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Yao syndrome (YAOS), formerly called NOD2-associated autoinflammatory disease, [1] is an autoinflammatory syndrome involving episodes of fever and abnormal inflammation affecting many parts of the body, particularly the skin, joints, and gastrointestinal system.
Diagnosis is generally based on symptoms. [2] Blood sugar levels are often normal or only mildly increased. [2] Other conditions that may present similarly include other causes of high anion gap metabolic acidosis including diabetic ketoacidosis. [2] Treatment is generally with intravenous normal saline and intravenous sugar solution. [2]
Without treatment mortality rates of autoimmune enteropathy are as high as 30%. [8] Many factors such as the need for parenteral nutrition, [61] age of presentation, and the severity of symptoms can impact long-term outcomes. [3] No one treatment has been proven successful in all cases and relapses are common. [4]
However, with proper treatment, including cessation of alcohol consumption and management of heart failure symptoms, the prognosis can improve significantly. [ 10 ] Research has shown that the mortality rate for people with ACM is higher than that of the general population, with a five-year survival rate of around 50%. [ 10 ]
Evans syndrome is an autoimmune disease in which an individual's immune system attacks their own red blood cells and platelets, the syndrome can include immune neutropenia. [ 1 ] [ 2 ] These immune cytopenias may occur simultaneously or sequentially.
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Calciphylaxis, also known as calcific uremic arteriolopathy (CUA) or “Grey Scale”, is a rare syndrome characterized by painful skin lesions.The pathogenesis of calciphylaxis is unclear but believed to involve calcification of the small blood vessels located within the fatty tissue and deeper layers of the skin, blood clots, and eventual death of skin cells due to lack of blood flow. [1]
Despite the clinic’s failure rate, she has not considered making the medication more accessible. “I don’t know how to answer that question,” she said. “We are an abstinence-based program by nature.” The state’s treatment providers have little idea how their patients fare once they walk out the door.
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