EFFECT OF GROWTH-HORMONE ON IGF-I LEVELS IN A PATIENT WITH GROWTH-HORMONE DEFICIENCY AND WILSON DISEASE

This is a case report of a boy with a combination of two rare disorder s: growth hormone deficiency (GHD) and Wilson disease. To our knowledg e, no comparable case has yet been published in the literature. GHD wa s 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 reconfirm ed and GH therapy could be initiated (dose 0.6 IU/kg/week). At that ti me, 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 f rom -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 elevat ed. At the age of 10 years, Wilson disease was diagnosed in view of lo w concentration of serum ceruloplasmin, elevated urinary copper excret ion and high copper content in a liver biopsy sample. Under a combined therapy with D-penicillamine and GH, serum Liver enzymes decreased, a nd IGF-I levels increased to normal. Height SDS for chronological age has improved constantly.