Congenital factor XIII deficiency: IV:
Prophylaxis:
Initial: 40 units/kg.
Maintenance: Adjust dose in 5 units/kg increments based on factor XIII activity trough levels (target level of 5% to 20% using Berichrom activity assay) and clinical response; repeat every 28 days.
Factor XIII Activity Trough Level (%):
One trough level <5%: Increase dosage by 5 units/kg.
Trough level 5% to 20%: No dosage change.
Two trough levels >20%: Decrease dosage by 5 units/kg.
One trough level >25%: Decrease dosage by 5 units/kg.
Treatment, perioperative management of surgical bleeding: Individualize dosing based on factor XIII activity level, type of surgery, and clinical response; monitor factor XIII activity levels during and after surgery:
If time since last prophylactic dose ≤7 days: Additional dose may not be needed.
If time since last prophylactic dose 8 to 21 days: Additional partial or full dose may be necessary based on factor XIII activity level.
If time since last prophylactic dose 21 to 28 days: Administer full prophylactic dose.
Congenital factor XIII deficiency: Infants, Children, and Adolescents: IV:
Prophylaxis:
Initial: 40 units/kg
Maintenance: Adjust dose in 5 units/kg increments based on factor XIII activity trough levels (target level of 5% to 20% using Berichrom activity assay) and clinical response; repeat every 28 days
Factor XIII Activity Trough Level (%):
One trough level <5%: Increase dosage by 5 units/kg
Trough level 5% to 20%: No dosage change
Two trough levels >20%: Decrease dosage by 5 units/kg
One trough level >25%: Decrease dosage by 5 units/kg
Treatment, perioperative management of surgical bleeding: Individualize dosing based on factor XIII activity level, type of surgery, and clinical response; monitor factor XIII activity levels during and after surgery:
If time since last prophylactic dose ≤7 days: Additional dose may not be needed.
If time since last prophylactic dose 8 to 21 days: Additional partial or full dose may be necessary based on factor XIII activity level.
If time since last prophylactic dose 21 to 28 days: Administer full prophylactic dose.
There are no dosage adjustments provided in the manufacturer's labeling.
There are no dosage adjustments provided in the manufacturer's labeling.
(For additional information see "Factor XIII, concentrate from human plasma: Drug information")
Congenital factor XIII deficiency:
Prophylaxis:
IV: Initial: 40 units/kg.
IV: Maintenance: Dose adjustment should be based on factor XIII activity trough levels (target level of 5% to 20% using Berichrom activity assay) and clinical response; repeat every 28 days.
One trough level of <5%: Increase dosage by 5 units/kg
Trough level of 5% to 20%: No dosage change
Two trough levels of >20%: Decrease dosage by 5 units/kg
One trough level of >25%: Decrease dosage by 5 units/kg
Perioperative management of surgical bleeding: IV: 10 to 50 units/kg has been reported in clinical practice but because congenital factor XIII deficiency is a rare disorder, data are limited (Janbain 2015; Naderi 2017). Individualize dosing based on factor XIII activity level, type of surgery, and clinical response; monitor factor XIII activity levels during and after surgery:
If time since last prophylactic dose ≤7 days: Additional dose may not be needed.
If time since last prophylactic dose 8 to 21 days: Additional partial or full dose may be necessary based on factor XIII activity level.
If time since last prophylactic dose 21 to 28 days: Administer full prophylactic dose.
There are no dosage adjustments provided in the manufacturer’s labeling.
There are no dosage adjustments provided in the manufacturer’s labeling.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Kit, Intravenous [preservative free]:
Corifact: 1000 - 1600 units [contains albumin human]
No
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Solution Reconstituted, Intravenous:
Corifact 250: 250 units (1 ea) [contains albumin human]
Corifact 1250: 1250 units (1 ea) [contains albumin human]
Potency is shown as a range for each vial (1,000 to 1,600 units/vial). The actual units of potency of FXIII are stated on each vial label and carton.
Parenteral: IV: Administer by IV infusion at a rate not to exceed 4 mL/minute. Administer through a separate infusion line.
