An Overview of Best Disease

Abstract

The German ophthalmologist Friedrich Best named Best vitelliform macular dystrophy (BVD), sometimes referred to as Best disease, in 1905 when he discovered a family with a history of early-onset macular degeneration. A Swedish study assessed the frequency of BVMD to be 2 in 10,000, whereas a Danish examination found 1.5 in 100,000 cases. If a parent with the condition has a kid with an unaffected spouse, there is a 50% chance that the child will inherit the condition from the parent. The syndrome is caused by a mutation in the VMD2 or BEST1 gene found at chromosome 11q12-q13. It is yet unclear how bestrophinopathies pathophysiologically operate. An ionic imbalance in the RPE milieu, which BEST1 gene mutations can bring on, can lead to impaired RPE functions. The best sickness is in five phases. There is currently no recognized treatment for the best sickness. One treatment option for CNV is VEFG injections, either used alone or in conjunction with photodynamic therapy (PDT). Anti-vascular endothelial growth factor (Anti- VEGF) treatments can prevent or reduce the creation of new blood vessels. This can postpone the onset of blindness and slow down the rate at which they leak.