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. Review (J Int Dent Med Res 2013; 6: (1), pp. 54-58)
References
- 1- Alagille D, Odièvre M, Gautier M, Dommergues JP. Hepatic
- ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental, and sexual development, Jan;86(1):63-71. J Pediatr. 1975 edition. Netherland: Saunders, Elsevier;
- 8- Gridley T. Notch signaling and inherited disease syndromes. Hum Mol Genet 2003; 12: R9-R13.
- 9- Martin SR, Garel L, Alvarez F. Alagille’s sindrome associated
- with cystic renal disease. Arch Dis Child. 1996; 74(3): 232-5.
- 10- Ciocca M, Alvarez F. Alagille syndrome Arch Argent Pediatr. 2012; 110(6): 509-515.
- 11- McElhinney DB, Kratz ID, Bason L, Piccoli DA, Emerick KM, Spinner NB, Goldmuntz E. Analysis of cardiovascular phenotype and genotype-phenotype correlation in individuals with a JAG1 mutation and/or Alagille syndrome. Circulation 2002; 106(20): 2567-74.
- 12- Rosenfield NS, Kelley MJ, Jensen PS, Cotlier E, Rosenfield AT, Riely CA. Arteriohepatic dysplasia: radiologic features of a new syndrome. AJR Am J Roentgenol 1980; 135:1217-23.
- 13- Hingorani M, Nischal KK, Davies A, Bentley C, Vivian A, Baker AJ, Mieli-Vergani G, Bird AC, Aclimandos WA. Ocular abnormalities in Alagille Syndrome. Ophthalmology 1999; 106(2): 330-7.
- 14- Amaral TH, Guerra Cde S, Bombonato-prado KF, Garcia de Paula E Silva FW, de Queiroz AM. Tooth pigmentation caused by bilirubin: a case report and histological evaluation. Spec Care Dent 2008; 28(6): 254-257.
- 15- Guadagni MG, Cocchi S, Tagariello T, Piana G. Case report: Alagille sindrome. Minerva Stomatol, 2005; 54 (10): 593-600.
- 16- Guimares LP, Silva TA. Green teeth associated with cholestasis caused by sepsis: A case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003; 95:446-51.
- 17- Al-Mutawa S, Mathews B, Salako N. Oral findings in Alagille syndrome. A case report. Med Princ Pract, 2002, 11(3): 161-3.
- 18- Sheehy EC, Roberts GJ, Beighton D, O’Brien G. Oral health in
- children undergoing liver transplantation, Int J Paediatr Dent 2000; 10: 109-119.
- 19- Yildiz TS, Yumuk NO, Baykal D, Solak M, Toker K. Alagille syndrome and anestesia management. Paediatr Anaesth 2007; 17: 87-97.
Abstract
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
Keywords
References
- 1- Alagille D, Odièvre M, Gautier M, Dommergues JP. Hepatic
- ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental, and sexual development, Jan;86(1):63-71. J Pediatr. 1975 edition. Netherland: Saunders, Elsevier;
- 8- Gridley T. Notch signaling and inherited disease syndromes. Hum Mol Genet 2003; 12: R9-R13.
- 9- Martin SR, Garel L, Alvarez F. Alagille’s sindrome associated
- with cystic renal disease. Arch Dis Child. 1996; 74(3): 232-5.
- 10- Ciocca M, Alvarez F. Alagille syndrome Arch Argent Pediatr. 2012; 110(6): 509-515.
- 11- McElhinney DB, Kratz ID, Bason L, Piccoli DA, Emerick KM, Spinner NB, Goldmuntz E. Analysis of cardiovascular phenotype and genotype-phenotype correlation in individuals with a JAG1 mutation and/or Alagille syndrome. Circulation 2002; 106(20): 2567-74.
- 12- Rosenfield NS, Kelley MJ, Jensen PS, Cotlier E, Rosenfield AT, Riely CA. Arteriohepatic dysplasia: radiologic features of a new syndrome. AJR Am J Roentgenol 1980; 135:1217-23.
- 13- Hingorani M, Nischal KK, Davies A, Bentley C, Vivian A, Baker AJ, Mieli-Vergani G, Bird AC, Aclimandos WA. Ocular abnormalities in Alagille Syndrome. Ophthalmology 1999; 106(2): 330-7.
- 14- Amaral TH, Guerra Cde S, Bombonato-prado KF, Garcia de Paula E Silva FW, de Queiroz AM. Tooth pigmentation caused by bilirubin: a case report and histological evaluation. Spec Care Dent 2008; 28(6): 254-257.
- 15- Guadagni MG, Cocchi S, Tagariello T, Piana G. Case report: Alagille sindrome. Minerva Stomatol, 2005; 54 (10): 593-600.
- 16- Guimares LP, Silva TA. Green teeth associated with cholestasis caused by sepsis: A case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003; 95:446-51.
- 17- Al-Mutawa S, Mathews B, Salako N. Oral findings in Alagille syndrome. A case report. Med Princ Pract, 2002, 11(3): 161-3.
- 18- Sheehy EC, Roberts GJ, Beighton D, O’Brien G. Oral health in
- children undergoing liver transplantation, Int J Paediatr Dent 2000; 10: 109-119.
- 19- Yildiz TS, Yumuk NO, Baykal D, Solak M, Toker K. Alagille syndrome and anestesia management. Paediatr Anaesth 2007; 17: 87-97.
Details
| Primary Language | Turkish |
|---|---|
| Journal Section | Articles |
| Authors | |
| Publication Date | March 1, 2013 |
| Published in Issue | Year 2013 Volume: 6 Issue: 1 |