IV: Administer by IV infusion at a rate not to exceed 4 mL/minute. Product should be brought to room temperature prior to infusing. Administer through a separate infusion line.
Store at 2°C to 8°C (36°F to 46°F); do not freeze. Protect from light. May be stored at room temperature (≤25°C [≤77°F]) for up to 6 months; do not return to refrigerator if stored at room temperature.
Prophylaxis against bleeding episodes and management of perioperative surgical bleeding in patients with congenital factor XIII deficiency (FDA approved in all ages).
Factor XIII may be confused with Factor VIII
The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified.
Frequency not defined.
>1%:
Central nervous system: Chills, headache
Dermatologic: Erythema, pruritus, skin rash
Endocrine & metabolic: Increased lactate dehydrogenase
Hematologic & oncologic: Hematoma
Hypersensitivity: Hypersensitivity reaction
Neuromuscular & skeletal: Arthralgia, arthritis
Miscellaneous: Fever
<1%, postmarketing, and/or case reports: Anaphylaxis, antibody development (factor XIII), infection, ischemia (acute), thromboembolism
History of anaphylaxis or severe systemic reactions to human plasma-derived products or hypersensitivity to any component of the formulation
Concerns related to adverse effects:
• Antibody formation: The development of factor XIII inhibitory antibodies has been reported. Factor XIII inhibitory antibodies should be measured when clinical response (breakthrough bleeding) and/or factor XIII trough levels are suboptimal after apparent adequate dosing.
• Hypersensitivity reactions: Hypersensitivity and anaphylactic reactions have been reported with use; discontinue immediately if develops and initiate appropriate management.
• Thrombotic events: Thromboembolic events have been reported. Use with caution in patients with known risk factors for thrombosis.
Dosage form specific issues:
• Human plasma: Product of human plasma; may potentially contain infectious agents which could transmit disease. Screening of donors, as well as testing and/or inactivation or removal of certain viruses, reduces the risk. Infections thought to be transmitted by this product should be reported to the manufacturer. Vaccination with hepatitis A and hepatitis B vaccines is recommended.
None known.
There are no known significant interactions.
Pregnant patients with factor XIII deficiency may have an increased risk of bleeding following abortion, antenatal procedures, and delivery. There is also a high rate of pregnancy loss without treatment; close surveillance is recommended. Maternal factor XIII concentrations decrease during pregnancy and dosing frequency should be increased. Additional treatment may be needed prior to delivery or procedures. Factor XIII concentrate (human) may be used in patients with a factor XIII deficiency (RCOG [Pavord 2017]).
Factor XIII trough levels in conjunction with clinical response to assess efficacy (ie, approximately every 28 days for prophylaxis, during and after surgery for perioperative management of surgical bleeding). Factor XIII inhibitory antibodies if inadequate clinical response and/or factor XIII trough levels are suboptimal. Signs/symptoms of hypersensitivity reactions, thrombotic events, and infection.
Factor XIII (FXIII) is an endogenous plasma glycoprotein found in platelets, monocytes and macrophages that is converted to activated factor XIII (FXIIIa) in the presence of calcium ions. Once activated, FXIIIa cross-links fibrin and cross-links plasmin inhibitor to protect and strengthen the hemostatic platelet plug.
Duration of effect: Plasma levels of FXIII: ~28 days; FXIII activity maintained at ≥5% in ≥97% of patients and ≥10% in ≥85% of patients
Distribution: Vd: 51.1 mL/kg
Metabolism: Factor XIII, a proenzyme, is converted to activated factor XIII
Half-life elimination: Children (<16 years): 5.7 ± 1 days; Adults: 7.1 ± 2.7 days
Time to peak: 1.7 hours postinfusion
Excretion: Clearance:
Pediatric patients <16 years: 0.29 ± 0.12 mL/hour/kg
Adults: 0.22 ± 0.07 mL/hour/kg
Kit (Corifact Intravenous)
1000-1600 unit (Price provided is per AHF Unit): $13.37
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