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Results for “Factor X Deficiency”

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At least 199 records · Page 11Linked to original sources
PubMed2007

Haemoperitoneum associated with ovulation in women with bleeding disorders: the case for conservative management and the role of the contraceptive pill.

Haemoperitoneum secondary to ruptured corpus luteum is a rare complication for women on anticoagulants and with certain congenital bleeding disorders. A surgical approach is often taken, leading to oophorectomy in many cases. We describe three patients presenting with haemoperitoneum in association with factor VII deficiency, factor X deficiency and sitosterolaemia. In two of the patients, recurrent episodes occurred prior to introduction of the oral contraceptive pill. Conservative management with blood product and factor concentrate support was successful in avoiding surgery in three of the five episodes of bleeding. These cases demonstrate that preservation of ovarian function is possible with a conservative approach and recurrent episodes may be prevented by suppression of ovulation.

Adult↗
PubMed1997

Normalization of plasma factor X levels in amyloidosis after plasma exchange.

Some patients with systemic light chain amyloidosis develop bleeding complications that can be caused by vascular infiltration with amyloid or by alterations of the coagulation or fibrinolytic systems. Factor X deficiency is the most common cause of bleeding manifestations, although deficiencies of other clotting factors, a disruption in the conversion of fibrinogen to fibrin, and circulating heparin-like anticoagulants have also been reported. Deficiency of factor X is a well-recognized cause of bleeding manifestations in patients with light chain amyloidosis. This acquired disorder appears to be secondary to adsorption of factor X to the amyloid fibrils. Previous studies have shown that infusion of plasma into patients with acquired factor X deficiency and amyloidosis induces a transitory improvement of the coagulation tests. However, there is a rapid return to pretransfusion levels. In this manuscript we report the clinical application of plasma exchange in the management of a patient with systemic light chain amyloidosis with acquired factor X deficiency.

Amyloidosis↗
PubMed1996

Rare inherited coagulation disorders in India.

Twenty four cases with rare coagulation disorders were diagnosed over a 4 year period. These included 8 patients with factor X deficiency, 7 with factor XIII deficiency, 4 each with fibrinogen and factor VII deficiency and 1 with factor V deficiency. All these patients had presented with bleeding manifestations. Two patients with factor X deficiency showed interesting clinical presentations, one patient had recurrent deep vein thrombosis and another patient had a pseudotumor of the thigh.

Adolescent↗
PubMed2003

In utero transplantation of wild-type fetal liver cells rescues factor X-deficient mice from fatal neonatal bleeding diatheses.

Factor X (FX)-deficient embryos suffer partial embryonic lethality with approximately 30% of the embryos arresting at midgestation. The remaining animals survive to term but die perinatally mainly from abdominal or intracranial hemorrhage. We have rescued FX-deficient mice by transplanting fetal liver cells from FX+/+, Rosa26 fetuses into midgestation embryos derived from FX+/- heterozygous crosses. FX-/- embryos were born at the expected frequency and approximately 50% of the FX-/- neonates survived longer than 4 months. FX-/- embryos receiving saline injections that survived to term died perinatally similar to untreated FX-deficient mice. The plasma levels of FX in the rescued 16-week-old FX-/- mice were approximately 1-6% of wild-type levels. beta-Galactosidase-staining cells derived from the donor Rosa26 fetal liver cells were detected in 47% of the livers of adult mice. In addition, donor-derived cells were also recovered in the bone marrow, spleen, lung, and occasionally in the brain and testis. These results suggest that in utero cell transplantation could be an effective therapeutic strategy to treat pathologies resulting from the deficiency of hepatic-expressed factors.

Animals↗
PubMed1982

Tissue factor-like activity of the human monocytic tumor cell line U937.

