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PubMed1980

Disturbances of desmofibrinogenesis in pateints suffering from acute myeloblastic leukemia.

Significantly decreased levels of blood plasma clotting factor XIII (FSF) were found in the blood of 20 patients with acute myeloblastic leukemia as compared to the control values. It was found that after administration of cytostatic drugs (Cerubidyne and Cytosar) FSF deficiency was higher. This effect was associated with a proteolytic activity detectable in plasma which destroys FSF in vitro. This proteolytic activity was neither inhibited by EACA nor by Trasylol. These results indicate that in patients with acute myeloblastic leukemia beside DIC the treatment with cytostatic drugs as well as the presence of proteases from leukemic cells in the plasma will cause an impairment of transformation of soluble fibrin polymer into insoluble desmofibrin.

Adolescent↗
PubMedDate not supplied

A new autologous fibrinogen-based adhesive for otologic surgery.

Many middle ear reconstructive procedures would be facilitated by use of a relatively safe surgical adhesive. A fibrinogen-based adhesive (Tisseel) has been effective in otologic surgery in Europe, but because it is derived from pooled human blood, it carries a risk of transmitting hepatitis, acquired immune deficiency syndrome, and other illnesses. This report details a new procedure for creating an autologous fibrinogen-based adhesive, obviating these risks. The fibrinogen and factor XIII component of the adhesive was isolated by polyethylene glycol precipitation from human plasma within a few hours, and was used either immediately or frozen for use up to 3 weeks later. Fifteen chinchillas had either the single donor adhesive, the commercial European adhesive, or saline placed on the oval and round windows, and no evidence of cochlear, mucosal, or ossicular damage was seen by light microscopy 30 days later. Auditory brain stem response thresholds remained stable, except in three animals that developed otitis media. Based on this investigation, autologous fibrinogen-based adhesive appears promising as a relatively safe, biological bonding material for otologic surgery, and is worthy of further study.

Animals↗
PubMed1997

Hemostatic treatment after tooth extraction in a patient with factor V deficiency.

BACKGROUND: Factor V deficiency is a very rare hereditary coagulation disorder and tooth extraction in the patient with factor V deficiency has not been reported except in one case. PATIENTS AND TREATMENT: A 38-year-old woman with factor V deficiency was referred for extraction of the impacted lower third molar. After intravenous administration of frozen fresh plasma (FFP) and recognition of an increase of factor V level from 1-31%, upper and lower third molars were extracted. Eighteen and 48 h after the extraction, factor V was intermittently supplemented by injection of 4 and 2 units of FFP, respectively, and factor V was maintained above 12%. To form fast coagula and to protect the wound, the lower extraction socket was filled with a fibrin glue composed of factor XIII and fibrinogen (Beriplast P) and a plastic splint was applied. The wound was healed and epithelized within 2 weeks after the extraction without any bleeding or infectious consequences. CONCLUSION: Extraction in the patient with factor V hereditary deficiency is safely performed by both supplementation of factor V and application of local hemostasis.

Adult↗
PubMed2001

A patient defines the interstitial 1q deletion syndrome characterized by antithrombin III deficiency.

A patient with microbrachycephaly, high forehead, long philtrum, thin upper lip, downturned corners of the mouth, low set ears with overlapping helix, fifth-finger clinodactyly, small hands and feet, bilateral transverse palmar crease, low total finger ridge count, hypotonia, severe growth and psychomotor delay, mild hypoplasia of corpus callosum, and Arnold-Chiari type 1 malformation is reported. The karyotype showed 46, XY, del(1)(q23q31.2). Coagulation factor V (F5, 1q23) and coagulation factor XIII (F13B, 1q31-q32.1) levels were normal. As expected, antithrombin III (AT3, 1q23-q25.1) serum level and activity were half of normal. We performed a review of the literature on proximal and intermediate deletion 1q syndrome, and we hypothesize the existence of only one 1q interstitial deletion syndrome, clinically characterized by ATIII deficiency.

Adolescent↗