Protein C Deficiency as a Risk Factor for Stroke in Young Adults: A Review

Majid, Zainab; Tahir, Faryal; Ahmed, Jawad; Arif, Taha Bin; Haq, Anwarul

doi:10.7759/cureus.7472

Cited by 13 publications

(14 citation statements)

References 44 publications

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“…Thrombophilia is a thrombotic disorder associated with a dysfunction in coagulation factors and in some of their cofactors, such as APC and PS [ 268 , 269 ]. Thrombophilia associated with vWF, FVIII, and FV is the result of an impaired turnover of these proteins, which favors the factors’ synthesis, due to an alteration in natural inactivators, such as ADAMTS13 for vWF [ 270 ] ( Figure 3 ) and APC and PS for FVIII ( Figure 4 ) and FV ( Figure 5 ), respectively [ 268 , 269 ]. This results in increased levels of vWF, FVIII, and FV, which may trigger a thrombotic process [ 271 ].…”

Section: Dysregulation Of Homeostasismentioning

confidence: 99%

“…In the case of thrombophilia, the dysfunction of homeostasis is associated with an increased rate of thrombosis. The causes may be related to a deficiency of natural anticoagulants, such as PC [ 268 ] or PS [ 269 ], which tends to result in excessive activity levels of coagulation factors, such as FVIII or FV, or to a state of hypercoagulability typically induced by a mutation in FV Leiden [ 226 ], which offers resistance to APC. It may also be due to a deficiency of the regulators of other clotting factors secreted at the level of the vascular endothelium, such as vWF.…”

Section: Homeostasis-modifying Treatments In Coagulationmentioning

confidence: 99%

“…PC deficiency drastically alters the homeostasis between procoagulant and anticoagulant proteins, which predisposes subjects to thromboembolism [ 268 ]. The role of natural anticoagulants is essential to avoid the consequences of long-term exposure of procoagulant proteins as a result of the low rate of blood flow in the venous circulation.…”

Section: Homeostasis-modifying Treatments In Coagulationmentioning

confidence: 99%

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The Vascular Endothelium and Coagulation: Homeostasis, Disease, and Treatment, with a Focus on the Von Willebrand Factor and Factors VIII and V

Pablo-Moreno

Serrano

Revuelta

et al. 2022

IJMS

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The vascular endothelium has several important functions, including hemostasis. The homeostasis of hemostasis is based on a fine balance between procoagulant and anticoagulant proteins and between fibrinolytic and antifibrinolytic ones. Coagulopathies are characterized by a mutation-induced alteration of the function of certain coagulation factors or by a disturbed balance between the mechanisms responsible for regulating coagulation. Homeostatic therapies consist in replacement and nonreplacement treatments or in the administration of antifibrinolytic agents. Rebalancing products reestablish hemostasis by inhibiting natural anticoagulant pathways. These agents include monoclonal antibodies, such as concizumab and marstacimab, which target the tissue factor pathway inhibitor; interfering RNA therapies, such as fitusiran, which targets antithrombin III; and protease inhibitors, such as serpinPC, which targets active protein C. In cases of thrombophilia (deficiency of protein C, protein S, or factor V Leiden), treatment may consist in direct oral anticoagulants, replacement therapy (plasma or recombinant ADAMTS13) in cases of a congenital deficiency of ADAMTS13, or immunomodulators (prednisone) if the thrombophilia is autoimmune. Monoclonal-antibody-based anti-vWF immunotherapy (caplacizumab) is used in the context of severe thrombophilia, regardless of the cause of the disorder. In cases of disseminated intravascular coagulation, the treatment of choice consists in administration of antifibrinolytics, all-trans-retinoic acid, and recombinant soluble human thrombomodulin.

