Ahlstrom's Syndrome with Type IV Choledochal Cyst.
Loading...
Date
2010-10
Journal Title
Journal ISSN
Volume Title
Publisher
Abstract
Ahlstrom's syndrome is an autosomal recessive, single gene disorder with multisystem involvement with
cone-rod retinal dystrophy leading to juvenile blindness, sensorineural hearing loss, obesity, insulin resistance
and type 2 Diabetes mellitus. The other less common involvements may severely affect the prognosis and
survival and include endocrine abnormalities, dilated cardiomyopathy(DCM) , pulmonary fibrosis and
restrictive lung disease, progressive hepatic and renal failure.Apart from its typical, well known clinical
features, hepatic & biliary involvement should be kept in mind. Though not known in literature, the biliary
tract abnormalities might not be so uncommon. A routine MRCP in a child of AS with typical or atypical
biliary tract involvement is thus advisable.
Description
Keywords
Ahlstrom's Syndrome, Obesity
Citation
Shah Nisar, Javid Gul, Parveen Shagufta, Riyaz u saif, Wani Zeeshan, Iqbal Mohd yousuf Asif, Shouket Abid. Ahlstrom's Syndrome with Type IV Choledochal Cyst. JK Science Journal of Medical Education and Research. 2010 Oct-Dec; 12(4): 191-193