Undisrupted cells of the human monocytic tumor cell line U937 have procoagulant activity that is Ca2+ dependent and is not demonstrable in Factor VII or Factor X deficient plasma. Furthermore, U937 cells when incubated with purified human Factor VII in the presence of Ca2+ and then repeatedly washed promoted coagulation of Factor VII deficient plasma in the absence of added tissue factor. Culture with endotoxin increased the procoagulant activity of U937 cells approximately 5-fold. In separate experiments, exposure to lymphokines obtained from phytohemagglutinin-stimulated lymphocytes enhanced the procoagulant activity of U937 cells 4 to 110-fold. Other cell lines (of myeloid and lymphoid origin) tested lacked the procoagulant activity found in U937 cells. These results indicate that the constitutive tissue factor-like activity of U937 cells resembles that of normal activated human monocytes.

Binding Sites↗
PubMed1992

Prevention of recurrent intracranial hemorrhage in a factor X-deficient infant.

We describe a case of a child with severe Factor X deficiency who had three episodes of intracranial hemorrhage during the first 6 months of life. Since that time he has been successfully managed with prophylactic therapy using prothrombin complex infusions via an indwelling central venous line. This prophylactic therapy may prevent the poor outcome usually described in these patients.

Blood Coagulation Factors↗
PubMed1994

Congenital coagulopathies and pregnancy: report of four pregnancies in a factor X-deficient woman.

Pregnancy in women with congenital coagulation factor deficiencies has been associated with adverse fetal outcomes. Recurrent spontaneous abortions, placental abruptions, and premature births are reported, the exact reasons for which are not clear, and management of such patients continues to be a challenge. We reviewed the outcome of four pregnancies in a patient with factor X deficiency, and looked at the effect of factor replacement therapy on pregnancy. Her first two pregnancies resulted in the birth of extremely premature babies at 21 and 25 weeks of gestation, both of which died in the neonatal period. The patient was treated with fresh frozen plasma for acute bleeding episodes during these pregnancies. In addition during her second conception she was given factor IX complex [Konyne] prophylactically, but only in the latter half of her pregnancy. During her next two pregnancies she was treated early on during pregnancy, with prophylactic replacement of factor X. She delivered healthy babies at 34 and 32 weeks of gestation, and they are both doing well. We therefore suggest possible mechanisms by which aggressive prophylactic factor support in a female with severe congenital coagulopathy, may improve on fetal outcome.

Adult↗
PubMed2004

Rare Bleeding Disorder Registry: deficiencies of factors II, V, VII, X, XIII, fibrinogen and dysfibrinogenemias.

A North American registry for rare bleeding disorders [factor (F)II, factor (F)VII, factor (F)X, factor (F)V, factor (F)XIII, fibrinogen deficiencies and dysfibrinogenemias] was established to gather information about disease prevalence, genotyping frequency, diagnostic events, clinical manifestations, treatment and prophylaxis strategies, as well as disease- and treatment-related complications. Questionnaires were sent to 225 hemophilia treatment centers in the USA and Canada. Among 26% of responding centers, 294 individuals [4.4% of the registered children (200/4583) and 2.4% of adults (94/3809)] were diagnosed with one or more of the rare bleeding disorders (RBDs) included in this survey. The ethnic distribution for each disorder paralleled that of the general US population with the exception of the disproportionately large number of Latinos with FII deficiency. Only 5.4% of affected individuals were genotyped. An abnormal preoperative bleeding screen most often led to diagnosis. The most common coagulopathy was FVII deficiency; however, 40% of homozygous patients were asymptomatic. FX and FXIII deficiencies caused the most severe bleeding manifestations. Among all RBDs, the most common sites of bleeding were skin and mucus membranes. Multiple products were used to treat hemorrhage; however, half of the bleeding episodes required no therapy. The majority of patients suffered no long-term complications from hemorrhage. Treatment-related complications included viral seroconversion, anemia, allergic reactions and venous access device-related events. This registry provides the most comprehensive information to date about North American individuals with RBDs and could serve as an important resource for both basic scientist and clinician.

Adolescent↗
PubMed1975

Factor X Friuli: An immunological study in plasma and in serum using several methods.

