Advancements in Managing Progressive Familial Intrahepatic Cholestasis Type 2
Progressive Familial Intrahepatic Cholestasis Type 2 (PFIC2) is a rare genetic liver disorder caused by mutations in the ABCB11 gene, which encodes the bile salt export pump (BSEP). This defect leads to impaired bile flow from liver cells, resulting in severe itching, jaundice, growth challenges, and progressive liver damage. Treatment strategies aim to relieve symptoms, slow disease progression, and improve long-term outcomes.
Medical therapy often begins with bile acid modulators and medications designed to reduce bile acid accumulation. Agents such as odevixibat, an ileal bile acid transporter (IBAT) inhibitor, have shown promise in reducing pruritus and improving quality of life. Nutritional management, including supplementation of fat-soluble vitamins (A, D, E, and K), is also critical due to malabsorption issues.
Ref - https://www.marketresearchfuture.com/reports/progressive-familial-intrahepatic-cholestasis-type-2-treatment-market-27160
Progressive Familial Intrahepatic Cholestasis Type 2 (PFIC2) is a rare genetic liver disorder caused by mutations in the ABCB11 gene, which encodes the bile salt export pump (BSEP). This defect leads to impaired bile flow from liver cells, resulting in severe itching, jaundice, growth challenges, and progressive liver damage. Treatment strategies aim to relieve symptoms, slow disease progression, and improve long-term outcomes.
Medical therapy often begins with bile acid modulators and medications designed to reduce bile acid accumulation. Agents such as odevixibat, an ileal bile acid transporter (IBAT) inhibitor, have shown promise in reducing pruritus and improving quality of life. Nutritional management, including supplementation of fat-soluble vitamins (A, D, E, and K), is also critical due to malabsorption issues.
Ref - https://www.marketresearchfuture.com/reports/progressive-familial-intrahepatic-cholestasis-type-2-treatment-market-27160
Advancements in Managing Progressive Familial Intrahepatic Cholestasis Type 2
Progressive Familial Intrahepatic Cholestasis Type 2 (PFIC2) is a rare genetic liver disorder caused by mutations in the ABCB11 gene, which encodes the bile salt export pump (BSEP). This defect leads to impaired bile flow from liver cells, resulting in severe itching, jaundice, growth challenges, and progressive liver damage. Treatment strategies aim to relieve symptoms, slow disease progression, and improve long-term outcomes.
Medical therapy often begins with bile acid modulators and medications designed to reduce bile acid accumulation. Agents such as odevixibat, an ileal bile acid transporter (IBAT) inhibitor, have shown promise in reducing pruritus and improving quality of life. Nutritional management, including supplementation of fat-soluble vitamins (A, D, E, and K), is also critical due to malabsorption issues.
Ref - https://www.marketresearchfuture.com/reports/progressive-familial-intrahepatic-cholestasis-type-2-treatment-market-27160
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