title: HYDROCORTISONE THERAPY AND GROWTH TRAJECTORY IN CHILDREN WITH CLASSICAL CONGENITAL ADRENAL HYPERPLASIA.
abstract: OBJECTIVE
  Poor linear growth is one of the main concerns in children with congenital adrenal hyperplasia (CAH). We aimed to analyze factors affecting growth trajectory in children with classical CAH.
  
  
  METHODS
  Clinical records of children followed from infancy up to the end of growth at two Italian tertiary referral hospitals were reviewed. Fifty-seven patients (31 males), treated with hydrocortisone and fludrocortisone only, were included. Clinical observations were divided into three groups: 0 to 2 years, 172 observations; from 2 years to puberty onset, 813 observations; after puberty onset, 527 observations. Height velocity, pubertal growth spurt, and final height were evaluated as outcomes.
  
  
  RESULTS
  Final height standard deviation score (SDS) was lower than target height SDS (-0.74 ± 1.1 versus -0.31 ± 1.01; P<.001). Target-adjusted final height SDS was -0.44 ± 1.8 in males and -0.13 ± 1.1 in females (P = .001). Total pubertal growth was 21.9 ± 7.3 cm in males and 19.2 ± 8.2 cm in females (P = .19). Hydrocortisone dose increased and height-velocity SDS decreased during puberty. At multivariable analysis, height-velocity SDS was adversely affected by hydrocortisone dose (P = .038) and directly related to adrenocorticotropic hormone (ACTH) levels (P = .023). Target-adjusted final-height SDS was adversely affected by hydrocortisone dose (P<.001) and positively related to mineralocorticoid therapy (P = .001) and ACTH levels (P = .02). Total pubertal growth (cm) was positively related to ACTH levels (P = .01).
  
  
  CONCLUSIONS
  Height outcome of CAH patients is now better than previously reported. During puberty, the lowest effective dose of hydrocortisone should be used to optimize pubertal growth spurt and final height.
  
  
  BACKGROUND
  17-OHP = 17-alpha-hydroxyprogesterone ACTH = adrenocorticotropic hormone BMI = body mass index CAH = congenital adrenal hyperplasia GH = growth hormone HPA = hypothalamus-pituitary-adrenal PRA = plasma renin activity SDS = standard deviation score SV = simple virilizing SW = salt-wasting.
authors.forename: Carla
authors.initials: C
authors.lastname: Bizzarri
authors.forename: Nicola
authors.initials: N
authors.lastname: Improda
authors.forename: Chiara
authors.initials: C
authors.lastname: Maggioli
authors.forename: Donatella
authors.initials: D
authors.lastname: Capalbo
authors.forename: Silvia
authors.initials: S
authors.lastname: Roma
authors.forename: Ottavia
authors.initials: O
authors.lastname: Porzio
authors.forename: Mariacarolina
authors.initials: M
authors.lastname: Salerno
authors.forename: Marco
authors.initials: M
authors.lastname: Cappa
mesh_terms: D000312:Adrenal Hyperplasia, Congenital / Q000188:drug therapy*; D001827:Body Height / Q000187:drug effects; D002648:Child; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000008:administration & dosage*; D008297:Male; D011627:Puberty / Q000187:drug effects; D012189:Retrospective Studies; D012741:Sexual Maturation
chemical_list: D006854:Hydrocortisone