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At least 685 records · Page 38Linked to original sources
PubMed1991

Fibrinogen date: congenital hypodysfibrinogenemia associated with decreased binding of tissue-plasminogen activator.

A new case of congenital hypodysfibrinogenemia associated with a thrombotic tendency (thrombosis of the peroneal artery, the pulmonary artery and the deep veins of the lower extremities), is reported. Clotting time using Reptilase was significantly prolonged. The low levels of plasma fibrinogen were demonstrated using both thrombin time (Clauss) method (45.0 mg/ml) and radial immunodiffusion technique (106.1 mg/ml). The fibrinogen is also characterized by a defective polymerization of fibrin monomers. Furthermore, the important functional property of this fibrinogen is that the patient's fibrin adsorbs approximately 31-38% of added tissue-plasminogen activator (t-PA) at the time of fibrin formation, whereas control fibrin can adsorb about 79% of added t-PA. This result could provide an explanation for the thrombotic tendency in this patient. This variant fibrinogen appears to have unique characteristics and has been designated as "Fibrinogen Date," according to the city where the proband has lived.

Afibrinogenemia↗
PubMed1995

Dysfibrinogenemia: a case with thrombosis (fibrinogen Richfield) and an overview of the clinical and laboratory spectrum.

Fibrinogen Richfield exemplifies a dysfibrinogen associated with a life-long thrombotic tendency. The evaluation of this novel case indicates that, like similar thrombotic dysfibrinogenemias, the abnormal protein polymerizes abnormally and demonstrates impaired clot dissolution. A survey of other cases of dysfibrinogenemia indicates that the relatively common abnormalities of Fibrinopeptide A release are generally asymptomatic or associated with bleeding, polymerization abnormalities are likely to be asymptomatic or associated with thrombosis (or occasionally bleeding), and complex abnormalities or additional, independent hemostatic defects are rather common. Thrombin and Reptilase clotting times are not helpful in distinguishing between the subsets, but clinical history, fibrinopeptide release, and polymerization studies may be useful. Abnormalities of fibrinogen function tend to correlate with changes in molecular domains related to binding and hydrolysis.

Afibrinogenemia↗
PubMedDate not supplied

Consumption coagulopathy associated with intrauterine fetal death: the role of heparin therapy.

A gravida with intrauterine fetal death who developed progressive chronic consumption coagulopathy was treated with heparin. When serial fibrinogen levels fell below 100 mg% and the prothrombin time was significantly prolonged, intravenously injected heparin corrected hypofibrinogenemia. A safe delivery followed administration of oxytocin. The authors emphasize the infrequent need for heparin therapy in the majority of cases of the intrauterine fetal death syndrome. Therapeutic guidelines for its use in selected cases are reviewed.

Adolescent↗
PubMed1978

L-Asparaginase-induced coagulopathy during therapy of acute lymphocytic leukemia.

L-Asparaginase-induced coagulation abnormalities were observed in all patients receivng this drug. All patients studied demonstrated low factor IX and fibrinogen levels and most (75%) had low levels of factor XI. Disturbances in other factors were much less frequent. Despite the presence of markedly abnormal coagulation studies, clinical bleeding was not observed.

Adolescent↗
PubMed2006

Delayed inflammatory responses to endotoxin in fibrinogen-deficient mice.

Severe inflammation leads to haemostatic abnormalities, such as the development of microvascular thrombi. As a result, ischaemia-related downstream organ damage can occur. The present study demonstrates that mice with a total deficiency of fibrinogen (Fg(-/-)) present with altered responses to challenge with Gram-negative lipopolysaccharide (LPS). Early survival in response to continuous LPS challenge was increased in Fg(-/-) mice and histological findings indicated that this improvement correlated with a lack of fibrin deposition in organs. Neutrophils appeared early in the lungs of challenged wild-type (WT) mice, but occurred in Fg(-/-) mice at later times. This delayed response in Fg(-/-) mice was confirmed by studies that showed a strong dependence on Fg of binding of neutrophils to endothelial cells in the presence of LPS. While cytokines were also elevated in both WT and Fg(-/-) mice, their levels were generally lower at early times in this latter group. The time course of MIP-2 expression correlated with the occurrence of pulmonary leakage after LPS challenge, which was delayed in Fg(-/-) mice. These results suggest that fibrin(ogen) plays a role as an early mediator in the cross-talk between coagulation and inflammation.

Afibrinogenemia↗
PubMedDate not supplied

The mechanism of loss of CR1 during maturation of erythrocytes is different between factor I deficient patients and healthy donors.

