Progressive Liver Disease in Patients With Ataxia Telangiectasia.

ABSTRACT: Ataxia telangiectasia (A-T) is a devastating multi-system disorder characterized by progressive cerebellar ataxia, immunodeficiency, genetic instability, premature aging and growth retardation. Due to better care the patients get older than in the past and new disease entities like disturbed glucose tolerance and liver disease emerge. The objective of the present investigation is to determine the evolution of liver disease and its relation to age and neurological deterioration. The study included 67 patients aged 1 to 38 years with classical A-T. At least two measurements of liver enzymes were performed within a minimum interval of 6 months in 56 patients. The median follow-up period was 4 years (1-16 years). A total of 316 liver enzyme measurements were performed. For analysis, patients w

SUBMITTER: Donath H 

PROVIDER: S-EPMC6856634 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature