17 Chapter 1 reports. So far, it has not yet yielded any clear answers. However, both case reports and complaints by OI patients on bleeding and bruising mentioned in clinical care are still relevant 64–69. The early detection of bleeding problems is very important because even mild bleeding tendencies can have an impact on operations, length of hospitalization, mortality, pregnancy, birth and miscarriages. Unexplained mild bleeding tendencies can also lead to anxiety. More clarity on the underlying cause of bleeding tendency would be crucial to providing more safety in the numerous operations and interventions that patients with OI undergo. Figure 1 Primary haemostasis Primary haemostasis involves two key processes: vasoconstriction and platelet adhesion to the injured vessel wall. (a) The plasma protein von-Willebrand-factor plays a vital role in capturing platelets from the rapidly flowing blood at the site of vessel wall damage. (b) Once adhered, platelets undergo activation and aggregation. (c) Concurrently, tissue factor present in the damaged vessel wall triggers secondary haemostasis. This leads to the activation of coagulation factors on the surface of the activated platelets, resulting in the generation of thrombin. (d) Ultimately, thrombin influences the formation of a fibrin network that reinforces the haemostatic plug and acts as a trap for erythrocytes, forming a blood clot. Following clot formation, the fibrinolytic system is responsible for breaking down the blood clot 70. d fibrin endothelial cell erythrocyte a b c direction of blood flow vascular wall lumen platelet von-Willebrand-factor thrombin ‘tissue factor’
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