Michele CALLEA,
Emrullah BAHSİ,
Marco Montanari,
Bayram INCE,
Giovanni E. MANCİNİ,
Yasemin YAVUZ,
Franco RADOVİCH,
Ayse GUNAY,
Gabriela PİANA,
Mehmet Unal,
Giovanni D’ALESSANDRO,
Mauro Caselli,
Gabriella CLARİCH
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Alagille Syndrome: A Review
Michele Callea1*, Emrullah Bahsi2, Marco Montanari3, Bayram Ince2, Giovanni E. Mancini5, Yasemin Yavuz2, Franco Radovich1, Ayse Gunay4, Gabriela Piana, Mehmet Unal4, Giovanni D’Alessandro3, Mauro Caselli6, Gabriella Clarich1
Alagille Syndrome: A Review
Alagille Syndrome (AGS) is a genetically determined multisystem disorder affecting liver, hearth, eyes, skeleton and facies, less commonly kidney and CNS. The prognosis depends on the severity of the associated anomalies. The liver pathology plays a central role in that most clinical complications are due to long standing cholestasis as a consequence of lack of bile excretion secondary to paucity/absence of interlobular bile ducts. That results in hyperbilirubinemia, hypercholesterolemia, hypertriglyceridemia, fat and liposoluble vitamin malabsorption, pruritus and cutaneous xanthoma. Liver transplantation represents the only curative therapy for the liver pathology. Most hepatic symptoms reverse after liver transplantation. Therapeutical education for oro-dental hygiene is required before and after liver transplantation. The green pigmentation of teeth requires dental rehabilitation. This paper reviews the clinical manifestations of AGS with special regard to the cephalic district, and highlights the necessity for a multidisciplinary approach in order to minimize complications and to ameliorate the quality of life in AGS patients. Review (J Int Dent Med Res 2013; 6: (1), pp. 54-58)
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