20 results match your criteria Alpha2-Plasmin Inhibitor Deficiency

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Alpha 2-antiplasmin deficiency in a Sudanese child: a case report.

J Med Case Rep 2021 May 7;15(1):238. Epub 2021 May 7.

Hematology Department, Faculty of Medical Laboratory Sciences, Port Sudan Ahlia College, Port Sudan, Sudan.

Background: The plasma serine protease inhibitor alpha 2-antiplasmin (α-AP, otherwise known as α-plasmin inhibitor) is a rapid-acting plasmin inhibitor recently found in human plasma, which seems to have a significant role in the regulation of in vivo fibrinolysis. Congenital deficiency of α-AP is extremely uncommon.

Case Presentation: We report here a case of absolute deficiency of α-AP in an 11-year-old Sudanese boy, who had a lifelong intermittent hemorrhagic tendency (gum bleeding, epistaxis, and exaggerated bleeding after trauma). Read More

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Pathological coagulation parameters in as many as 54 patients with autoimmune acquired factor XIII deficiency due to anti-factor XIII autoantibodies.

Haemophilia 2021 May 12;27(3):454-462. Epub 2021 Apr 12.

Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine, Yamagata, Japan.

Introduction: Autoimmune factor XIII (FXIII) deficiency (AiF13D) due to anti-FXIII autoantibodies is an extremely rare, life-threatening bleeding disorder that mostly occurs in the elderly. The number of patients diagnosed with AiF13D has been increasing in Japan, probably because of the nationwide survey on AiF13D supported by the Japanese Ministry of Health, Labour and Welfare.

Aim: To explore the pathologic characteristics of coagulation parameters in AiF13D. Read More

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Molecular pathogenesis of plasminogen Hakodate: the second Japanese family case of severe type I plasminogen deficiency manifested late-onset multi-organic chronic pseudomembranous mucositis.

J Thromb Thrombolysis 2016 Aug;42(2):218-24

Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine, Iida-Nishi 2-2-2, Yamagata, 990-9585, Japan.

A 64-year-old man first developed ligneous conjunctivitis at the age of 58 years after right pulmonary resection because of suspected cancer; otherwise, he had been healthy. Since then, he began to suffer from various forms of chronic pseudomembranous mucositis. Laboratory tests demonstrated that he had 7. Read More

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Natural heterogeneity of α2-antiplasmin: functional and clinical consequences.

Blood 2016 Feb 1;127(5):538-45. Epub 2015 Dec 1.

Department of Hematology, Erasmus University Medical Center, Rotterdam, The Netherlands.

Human α2-antiplasmin (α2AP, also called α2-plasmin inhibitor) is the main physiological inhibitor of the fibrinolytic enzyme plasmin. α2AP inhibits plasmin on the fibrin clot or in the circulation by forming plasmin-antiplasmin complexes. Severely reduced α2AP levels in hereditary α2AP deficiency may lead to bleeding symptoms, whereas increased α2AP levels have been associated with increased thrombotic risk. Read More

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February 2016

Fli1 deficiency contributes to the downregulation of endothelial protein C receptor in systemic sclerosis: a possible role in prothrombotic conditions.

Br J Dermatol 2016 Feb 21;174(2):338-47. Epub 2015 Nov 21.

Department of Dermatology, University of Tokyo Graduate School of Medicine, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan.

Background: Endothelial protein C receptor (EPCR), expressed predominantly on endothelial cells, plays a critical role in the regulation of the coagulation system and also mediates various cytoprotective effects by binding and activating protein C. So far, the role of EPCR has not been studied in systemic sclerosis (SSc).

Objectives: To investigate the potential contribution of EPCR to the development of SSc. Read More

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February 2016

Severe congenital factor XIII deficiency caused by novel W187X and G273V mutations in the F13A gene; diagnosis and classification according to the ISTH/SSC guidelines.

Haemophilia 2014 Mar 29;20(2):255-62. Epub 2013 Nov 29.

Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine, Yamagata, Japan.

Factor XIII (FXIII) consists of the A and B subunits (FXIII-A and FXIII-B) and stabilizes fibrin clots. Defects in either the FXIII-A or FXIII-B gene lead to congenital FXIII deficiency, which manifests a life-long haemorrhagic tendency. Thus, prophylactic FXIII replacement therapy is recommended. Read More

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Changes of proteases and proteinase inhibitors in androgen-dependent advanced prostate cancer patients with alpha2-macroglobulin deficiency.

Clin Lab 2012 ;58(3-4):217-25

Department of Laboratory Medicine, School of Medicine, Kitasato University, 1-15-1 Kitasato, Sagamihara, Minami-ku, Kanagawa 252-0374, Japan.

Background: It is thought that the quantitative imbalance between proteases and their inhibitors is a causative factor in invasion and metastasis of cancer cells. We previously reported on a number of androgen-dependent advanced prostate cancer (PCa) patients in which serum alpha2-macroglobulin (alpha2M) levels were markedly decreased to < 20 mg/dL (defined as alpha2M deficiency). Anti-androgen therapy is at first generally very effective for androgen-dependent advanced PCa, yielding survival benefits for most patients. Read More

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[Interpretation of hemostatic and fibrinolytic markers].

Hidesaku Asakura

Rinsho Byori 2011 Oct;59(10):970-7

Department of Internal Medicine (III), Kanazawa University School of Medicine, Kanazawa 920-8641, Japan.

