Factor VII deficiency and its treatment in delivery with recombinant factor VII

Pehlivanov, B; Milchev, N; Kroumov, G.

doi:10.1016/j.ejogrb.2003.12.026

Cited by 15 publications

(28 citation statements)

References 4 publications

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“…Factor VII deficiency may be critical to the temporal effect of radiation on PT values which was observed in the 200 cGy doses when plasma was isolated at 3 h or 7 h post-irradiation. Factor VII has a half-life of 3–6 h (Pehlivanov et al 2004), which may contribute to the increase in PT values observed at 3 h post-irradiation, but not at 7 h post-irradiation.…”

Section: Discussionmentioning

confidence: 99%

The effects of proton radiation on the prothrombin and partial thromboplastin times of irradiated ferrets

Krigsfeld

Sanzari

Kennedy

2012

International Journal of Radiation Biology

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Purpose To determine whether proton radiation affects coagulation. Material and methods Ferrets were exposed to solar particle event-like proton radiation at doses of 0, 25, 100, or 200 centigray (cGy), and dose rates of 50 cGy/minute (high dose rate or HDR) or 50 cGy/hour (low dose rate or LDR). Plasma was isolated from blood collected prior to radiation exposure and at 3–7 h post-radiation. Prothrombin time (PT) assays and activated partial thromboplastin time (aPTT) assays were performed as were mixing studies to determine the coagulation factors involved. Results HDR and LDR exposure led to statistically significant increases in PT values. It was determined that the HDR-induced increase in PT was due to Factor VII, while Factors II, V, and VII contributed to the LDR-induced increase in PT values. Only acute LDR exposure caused an increase in aPTT values, which remained elevated for 48 h post-irradiation (which was the latest time assayed in these studies). Mixing studies revealed that Factor IX contributed to the increased aPTT values. A majority of the animals exposed at the LDR had an International Normalized Ratio approaching or surpassing 2.0. Conclusions PT/aPTT assays resulted in increased clotting times due to different coagulation factors, indicating potential radiation-induced coagulopathy.

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Section: Discussionmentioning

confidence: 99%

The effects of proton radiation on the prothrombin and partial thromboplastin times of irradiated ferrets

Krigsfeld

Sanzari

Kennedy

2012

International Journal of Radiation Biology

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“…INR ( I ) is related to the clotting factor concentration as a fraction of normal ( F ) and is associated with the following parameters: Clotting factor VII is cleared with rate β f = 5/day 22 The minimum clotting factor concentration is F min = 0.75. …”

Section: Methodsmentioning

confidence: 99%

Mathematical modeling of liver injury and dysfunction after acetaminophen overdose: Early discrimination between survival and death

et al. 2012

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Acetaminophen (APAP) is the leading cause of acute liver injury in the developed world. Timely administration of N-acetylcysteine (N-Ac) prevents the progression of serious liver injury and disease, whereas failure to administer N-Ac within a critical time frame allows disease progression and in the most severe cases may result in liver failure or death. In this situation, liver transplantation may be the only life-saving measure. Thus, the outcome of an APAP overdose depends on the size of the overdose and the time to first administration of N-Ac. We developed a system of differential equations to describe acute liver injury due to APAP overdose. The Model for Acetaminophen-induced Liver Damage (MALD) uses a patient's aspartate aminotransferase (AST), alanine aminotransferase (ALT), and international normalized ratio (INR) measurements on admission to estimate overdose amount, time elapsed since overdose, and outcome. The mathematical model was then tested on 53 patients from the University of Utah. With the addition of serum creatinine, eventual death was predicted with 100% sensitivity, 91% specificity, 67% positive predictive value (PPV), and 100% negative predictive value (NPV) in this retrospective study. Using only initial AST, ALT, and INR measurements, the model accurately predicted subsequent laboratory values for the majority of individual patients. This is the first dynamical rather than statistical approach to determine poor prognosis in patients with life-threatening liver disease due to APAP overdose. Conclusion: MALD provides a method to estimate overdose amount, time elapsed since overdose, and outcome from patient laboratory values commonly available on admission in cases of acute liver failure due to APAP overdose and should be validated in multicenter prospective evaluation. (HEPATOLOGY 2012;56:727-734)

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“…Subjects with slightly less severe reduction of factor VII level to 1% or higher may escape this complication and present later with joint bleeding, epistaxis, or some other hemorrhagic manifestation. 2,3 …”

Section: Introductionmentioning

confidence: 99%

“…4 The situation is worse in the developing world, where treatment of bleeding episodes is limited to plasma-derived products, which are in scarce supply or contaminated with blood-borne pathogens. 2,3,5 …”

Section: Introductionmentioning

confidence: 99%

AAV-mediated gene transfer in the perinatal period results in expression of FVII at levels that protect against fatal spontaneous hemorrhage

Binny

McIntosh

Peruta

et al. 2012

Blood

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We explored adeno-associated viral vector (AAV)–mediated gene transfer in the perinatal period in animal models of severe congenital factor VII (FVII) deficiency, a disease associated with early postnatal life-threatening hemorrhage. In young adult mice with plasma FVII < 1% of normal, a single tail vein administration of AAV (1 × 1013 vector genomes [vg]/kg) resulted in expression of murine FVII at 266% ± 34% of normal for ≥ 67 days, which mediated protection against fatal hemorrhage and significantly improved survival. Codon optimization of human FVII (hFVIIcoop) improved AAV transgene expression by 37-fold compared with the wild-type hFVII cDNA. In adult macaques, a single peripheral vein injection of 2 × 1011 vg/kg of the hFVIIcoop AAV vector resulted in therapeutic levels of hFVII expression that were equivalent in males (10.7% ± 3.1%) and females (12.3% ± 0.8%). In utero delivery of this vector in the third trimester to fetal monkeys conferred expression of hFVII at birth of 20.4% ± 3.7%, with a gradual decline to > 1% by 7 weeks. Re-administration of an alternative serotype at 12 months postnatal age increased hFVII levels to 165% ± 6.2% of normal, which remained at therapeutic levels for a further 28 weeks without toxicity. Thus, perinatal AAV-mediated gene transfer shows promise for disorders with onset of pathology early after birth.

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Factor VII deficiency and its treatment in delivery with recombinant factor VII

Cited by 15 publications

References 4 publications

The effects of proton radiation on the prothrombin and partial thromboplastin times of irradiated ferrets

The effects of proton radiation on the prothrombin and partial thromboplastin times of irradiated ferrets

Mathematical modeling of liver injury and dysfunction after acetaminophen overdose: Early discrimination between survival and death

AAV-mediated gene transfer in the perinatal period results in expression of FVII at levels that protect against fatal spontaneous hemorrhage

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