Alagille syndrome: Experience of a tertiary care center in North India

Indian Journal of Gastroenterology

Volumn 33, Issue 1, 2014, Pages 59-62

  Srivastava, Anshu ^a   Goel, Deepak ^a   Bolia, Rishi ^a   Poddar, Ujjal ^a   Yachha, Surender Kumar ^a  

^a SANJAY GANDHI POSTGRADUATE INSTITUTE OF MEDICAL SCIENCES   (India)

Author keywords

Bile ductular paucity; Childhood; Cholestasis; Liver disease; Pruritus

Indexed keywords

ALKALINE PHOSPHATASE; BILIRUBIN; COLESTYRAMINE; GAMMA GLUTAMYLTRANSFERASE; RIFAMPICIN; URSODEOXYCHOLIC ACID;

ALAGILLE SYNDROME; ARTICLE; CHILD; CHOLESTASIS; CLINICAL ARTICLE; CLINICAL FEATURE; CONGENITAL HEART DISEASE; FEMALE; FOLLOW UP; HEPATOMEGALY; HUMAN; INDIA; INFANT; LIVER BIOPSY; LIVER FUNCTION TEST; MALE; MEDICAL RECORD REVIEW; PRESCHOOL CHILD; PRURITUS; SCHOOL CHILD; SINGLE DRUG DOSE; SPLENOMEGALY; TERTIARY CARE CENTER; TREATMENT OUTCOME; TREATMENT PLANNING; VERTEBRA MALFORMATION;

ALAGILLE SYNDROME; CALCIUM, DIETARY; CHILD; CHILD, PRESCHOOL; CHOLAGOGUES AND CHOLERETICS; CHOLESTASIS; CHOLESTYRAMINE RESIN; ENERGY INTAKE; FEMALE; HUMANS; INDIA; INFANT; MALE; PROGNOSIS; PRURITUS; RECOMMENDED DIETARY ALLOWANCES; RIFAMPIN; TERTIARY CARE CENTERS; TRIGLYCERIDES; URSODEOXYCHOLIC ACID; VITAMINS;

EID: 84895072152     PISSN: 02548860     EISSN: 09750711     Source Type: Journal    
DOI: 10.1007/s12664-013-0392-4     Document Type: Article

References (10)

1. Turnpenny PD, Eland S. Allagille syndrome: pathogenesis, diagnosis and management. Eur J Human Genet. 2012;20: 251-7.
2. Emerick KM, Rand EB, Goldmuntz E, et al. Features of Alagille syndrome in 92 patients, frequency and relation to prognosis. Hepatology. 1999;29: 822-9.
3. Lykavieris P, Hadchouel M, Chardot C, Bernard O. Outcome of liver disease in children with Alagille syndrome: a study of 163 patients. Gut. 2001;49: 431-5.
4. Kaye AJ, Rand EB, Munoz PS, Spinner NB, Flake AW, Kamath BM. Effect of Kasai procedure on hepatic outcome in Alagille syndrome. J Pediatr Gastroenterol Nutr. 2010;51: 319-21.
5. Shendge H, Tullu MS, Shenoy A, et al. Alagille syndrome. Indian J Pediatr. 2002;69: 825-7.
6. Sengupta S, Das JK, Gangopadhyay A. Alagille syndrome with prominent skin manifestations. Indian J Dermatol Venereol Leprol. 2005;71: 119-21.
7. Subramaniam P, Knisely A, Portmann B, et al. Diagnosis of Alagille syndrome-25 years of experience at King's College Hospital. J Pediatr Gastroenterol Nutr. 2011;52: 84-9.
8. Kamath BM, Bason L, Piccoli DA, et al. Consequences of JAG1 mutations. J Med Genet. 2003;40: 891-5.
9. Emerick KM, Whitington PF. Partial external biliary diversion for intractable pruritus and xanthomas in Alagille syndrome. Hepatology. 2002;35: 1501-6.
10. Kamath BM, Schwarz KB, Hadzic N. Alagille syndrome and liver transplantation. J Pediatr Gastroenterol Nutr. 2010;50: 11-5.