Alagille Syndrome: Clinical and Ocular Pathognomonic Features
- Resource Type
- Authors
- Nehal El-Koofy; Mona E. Fahmy; Ahmed El-Hennawy; Randa El-Mahdy; Hanaa El-Karaksy; Mohamed Y. Farag
- Source
- European Journal of Ophthalmology. 21:199-206
- Subject
- Male
Pathology
medicine.medical_specialty
Adolescent
Cholestasis, Intrahepatic
Hepatitis
03 medical and health sciences
0302 clinical medicine
Liver Function Tests
Cholestasis
Pathognomonic
Alagille syndrome
Humans
Medicine
Child
medicine.diagnostic_test
Optic Disk Drusen
business.industry
Infant, Newborn
Infant
General Medicine
medicine.disease
Alagille Syndrome
Interlobular bile ducts
Neonatal hepatitis
Ophthalmology
Stenosis
Child, Preschool
Liver biopsy
030221 ophthalmology & optometry
Female
business
Liver function tests
030217 neurology & neurosurgery
- Language
- ISSN
- 1724-6016
1120-6721
Pur P ose . This study was carried out to determine the frequency of clinical diagnostic criteria in patients with Alagille syndrome (AGS) in comparison to a group of children with cholestasis and histologically proven neonatal hepatitis (NH). The type and frequency of ocular manifestations are highlighted. Methods . According to histologic findings on liver biopsy, the 32 patients included in the study were classified into 2 groups: group 1 had paucity of interlobular bile ducts (PILBD) (n=13) and at least 3 of 5 characteristics of AGS and group 2 had NH on liver biopsy (n=19). results . The mean age of patients with AGS was higher than in group 2 (3.9 vs 1.6 years) (p