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At least 1,351 records · Page 75Linked to original sources
PubMed1993

A new type of congenital dysfibrinogen, fibrinogen Bremen, with an A alpha Gly-17 to Val substitution associated with hemorrhagic diathesis and delayed wound healing.

We have identified a new type of A alpha Gly-17 to Val substitution in a congenital dysfibrinogen, fibrinogen Bremen, derived from a 15-year-old boy having manifested easy bruising and delayed wound healing. The functional abnormality was characterized by altered fibrin monomer polymerization, which became evident by increasing the salt concentration and pH. A synthetic tetrapeptide with a sequence of the amino-terminal segment of normal fibrin alpha-chain, Gly-Pro-Arg-Val, substantially inhibited polymerization of both normal and the patient-derived fibrin monomers. A synthetic tetrapeptide with the Bremen type sequence of Val-Pro-Arg-Val inhibited polymerization of the patient's fibrin monomers partially at a peptide: fibrin monomer molar ratio of 4,000:1, and that of normal one at a much higher ratio of 10,000:1. Likewise, a synthetic peptide Ala-Pro-Arg-Val with a replacement of the Gly residue by another aliphatic amino acid Ala inhibited similarly the patient's fibrin monomer polymerization. Thus, the hypothetical two-pronged socket-like structure consisting of the alpha-amino group of the amino-terminal Gly and the guanidino group of an Arg at position 3 of the normal fibrin alpha-chain seems to be restored considerably in the mutant fibrin alpha-chain at low ionic strengths and pH's, despite the replacement of the amino-terminal Gly by another aliphatic amino acid Val.

Adolescent↗
PubMed1995

[Desmopressin in the treatment of hemorrhagic diathesis].

Over the past several years, the use of 1-deamino-8-D-arginine vasopressin (DDAVP), a synthetic analogue of vasopressin, has been found to be useful in the treatment of patients with abnormal bleeding tendency. This article is a review of inherited and acquired disorders with prolonged bleeding time in which DDAVP is supposed to shorten the bleeding time. DDAVP is established as effective therapy of the abnormal haemostasis in mild or moderate haemophilia A and von Willebrand's disease. Frequently, DDAVP infusions are used to control bleeding in patients with uraemia. Bleeding time is also significantly shortened in patients with liver cirrhosis, although randomized trials of DDAVP therapy of gastrointestinal bleeding in this group of patients are still needed. Shortening or normalization of the bleeding time with DDAVP has also been observed in patients with inherited platelet dysfunctions, acquired disorders of haemostasis and abnormal haemostasis in chronic myeloproliferative diseases. In addition, preoperative treatment with DDAVP seems to reduce blood loss during surgery.

Deamino Arginine Vasopressin↗
PubMed1995

Fibrin sealant in surgery of patients with a hemorrhagic diathesis.

The utilization of fibrin sealants in patients with hemostatic disorders has hitherto been very limited. Our experiences from 40 major and 8 minor surgical procedures, 10 circumcisions and 118 tooth extractions in 106 patients with hemophilia A, B or von Willebrand's disease, almost exclusively of the severe form, respectively, demonstrate the benefits regarding reduction of blood loss and requirements for systemic replacement therapy. The literature on the use of fibrin sealants in this group of patients, which mainly concerns dental procedures, is reviewed and compared with our data. The importance of the composition of the glue is also discussed in detail.

Blood Loss, Surgical↗
PubMed1995

[Hysteroscopic endometrium ablation in "high-risk" situations and in hemorrhagic diathesis].

