title: Vascular endothelial growth factor (VEGF) levels in short, GH treated children: a distinct pattern of VEGF-C in Noonan syndrome.
abstract: BACKGROUND
  Noonan syndrome (NS) is characterized by short stature and elevated risk of lymphedema. The mechanism underlying lymphedema may be mediated by vascular endothelial growth factors (VEGFs).
  
  
  OBJECTIVE
  To assess the effect of growth hormone (GH) treatment on plasma insulin-like growth factor (IGF)-1, VEGF-A and VEGF-C levels in patients with NS as compared to short GH-sufficient children.
  
  
  METHODS
  Retrospective, comparative.
  
  
  METHODS
  Endocrinology department of a tertiary pediatric medical center.
  
  
  METHODS
  Plasma IGF-1, VEGF-A and VEGF-C levels were measured before and during GH treatment in 6 patients with NS and 18 age-matched short subjects (Turner, idiopathic short stature and small for gestational age).
  
  
  METHODS
  Changes in plasma VEGF and IGF-1 levels.
  
  
  RESULTS
  Baseline IGF-1 SDS levels were slightly lower in NS patients compared with controls; IGF-1 response to GH therapy was markedly lower in NS patients compared with controls (p = 0.017). Mean baseline VEGF-A levels were similar in NS patients and controls whilst mean baseline VEGF-C levels were significantly lower in the NS group as compared with controls (p = 0.022). Plasma VEGF-A and VEGF-C levels did not significantly change during GH treatment in the study cohort. No correlation was found between VEGF-C levels and levels of IGF-1, VEGF-A and auxological parameters, either before or during GH administration.
  
  
  CONCLUSIONS
  Children with NS have a distinct growth factor profile including low basal VEGF-C and flattened IGF-1 response to GH. Further studies are needed to confirm our findings and to elucidate the interaction between VEGF-C levels and lymphedema.
authors.affiliation: The Jesse Z. and Sara Lea Shafer Institute for Endocrinology and Diabetes, National Center for Childhood Diabetes, Schneider Children's Medical Center of Israel, 14 Kaplan Street, 49202, Petah Tikva, Israel.|Department of Molecular Genetics, Weizmann Institute of Science, 76100, Rehovot, Israel.
authors.forename: S
authors.initials: S
authors.lastname: Fuchs
authors.affiliation: The Jesse Z. and Sara Lea Shafer Institute for Endocrinology and Diabetes, National Center for Childhood Diabetes, Schneider Children's Medical Center of Israel, 14 Kaplan Street, 49202, Petah Tikva, Israel.|Felsenstein Medical Research Center, 49100, Petah Tikva, Israel.|Sackler Faculty of Medicine, Tel Aviv University, 69978, Tel Aviv, Israel.
authors.forename: G
authors.initials: G
authors.lastname: Gat-Yablonski
authors.affiliation: Felsenstein Medical Research Center, 49100, Petah Tikva, Israel.|Sackler Faculty of Medicine, Tel Aviv University, 69978, Tel Aviv, Israel.
authors.forename: B
authors.initials: B
authors.lastname: Shtaif
authors.affiliation: The Jesse Z. and Sara Lea Shafer Institute for Endocrinology and Diabetes, National Center for Childhood Diabetes, Schneider Children's Medical Center of Israel, 14 Kaplan Street, 49202, Petah Tikva, Israel.|Sackler Faculty of Medicine, Tel Aviv University, 69978, Tel Aviv, Israel.
authors.forename: L
authors.initials: L
authors.lastname: Lazar
authors.affiliation: The Jesse Z. and Sara Lea Shafer Institute for Endocrinology and Diabetes, National Center for Childhood Diabetes, Schneider Children's Medical Center of Israel, 14 Kaplan Street, 49202, Petah Tikva, Israel.|Felsenstein Medical Research Center, 49100, Petah Tikva, Israel.|Sackler Faculty of Medicine, Tel Aviv University, 69978, Tel Aviv, Israel.
authors.forename: M
authors.initials: M
authors.lastname: Phillip
authors.affiliation: The Jesse Z. and Sara Lea Shafer Institute for Endocrinology and Diabetes, National Center for Childhood Diabetes, Schneider Children's Medical Center of Israel, 14 Kaplan Street, 49202, Petah Tikva, Israel. yael_lebenthal@walla.co.il.|Sackler Faculty of Medicine, Tel Aviv University, 69978, Tel Aviv, Israel. yael_lebenthal@walla.co.il.
authors.forename: Y
authors.initials: Y
authors.lastname: Lebenthal
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D019382:Human Growth Hormone / Q000008:administration & dosage / Q000494:pharmacology*; D006801:Humans; D007334:Insulin-Like Growth Factor I / Q000032:analysis*; D008209:Lymphedema / Q000097:blood* / Q000188:drug therapy; D008297:Male; D009634:Noonan Syndrome / Q000097:blood* / Q000188:drug therapy; D012189:Retrospective Studies; D016896:Treatment Outcome; D042461:Vascular Endothelial Growth Factor A / Q000097:blood*; D042582:Vascular Endothelial Growth Factor C / Q000097:blood*
chemical_list: C583557:IGF1 protein, human; C467484:VEGFA protein, human; C467486:VEGFC protein, human; D042461:Vascular Endothelial Growth Factor A; D042582:Vascular Endothelial Growth Factor C; D019382:Human Growth Hormone; D007334:Insulin-Like Growth Factor I
keywords: Growth Hormone (GH); Insulin-like growth factor-1 (IGF-1); Lymphedema; Noonan Syndrome; Vascular Endothelial Growth Factor (VEGF)