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At least 1,189 records · Page 66Linked to original sources
PubMed1975

Platelet and fibrinogen survival with 75Se selenomethionine in acute infectious hepatitis.

Simultaneous platelet and fibrinogen survival with 75Se selenomethionine was determined in eight patients with acute infectious hepatitis of intermediate severity. Fibrinogen survival alone was estimated in another nine patients, seven of whom were receiving heparin treatment. Platelet survival was found to be normal (7-9 days) in seven of the 8 patients; it was reduced 4,6 days) only in one patient, who was also affected by measles. Fibrinogen survival was markedly reduced (1-3.7 days) and fibrinogen turnover sharply increased (0.59-2.80 mg/ml/day) in all but one patient, who had thalassaemia major, with normal fibrinogen survival and fibrinogen turnover. Heparin treatment did not affect either platelet survival or fibrinogen turnover. In all patients the coagulation defect was mild and no sign of disseminated intravascular coagulation or of increased fibrinolytic activity could be demonstrated by routine tests. These results are consistent with the hypothesis that in acute infectious hepatitis the decreased survival and increased turnover of fibrinogen might be due to a pathological pathway of defibrination in dependent of thrombin of plasmin.

Afibrinogenemia↗
PubMed2001

A frameshift mutation in the human fibrinogen Aalpha-chain gene (Aalpha(499)Ala frameshift stop) leading to dysfibrinogen San Giovanni Rotondo.

We have investigated a 53-yr-old asymptomatic white man with decreased functional, but not immunologic, fibrinogen plasma levels together with prolonged thrombin and reptilase times, detected through routine coagulation studies prior to a surgical procedure. A new heterozygous single nucleotide deletion (C) at position Ala499 within the Aalpha-chain gene was identified, which predicted changes of the corresponding amino acids encoded by the subsequent portion of the exon V and the appearance of a premature stop codon at position 518 (Aalpha[499]Ala frameshift stop). The new dysfunctional fibrinogen, San Giovanni Rotondo variant, was confirmed in vivo by SDS-PAGE analysis of HPLC-purified fibrinogen chains. Mass spectrum examination of the abnormal HPLC-purified peak gave an estimated mass (56,088 Da) similar to that predicted by DNA analysis of the mutated Aalpha-chain gene (56,088 Da) and, after tryptic digestion, the truncated Aalpha-chain was shown only in the propositus, who also carried normal Aalpha-chain. In addition, mass spectrum analysis of the tryptic digest of the abnormal chain confirmed the presence of a new and unpaired cysteine at the last position that was predicted to form a disulfide bridge with human serum albumin. Immuno-blot analysis confirmed that fibrinogen San Giovanni Rotondo variant, but not normal fibrinogen. contained substantial amounts of albumin. Present findings confirm that truncated Aalpha-chain lacking part of the terminal domain may be incorporated into mature fibrinogen molecules and normally secreted in the bloodstream.

Afibrinogenemia↗
PubMedDate not supplied

Leg ulcer presenting in a patient with congenital afibrinogenaemia.

Congenital afibrinogenaemia is a rare hemorrhagic disorder characterized by the absence of fibrinogen. We report a case of congenital afibrinogenaemia presented with leg ulcer. A 30-year-old man presented with a history of prolonged bleeding from birth. His parents are cousins. He repeatedly showed haematoma after traumas on his leg. He was diagnosed as having congenital afibrinogenaemia because of plasma fibrinogen deficiency. Because his leg ulcer gradually increased in size, he was admitted to our department for treatment. Laboratory examinations revealed prolonged bleeding time, prolonged coagulation time, prolonged prothrombin time, prolonged activated partial thromboplastin time and plasma fibrinogen was not measurable. Histological examination revealed hyperkeratosis, acanthosis and severe fibrotic change in the whole dermis. Severe hemosiderin deposit was found in the middle dermis. His leg ulcer cured 2 months after the beginning of fresh frozen plasma administration (FFP), but recurrence of the leg ulcer after FFP treatment was found. This is the second reported case of congenital afibrinogenaemia presented with leg ulcer.

Adult↗
PubMed1975

The pathogenesis of Vaccinia virus toxicity. I. The role of virus-platelet interaction.

When mice are injected intravenously with a large dose of vaccinia virus, prepared in the Ehrlich ascites carcinoma of the mouse, there is a precipitous loss of plasma fibrinogen and blood platelets. Death occurs usually within 24 hours. A specific role of the virus in this toxic syndrome can be demonstrated when heparin is employed to circumvent intravascular coagulation and fibrinogen loss. Heparin does not prevent a profound thrombocytopenia from occurring, but it modifies the rate of platelet loss. Toxicity is prevented when heparinized virus preparations are pretreated with beta-propiolactone or specific antibody, although a mild thrombocytopenia occurs. Thrombocytopenia does not occur in mice injected with heparinized material prepared from uninfected tumors. These studies indicate that the basic mechanism of vaccinia virus toxicity is an early interaction between infectious virus and blood platelets, with marked thrombocytopenia and consequential pathophysiologic changes.

Afibrinogenemia↗
PubMed2002

[Detection of platelet fibronectin from congenital fibrinogenopenic patients and its clinical significance].

