Alagille Syndrome: A review

Michele Callea, Emrullah Bahsi, Marco Montanari, Bayram Ince, Giovanni E. Mancini, Yasemin Yavuz, Franco Radovich, Ayse Gunay, Gabriela Piana, Mehmet Unal, Giovanni D'Alessandro, Mauro Caselli, Gabriella Clarich

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)54-58
Number of pages5
JournalJournal of International Dental and Medical Research
Volume6
Issue number1
Publication statusPublished - 2013

Keywords

  • Alagille syndrome
  • Liver transplantation
  • Oro-dental complication
  • Treatment

ASJC Scopus subject areas

  • Dentistry(all)

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