Blood dose not normally coagulate in the blood vessels covered with endothelial cells, because these cells contain some substances responsible for antithrombotic action such as thrombomodulin, heparin-like substance, prostacyclin, nitric oxide and tissue plasminogen activator. Most important role of blood coagulation is hemostasis. Blood can coagulate in two ways: intrinsic coagulation pathway and extrinsic coagulation pathway that is activated by negatively charged substances and FVIIa-tissue-factor (TF) complex, respectively. Read More

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October 2011

Rare and unusual bleeding manifestations in congenital bleeding disorders: an annotated review.

Clin Appl Thromb Hemost 2012 Mar-Apr;18(2):121-7. Epub 2011 Aug 25.

Department of Medical and Surgical Sciences, University of Padua Medical School, Italy.

Epistaxis, superficial and deep hematomas, hemarthrosis, gastrointestinal bleeding, hematuria represent the most frequent hemorrhagic events in congenital coagulation disorders. Occasionally, bleeding manifestations occur in unusual sites or are peculiar. A clotting defect may alter the clinical aspect of skin conditions or infections (hemorrhagic scabies or varicella). Read More

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Acute myocardial ischemia in a patient with heterozygous alpha-2-plasmin inhibitor deficiency.

Blood Coagul Fibrinolysis 2009 Oct;20(7):599-600

Department of Hematology, University Medical Center Groningen, Hanzeplein 1, 97013 GZ Groningen, The Netherlands.

In this brief report we present a patient with heterozygous alpha2 plasmin inhibitor (alpha2PI) deficiency who developed atherosclerosis and myocardial ischemia in the presence of multiple classical risk factors. Management was complicated by fear of bleeding complications with the use of antiplatelet agents and serious comorbidity.The role of alpha2PI in atherosclerosis and pathological clot formation is still not clear. Read More

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October 2009

Role of interleukin-6 in fibrinolytic changes induced by lipopolysaccharide in mice.

Blood Coagul Fibrinolysis 2006 Jun;17(4):307-9

Inhalation Toxicology & Pathophysiology Research Team, National Institute for Environmental Studies, Tsukuba, Ibaraki, Japan.

We have recently demonstrated that interleukin (IL)-6 is protective against coagulatory and hemostatic disturbance and subsequent pulmonary hemorrhage induced by bacterial endotoxin, at least partly, via the inhibition of proinflammatory cytokines and chemokines using IL-6-null [IL-6(-/-)] mice and corresponding wild-type mice. Its role in fibrinolytic systems remains undefined, however. The present study elucidated the role of IL-6 in the activity of alpha(2)-plasmin inhibitor, an inhibitor of fibrinolysis, during inflammation induced by intraperitoneal administration of lipopolysaccharide in IL-6(-/-) and wild-type mice. Read More

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Discovery of alpha2-plasmin inhibitor and its congenital deficiency.

N Aoki

J Thromb Haemost 2005 Apr;3(4):623-31

Tokyo Medical and Dental University, Tokyo, Japan.

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[Alpha2-plasmin inhibitor (alpha2PI)].

Jun Mimuro

Nihon Rinsho 2004 Dec;62 Suppl 12:708-10

Cell and Molecular Medicine, Center for Molecular Medicine, Jichi Medical School.

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December 2004

Haemostatic management of intraoral bleeding in patients with congenital deficiency of alpha2-plasmin inhibitor or plasminogen activator inhibitor-1.

Haemophilia 2004 Sep;10(5):669-74

Department of Dental Anesthesiology, Graduate School of Dentistry, Osaka University, Suita, Japan.

Haemostatic management of intraoral bleeding was investigated in patients with congenital alpha2-plasmin inhibitor (alpha2-PI) deficiency or congenital plasminogen activator inhibitor- 1 (PAI-1) deficiency. When extracting teeth from patients with congenital alpha2-PI deficiency, we advocate that 7.5-10 mg kg(-1) of tranexamic acid be administered orally every 6 h, starting 3 h before surgery and continuing for about 7 days. Read More

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September 2004

A case of intramedullary haematoma associated with congenital alpha2-plasmin inhibitor deficiency.

Pediatr Radiol 1998 Dec;28(12):978-80

Service de Radiologie Pédiatrique, Hôpital d'Enfants Armand-Trousseau, 26 avenue du Dr Arnold-Netter, F-75 571 Paris Cedex 12, France.

The association of intramedullary haematoma of the left femoral diaphysis and congenital alpha2-plasmin inhibitor deficiency in a 6-year-old boy is reported. Congenital deficiency of this fibrinolytic system component is very rare and can result in severe bleeding. Clinically, an intramedullary haematoma causes pain but no swelling, and there is no history of trauma. Read More

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December 1998

Alpha2-plasmin-inhibitor deficiency (Miyasato disease).

Lancet 1978 Dec 23-30;2(8104-5):1334-6

A 25-year-old man, born in Okinawa, Japan, had a haemorrhagic diathesis characterised by prolonged bleeding and ecchymoses after minor trauma and spontaneous joint haemorrhage. The frequency and severity of these episodes were reduced by an antiplasminic drug. Routine coagulation studies revealed no abnormalities except for significantly sshortened euglobulin-lysis time and whole-blood clot lysis time. Read More

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February 1979
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