OBJECTIVE: To assess the platelet and plasma concentrations of fibronectin (Fn) and fibrinogen (Fg) in congenital fibrinogenopenic (FgP) patients and explore their role in inducing platelet adhesion and aggregation. METHODS: A FgP family was selected as study group and the platelets isolated and purified to assess concentrations of Fn and Fg in platelets, alpha-granules and plasma with Western blotting, immuofluoresence staining and flow cytometry (FACS), respectively, the expression of platelets GP II b/III a by FACS. RESULTS: The concentration of platelets Fn in FgP patients is higher than that in controls, and is higher in homozygote than in heterozygote. In contrast, plasma Fn levels were identical in all samples. The amount of platelet Fg from FgP patients is lower than that from the controls and positively correlated with the concentration of their plasma Fg. No difference in the expression of platelet GP II b/III a had been found. CONCLUSION: It suggested that increased platelet Fn could partially compensate the lack of Fg and lead the platelet adhesion and aggregation.

Afibrinogenemia↗
PubMed2002

[Thrombophilias associated with cerebral venous thrombosis].

Many coagulation disorders have been associated with cerebral venous thrombosis. These disorders may be primary like protein C and S deficiency, antithrombine III deficiency and activated protein C resistance. Antiphospholipid antibodies represent an acquired disorders of coagulation. A prothrombotic state induced by more common factor including oral contraceptive, pregnancy and puerperium increases the venous thrombosis risk.

3' Untranslated Regions↗
PubMed1975

[Heparin in intravascular coagulation].

1. The diagnostic criteria of intravascular coagulation are critically reviewed. Before a potentially dangerous drug is administered, the diagnosis should be more securely established than has been the case in the past. In particular, fibrinogen loss from the circulation must be excluded whenever this protein shows unduly rapid rapid disappearance from the blood. 2. Even when the diagnosis is firmly secured, advocacy of heparin therapy should not be based on theoretical considerations or on the huge amount of accumulated case reports but on figures derived from controlled clinical trials, the essential data being nortality and bleeding complications and not the course of laboratory pattern.

Afibrinogenemia↗
PubMed2002

Novel fibrinogen truncation with deletion of Bbeta chain residues 440-461 causes hypofibrinogenaemia.

A 24-year-old male with hepatitis C was initially diagnosed with hypofibrinogenaemia during investigations prior to a liver biopsy. He had a low functional and gravimetric fibrinogen concentration of 1.0 mg/mL and DNA sequencing of all exons, exon-intron boundaries and promoter regions of the fibrinogen Aalpha, Bbeta, and gamma genes revealed a single heterozygous g-->a mutation at nucleotide 8035 of the Bbeta gene. This creates a premature stop at the Trp 440 codon and results in a 22-residue truncation of the Bbeta chain. Analysis of purified plasma fibrinogen by SDS PAGE, reverse phase HPLC and ESI MS, however, failed to detect any of the truncated chains in the plasma fibrinogen. The non-expression of aberrant molecules was further confirmed by functional analysis, which revealed normal fibrin polymerisation. The principal structural feature of the independently folding betaD domain is its five-stranded anti-parallel beta sheet. The deletion here of residues 440 to 461 removes the second strand from this sheet structure and appears to impact on the viability of the nascent chain and its ability to be incorporated into mature fibrinogen molecules. The mutation does not however provoke the formation of hepatic inclusion bodies.

Adult↗
PubMed1975

[Giant occipital hemangioendothelioma with thrombocytopenia, anemia and hypofibrinogenemia treated by total excision (author's transl)].

An infant with a large occipital hemangioendothelioma with thrombocytopenia, anemia, and hypofibrinogenemia--Kasabach-Merritt syndrome--was reported. The case, a male neonate is unique, for this is the first report with this syndrome in whom the large hemangioma was noted at birth on the midocciput simulating the occipital encephalomeningocele. With the development of thrombocytopenia of 84,000 per mm3, hypofibrinogenemia of 92 mg/dl, anemia (erythrocyte 193 X 10(4) per mm3, hemoglobin 5.9 g/dl, hematocrit 16 vol%), hepato-splenomegalia, enlargement and bluish discoloration of the tumor noted on the 21/2 months of life, total excision was intended prior to the expected occurence of the systemic purpura. The patient received fresh whole blood transfusion immediately prior to surgery, and the total excision was successfully performed. Excessive bleeding was not encountered. Abrupt rise in the platelet count, red blood cell count, hemoglobin and hematocrit to normal range was noted at the first postoperative day; he was discharged on the 17th day after surgery. Nineteen months' follow-up showed normal hematologic findings with good somatic and mental development. The specimen weighing 250 g. revealed benigh hemangioendothelioma. Silver impregnation demonstrated lobular aggregates of small vascular channels. Papillary projection of interstitial cells into the lumen, reaction of the endothelium of the vessels, newly formed thrombus, ishemic necrosis and hemorrhage, hyaline degeneration of interstitial tissue were noted. These findings suggested the disseminated intravascular coagulation within the tumor followed by fibrinolysis accounts for loss of blood corpuscles, platelet, fibrinogen and clotting factors, which leads ultimately to the consumption coagulopathy and diffuse bleeding.

Afibrinogenemia↗