Body composition, muscle strength and hormonal status in patients with ataxia telangiectasia: a cohort study.

ABSTRACT:

Background

Ataxia-telangiectasia (A-T) is a devastating human autosomal recessive disorder that causes progressive cerebellar ataxia, immunodeficiency, premature aging, chromosomal instability and increased cancer risk. Affected patients show growth failure, poor weight gain, low body mass index (BMI), myopenia and increased fatigue during adolescence. The prevalence of alterations in body composition, muscle strength and hormonal status has not been well described in classical A-T patients. Additionally, no current guidelines are available for the assessment and management of these changes.

Methods

We analyzed body composition, manual muscle strength and hormonal status in 25 A-T patients and 26 age-matched, healthy controls. Bioelectrical impedance analysis (BIA) was perfo

SUBMITTER: Pommerening H 

PROVIDER: S-EPMC4673730 | biostudies-literature | 2015 Dec

REPOSITORIES: biostudies-literature