title: [Long-term follow-up of isolated-growth hormone deficiency typeIA: the clinical analysis of 2-sister cases].
abstract: OBJECTIVE
  To elucidate the curative and adverse effect of recombinant human growth hormone (rhGH) in 2 patients with isolated-growth hormone deficiency type IA (IGHDIA), to track sexual development and pregnancy, and reassess the quality of life in the adulthood.
  
  
  METHODS
  The authors summarized the data of 2-sister cases with IGHDIA; followed up for assessment of height, weight, blood pressure and sexual development; detected fasting blood lipids, glucose, insulin, insulin growth factor-1 (IGF-1) and insulin-like growth factor binding protein-3 (IGFBP-3); made an investigation of education and occupation, and so on.
  
  
  RESULTS
  After 6.2 and 7.3 years treatment with rhGH, the two sisters had considerably improved height from -7.8 SDS, -8.8 SDS to -2.6 SDS and -1.3 SDS respectively. No evident side effect was observed. They had normal sexual development and pregnancy. The levels of IGF-1 and IGFBP-3 were still low, in the elder sister they were 46.6 µg/L, 2460 µg/L, and in the younger 52.4 µg/L, 2430 µg/L. No hyperlipidemia, diabetes or obesity occurred.
  
  
  CONCLUSIONS
  Long term therapy with rhGH may improve final adult height of individuals with IGHDIA. They can have normal sexual development and pregnancy. Metabolic syndrome did not occur during the follow-up period.
authors.affiliation: Department of Pediatrics, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China.
authors.forename: Guo-ying
authors.initials: GY
authors.lastname: Chang
authors.forename: Zhi-ya
authors.initials: ZY
authors.lastname: Dong
authors.forename: Wei
authors.initials: W
authors.lastname: Wang
authors.forename: De-fen
authors.initials: DF
authors.lastname: Wang
mesh_terms: D002648:Child; D004393:Dwarfism, Pituitary / Q000145:classification* / Q000628:therapy; D005260:Female; D005500:Follow-Up Studies; D019382:Human Growth Hormone / Q000627:therapeutic use; D006801:Humans; D035781:Siblings
chemical_list: D019382:Human Growth Hormone