We evaluated 12 children with TS (age 15-23 yr) who received GH(0.375mg/kg/day) for 5-7 years, as participants in a larger multicenter trial; 4 were also receiving Oxandrolone (0.0625mg/kg/day). All except one currently are on HRT. M-mode and two dimensional echocardiographic studies were performed using standard views. When compared to age related controls, and corrected for body surface area, no evidence of VH was seen in serial evaluations.Follow up echo studies up to 7 yrs after discontinuation of GH show no VH or aortic root dilatation.

It had been previously shown that glucose stimulated insulin response is increased in patients with TS, and that these alterations are further exaggerated by treatment with GH(J. Ped. 1992, 120:238). Typically, insulin levels rose into the high normal range for postprandial insulin levels, plateauing at 36 mos. Therefore, carbohydrate tolerance and insulin levels were evaluated in the same cohort at a mean of 44 months(range= 5-81 mos.) after completion of growth promoting therapy. In nine patients, both fasting and postprandial (glucose load 1.75g/kg) insulin levels after GH treatment returned to pretreatment levels (mean pre-GH insulin(mU/L) fasting 8.6±3.8,postprand. 30±13.2; post-GH insulin 11.1±8.9, 47.8±16.6 p:ns). Fasting and postprandial glucose levels as well as HgbA1c did not change during GH therapy when compared to baseline. One patient with persistent hyperinsulinism was obese with a BMI of 33. In summary, in this cohort of patients with TS, GH treatment did not lead to VH or irreversible carbohydrate intolerance.(Partial funding by Genentech, Inc.)

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