title: Metabolic effects of growth hormone (GH) replacement in children and adolescents with severe isolated GH deficiency due to a GHRH receptor mutation.
abstract: BACKGROUND
  The interpretation of the true effect of GH replacement therapy (GHRT) on metabolic status in GH deficiency (GHD) is often complicated by differing aetiologies of GHD and by the presence of additional hormone deficits.
  
  
  OBJECTIVE
  To study the growth and response of the lipid profile and body composition to GHRT in a cohort of children with the same mutation in the GHRH receptor gene. Design Nine GH-deficient subjects (mean age 12.8 years, range 5-17.5 years; three male) in a rural community in Northeast Brazil were treated with GHRT for 2 years and compared with indigenous normal controls.
  
  
  METHODS
  Total cholesterol (TC), low density lipoprotein cholesterol (LDL-C), high density lipoprotein cholesterol (HDL-C), triglycerides (TG) and body composition were measured at baseline and after 3, 12 and 24 months of GHRT.
  
  
  RESULTS
  At baseline, the subjects with GHD had an adverse lipid profile, including elevated TC, elevated LDL-C and elevated TG. GHRT normalized TG in 3 months, LDL-C in 12 months and TC in 24 months. At baseline, older pubertal subjects with GHD had adverse body composition, including higher percentage fat mass (%FM), and GHRT induced a reduction in %FM that was maintained after 24 months. By contrast, younger prepubertal subjects did not have an adverse body composition.
  
  
  CONCLUSIONS
  Lipid profile was abnormal at baseline, while abnormal body composition was only seen in older subjects in late puberty, indicating that body composition is less sensitive to the effect of GHD than lipid profile. GHRT improves lipid profile at all ages, while it affects body composition only towards the end of growth, emphasizing its importance in achieving normal somatic development in the transition period.
authors: Christie Hospital, Department of Endocrinology, Manchester, UK. helena@kgleeson99.freeserve.co.uk
authors: Helena
authors: H
authors: Gleeson
authors: Elenilde S de A
authors: ES
authors: Barreto
authors: Roberto
authors: R
authors: Salvatori
authors: Liana
authors: L
authors: Costa
authors: Carla R P
authors: CR
authors: Oliveira
authors: Rossana M C
authors: RM
authors: Pereira
authors: Peter
authors: P
authors: Clayton
authors: Manuel H
authors: MH
authors: Aguiar-Oliveira
mesh_terms: D000293:Adolescent; D000704:Analysis of Variance; D001823:Body Composition / Q000187:drug effects; D016022:Case-Control Studies; D002648:Child; D002784:Cholesterol / Q000097:blood; D008076:Cholesterol, HDL / Q000097:blood; D008078:Cholesterol, LDL / Q000097:blood; D005260:Female; D017353:Gene Deletion; D006442:Glycated Hemoglobin / Q000032:analysis; D006130:Growth Disorders / Q000097:blood / Q000188:drug therapy / Q000235:genetics*; D013006:Growth Hormone / Q000172:deficiency*; D006720:Homozygote; D020249:Hormone Replacement Therapy; D019382:Human Growth Hormone / Q000627:therapeutic use; D006801:Humans; D007334:Insulin-Like Growth Factor I / Q000032:analysis; D008297:Male; D009154:Mutation; D018013:Receptors, Neuropeptide / Q000235:genetics*; D018017:Receptors, Pituitary Hormone-Regulating Hormone / Q000235:genetics*; D016896:Treatment Outcome; D014280:Triglycerides / Q000097:blood
chemical_list: D002784:Cholesterol; D008076:Cholesterol, HDL; D008078:Cholesterol, LDL; D006442:Glycated Hemoglobin; D013006:Growth Hormone; D019382:Human Growth Hormone; D007334:Insulin-Like Growth Factor I; D018013:Receptors, Neuropeptide; D018017:Receptors, Pituitary Hormone-Regulating Hormone; D014280:Triglycerides; C048250:somatotropin releasing hormone receptor