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Epidermolysis bullosa (EB) is a group of rare genetic skin diseases of which Dystrophic EB (DEB) is one of the most severe forms. There are two main types of DEB – the dominant form (DDEB) when the affected person inherits the altered gene from only one parent and the recessive form (RDEB) when the affected person inherits one copy of the altered gene from each parent.

RDEB tends to be generally more severe than DDEB. People with DEB have blistering of skin and mucosal membranes that line many tracts and structures of the body, such as the mouth, food pipe etc. Children with DEB are often called ‘Butterfly children’ as their skin is as fragile as the wings of a butterfly. This fragility results from a very weak connection between the dermis (inner layer) and the epidermis (outer layer) of the skin. People with DEB live with constant pain and have a high risk of malnutrition and infections. Symptoms include poorly healing wounds, skin infections, fusion of fingers and toes (pseudosyndactyly), anemia, gastrointestinal tract problems and with adulthood some develop very aggressive forms of squamous cell carcinoma (skin cancer).

Backup up your current firmware (this saves a copy of your current firmware) press menu---tool---dump by usb Save your dump file 2. Upgrad to new firmware 3.on your openbox/skybox remote press menu---tool---information( for openbox s11 press 8888 for openbox s10 press ok)---press 8888 (7777 for openbox S9) If chip ID is one of these numbers 00D0 00EA 085B 0D0C 98A1 98F2 9CF4 9D1A 1015 0D7E 38EC 10AA 1466 113c Congratulatations! HOW TO IDENTIFY IF YOUR OPENBOX/SKYBOX IS A CLONE OR AN ORIGINAL If you have Openbox/Skybox S9(S10) HD PVR with Ali3602 BGA. To identify if it is original or not as follows: If you have a firmware later than go to step 3, otherwise 1. Openbox sx6 hd firmware update.

What causes DEB? Currently, there are no approved treatments available that target the underlying cause of DEB. Patients receive treatments that focus on disease management, prevention of complications and prevention of the formation of new blisters. There are palliative treatment options focused on wound care (bandaging, prevention of infection, better wound healing), pain management and itch management. Other points of attention are bone care, nutrition (due to eating difficulties), oral care and eye care. In addition there is a close surveillance for skin cancer, to which some DEB patients are especially vulnerable.

ProQR is developing an investigational drug called QR-313 for people that have DEB due to a mutation in a specific part of the COL7A1 gene called exon 73. We estimate there are approximately 2,000 patients in the Western world that have DEB due to a mutation in this specific part of the gene. QR-313 is designed to exclude exon 73 from the COL7A1 RNA. This approach is also known as exon skipping. RNA is the 'blueprint' for protein synthesis.