Challenges Regarding the Management of Gynecological and Obstetric Complications in Women with Inherited Factor XIII Deficiency

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.

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Determination of Factor XIII Activity and of Factor XIII Inhibitors Using an Ammonium-Sensitive Electrode

Thrombosis and Haemostasis ◽

10.1055/s-0038-1665256 ◽

1983 ◽

Vol 50 (02) ◽

pp. 563-566 ◽

Cited By ~ 3

Author(s):

P Hellstern ◽

K Schilz ◽

G von Blohn ◽

E Wenzel

Keyword(s):

Factor Xiii ◽

Normal Subjects ◽

The Other ◽

Activity Measurement ◽

Factor Xiii Deficiency ◽

Assay Procedure ◽

Stabilization Factor ◽

Release Method ◽

Sensitive Electrode

SummaryAn assay for rapid factor XIII activity measurement has been developed based on the determination of the ammonium released during fibrin stabilization. Factor XIII was activated by thrombin and calcium. Ammonium was measured by an ammonium-sensitive electrode. It was demonstrated that the assay procedure yields accurate and precise results and that factor XIII-catalyzed fibrin stabilization can be measured kinetically. The amount of ammonium released during the first 90 min of fibrin stabilization was found to be 7.8 ± 0.5 moles per mole fibrinogen, which is in agreement with the findings of other authors. In 15 normal subjects and in 15 patients suffering from diseases with suspected factor XIII deficiency there was a satisfactory correlation between the results obtained by the “ammonium-release-method”, Bohn’s method, and the immunological assay (r1 = 0.65; r2= 0.70; p<0.01). In 3 of 5 patients with paraproteinemias the values of factor XIII activity determined by the ammonium-release method were markedly lower than those estimated by the other methods. It could be shown that inhibitor mechanisms were responsible for these discrepancies.

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Defective Fibrin Crosslinking in Acute Leukemia

Thrombosis and Haemostasis ◽

10.1055/s-0038-1661210 ◽

1984 ◽

Vol 52 (03) ◽

pp. 343-346 ◽

Cited By ~ 1

Author(s):

F Rodeghiero ◽

T Barbui ◽

A Dal Belin-Peruffo ◽

E Dini

Keyword(s):

Acute Leukemia ◽

Factor Xiii ◽

Factor Xiii Deficiency ◽

Coagulation Abnormalities ◽

Normal Plasma ◽

Transamidating Activity

SummaryFibrin crosslinking was assayed in 22 patients with acute leukemia showing secondary coagulation abnormalities of variable severity. In 9 patients fibrin crosslinking was found to be normal, whereas 10 patients presented impaired polymerization of α-chains and 3 of both α- and γ-chains.Only a rough correlation was found between transamidating activity of factor XIII and the fibrin crosslinking pattern in these patients. Moreover, incomplete fibrin crosslinkage occurred at levels of factor XIII far in excess of that required for full polymerization of fibrin in “normal” plasma. This latter finding suggests that, in addition to factor XIII deficiency, other causes are responsible for thehigh rate of fibrin crosslinking impairment in acute leukemia.

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