title: Effect of growth hormone on IGF-I levels in a patient with growth hormone deficiency and Wilson disease.
abstract: This is a case report of a boy with a combination of two rare disorders:growth hormone deficiency (GHD) and Wilson disease. To our knowledge, no comparable case has yet been published in the literature. GHD was diagnosed at the age of 4.5 years (height standard deviation score (SDS) -4.85). However, because of a difficult family background, growth hormone (GH) therapy could not be started. The boy was not seen again until the age of 7.7 years (height SDS -4.77), when GHD was reconfirmed and GH therapy could be initiated (dose 0.6 IU/kg/week). At that time, elevated liver enzymes (GPT 128 U/l, GOT 67 U/l, gamma-GT 28 U/l) confused diagnostic procedures. On GH, growth velocity SDS increased from -1.86 to +4.50 in the first year and +3.87 in the second year, and height SDS increased to -4.26 and -3.59. However, serum IGF-I levels did not normalize (max. 67 ng/ml), and liver enzymes were still elevated. At the age of 10 years, Wilson disease was diagnosed in view of low concentration of serum ceruloplasmin, elevated urinary copper excretion and high copper content in a liver biopsy sample. Under a combined therapy with D-penicillamine and GH, serum liver enzymes decreased, and IGF-I levels increased to normal. Height SDS for chronological age has improved constantly.
authors.affiliation: Children's Hospitals, Division of Pediatric Endocrinology, Frankfurt, Germany.
authors.forename: A
authors.initials: A
authors.lastname: Koch
authors.forename: H G
authors.initials: HG
authors.lastname: Dörr
authors.forename: S
authors.initials: S
authors.lastname: Gerling
authors.forename: R
authors.initials: R
authors.lastname: Behrens
authors.forename: H J
authors.initials: HJ
authors.lastname: Böhles
mesh_terms: D002570:Ceruloplasmin / Q000378:metabolism; D002675:Child, Preschool; D003300:Copper / Q000032:analysis / Q000652:urine; D013006:Growth Hormone / Q000172:deficiency* / Q000627:therapeutic use*; D006527:Hepatolenticular Degeneration / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D007334:Insulin-Like Growth Factor I / Q000378:metabolism*; D008099:Liver / Q000737:chemistry / Q000201:enzymology; D008297:Male; D010396:Penicillamine / Q000627:therapeutic use; D015430:Weight Gain
chemical_list: D007334:Insulin-Like Growth Factor I; D003300:Copper; D013006:Growth Hormone; D002570:Ceruloplasmin; D010396:Penicillamine