224 Final Height in Two Growth Hormone-Deficient Patients with Barter's Syndrome Treated with Growth Hormone in Combination with Lutenizing Hormone-Releasing Hormone Analog

We report two patients with Barter's syndrome who have attained to normal adult height by treated with growth hormone (GH) and lutenizing hormone-relasing hormone analog (LHRHa). One is a female patient who was diagnosed as having Barter's syndrome at the age of 9 months, the other is a male patient diagnosed at 5 years old. Despite administration of KCl, spironolactone and indomethacin, hypokalaemia was persistent and growth retardation was not improved in each patient. Their GH responses to insulin, clonidine and GRF stimulation test showed less than normal, so the female patient was also diagnosed as having growth hormone deficiency (GHD) at the age of 8 years and 6 months with -3.4 height SD score and the male patient wag diagnosed at the age of 11 years and 9 months with -2.5 height SD score. After starting GH replacement followed by administration of LHRHa, linear growth eatch-up occurred in the both patients. The female patient's height SD score increased by 2.9SD to -0.5SD (154.4cm) at the age of 18 years and the male patient's height SD score increased by 2.2SD to -0.3SD (168.8cm) at the age of 20 years. Prolonged hypokalaemia did not disturb the efficacy of GH replacement. Serious growth retardation during childhood is regulary observed in Barter's syndrome. When diagnosed as also having GHD, administration of GH in combination with LHRHa may have contributed to the encouraging outcome in final height.