Koert Gooijer

136 Prevalence of bleeding in a large cohort such as the one in Chapter 5 has never been studied. However, recent studies have looked at bleeding around pregnancy 74, confirming the increased risk of bleeding around pregnancy for patients with OI. An increase in the incidence of major bleeding during surgery in children has also recently been described 75. What are the clinical manifestations of bleeding tendency in OI? Chapter 5 shows that bleeding in OI typically occurs as spontaneous or easy bruising, prolonged bleeding after trauma or surgery, and excessive bleeding during menstruation. Post-extraction bleeding and gastrointestinal bleeding have also been reported. Spontaneous and easy bruising is a symptom of OI that is often mentioned by patients in the consulting room. This is often associated with concerns about bleeding during or after surgery. There are case reports in the literature of bleeding events with serious complications due to aneurysms 70,76–78. These complications were not found in our study. Chapter 5 evaluates all the different bleeding events and shows that abnormal bleeding events occur much more frequently than would be expected in a control population. The self-reported questionnaire used was analysed to identify clinically relevant bleeding symptoms. The underlying mechanism was not readily apparent from the symptoms. By focusing on clinically relevant bleeding symptoms, it was possible to estimate the severity of the bleeding tendency (Chapter 5). Which bleeding events are most clinically relevant in OI? The relevance of bleeding events in OI can be determined by considering the types of events that lead to bleeding. People with OI experience many events that can cause bleeding, including fractures, corrective surgeries for bone deformities, and tooth extractions due to dentinogenesis imperfecta. While this may lead to a higher number of reported bleeding events compared to the healthy population, it also raises the question of why bleeding occurs so frequently in OI. Assessing the severity of these bleeding events is key in determining whether precautions should be taken during procedures like surgery or tooth extraction. In addition to these commonly reported bleeding events, there are also cases of intracranial bleeding after head injury that result in severe complications 79. While these events were not reported in our study, they are highly relevant and require prompt medical attention. Based on our study outcome (Chapter 5), we recommend that individuals with OI who experience even minimal head trauma should receive a CT scan to exclude fractures and post-traumatic brain injury. Treatment for heavy menstrual bleeding might also be considered proactively with medication like desmopressin

RkJQdWJsaXNoZXIy MTk4NDMw