Management of cholestatic pruritus in children with Alagille syndrome: Case report and literature review

Alagille syndrome causes intractable pruritus and disfiguring xanthomas because of retained bile acids and cholesterol. Drug therapy in addition to surgical intervention may be effective in many patients in reducing serum bile acids, cholesterol levels, pruritus, and skin xanthomas. In this report,...

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Bibliographic Details
Published in:Archives de pédiatrie : organe officiel de la Société française de pédiatrie 2016-12, Vol.23 (12), p.1247-1250
Main Authors: Ben Ameur, S., Chabchoub, I., Telmoudi, J., Belfitouri, Y., Rebah, O., Lacaille, F., Aloulou, H., Mehrzi, A., Hachicha, M.
Format: Article
Language:English
Subjects:
Alagille Syndrome - complications
Child
Cholestasis - etiology
Cholestasis - therapy
Humans
Hypercholesterolemia - complications
Hypercholesterolemia - genetics
Male
Pruritus - etiology
Pruritus - therapy
Xanthomatosis - etiology
Xanthomatosis - therapy
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Summary:Alagille syndrome causes intractable pruritus and disfiguring xanthomas because of retained bile acids and cholesterol. Drug therapy in addition to surgical intervention may be effective in many patients in reducing serum bile acids, cholesterol levels, pruritus, and skin xanthomas. In this report, we describe a child with Alagille syndrome who presented with severe pruritus and xanthomas as a consequence of severe hypercholesterolemia and discuss the treatment modalities. Le syndrome d’Alagille, ou paucité ductulaire syndromique, est une affection multisystémique héréditaire, de transmission autosomique dominante. Il est caractérisé par l’association d’une cholestase chronique à une atteinte cardiaque, oculaire, squelettique et un faciès caractéristique. Il est souvent associé à prurit et à la formation de xanthomes secondaires à l’hypercholestérolémie. Nous discutons à travers une observation les modalités thérapeutiques chez ces patients souffrant souvent de prurit invalidant.
ISSN:0929-693X
1769-664X
DOI:10.1016/j.arcped.2016.09.004