During the in vivo maturation of erythrocytes, the number of CR1 per cell decreases by approximately two-thirds in 30 days. The CR1 loss is enhanced in several diseases such as SLE, AIDS, and particularly in factor I deficiency. Microvesicles enriched in CR1 and DAF are released from erythrocytes matured in vitro, leading to the same loss of both molecules. When comparing reticulocytes and erythrocytes, CR1 and DAF were lost similarly in 15 normal individuals, suggesting that vesiculation may be at the origin of CR1 loss in vivo. However, the enhanced loss of CR1 in 3 patients with factor I deficiency was contrasted with a normal loss of DAF, raising the possibility that, in this pathological condition, CR1 might be proteolytically cleaved, leaving small CR1 fragments on the erythrocytes. To answer this question, a rabbit polyclonal antibody was raised against the cytoplasmic (tail) domain of CR1, which recognised specifically CR1 of erythrocytes and urinary vesicles on Western blots. However, no CR1 fragments could be detected on erythrocytes of the factor I deficient patients although this antibody was able to recognise CR1 fragments after treatment of normal erythrocytes or urinary vesicles with elastase. These data suggest that cell surface domains rich in CR1, but not in DAF, are specifically lost in factor I deficiency.

Afibrinogenemia↗
PubMed1994

The effect of hypofibrinogenemia and fibrinolysis on skeletal muscle function after ischemia and reperfusion.

Isometric contraction to direct supramaximal tetanic stimulation of the anterior tibialis (AT) muscle was measured in 50 New Zealand White rabbits after ischemia and reperfusion. Ischemia was produced unilaterally by collateral ligation and temporary inflow control until AT muscle function decreased to < 5% of contralateral (control) AT muscle and the ischemic interval was recorded. Reperfusion was carried out in one of the following ways: group I (n = 20), release of vascular clamps (blood reperfusion [BR]); group II (n = 10), release of vascular clamps and simultaneous intraarterial administration of 50,000 units of urokinase (urokinase reperfusion [UR]); group III (n = 10), release of vascular clamps and simultaneous administration of 50,000 units of urokinase and 28 mg (5 units) of purified rabbit plasminogen (urokinase plasminogen reperfusion [UPR]); and group IV (n = 10), animals defibrinated to < 50 mg/dl with ancrod prior to ischemia and received BR (ancrod blood reperfusion [ABR]). During reperfusion, function was recorded every 60 min for 2 hr. Recovery of experimental muscle function is expressed as the percentage of contralateral control limb function. The mean ischemic interval (mean +/- SEM), to achieve < 5% of contralateral control limb function, was 206.7 +/- 9.9, 209.5 +/- 16.6, 221.7 +/- 12.5, and 272.0 +/- 14.2 min for animals in groups I-IV, respectively. The mean experimental muscle function (mean +/- SEM) following the ischemic interval was 3.2 +/- 0.8, 4.5 +/- 1.4, 4.4 +/- 1.2, and 3.3 +/- 1.0 for groups I-IV, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

Afibrinogenemia↗
PubMed1990

Native fibrin gel networks and factors influencing their formation in health and disease.

Hydrated fibrin gels were studied by confocal laser 3D microscopy, liquid permeation and turbidity. The gels from normal fibrinogen were found to be composed of straight rod-like fiber elements which sometimes originated from denser nodes. In gels formed at increasing thrombin or fibrinogen concentrations, the gel networks became tighter and the porosity decreased. The fiber strands also became shorter. Gel porosity of the network decreased dramatically in gels formed at increasing ionic strengths. Shortening of the fibers were observed and fiber swelling occurred at ionic strength above 0.24. Albumin and dextran, when present in the gel forming system, affected the formation of more porous structures with strands of larger mass-length ratio and fiber thickness. This type of gels were also formed in plasma. Albumin and lipoproteins may be among the determinants for the formation of this type of gel structure in plasma. Gels formed when factor XIIIa instead of thrombin was used as catalyst for gelation showed a completely different structure in which lumps of polymeric material were held together by a network of fine fiber strands. Our studies have also shown that the methodologies employed may be useful in studies of gel structures in certain dysfibrinogenemias as well as in other diseases. We give examples of two patients with abnormal fibrinogen and of patients with ischaemic heart disease.

Adult↗
PubMed1992

Hypofibrinogenemia due to fibrin formation in subserosal type eosinophilic gastroenteropathy.

A 33-year-old woman presented with abdominal pain and distention, diarrhoea and marked eosinophilia in blood and ascites. As other causes could be excluded, the subserosal type of eosinophilic gastroenteropathy was diagnosed. The low plasma fibrinogen level (less than 100 mg/100 ml) found in this patient is an as yet undescribed feature. During prednisolone therapy it increased concurrently with the fall of blood eosinophils and the relief of clinical symptoms. Interest was further directed to the ascitic fluid where not only the presence of eosinophils (74%) enveloped in fibrin yarn and of basophils (2%) but also of 24% T lymphocytes (among them 75% CD4+, 24% CD8+, 4% CD25+, less than 1% CD19+, less than 1% natural killer cells) could be demonstrated. These lymphocytes are likely to be the source for lymphokine production chemoattracting eosinophils into the intestine. In addition they seem to be involved in IgE hyperproduction, which after adequate therapy and complete resolution of the clinical symptoms, tended to decrease slowly.

