摘要:

&NA;We report a family with a combined factor VII Padua defect and von Willebrand's disease (vWd). The propositus is a 9‐year‐old child with a moderate bleeding tendency who appeared to be heterozygous for both factor VII Padua and type I vWd. The diagnosis of factor VII Padua was based on a normal factor VII antigen and factor VII activity which was low with rabbit brain thromboplastin but normal with ox brain thromboplastin. Type I vWd was diagnosed because of a concomitant decrease of von Willebrand factor antigen (vWf:Ag) and vWf ristocetin‐cofactor activity (vWf:RCoF), associated with the presence of vWf multimers of all sizes in plasma and platelets. The parents were not consanguineous but came from the same isolated river Piave valley in North Eastern Italy where the factor VII Padua defect was first described. The father had the factor VII Padua defect but was clinically asymptomatic in accordance with the heterozygous state. The propositus's mother had type I vWd and was mildly symptomatic. The propositus' sisters, who were clinically asymptomatic, were both heterozygotes for factor VII Padua. The infusion of DDAVP normalized the factor VIII/vWf pattern in all patients. In the propositus, in contrast to the mother and normal subjects, showed a more rapid clearance both of vWf and factor VIII. The same pattern, albeit to a lesser degree, was also observed in the father.

ISSN:0957-5235    

出版商:OVID    

年代:1993

数据来源: OVID

摘要:

&NA;We have investigated the effects of low dose nitroglycerine on the activated partial thromboplastin time (APTT), plasma heparin concentration, antithrombin III activity (AT‐III) and platelet factor 4 (PF4) levels in a group of 42 patients receiving intravenous heparin and low dose nitroglycerine (GTN) following percutaneous transluminal coronary angioplasty (PTCA). Venous samples were taken before PTCA and at 2, 4 and 24 h after the start of the infusions. Despite the heparin infusion being constant, the median APTT ratio (interquartile range) was significantly lower at the 4 h sample time compared to the 2 h sample time (4.4 [3.8‐4.5] vs 2.6 [1.8‐4.0],P< 0.05). At this time there was also a significantly lower median plasma heparin concentration compared to the 2 h sample (0.35 [0.2‐0.7]vs0.17 [0.1‐0.3]P< 0.05). There were no significant differences in AT‐III activity or PF4 levels at 4 h compared to the 2 h sampling time. In another group of patients (n= 20) who received intravenous heparin alone following PTCA also at 1000 U/h there were no significant differences in median APTT ratios (4.4 [4.3‐4.5]vs4.2 [2.9‐4.5]), or in median plasma heparin concentrations (0.26 [0.14‐0.96]vs0.22 [0.18‐0.87]) at 4 h compared to 2 h. Our observations confirm that nitroglycerine can interfere with the anticoagulant effect of heparin even at low doses. Although the exact mechanism involved remains unknown, this study suggests it is likely to be as a result of a reduction in plasma heparin levels, perhaps through acceleration of normal heparin climination.

ISSN:0957-5235    

出版商:OVID    

年代:1993

数据来源: OVID