An immunological study of factor X Friuli was carried out using an anti-human-factor X antiserum. The results obtained with different methods were compared. Only an ill-defined factor X band or precipitate was evident on standard immuno-electrophoresis and on immunodiffusion. Using a non-absorbed antiserum a precipitate was evident on eletroimmunoassay, in normal, factor X Friuli and in factor X deficient plasma. On the contary, using an antiserum which had been previously absorbed with factor X deficient plasma, a precipitate was evident only with factor X Friuli plasma or normal plasma. Excellent results were obtained with the cross-over electrophoresis. Using this method a major or factor X band was evident in normal plasma, factor II,P FACTOR VII, factor IX deficient plasmas and in the factor X FRIULI PLASMA. No factor X band was evident in factor X deficient plasmas and in absorbed normal plasma. Lighter, more cathodic bands were seen in all plasmas. These were due to secondary activities of the antiserum and could be disregarded. Friuli serum factor X and normal serum factor X showed a slightly more anodic migration as compared to their plasma counterparts. In coumarin plasma two factor X bands were evident; one normal in position and one slightly more catholidic. In the bidimensional immunoelectrophoresis two precipitates are visible in normal plasma and in factor X Friuli plasma. In factor X deficient plasma only one precipitate is evident. This latter precipitate is due to the secondary activity of the antiserum used. On the basis of these data it is concluded that factor X Friuli behaves, immunologically, as normal factor X.

Blood Coagulation Disorders↗
PubMed1989

Molecular characterization of human factor XSan Antonio.

Enzymatic amplification technique was used to isolate all eight exons and sequences around the splice junctions, putative promoter, and polyadenylation sites of human factor X DNA from a patient with factor X deficiency. Two genetic changes in factor X have been observed in this patient. The patient is most likely a compound heterozygote since there is only 14% activity associated with factor X. A point mutation that resulted in the substitution of cysteine (TGC) for arginine (CGC) at amino acid 366 was found in exon VIII of one allele of the factor X gene. This mutation, which occurs in the catalytic domain, can affect the formation of a disulfide bridge and thus could result in a reduction in factor X activity. Sequencing all the regions revealed a second mutation: a deletion of one nucleotide (TCCT to TCT) in exon VII that would cause a frame shift at amino acid 272 followed by termination. We have also shown that the point mutation in exon VIII creates an ApaL1 restriction site and destroys the HinP1 site. Enzymatic DNA amplification followed by restriction digestion provides a quick, reliable, and sensitive method for carrier detection and antenatal diagnosis in affected kindreds. This is the first characterization of factor X deficiency at the molecular level. We propose to name this mutation Factor XSan Antonio.

Alleles↗
PubMed2002

The influence of acetaldehyde and glycosaminoglycans upon factor Xa- and factor X-deficient plasma.

The comparative effects of glycosaminoglycans and acetaldehyde (AcH)--glycosaminoglycan (GAG) mixtures upon Factor Xa- (FXa) and Factor X-deficient plasma (FXDP) have been studied by activated partial thromboplastin time (APTT) studies. Heparin at 0.025, 0.030, 0.04, and 0.05 U statistically prolonged the APTT when pre-incubated with FXa at 37 degrees C for 3 min prior to addition to FXDP and subsequent addition of Ca2+. Upon addition of 0.25, 0.375, and 0.5 microg heparin-6000 (H6k) to FXa, significant increases in APTT were observed. Similarly, profound increases in APTT were observed when 0.5, 0.75, and 1.0 microg heparin-3000 (H3k) was added to FXa. The chondroitin sulfates (CSA, CSB, CSC) had far less impact upon APTT with the FXa-FXDP system. In examining the effects of AcH-GAG mixtures upon the clotting factor, it was observed that 44.3 and 443 mM AcH synergistically prolonged the APTT in a statistically significant manner regardless of the order of premixing the three components. Hence, AcH may play a role in prolonging APTT in alcoholics. It synergistically prolonged APTT in concert with GAGs and FXa at the AcH levels used in this study. The effect of the GAGs upon FXDP is far less than its effect upon FXa.

Acetaldehyde↗