title: Measures of growth in children at risk for Huntington disease.
abstract: OBJECTIVE
  The effect of mHTT on human development was examined by evaluating measures of growth in children at risk for Huntington disease (HD).
  
  
  METHODS
  Children at risk for HD with no manifest symptoms (no juvenile HD included) were enrolled and tested for gene expansion for research purposes only. Measurements of growth (height, weight, body mass index [BMI], and head circumference) in children tested as gene-expanded (n = 20, 7-18 years of age, CAG repeats ≥39) were compared to those of a large database of healthy children (n = 152, 7-18 years of age).
  
  
  RESULTS
  Gene-expanded children had significantly lower measures of head circumference, weight, and BMI. Head circumference was abnormally low even after correcting for height, suggesting a specific deficit in brain growth, rather than a global growth abnormality.
  
  
  CONCLUSIONS
  These results indicate that, compared to a control population, children who were estimated to be decades from HD diagnosis have significant differences in growth. Further, they suggest that mHTT may play a role in atypical somatic, and in particular, brain development.
authors: Department of Psychiatry, University of Iowa, Carver College of Medicine, Iowa City, USA. jessica-k-lee@uiowa.edu
authors: Jessica K
authors: JK
authors: Lee
authors: Kathy
authors: K
authors: Mathews
authors: Bradley
authors: B
authors: Schlaggar
authors: Joel
authors: J
authors: Perlmutter
authors: Jane S
authors: JS
authors: Paulsen
authors: Eric
authors: E
authors: Epping
authors: Leon
authors: L
authors: Burmeister
authors: Peg
authors: P
authors: Nopoulos
mesh_terms: D000293:Adolescent; D041923:Adolescent Development / Q000502:physiology*; D001827:Body Height / Q000502:physiology*; D015992:Body Mass Index; D001835:Body Weight / Q000502:physiology*; D002508:Cephalometry; D002648:Child; D002657:Child Development / Q000502:physiology*; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000503:physiopathology*; D008297:Male; D012306:Risk