pituitrin and von-Willebrand-Diseases

Primary blood coagulation and wound sealing are orchestrated by von Willebrand factor (VWF), a large multimeric glycoprotein. Upon release of arginine vasopressin (AVP), VWF containing high molecular weight multimers is secreted. By measuring copeptin, the C-terminal part of the AVP prohormone, we recently found strongly increased AVP levels in children with febrile seizures (FS) as compared to children with fever but without seizures. It is unknown if increased AVP levels in FS are of any biological function. Therefore, our a priori hypothesis was that children with FS have increased VWF parameters in parallel with higher AVP levels.. We conducted a prospective, cross-sectional study of children aged between 6 months and 5 years. Children that presented at our emergency department with fever or a recent FS (within four hours) were evaluated to be included to the study. We measured serum copeptin and VWF parameters, including analyses of VWF:Antigen (WVF:Ag), VWF:collagen binding activity (VWF:CB) and VWF multimers in children with FS, febrile infections without seizures and additionally, in a non-febrile control group.. We included 54 children in our study, 30 with FS, 10 in the febrile control group, and 14 in the non-febrile control group. Serum copeptin levels were significantly higher in children with FS (median [IQR] 24.73 pmol/l [13.65-68.65]) compared to the febrile control group (5.66 pmol/l [4.15-8.07], p = 0.002) and the non-febrile control group (4.78 pmol/l [3.33-5.3], p<0.001). VWF:CB levels were also significantly higher in children with FS (VWF:CB 2.29 U/ml [1.88-2.97]) as compared to the febrile (VWF:CB 1.41 U/ml [1.27-1.93], p = 0.048) and the non-febrile control group (VWF:CB 1.15 U/ml [0.98-1.21], p<0.001). VWF:Ag tended to be higher in children with FS compared to both control groups. Multivariate regression analysis revealed FS and copeptin as major determinants of VWF:CB.. Our results suggest that increased secretion of AVP in children with FS is associated with higher plasma levels of VWF parameters. Especially VWF:CB may serve as additional biomarker in the diagnosis of FS.

Topics: Biomarkers; Child, Preschool; Cross-Sectional Studies; Female; Fever; Glycopeptides; Humans; Infant; Male; Neurophysins; Prospective Studies; Protein Precursors; Seizures, Febrile; Vasopressins; von Willebrand Diseases; von Willebrand Factor

Topics: Adult; Cerebral Arterial Diseases; Contraceptives, Oral, Combined; Factor VIII; Female; Humans; Intracranial Thrombosis; Risk Factors; Smoking; Vasopressins; von Willebrand Diseases; von Willebrand Factor

Topics: Blood Coagulation Factors; Cells, Cultured; Deamino Arginine Vasopressin; Dexamethasone; Endothelium; Epinephrine; Humans; Hydrocortisone; Ristocetin; Umbilical Veins; Vasopressins; von Willebrand Diseases; von Willebrand Factor

1-Deamino-8-d-arginine vasopressin (D.D.A.V.P.) infusion causes a marked increase in factor-VIII (antihaemophilic-factor)-related properties in patients with moderate and mild haemophilia and von Willebrand's disease (vWd). The possibility was therefore evaluated that such an autologous factor-VII response might be haemostatically effective, allowing patients to undergo surgery without plasma concentrates. 0.3 microng/kg of D.D.A.V.P. given before dental surgery and repeated in the early postoperative period was followed by a two to three fold rise in factor-VIII coagulant activity (VII C.A.) in four patients with moderate and mild haemophilia. In two, there was no abnormal bleeding after dental extraction, whereas plasma concentrates were necessary to control oozing from the sockets in the remaining two patients. A higher D.D.A.V.P. dosage (0.4-0.5 microng/kg) in patients with higherstarting VII C.A. (9% or more) was followed by a more marked response (four to six fold). VII C.A. levels up to 100% of average normal were achieved and dental extraction and major surgery (such as cholecystectomy, thoracotomy, and two tonsillectomies) were carried out successfullly in six patients with mild haemophilis and in two with vWd. The mean half-life of autologous VII C.A. was 9.4 h (range 7.5-11.6). Plasma and urine osmolality showed no consistent variation after drug administration. Thus D.D.A.V.P. appears a promision pharmacological alternative to plasma concentrates in the management of some patients with haemophilis and vWd.

Topics: Adolescent; Adult; Biopsy; Blood Coagulation Tests; Cholecystectomy; Deamino Arginine Vasopressin; Drug Evaluation; Factor VIII; Female; Hemophilia A; Hemorrhage; Hemostasis, Surgical; Humans; Infusions, Parenteral; Male; Middle Aged; Postoperative Care; Postoperative Complications; Preoperative Care; Thoracic Surgery; Thorax; Tonsillectomy; Tooth Extraction; Vasopressins; von Willebrand Diseases

Topics: Blood Coagulation; Deamino Arginine Vasopressin; Hemophilia A; Hemorrhage; Humans; Vasopressins; von Willebrand Diseases

Topics: Arginine Vasopressin; Drug Evaluation; Female; Hemophilia A; Hemorrhage; Hemostasis, Surgical; Hemostatics; Humans; Postoperative Care; Postoperative Complications; Preoperative Care; Vasopressins; von Willebrand Diseases

1-Deamino-8-D-arginine vasopressin (DDAVP), a vasopressin analogue, increases factor VIII levels in plasma. A female carrier of haemophilia A with known bleeding diathesis and markedly reduced factor VIII activity was successfully treated with DDAVP during bilateral Caldwell-Luc's operation for chronic maxillary sinusitis. An estimated 5000 U factor VIII concentrate was calculated to have been saved. DDAVP is the first alternative to replacement therapy in the treatment of moderate and mold forms of haemophilia A and von Willebrand's disease.

Topics: Arginine Vasopressin; Factor VIII; Female; Hemophilia A; Humans; Maxillary Sinus; Middle Aged; Sinusitis; Vasopressins; von Willebrand Diseases

Topics: Blood Coagulation; Factor VIII; Female; Humans; Male; Platelet Adhesiveness; Ristocetin; Vasopressins; von Willebrand Diseases

Topics: Arginine; Blood Coagulation Tests; Blood Platelets; Factor VIII; Hemophilia A; Humans; Ristocetin; Vasopressins; von Willebrand Diseases