Treatment of Factor XIII Deficiency with Cryoprecipitate

1968 ◽  
Vol 20 (03/04) ◽  
pp. 528-533 ◽  
Author(s):  
C. J Amris ◽  
M Hilden
Keyword(s):  
Factor Xiii ◽  
Prophylactic Treatment ◽  
Factor Xiii Deficiency ◽  
Intravenous Injections ◽  
Congenital Factor

SummaryIn in vitro and in vivo investigations of two patients with congenital factor XIII-deficiency it was demonstrated that cryoprecipitate contains a high factor XIII-activity. Cryoprecipitate in small amounts, given as intravenous injections every 3 or 4 weeks seems to be useful as a prophylactic treatment of patients with severe haemorrhagic due to factor XHI-deficiency.

Prevention Of Shwartzman’S Phenomenon In Factor XIII Deficiency

1981 ◽  
Author(s):  
Soo Young Lee ◽  
Sung Keun Chang ◽  
Soo II Chung
Keyword(s):  
Factor Xiii ◽  
Rabbit Platelet ◽  
Factor Xiii Deficiency ◽  
Platelet Factor ◽  
Cortical Necrosis ◽  
Rate Of Increase ◽  
Shwartzman Reaction

Cross-linked clots formed in vitro are reported to be more resistant to fibrinolysis but the relevance of these observations to the situation in vivo is uncertain. The possible role of Factor XIII in the formation of diffuse intravascular fibrin deposition was examined in experimentally induced Factor XIII deficient rabbits. Factor XIII deficiency was induced by intravenous infusion of IgG isolated from goat anti-rabbit platelet Factor XIII. Control received normal goat IgG. The Shwartzman reaction was produced by two injections of bacterial endotoxin given 24 hours apart.The most striking histological differences were observed after 48 hours. A large number of glomerular loops were enlarged and engorged with red blood cells and platelet- fibrin thrombi; extensive bilateral cortical necrosis was observed in 8 out of 10 endotoxin injected control rabbits but none in the Factor XIII deficient group.Fibrinogen levels in control rabbits were increased 3-4 fold (1.1g/100 ml), at 24 hours and slightly decreased at 48 hours after endotoxin injection, whereas in Factor XIII deficient animals, the rate of increase was slower but reached similar levels at 48 hours. Fibrinolytic activity in vivo, studied by the degradation of infused 125I-fibrinogen, was significantly increased in both endotoxin injected groups, irrespective of Factor XIII levels.These results strongly suggest that cross-linked thrombi are more resistant to fibrinolysis in vivo as well as in vitro.

Factor XIII Concentrate in the Long Term Management of Congenital Factor XIII Deficiency

1975 ◽  
Author(s):  
K. Miloszewski ◽  
M. S. Losowsky
Keyword(s):  
Half Life ◽  
Factor Xiii ◽  
Blood Levels ◽  
Factor Xiii Deficiency ◽  
Plasma Factor ◽  
Term Management ◽  
Plasma Infusions ◽  
Congenital Factor

Congenital Factor XIII deficiency causes a serious and disabling bleeding diathesis with a high risk of fatal intracranial haemorrhage. Blood levels of Factor XIII of a few percent of normal are sufficient to control bleeding and the in vivo half-life of Factor XIII is long, making permanent prophylaxis practicable.We have kept 5 patients with congenital Factor XIII deficiency free from bleeding manifestations for 3 to 10 years by regular plasma infusions. Their lives have been transformed. Plasma as a long term source of Factor XIII has disadvantages and a purified Factor XIII has been derived from human placenta (Hoechst), but its in vivo half-life was said to be only 70 hours. The only detailed report of the use of this preparation is of two injections in a single patient. We have used this preparation regularly for two years in two patients and we find that its half-life is similar to that of plasma Factor XIII i.e. 12–14 days. Both patients have remained free from bleeding manifestations or side effects.We conclude that long term prophylaxis for congenital Factor XIII deficiency is possible and the placental Factor XIII is preferable to plasma.

New Families with Hereditary Hemorrhagic Trait due to Deficiency of Fibrin Stabilizing Factor (F. XIII)

1968 ◽  
Vol 20 (03/04) ◽  
pp. 534-541 ◽  
Author(s):  
O Egeberg
Keyword(s):  
Factor Xiii ◽  
Family Members ◽  
Recessive Inheritance ◽  
Factor Xiii Deficiency ◽  
History Of ◽  
Congenital Factor

SummarySevere hemorrhagic disorder due to congenital factor XIII deficiency is described in two unrelated Norwegian girls.Plasma cephalin time was for both patients extraordinarily short during episodes of bleeding and hematomas. No such hyperactivity reaction was demonstrable in unaffected condition some months later.Estimations of blood factor XIII levels revealed a partial defect in the parents of both children, and also in some other family members, consistent with an autosomal incompletely recessive inheritance of the defect. Some of the presumptive heterozygotes had a history of light bleeding phenomenons; whether this was related to their partial lack of factor XIII is so far uncertain.

