2022
DOI: 10.1097/mop.0000000000001156
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The cholestatic infant: updates on diagnosis and genetics

Abstract: Purpose of reviewCholestasis in infants can indicate a serious hepatobiliary disease and requires timely assessment, diagnosis and intervention to prevent progression to serious liver decompensation. This report aims to highlight recently published studies regarding diagnosis and treatment of cholestasis in infants.Recent findingsThe evaluation of neonatal cholestasis can be challenging, requiring the assessment of a broad differential diagnosis in timely fashion. The Italian Society of pediatric gastroenterol… Show more

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Cited by 2 publications
(2 citation statements)
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“…Up until recently, patients with AGLS did not have any treatment options for refractory pruritus. As of now, maralixibat, an IBAT inhibitor, is considered a strong candidate by the Food and Drug Administration (FDA) as a Breakthrough Therapy and Orphan Drug for pruritus associated with AGLS among patients 1 year or older, building the roadmap for its approval by the FDA and gathering support from data from clinical trials for its strong efficacy [33,34]. Our findings support both maralixibat and odevixibat, both IBAT blockers, as important treatment options for AGLS, the use of which can lead to significant reductions in the severity of pruritus.…”
Section: Discussionmentioning
confidence: 99%
“…Up until recently, patients with AGLS did not have any treatment options for refractory pruritus. As of now, maralixibat, an IBAT inhibitor, is considered a strong candidate by the Food and Drug Administration (FDA) as a Breakthrough Therapy and Orphan Drug for pruritus associated with AGLS among patients 1 year or older, building the roadmap for its approval by the FDA and gathering support from data from clinical trials for its strong efficacy [33,34]. Our findings support both maralixibat and odevixibat, both IBAT blockers, as important treatment options for AGLS, the use of which can lead to significant reductions in the severity of pruritus.…”
Section: Discussionmentioning
confidence: 99%
“…It can result in serious outcomes and requires prompt intervention. [2] Biliary atresia (BA), an infantile hepatobiliary condition that includes both extrahepatic bile duct blockage and intrahepatic fibrosing cholangiopathy, is the most common cause of NC. Surgically releasing the extrahepatic obstruction by portoenterostomy may avoid cirrhosis developing with BA.…”
Section: Introductionmentioning
confidence: 99%