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Alagille Syndrome
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Several oral manifestations associated with congenital neonatal cholestasis

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Oral manifestations, including jaw morphology, dental structure, and periodontal health are associated with congenital neonatal cholestasis, including biliary atresia (BA), Alagille syndrome (ALGS), according to a study.

The presence of facial dysmorphism in ALGS and tooth discoloration resulting from bilirubin exposure are particularly noteworthy, the authors said.

In this systematic review of case reports and case series, 19 studies, including 16 case reports and 3 case series, were included.

In addition to the impact on jaw morphology, with facial dysmorphism being specific to ALGS, exposure to elevated levels of bilirubin during dental calcification led to distinct tooth discoloration were also noted. These manifestations underscore the importance of early involvement of dental professionals in the care team for congenital cholestasis patients.

Reference
Reynal F, Camoin A, Tardieu C, et al. Oral findings in children with congenital cholestatic disease: A systematic review of case reports and case series. Arch Pediatr. 2023;S0929-693X(23)00093-3. doi: 10.1016/j.arcped.2023.06.003. Epub ahead of print. PMID: 37394364.

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