Although GH treatment for short stature in Turner syndrome is an accepted treatment in many countries, which GH dosage to use and which age to start puberty induction are issues of debate. This study shows final height (FH) in 60 girls with Turner syndrome treated in a randomized dose-response trial, combining GH treatment with low dose estrogens at a relatively young age.Girls were randomly assigned to group A (4 IU/m 2 ⅐d; ϳ0.045 mg/kg/d), group B (first year, 4 IU/m 2 ⅐d; thereafter 6 IU/m 2 ⅐d), or group C (first year, 4 IU/m 2 ⅐d; second year, 6 IU/m 2 ⅐d; thereafter, 8 IU/m 2 ⅐d). After a minimum of 4 yr of GH treatment, at a mean age of 12.7 ؎ 0.7 yr, low dose micronized 17␤-estradiol was given orally. After a mean duration of GH treatment of 8.6 ؎ 1.9 yr, FH was reached at a mean age of 15.8 ؎ 0.9 yr. FH, expressed in centimeters or SD score, was 157.6 ؎ 6.5 or ؊1.6 ؎ 1.0 in group A, 162.9 ؎ 6.1 or ؊0.7 ؎ 1.0 in group B, and 163.6 ؎ 6.0 or -0.6 ؎ 1.0 in group C. The difference in FH in centimeters, corrected for height SD score and age at start of treatment, was significant between groups A and B [regression coefficient, 4.1; 95% confidence interval (CI), 1.4, 6.9; P < 0.01], and groups A and C (coefficient, 5.0; 95% CI, 2.3, 7.7; P < 0.001), but not between groups B and C (coefficient, 0.9; 95% CI, ؊1.8, 3.6). Fifty of the 60 girls (83%) had reached a normal FH (FH SD score, more than ؊2). After starting estrogen treatment, the decrease in height velocity (HV) changed significantly to a stable HV, without affecting bone maturation (change in bone age/change in chronological age). The following variables contributed significantly to predicting FH SD score: GH dose, height SD score (ref. normal girls), chronological age at start of treatment, and HV in the first year of GH treatment. GH treatment was well tolerated.In conclusion, GH treatment leads to a normalization of FH in most girls, even when puberty is induced at a normal pubertal age. The optimal GH dosage depends on height and age at the start of treatment and first year HV. (2), subnormal levels of GH and IGF-I have been reported (3, 4). It has been postulated that a diminished sensitivity for growth factors might explain their growth retardation (5, 6). Nevertheless, GH treatment in a supraphysiological dosage has been shown to accelerate growth (4, 7). Another clinical feature in most girls with TS is the absence of spontaneous pubertal development, for which estrogen substitution is necessary. Although GH treatment for short stature in TS is now an accepted treatment in many countries, reports on final height are inconsistent (8,9), and which dosage to use and which age to start puberty induction are issues of debate.Previously, we have demonstrated that long-term GH treatment in TS leads to normalization of height (4, 10). This study shows final height (FH) results in 60 girls with TS treated in a randomized dose-response trial comparing 3 dosage schedules. In addition, we show the effect of low dose estrogen treatment begun at a relatively ...

Background: Knowledge about the effects of GH treatment on cognitive functioning in children with Prader-Willi syndrome (PWS) is limited.

This 8-year study demonstrates that GH treatment is a potent force for counteracting the clinical course of obesity in children with PWS.

Our study in children with PWS shows that 4 yr of continuous GH treatment (1 mg/m(2) . d) improves body composition by decreasing fat%SDS and stabilizing LBMSDS and head circumference SDS and normalizes heightSDS without adverse effects. Thus, long-term continuous GH treatment is an effective and safe therapy for children with PWS.

SummaryOBJECTIVE In order to determine if a serious disease like childhood acute lymphoblastic leukaemia (ALL) and the treatment necessary to cure the patients has long term effects on bone mass, we assessed bone mineral density (BMD) and several parameters involved in bone formation in a group of young adult survivors of ALL. DESIGN AND PATIENTS Fourteen male and ten female survivors, treated for ALL in childhood, were cross-sectionally studied, at a mean age of 25·1 years (range 20·1-34·9). All patients, except for two, had received cranial irradiation as part of their treatment (mean radiation dose 2460 cGy).MEASUREMENTS Height and weight were measured. Bone mineral density (BMD) was assessed using dual energy X-ray absorptiometry in the lumbar spine, femoral neck, femoral trochanter and at 1/3 distal and ultradistal in the radius. Early morning serum levels of LH, FSH, oestradiol or testosterone, IGF-1 and IGF-BP3 were determined as well as several specific markers of bone turnover.RESULTS Mean height, expressed as standard deviation score (SDS) was ¹1·12, significantly reduced. BMD in the lumbar spine, femoral neck and at 1/3 distal and ultradistal in the radius, was significantly lower compared to the reference population (P < 0·05). No correlation was found between the BMD values and the cumulative dose of administered cytotoxic drugs, the age at diagnosis of ALL or the duration of follow-up. Mean IGF-1 and IGF-BP3 SDS-scores were ¹1·24 and ¹0·78 respectively, significantly reduced. GH stimulation tests performed in a subgroup of 9 patients showed an insufficient peak GH response in at least one test in all tested patients. The values of LH, FSH oestradiol or testosterone were within the normal adult range. Serum markers of bone formation and bone resorption were in the normal range, indicating that bone turnover was normal at the time of the study.CONCLUSIONS Bone development in patients cured of acute lymphoblastic leukaemia is disturbed, resulting in a significantly reduced bone mineral density. Impaired growth hormone activity, as a long term effect of cranial irradiation, may be one of the underlying causes as well as the illness itself and the administered cytotoxic drugs. Since a reduced bone mineral density predispose patients to osteoporosis, intervention in order to improve bone mass should be considered.