Successful pregnancy in a woman with congenital factor XIII deficiency treated with substitutive therapy

1987 ◽  
Vol 55 (1) ◽  
pp. 45-48 ◽  
Author(s):  
F. Rodeghiero ◽  
G. C. Castaman ◽  
E. Bona ◽  
M. Ruggeri ◽  
E. Dini
Keyword(s):  
Factor Xiii ◽  
Factor Xiii Deficiency ◽  
Successful Pregnancy ◽  
Congenital Factor

scholarly journals A Novel Molecular Mechanism of Severe Congenital Factor XIII Deficiency

2020 ◽  
Author(s):  
Jingjing Han ◽  
Miao Jiang ◽  
Jian Su ◽  
Ziqiang Yu ◽  
Xia Bai ◽  
...  
Keyword(s):  
Molecular Mechanism ◽  
Factor Xiii ◽  
Factor Xiii Deficiency ◽  
Congenital Factor

Noninvasive prenatal diagnosis of congenital factor XIII deficiency in Iran

2022 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Hoda Motlagh ◽  
Akbar Dorgalaleh ◽  
Shadi Tabibian ◽  
Majid Naderi ◽  
Farhad Zaker
Keyword(s):  
Prenatal Diagnosis ◽  
Factor Xiii ◽  
Factor Xiii Deficiency ◽  
Noninvasive Prenatal Diagnosis ◽  
Congenital Factor

scholarly journals In vivo radiolabeling of platelet proteins: a new method with identification of platelet factor XIII

Blood ◽  
1980 ◽  
Vol 55 (4) ◽  
pp. 559-563 ◽  
Author(s):  
DM Rider ◽  
RP McDonagh ◽  
J McDonagh
Keyword(s):  
Gel Electrophoresis ◽  
Factor Xiii ◽  
Soluble Fraction ◽  
Polyacrylamide Gel Electrophoresis ◽  
Fold Increase ◽  
Platelet Factor ◽  
Intravenous Injections ◽  
Molecular Weights ◽  
The Third

Abstract A method for radiolabeling platelets in vivo was developed in which 3H- arginine was injected into the bone marrow of normal dogs. On the third day after injection, a maximum of 6%--7% of the radioactivity had been incorporated into the total platelet mass. This method of isotope administration resulted in a 50--60-fold increase in maximum uptake of radiolabel by platelets, as compared to values obtained by others using intravenous injections of various radioactive compounds. Tritium- labeled platelets were harvested from the animals and then were washed to remove unbound 3H-arginine. On polyacrylamide gel electrophoresis 7 labeled protein bands, with molecular weights ranging from 29,000to 132,000, were obtained from the platelet-soluble fraction. One 3H- containing protein with a molecular weight of 81,000 was identified immunologically and enzymatically as platelet factor XIII.

Platelets Exposed to Elevated Levels of Endogenous Thrombopoietin In Vivo Have a Reduced Response to Megakaryocyte Growth and Development Factor In Vitro

2001 ◽  
Vol 85 (01) ◽  
pp. 152-159 ◽  
Author(s):  
Uichi Nishiyama ◽  
Haruhiko Morita ◽  
Yoshifumi Torii ◽  
Tomoaki Kuwaki ◽  
Eiko Shimizu ◽  
...  
Keyword(s):  
Growth And Development ◽  
Platelet Reactivity ◽  
Biochemical Properties ◽  
Human Platelets ◽  
Janus Kinase ◽  
Intravenous Injections ◽  
Development Factor ◽  
Lower Reactivity

SummaryThrombopoietin (TPO), or megakaryocyte growth and development factor (MGDF), has been shown to potentiate the sensitivity of normal human platelets to various agonists in vitro. The present study investigated the functional and biochemical properties of platelets from mice rendered thrombocytopenic by sublethal irradiation with regard to the reactivity to recombinant murine MGDF (rmMGDF) in vitro. During the course of reversible thrombocytopenia following irradiation, platelets from irradiated mice which had lower platelet counts and reciprocally higher plasma TPO levels showed lower reactivity to rmMGDF in agonist-induced platelet aggregation. Intravenous injections of recombinant soluble murine c-Mpl (sMpl), which has the ability to capture TPO, after irradiation restored the reactivity of platelets at the platelet nadir to rmMGDF. On the other hand, platelets prepared from normal mice 3 h after a single intravenous injection of pegylated rmMGDF did not respond to rmMGDF. There was a marked decrease in c-Mpl and Janus kinase 2 (JAK2) in platelets from irradiated mice at the platelet nadir. Similar results were observed with platelets from mice administered pegylated rmMGDF. JAK2 was only moderately decreased, however, in platelets from mice given sMpl after irradiation. These results indicate that exposure of platelets to increased endogenous TPO levels in vivo in thrombocytopenic mice leads to a reduction in the platelet reactivity to rmMGDF in vitro. Further, these results suggest that the c-Mpl-mediated signaling pathway, which is essential for the priming effect of rmMGDF, is defective in thrombocytopenic murine platelets.

Sign in / Sign up

Export Citation Format

Share Document