Intense pruritus and failure to thrive in Alagille syndrome

Journal of the American Academy of Dermatology

Volumn 58, Issue 2 SUPPL. 1, 2008, Pages

  Schwartz, Rodrigo ^a   Rehder, Kyle ^a   Parsons, Daniel J ^a   Morrell, Dean S ^a  

^a UNIVERSITY OF NORTH CAROLINA SCHOOL OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ALAGILLE SYNDROME; ARTICLE; CASE REPORT; FEMALE; HEART ATRIUM SEPTUM DEFECT; HEART VENTRICLE SEPTUM DEFECT; HUMAN; HYPERTELORISM; JAUNDICE; LIVER BIOPSY; LIVER FIBROSIS; PHYSICAL EXAMINATION; PRESCHOOL CHILD; PRIORITY JOURNAL; PRURITUS; PULSE OXIMETRY; SKIN EXAMINATION; SLIT LAMP;

ALAGILLE SYNDROME; CHILD, PRESCHOOL; FAILURE TO THRIVE; FEMALE; HUMANS; PRURITUS;

EID: 38049008488     PISSN: 01909622     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.jaad.2007.03.029     Document Type: Article

References (20)

1. Alagille D., Estrada A., Hadchouel M., Gautier M., Odievre M., and Dommergues J.P. Syndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia): review of 80 cases. J Pediatr 110 (1987) 195-200
2. Alagille D., Odievre M., Gautier M., and Dommergues J.P. Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental, and sexual development, and cardiac murmur. J Pediatr 86 (1975) 63-71
3. Oda T., Elkahloun A.G., Meltzer P.S., and Chandrasekharappa S.C. Identification and cloning of the human homolog (JAG1) of the rat Jagged1 gene from the Alagille syndrome critical region at 20p12. Genomics 43 (1997) 376-379
4. Roberts E.A. The jaundiced baby. In: Kelly D.A. (Ed). Diseases of the liver and biliary system in children (1999), Blackwell Science, London 49-50
5. Emerick K.M., Rand E.B., Goldmuntz E., Krantz I.D., Spinner N.B., and Piccoli D.A. Features of Alagille syndrome in 92 patients: frequency and relation to prognosis. Hepatology 29 (1999) 822-829
6. Kamath B.M., Loomes K.M., Oakey R.J., Emerick K.E., Conversano T., Spinner N.B., et al. Facial features in Alagille syndrome: specific or cholestasis facies?. Am J Med Genet 112 (2002) 163-170
7. Moreno J.C., Del Rio L., and Camacho F. Le syndrome d′Alagille. Etude clinique et histo-pathologique. Ann Dermatol Venereol 111 (1984) 225-229
8. Martin S.R., Garel L., and Alvarez F. Alagille's syndrome associated with cystic renal disease. Arch Dis Child 74 (1996) 232-235
9. García M.A., Ramonet M., Ciocca M., Cabrera H., Lapunzina P., Álvarez E., et al. Alagille syndrome: cutaneous manifestations in 38 children. Pediatr Dermatol 22 (2005) 11-14
10. Ho N.C., Lacbawan F., Francomano C.A., and Ho V. Severe hypodontia and oral xanthomas in Alagille syndrome. Am J Med Genet 93 (2000) 250-252
11. Kamath B.M., Loomes K.M., Oakey R.J., and Krantz I.D. Supernumerary digital flexion creases: an additional clinical manifestation of Alagille syndrome. Am J Med Genet 112 (2002) 171-175
12. Watson G., and Miller V. Arteriohepatic dysplasia. Familial pulmonary arterial stenosis with neonatal liver disease. Arch Dis Child 48 (1973) 459-466
13. Ferrando J., Bassas S., Rodés J., and Mascaró J.M. Displasia arteriohepatica (S. de Alagille), con manifestaciones cutáneas. Actas Dermosif 74 (1983) 87-92
14. Gottrand F., Clavey V., Fruchart J.C., and Farriaux J.P. Lipoprotein pattern and plasma lecithin cholesterol acyl transferase activity in children with Alagille syndrome. Atherosclerosis 115 (1995) 233-241
15. Alagille D. Management of paucity of interlobular bile ducts. J Hepatol 1 (1985) 561-565
16. Buckley D.A., Higgins E.M., and du Vivier A.W. Resolution of xanthomas in Alagille syndrome after liver transplantation. Pediatr Dermatol 15 (1998) 199-202
17. Krawinkel M.B., Santer R., and Oldigs H.D. Ursodesoxycholic acid: effect on xanthomas in Alagille-Watson syndrome. J Pediatr Gastroenterol Nutr 19 (1994) 476-477
18. Emerick K.M., and Whitington P.F. Partial external biliary diversion for intractable pruritus and xanthomas in Alagille syndrome. Hepatology 35 (2002) 1501-1506
19. Hoffenberg E.J., Narkewicz M.R., Sondheimer J.M., Smith D.J., Silverman A., and Sokol R.J. Outcome of syndromic paucity of interlobular bile ducts (Alagille syndrome) with onset of cholestasis in infancy. J Pediatr 127 (1995) 220-224
20. Lykavieris P., Hadchouel M., Chardot C., and Bernard O. Outcome of liver disease in children with Alagille syndrome: a study of 163 patients. Gut 49 (2001) 431-435