الفهرس 
Material and methodsOnly 14 pages are availabe for public view
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Abstract
(~ON(~L(JSIONAND IlI~CO)IHENDA1’IONS
This study was conducted on 19 patients with different causes of pH
attending the pediatric clinic at the Benha hospital, Benha Faculty of
.
Medicine. Their ages ranged from 18 days to 16 years. The study include
also 10 apparently healthy, age and sex matched subjects as control group.
The aim of this study was to investigate changes in the level of
circulating vWF in patients with pH.
The studied subjects were grouped into 3 groups:
• Group I: Patients with pH secondary to venous causes (Rheumatic heart
disease), 14 patients.
• Group II: Patients with pH secondary to arterial causes (Congenital
anomalies with left to right shunt), 5 patients.
• Group III: Normal subjects serving as a control group, 10 subjects.
All these groups were subjected to medical history taking, thorough
clinical examination, echocardiography, and laboratory tests which
included vWF : Ag level assay by ELISA technique.
The mean values of plasma vWF : Ag level showed significant
increase in group I and group II as compared to group III (F=16.91,
P < 0.0001).
Conclusion and RecommendatiOns 99.
The etiology of pH (whether due to arterial or venous causes) had no
effect on the degree of rise of circulating vWF : Ag. This was evident by
the absence of significant difference in the circulating vWF : Ag levels
between group I and group II (P > 0.05).
The degree of severity of pH is an important determinant ofvWF :
Ag rise. There was a significant positive correlation between vWF : Ag
levels and the corresponding values of pulmonary artery pressure in all
patients with pH (P > 0.05).
There was significant correlation between the rise ofvWF : Ag level
with the duration of the disease (P < 0.05).
from this study we concluded that:
vWF proved to be a sensitive indicator of endothelial cell injury and the
degree of hemodynamic abnormalities in patients with pH.
Inspite of high levels of vWF in patients with pH, it has a defective
,
structure in these patients. However, insitu thrombosis occurs due to
high shear stress, which leads to coagulation abnormalities and
thrombin release, leading to the formation of abnormal circulating
aggregates with more vascular injury and perpetuation of pH.
Conclusion and RecommendatiOns
100.
lU~(~O)I)I1~N))l’l’IONS
• Measurement ofvWF: Ag is recommended in follow up of patients with
rheumatic or congenital heart diseases at risk of developing pulmonary
hypertension for early detection of abnormal hemodynamics and
endothelial injury in such patients.
• Further research is needed to study the use of anticoagulant therapy and
antiplatelet drugs to stop the vicious circle of insitu thrombosis and
improve survival in patients with pulmonary hypertension.