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Section: Dysregulation Of Homeostasismentioning

confidence: 99%

Section: Homeostasis-modifying Treatments In Coagulationmentioning

confidence: 99%

Section: Homeostasis-modifying Treatments In Coagulationmentioning

confidence: 99%

See 1 more Smart Citation

The Vascular Endothelium and Coagulation: Homeostasis, Disease, and Treatment, with a Focus on the Von Willebrand Factor and Factors VIII and V

Pablo-Moreno

Serrano

Revuelta

et al. 2022

IJMS

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“…However, the standard treatment for cerebral venous thrombosis caused by protein C deficiency is the administration of heparin in the acute phase [ 1 ]. Additionally, continuous anticoagulant use is required to prevent the development of thromboembolic events in the chronic phase [ 5 , 6 ]. Conventionally, warfarin is considered a standard anticoagulant but can produce some problems in general and specific problems in protein C deficiency patients, such as warfarin-induced skin necrosis [ 7 , 8 ].…”

Section: Introductionmentioning

confidence: 99%

A Case of Treatment With Dabigatran for Cerebral Venous Thrombosis Caused by Hereditary Protein C Deficiency

Fukushima

Shimomura

Nagaya

et al. 2021

Cureus

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A 37-year-old woman was admitted to our hospital with involuntary movements. She had no medical or family history of thromboembolism, nor was she on any medication. She showed no impaired consciousness, cranial nerve abnormalities, abnormal breathing, stiff neck or paralysis. Magnetic resonance venography exhibited poor visualization of intracranial vein. The protein C activity level reduced but the protein C antigen level was normal. Genetic analysis revealed a heterozygous mutation in exon 7 c.577-579delAAG, p.Lys193del on protein C gene. She was diagnosed with cerebral venous thrombosis and hereditary protein C deficiency type II. She received heparin in acute phase, and switched to dabigatran in chronic phase. Consequently, she had no recurrence of cerebral venous thrombosis and other complications. Dabigatran might be one of the alternative choices for patients with cerebral venous thrombosis and protein C deficiency.

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“…There are few reports [14,15] that a low level of plasma protein C might be among risk factors contributing to recurrent thrombotic disease. This protein is recognized to exert an important role in anticoagulation [16].…”

Section: A C C E P T E D a R T I C L Ementioning

confidence: 99%

Effects of IgG from the serum of ischemic stroke patients on hemostasis

et al. 2021

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Ischemic stroke is among the top diseases leading to mortality and disability in the world. The detailed investigation of the mechanisms underlying this pathology and especially mediating the tendency to relapse during the first year after stroke incident undoubtedly belongs to important tasks of modern medicine and biology. The current study aims to analyze the influence of IgG derived from the blood serum of ischemic stroke patients on some hemostasis factors. In total, 123 participants with IS, 62 with atherothrombotic ischemic stroke, 61 with cardioembolic ischemic stroke, and 57 subjects as control have been examined. The same patients have participated in the research a year after stroke. IgG from serum was isolated by affinity chromatography on protein A Sepharose column. The activity of key hemostasis factors under the influence of IgG was analyzed. Obtained results revealed that IgG of stroke patients but not healthy subjects caused the inhibition of the amidolytic activity of endogenously generated thrombin, protein C, factor Xa, and led to an increase in the degree of ADP-induced platelet aggregation. The reduction of clotting time in the test "Thrombin time" by IgG of patients at the acute phase of disease was also observed; IgG of healthy subjects mediated the opposite effect. In contrast to acute ischemic stroke IgG, IgG of patients one year after both atherothrombotic and cardioembolic ischemic stroke influenced only the activity of endogenously generated thrombin and factor Xa resulting in inhibition of their activities. It was also established that IgG of ischemic stroke patients, as well as healthy subjects, stimulated the secretion of tissue plasminogen activator by endotheliocytes.

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Protein C Deficiency as a Risk Factor for Stroke in Young Adults: A Review

Cited by 13 publications

References 44 publications

The Vascular Endothelium and Coagulation: Homeostasis, Disease, and Treatment, with a Focus on the Von Willebrand Factor and Factors VIII and V

The Vascular Endothelium and Coagulation: Homeostasis, Disease, and Treatment, with a Focus on the Von Willebrand Factor and Factors VIII and V

A Case of Treatment With Dabigatran for Cerebral Venous Thrombosis Caused by Hereditary Protein C Deficiency

Effects of IgG from the serum of ischemic stroke patients on hemostasis

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