab.

16.2Storage and Handling When stored at temperatures of 2-25°C (36-77°F), Berinert is stable for the period indicated by the expiration date on the carton and vial label (up to 30 months). Keep Berinert in its original carton until ready to use. Do not freeze. Protect from light.

📦 Storage and Handling 48 words

16.2Storage and Handling When stored at temperatures of 2-25°C (36-77°F), Berinert is stable for the period indicated by the expiration date on the carton and vial label (up to 30 months). Keep Berinert in its original carton until ready to use. Do not freeze. Protect from light.

📋 Description ~2 min read

11 DESCRIPTION Berinert is a human plasma-derived, purified, pasteurized, lyophilized concentrate of C1 esterase inhibitor to be reconstituted for intravenous administration. Berinert is prepared from large pools of human plasma from US donors. One standard unit of C1 esterase inhibitor concentrate is equal to the amount of C1 esterase inhibitor in 1 mL of fresh citrated human plasma, which is equivalent to 270 mg/L or 2.5 µM/L.

No international laboratory standard for quantifying C1 esterase inhibitor. An in-house standard is used to assure lot-to-lot consistency in product potency. C1 esterase inhibitor is a soluble, single-chain glycoprotein containing 478 amino acid residues organized into three beta-sheets and eight or nine alpha-helices.

3 The heavily glycosylated molecule has an apparent molecular weight of 105 kD, of which the carbohydrate chains comprise 26% to 35%. 4 Each vial of Berinert contains 500 units C1 esterase inhibitor, 50 to 80 mg total protein, 85 to 115 mg glycine, 70 to 100 mg sodium chloride, and 25 to 35 mg sodium citrate. All plasma used in the manufacture of Berinert is obtained from US donors and is tested using serological assays for hepatitis B surface antigen and antibodies to HIV-1/2 and HCV.

Additionally, the plasma is tested with Nucleic Acid Testing (NAT) for HCV and HIV-1 and found to be non-reactive (negative). In addition, the plasma is tested by NAT for HAV and Human Parvovirus B19. Only plasma that has passed virus screening is used for production, and the limit for Parvovirus B19 in the fractionation pool is set not to exceed 10 4 IU of Parvovirus B19 DNA per mL.

The manufacturing process for Berinert includes multiple steps that reduce the risk of virus transmission. The virus inactivation/reduction capacity of three steps (pasteurization in aqueous solution at 60°C for 10 hours, hydrophobic interaction chromatography, and the combination of ion exchange chromatographies and ammonium sulphate precipitation) was evaluated in a series of in vitro spiking experiments. The total mean cumulative virus inactivation/reduction is shown in Table 5.

Table 5: Mean Virus Inactivation/Reductions in Berinert Virus Studied Pasteurization [log 10 ] Hydrophobic Interaction Chromatography [log 10 ] DEAE-Sephadex A50 Chromatography QAE-Sephadex Chromatography and Ammonium Sulphate Precipitation [log10] Total Cumulative [log 10 ] HIV-1, Human immunodeficiency virus type 1, a model for HIV-1 and HIV-2 BVDV, Bovine viral diarrhea virus, a model for HCV PRV, Pseudorabies virus, a model for large enveloped DNA viruses (eg, herpes virus) WNV, West Nile virus HAV, Hepatitis A virus CPV, Canine parvovirus B19V, Human Parvovirus B19 ND, Not determined NA, Not applicable Enveloped Viruses HIV-1 ≥6.6 ≥4.5 4.3 ≥15.4 BVDV ≥9.2 ≥4.6 NA ≥13.8 PRV 6.3 ≥6.5 ≥7.7 ≥20.5 WNV ≥7.0 ND NA NA Non-Enveloped Viruses HAV ≥6.4

4.5NA ≥10.9 CPV 1.4

6.1 NA

7.5 B19V

3.9 ND NA NA

💬 Information for Patients 209 words

17 PATIENT COUNSELING INFORMATION Inform patients to immediately report the following to their physician: Signs and symptoms of allergic hypersensitivity reactions, such as hives, urticaria, tightness of the chest, wheezing, hypotension and/or anaphylaxis experienced during or after injection of Berinert (see WARNINGS AND PRECAUTIONS/Hypersensitivity [5.1] ) Signs and symptoms of thrombosis, such as new onset swelling and pain in the limbs or abdomen, new onset chest pain, shortness of breath, loss of sensation or motor power, or altered consciousness, vision, or speech (see WARNINGS AND PRECAUTIONS/Thrombotic Events [5.2] ) Advise female patients to notify their physician if they become pregnant or intend to become pregnant during the treatment of acute abdominal or facial attacks of HAE with Berinert.

Advise patients to notify their physician if they are breastfeeding or plan to breastfeed. Advise patients to consult with their healthcare professional prior to travel. Advise patients that, because Berinert is made from human blood, it may carry a risk of transmitting infectious agents, eg, viruses, and, theoretically, the Creutzfeldt-Jakob (CJD) agent (see WARNINGS AND PRECAUTIONS/Transmission of Infectious Agents [5.3] and Description [11] ).

Inform patients of the risks and benefits of Berinert before prescribing or administering it to the patient .

17.1 FDA-Approved Patient Labeling – Patient Product Information (PPI)

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