A hysteroscopic endometrial ablation (HEA) under maximal anesthesiologic surveillance was performed on 34 high-risk patients (group I: chronic anticoagulant therapy n = 26; group II: endogenous coagulopathy n = 8) with therapy resistant meno-metrorrhagia to avoid a hysterectomy (HE). Total amenorrhea, or a least hypomenorrhea respectively cyclic spotting could be attained primarily in 22 patients (group I: 19; group II: 3), after a repeat procedure in further 6 patients (4 in group I, 2 in group II). Subjective evaluation of surgical results (overall 22 patients primarily satisfied, 6 secondarily) also differed between the two subgroups (group I: p < 0.01 primarily satisfied; p < 0.05 secondarily satisfied vs. p < 0.05 and p < 0.01 in group II). A HE had to be performed on two patients due to extensive adenomyosis uteri interna (group II). The significantly better results in the anticoagulation group were probably due to the basic illness. Larger groups will, however, be necessary before any conclusions from this difference can be drawn. No surgical or anesthesiological complications occurred. There also were no major postoperative complications (1 endomyometritis, 2 cervical stenoses). Endometrial ablation was found to be a valuable treatment alternative for this specific group of patients with severe coagulopathy, thrombo-embolic or thrombotic disease.

Adult↗
PubMed1996

[Factor XI deficiency: do patients with hemorrhagic diathesis also have hemostasis defects?].

It is well known that the extent of bleeding in patients with a deficiency of factor XI does not parallel the residual factor activity. Therefore, additional hemostatic aspects must be taken into consideration. 27 patients of 18 different families--6 with severe (FXI < 0.01 to 0.09 U/ml) and 21 with moderate (FXI 0.20 to 0.252 U/ml) factor XI deficiency--were reinvestigated regarding hemostasis including bleeding time and platelet function. 16 had an enhanced bleeding tendency, while the others--including 3 with severe FXI deficiency of < 0.01 U/ml--never suffered from bleeding complications despite delicate surgery in some. All 16 of the symptomatic patients had, besides the reduction of factor XI activity to various extents, an additional hemostatic defect: 3 had a moderate alpha/delta storage pool detect of the platelets, 11 a platelet anomaly mainly characterized by reduced ADP aggregation and 2 an isolated prolonged bleeding time of unknown cause. Synthesis of platelet thromboxane was unimpaired in all patients as tested by formation of malondialdehyde after stimulation with N-ethyl maleimide. Hence, in patients with a known factor XI deficiency diagnostic and prophylactic measures before surgery should concentrate on such additional findings. Preoperative administration of desmopressin, and not replacement of factor XI, will be the treatment of choice in these cases.

Adult↗
PubMed1996

Factor X Stockton: a mild bleeding diathesis associated with an active site mutation in factor X.

A unique blood coagulation factor X variant has been identified in a family with a history of bleeding. Plasma from affected family members had prolonged prothrombin times and activated partial thromboplastin times, low to below normal factor X coagulant activity, and normal factor X antigen levels. Sequencing of DNA from the propositus revealed a single G to A substitution in one allele of factor X at base 964 resulting in an amino acid substitution of Asn for Asp at residue 282. This residue corresponds with the active site Asp102 of chymotrypsin. The substitution eliminates a TaqI restriction site and provided the basis for a screening assay to detect the mutation in polymerase chain reaction (PCR) amplified factor X exon VIII DNA. Fourteen additional family members were identified as having the mutation at base 964. Plasma factor X purified from the proposita using an anti-factor X monoclonal antibody immunoadsorbent exhibited an approximately 50% decrease in specific activity compared with factor X purified from a normal individual in a similar manner. Bleeding in family members with the mutation, termed factor X Stockton, appears to be due to disruption of normal hemostasis by the presence in plasma of circulating abnormal factor X. Factor X Stockton is the first naturally occurring substitution at the active site Asp of a serine protease and underscores the importance of this amino acid residue in factor Xa coagulant activity.

Amino Acid Sequence↗
PubMed1996

[Hemorrhagic diathesis as initial clinical manifestation of celiac disease].

Coeliac disease is the most frequent malabsortive syndrome in daily practice. However, its true prevalence is not exactly known. The symptoms vary from patient to patient, ranging from the classic presentation with diarrhea, weight loss and fatigue to oligosymptomatic forms, being the latter more common in adults. We report two patients in which urinary and skin spontaneous hemorrhage were the only symptoms, and led to the diagnosis. We insist on the importance of considering the diagnosis of coeliac disease in patients with this kind of clinical manifestations. The early diagnosis will allow us to reduce both the morbidity and mortality in coeliac patients.

Adult↗