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Complications of Wilson's disease can include liver failure and kidney problems. A liver transplant may be helpful to those for whom other treatments are not effective or if liver failure occurs. [1] Wilson's disease occurs in about one in 30,000 people. [1] Symptoms usually begin between the ages of 5 and 35 years. [1]
Micrograph showing a Mallory body with the characteristic twisted-rope appearance (centre of image - within a ballooning hepatocyte). H&E stain.. In histopathology, a Mallory body, Mallory–Denk body (MDB), or Mallory's hyaline is an inclusion found in the cytoplasm of liver cells. [1]
The Alzheimer type II astrocyte is thought to be a pathological type of cell in the brain; however, its exact pathology remains unknown. Like other astrocytes, it is a non-neuronal glial cell. It's mainly seen in diseases that cause increased levels of ammonia (hyperammonemia), such as chronic liver disease and Wilson's disease. [1]
Wilson disease protein (WND), also known as ATP7B protein, is a copper-transporting P-type ATPase which is encoded by the ATP7B gene. The ATP7B protein is located in the trans-Golgi network of the liver and brain and balances the copper level in the body by excreting excess copper into bile and plasma.
Signs and symptoms depend largely upon the primary lesions giving rise to the condition. In addition to the heart or lung symptoms, there will be a sense of fullness and tenderness in the right hypochondriac region.
UX701 is an experimental gene therapy for Wilson disease, delivered via adeno-associated virus, that restores a functional version of the ATP7B gene. [1] [2] [3]
Preoperative treatment with regimen DD-4A, followed by nephron sparing surgery or nephrecomy, staging of tumors, and chemotherapy and/or radiotherapy based on pathology and staging In case of relapse of Wilms' tumor, the 4-year survival rate for children with a standard-risk has been estimated to be 80%.
The Wilson Disease Association (WDA) is a nonprofit volunteer organization which has been established to support the people who suffer from Wilson's disease. The Association's main mission is to finance scientific researches on this disease and enlightening people about it.