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Results for “Factor XIII Deficiency”

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At least 415 records · Page 23Linked to original sources

PubMed1996

Characterization of an acquired IgG inhibitor of coagulation factor XIII in a patient with systemic lupus erythematosus.

An acquired IgG inhibitor to factor XIII was identified in a 30-year-old Vietnamese woman with systemic lupus erythematosus. The IgG fraction was isolated from plasma by chromatography on an agarose gel column and by protein G adsorption. The anti-factor XIII activity, identified within the IgG fraction, inhibited factor XIII from both normal plasma (a2b2) and platelets (a2). A normal level of the b subunit was measured by immunoelectrophoresis of the patient's plasma, but the a subunit was not detected. These results suggested the presence of an IgG immunoglobulin which recognized the a subunit and interfered with its activity.

Adult↗

PubMed2002

Mutations in coagulation factor XIII A gene in three Turkish patients: two novel mutations and a known insertion.

Molecular analysis of factor XIII A gene on three unrelated Turkish families identified two novel and one known mutations. One novel mutation is a substitution of cytidine by guanine at codon 541 in exon 12, beta barrel 1 domain of the coagulation factor XIII A subunit gene resulting in the conversion of asparagine to lysine. The mutation alters the restriction site of the enzyme MboII. The second novel mutation, a 4 bp (-CAAA) deletion located in a direct repetitive sequence (CAAACAAA) between codons 466-469, results in premature termination of translation at codon 474. The third mutation is a previously reported single nucleotide (cytidine) insertion at codon 400 in exon 9 of the factor XIII gene.

Child↗

PubMed1992

Absence of human immunodeficiency virus (HIV) proviral sequences in seronegative hemophilic men and sexual partners of HIV-seropositive hemophiliacs.

To detect latent infection with human immunodeficiency virus (HIV), specimens of peripheral blood leukocytes from HIV-seronegative hemophiliacs and from sexual partners of HIV-seropositive hemophiliacs were examined by polymerase chain reaction (PCR). The primer pair SK 38/39 derived from the gag region and/or the primer pair SK 68/69 corresponding to a conserved region of the env gene were used. Whereas HIV proviral DNA was detected by PCR in samples from 86 (97%) of 89 HIV-seropositive hemophiliacs, no HIV-DNA was found in blood samples of 198 HIV-seronegative hemophiliacs at risk. Of 40 HIV-seronegative sexual partners of HIV-infected hemophiliacs, none was PCR positive. Thus, PCR is proving to be a sensitive method by which to confirm infection in seropositive hemophiliacs, while the negative results in HIV-seronegative hemophiliacs and HIV-seronegative sexual partners of HIV-seropositive hemophiliacs suggest that a prolonged seronegative period of latent HIV infection is the exception.

Algorithms↗

PubMed1978

Alterations of hemostasis associated with malignancy: etiology, pathophysiology, diagnosis and management.

As outlined in this paper, the patient with disseminated malignancy suffers many alterations of hemostasis; in addition, hemorrhage or less commonly thrombosis is the final clinical event in many of these patients. Patients with malignancy present a major clinical challenge in this day of new oncological awareness and more aggressive care. Thus, it is important to realize that these alterations of hemostasis do exist and they must be approached in a logical manner with respect to diagnosis as well as efficacious therapy. By far the most common alteration of hemostasis in malignancy is that of hemorrhage associated with thrombocytopenia either drug-induced or from bone marrow invasion. However, hemorrhage due to disseminated intravascular coagulation is also quite common. In addition, many antineoplastic drugs, as well as radiotherapy, may lead to hemorrhage in these patients. Thrombosis, which is also commonly seen in the patient with malignancy, is usually a manifestation of disseminated intravascular coagulation manifest as an intravascular thrombotic rather than an intravascular proteolytic event. When suspecting this, confirmatory laboratory evidence must be sought and the patient treated apropriately. When approaching the patient with malignancy and either hemorrhage or thrombosis, all of the potential defects in hemostasis must be taken into account, defined from the laboratory standpoint, and treated in as precise a manner as possible.

Blood Coagulation Disorders↗

PubMedDate not supplied

Decreased factor XIII activity during severe Henoch-Schoenlein purpura -- does it play a role?

BACKGROUND: In patients with Henoch-Schoenlein purpura (HSP), particularly with severe gastrointestinal symptoms, an associated decrease of plasma factor XIII has been observed. PATIENT: The authors report a case of HSP in a boy and describe the development of factor XIII activities throughout the course of the disease. Every relapse of severe gastrointestinal manifestation was associated with a decrease of factor XIII. No improvement was seen after treatment with prednisone. The symptoms resolved each time factor XIII concentrate was administered. With the return of factor XIII to normal values eight weeks after admission abdominal symptoms ceased. CONCLUSION: The documented course supports the hypothesis that factor XIII activity correlates well with the severity of abdominal symptoms. Measuring factor XIII activity helps to identify those patients with severe gastrointestinal manifestation who may benefit from substitution therapy.

Child↗

PubMed1991