Special Lecture - VHL 1984-2024: Evolution of Diagnosis, Management, Surgery and Therapy скачать в хорошем качестве

Special Lecture - VHL 1984-2024: Evolution of Diagnosis, Management, Surgery and Therapy

Dr. Ramaprasad Srinivasan highlights significant breakthroughs that have transformed VHL patient care. In the past, kidney tumors associated with VHL often led to the removal of entire kidneys, resulting in serious consequences like the need for lifelong dialysis. Over time, surgical techniques improved, allowing doctors to remove only the tumors and preserve kidney function. However, patients still faced frequent surgeries for other tumors. A major advancement came with the discovery of the VHL gene mutation responsible for the disease. Scientists found that this mutation leads to the overproduction of a protein called hypoxia-inducible factor (HIF), specifically HIF-2 alpha, which promotes tumor growth by encouraging blood vessel formation. Belzutifan (Welireg) is a groundbreaking medication designed to inhibit HIF-2 alpha. Clinical trials have shown that belzutifan can significantly reduce the size of tumors associated with VHL disease, offering a non-surgical treatment option for patients. Many patients experience significant shrinkage of kidney tumors on belzutifan, with some tumors disappearing entirely. Tumors in the brain, spinal cord, pancreas, and eyes have also responded well to the treatment, with many patients reporting quick improvements in symptoms like headaches and vision problems. While belzutifan is generally well-tolerated, some patients experience side effects. Low red blood cell count (anemia) is common, but can usually be managed with medical support. Some patients also report feeling tired, but this is typically mild. Low oxygen levels (hypoxia) have also occurred, but were similarly treatable with appropriate care. Doctors are still working to develop strategies to manage these side effects effectively, ensuring patients can continue the full-dose treatment comfortably. Dr. Srinivasan cautions that in some cases, tumors can become resistant to belzutifan treatment, making the drug less effective over time. Studies are ongoing to understand what causes this belzutifan resistance and identify the treatment combinations that could help delay, overcome, or reverse this effect. Despite the promising results of belzutifan, Dr. Srinivasan explains that several questions remain: Determining the best timing, dosage, and duration of treatment. Understanding the long-term effects and safety of the medication. Why some tumors may become less responsive to the medication over time. How to make the drug more accessible and affordable for patients worldwide.