{"id":48255,"date":"2022-09-12T16:01:50","date_gmt":"2022-09-12T14:01:50","guid":{"rendered":"https:\/\/pharma-trend.com\/en\/aicardi-goutieres-syndrome-epidemiology-forecasts-report-2019-2032-focus-on-7mm-united-states-eu5-germany-spain-italy-france-and-the-united-kingdom-and-japan-researchandmarkets-com\/"},"modified":"2022-09-12T16:01:50","modified_gmt":"2022-09-12T14:01:50","slug":"aicardi-goutieres-syndrome-epidemiology-forecasts-report-2019-2032-focus-on-7mm-united-states-eu5-germany-spain-italy-france-and-the-united-kingdom-and-japan-researchandmarkets-com","status":"publish","type":"post","link":"https:\/\/pharma-trend.com\/en\/aicardi-goutieres-syndrome-epidemiology-forecasts-report-2019-2032-focus-on-7mm-united-states-eu5-germany-spain-italy-france-and-the-united-kingdom-and-japan-researchandmarkets-com\/","title":{"rendered":"Aicardi Goutieres Syndrome Epidemiology Forecasts Report 2019-2032: Focus on 7MM – United States, EU5 (Germany, Spain, Italy, France, and the United Kingdom), and Japan – ResearchAndMarkets.com"},"content":{"rendered":"
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DUBLIN–(BUSINESS WIRE)–The “Aicardi Goutieres Syndrome – Epidemiology Forecast – 2032”<\/a> report has been added to ResearchAndMarkets.com’s<\/strong> offering.\n<\/p>\n

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\nThis ‘Aicardi-Goutieres Syndrome (AGS) – Epidemiology Forecast – 2032’ report delivers an in-depth understanding of the historical and forecasted epidemiology of Aicardi-Goutieres Syndrome (AGS) in the United States, EU5 (Germany, Spain, Italy, France, and the United Kingdom), and Japan.\n<\/p>\n

\nAicardi-Goutieres Syndrome (AGS): Disease UnderstandingAicardi-Goutieres Syndrome (AGS) Overview<\/strong>\n<\/p>\n

\nAccording to the National Organization for Rare Diseases, Aicardi-Goutieres syndrome (AGS) is an inherited disease mainly affecting the brain, immune system, and skin. The loss of white matter (leukodystrophy) and abnormal deposits of calcium (calcification) in the brain lead to early-onset severe brain dysfunction (encephalopathy) usually resulting in severe intellectual and physical disability.\n<\/p>\n

\nThere are two forms of the syndrome: a severe, early-onset form and a late-onset form with less impact on neurological function. The early-onset form affects about 20% of all Aicardi-Goutieres Syndrome (AGS) babies. The signs and symptoms vary widely among affected people.\n<\/p>\n

\nIn mild cases, symptoms appear after the first weeks or months of normal development like the decline in head growth, weak or stiffened muscles (spasticity), and cognitive and developmental delays. However, neurological and liver abnormalities, such as liver and spleen enlargement and elevated liver enzymes are reported in severe cases.\n<\/p>\n

\nThe disease occurs due to mutations in the TREX1, RNASEH2A, RNASEH2B, SAMHD1, ADAR, IFIH1, and RNASEH2C genes that lead to a buildup of small pieces of DNA in the brain, which triggers the immune response. It is most frequently inherited in an autosomal recessive manner whereas, in a few instances, the disease results from specific de novo or inherited autosomal dominant pathogenic variants in ADAR or TREX1 and a variety of heterozygous autosomal dominant pathogenic variants in IFIH1.\n<\/p>\n

\nIt is difficult to diagnose Aicardi-Goutieres Syndrome (AGS) since several symptoms appear similar to other diseases. The diagnosis is based on the clinical symptoms of the disease, as well as characteristic brain abnormalities observed in an MRI brain scan. Additionally, Cerebrospinal fluid (CSF) is tested for increased levels of a specific immune system cell (a lymphocyte), indicating chronic lymphocytosis.\n<\/p>\n

\nCurrently, no curative therapy is available. The present treatment focuses on managing symptoms and complications based on the severity of AGS. Chest physiotherapy, treatment for respiratory complications, and anticonvulsant medications manage seizures.\n<\/p>\n

\nDespite advances in understanding the Aicardi-Goutieres Syndrome (AGS) pathology spectrum, a systemic cure is still not developed. Hence, there is an urgent unmet medical need to diagnose better, treat, and eradicate AGS.\n<\/p>\n

\nHowever, many aspects of the Aicardi-Goutieres Syndrome (AGS) pathogenesis have been clarified to hypothesize new therapeutic strategies. A few studies have highlighted the possible positive effects of JAK inhibitors and antiretroviral on the CNS and, therefore, on AGS.\n<\/p>\n

\nAicardi-Goutieres Syndrome (AGS) : Epidemiology<\/strong>\n<\/p>\n

\nThe Aicardi-Goutieres Syndrome (AGS) epidemiology division provides insights into the historical and current patient pool, along with the forecast trend for every seven major countries. It helps recognize the causes of current and forecasted trends by exploring numerous studies and views of key opinion leaders. This part of the report also provides the diagnosed patient pool and their trends, along with assumptions undertaken.\n<\/p>\n

\nCountry-wise Aicardi-Goutieres Syndrome (AGS) Epidemiology<\/strong>\n<\/p>\n

\nThe total prevalent patient population of Aicardi-Goutieres Syndrome (AGS) in the 7MM countries was more than 850 cases in 2021.\n<\/p>\n

\nAs per the estimates, the US had the highest total prevalent patient population of Aicardi-Goutieres Syndrome (AGS) population in 2021. Among the EU5 countries, Germany had the highest total prevalent patient population of Aicardi-Goutieres Syndrome (AGS) with 89 cases, followed by France in 2021.\n<\/p>\n

\nOn the other hand, Spain had the lowest total prevalent patient population of Aicardi-Goutieres Syndrome (AGS) close to 50 cases in 2021.\n<\/p>\n

\nScope of the Report<\/strong>\n<\/p>\n