Adult↗
PubMed1978

Blood coagulation studies in patients with advanced carcinoma of the prostate treated with 2,6-cis-diphenylhexamethylcyclotetrasiloxane or estramustine-17-phosphate.

Two drugs, 2,6-cis-diphenylhexamethylcyclotetrasiloxane (Cisobitan) and estramustine-17-phosphate (Estracyt) were given to patients with poorly differentiated metastatic carcinoma of the prostate. The effect of the drugs on blood coagulation was investigated. Some parameters showed changes during the treatment: Antithrombin III decreased in the Estracyt treated patients to a level which might imply a thrombogenic effect. Fibrinogen decreased, whereas factor VIII showed no consistent change. Normotest changes appeared to correlate with liver damage whereas antithrombin III showed no change. Increased levels of fibrinogen degradation products and fibrinopeptide A (FPA) were more frequent in the group of deteriorating patients. However, the number of FPA analyses were too small for any definite conclusions regarding possible disseminated intravascular coagulation.

Afibrinogenemia↗
PubMed1993

Fibrinogen Osaka IV: a congenital dysfibrinogenemia found in a patient originally reported in relation to surgery, now defined to have an A alpha arginine-16 to histidine substitution.

We discovered a congenital heterozygous dysfibrinogen in a patient and reported this case in relation to surgery some time ago (Jpn J Surg (1988) 18:43-46). Further studies on the isolated abnormal population of fibrinogen derived from this patient have revealed that fibrinopeptide A was not cleaved by ancrod, a snake venom-derived thrombin-like enzyme, but by thrombin, slowly but completely. The released fibrinopeptide A components, being the A, AY, and AP peptides, were all found to be abnormal, as evidenced by slightly earlier elution positions on high-performance liquid chromatography, compared with the normal counterparts. By analyzing their amino acid sequence, we have identified an arginine to histidine substitution at position 16 of the A alpha chain, the thrombin cleavage site. Utilizing insolubilized abnormal fibrinogen, we confirmed that the polymerization site assigned to the central E domain, the "A" site, was exposed by thrombin, but not by ancrod. This dysfibrinogen, designated as fibrinogen Osaka IV, is the second abnormal molecule with an A alpha arginine-16 to histidine substitution identified among Japanese families.

Afibrinogenemia↗
PubMed1995

Heterozygous protein C deficiency and dysfibrinogenemia acquired by liver transplantation.

Orthotopic liver transplantation is now a successful treatment for end-stage liver diseases. Since most components of the coagulation system are synthesized by liver parenchymal cells, there is always a risk of genetic defects of hemostasis being transmitting by liver transplantation. Some coagulation factor defects, such as protein C deficiency, do not induce abnormalities in routine coagulation tests and, thus, go undetected before organ procurement. We report the first case, to our knowledge, of the transmission of heterozygous protein C deficiency, an autosomal recessive genetic defect, associated with dysfibrinogenemia, an autosomal dominant trait, by liver transplantation. Both the recipient and the donor presented with severe thrombotic complications. This case shows that potentially morbid genetic defects can be transmitted by organ transplantation, and it emphasizes the difficulty associated with organ procurement criteria, particularly for liver transplantation, in which routine blood tests appear insufficient for determining whether or not organs can or should be procured from a given donor.

Afibrinogenemia↗
PubMed1977

Haemorrhagic diathesis as a possible early sign of hereditary fructose intolerance.

An infant girl three weeks of age with the leading symptom of skin haemorrhages is presented. On further investigation, the signs of severe hepatic damage with hypofibrinogenaemia and prothrombin complex impairment, and renal tubular dysfunction were disclosed. All these pathological symptoms, which were reversed on fructose free diet, were caused by hereditary fructose intolerance.

Afibrinogenemia↗
PubMed1983

Hereditary hypofibrinogenemia with fibrinogen storage in the liver.

A family with hereditary autosomal dominant hypofibrinogenemia is described. The outstanding feature is massive deposition of fibrinogen/fibrin within hepatocytes, faintly visible in routine microscopic sections, but clearly demonstrable by immunohistologic techniques. Circulating fibrinogen shows normal electrophoretic mobility of A alpha-, B beta-, and gamma-chains. We assume that the hereditary defect in this family interferes with fibrinogen release from hepatocytes. Clinically there are fluctuating slight elevations of serum transaminase levels. Hemostasis and wound healing are undisturbed.

Adult↗