427 Results

Human plasma fibrinogen is synthesized in the liver.

Hereditary systemic amyloidosis caused by fibrinogen Aalpha-chain gene mutations is an autosomal dominant condition with variable penetrance, usually of late onset, and typically presents with nephropathy leading to renal failure.

Monitoring and Treatment of Coagulation Disorders in End-Stage Liver Disease.

Background: Patients with end-stage liver disease (ESLD) are assumed to be at high risk of bleeding when undergoing any kind of invasive intervention (any kind of operation, including transplantation or minimally invasive...

Budd-Chiari syndrome in an afibrinogenemic patient: a paradoxical complication.

Qualitative and quantitative abnormalities of fibrinogencan result in hemostatic disorders (1). Congenital afibrinogenemia is a rare coagulation disorder transmitted as an autosomal recessive trait (2). The incidence of  afibrinogenemia is...

Perioperative coagulation management in a patient with afibrinogenemia undergoing liver transplantation.

Afibrinogenemia is a rare hereditary coagulation disorder characterized by a propensity toward bleeding. A 21-year-old Hispanic woman with afibrinogenemia developed ascites, a distended abdomen, an enlarged liver...

Hyperfibrinolysis in liver disease.

The incidence of hyperfibrinolysis in patients with cirrhosis is still debated. The reasons for this uncertainty probably lie in the lack of appropriate laboratory tests for its evaluation. There is a relative consensus, however...

Fibrinogen angers with a new deletion (gamma GVYYQ 346-350) causes hypofibrinogenemia with hepatic storage.

Introduction: This study reports a family with chronically abnormal blood liver function tests (LFT) and congenital hypofibrinogenemia. The proposita had cirrhosis initially related to alcohol abuse and chronic viral hepatitis C...

Novel fibrinogen mutation γ314Thr→Pro (fibrinogen AI duPont) associated with hepatic fibrinogen storage disease and hypofibrinogenaemia.

Mutation in fibrinogen genes may lead to quantitative or qualitative disorders that result in bleeding, thrombosis or hepatic fibrinogen storage disease. Only three mutations in the fibrinogen γ gene have been identified that cause...

Novel fibrinogen gamma375 Arg-->Trp mutation (fibrinogen aguadilla) causes hepatic endoplasmic reticulum storage and hypofibrinogenemia.

The proposita and her sister had chronically elevated liver function test results, and needle biopsy specimens showed scattered eosinophilic inclusions within the hepatocytes. On immunoperoxidase staining, the inclusions reacted...

Fibrinogen brescia: hepatic endoplasmic reticulum storage and hypofibrinogenemia because of a gamma284 Gly-->Arg mutation.

The proposita suffered from liver cirrhosis and biopsy showed type 1 membrane-bound fiberglass inclusions. The hepatic inclusion bodies were weakly periodic acid-Schiff diastase-positive, and on immunoperoxidase staining reacted...

Do we still need cryoprecipitate? Cryoprecipitate and fibrinogen concentrate as treatments for major hemorrhage - how do they compare?

This paper examines the current practice and evidence regarding the role of different modes of fibrinogen replacement in major hemorrhage in 3 distinct clinical settings: trauma, obstetric hemorrhage, and gastrointestinal...

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