Response to growth hormone with respect to pubertal status on increased dose in idiopathic growth hormone deficiency: an analysis of Turkish children in the KIGS database (Pfizer International Growth Study).

AIM: To compare the growth response to growth hormone (GH) treatment in patients with idiopathic GH deficiency (IGHD) who were prepubertal with the response of those who were pubertal at the onset of GH therapy on an increased GH dose. PATIENTS AND METHODS: Among the Turkish patients enrolled in the Pfizer International Growth Study (KIGS) database with the diagnosis of IGHD, the growth data over 2 years of GH therapy were analyzed longitudinally of 113 (79 M) prepubertal (Group 1) and 44 (33 M) pubertal (Group 2) patients. Pubertal signs were reported to be present initially or to have appeared within 6 months of GH therapy in Group 2. Mean +/- SD age at onset of therapy was 8.7 +/- 3.5 and 13.5 +/- 1.8 years; height SDS -4.2 +/- 1.4 and -3.2 +/- 1.1 (p < 0.05) in Groups 1 and 2, respectively. Mid-parental height (MPH) SDS did not show a significant difference between the two groups (-1.5 +/- 1.1 vs -1.7 +/- 1.1). RESULTS: Delta height SDS over 2 years of therapy was significantly higher in Group 1 (1.1 +/- 1.0) than in Group 2 (0.7 +/- 0.6) (p <0.05) in spite of a significantly lower dose of GH (14.6 +/- 3.3 in Group 1 vs 17.0 +/- 3.1 IU/m2/week in Group 2, p < 0.05). Ht--MPH SDS showed an increase from -2.4 +/- 1.7 to -1.4 +/- 1.5 in Group 1 and from -1.5 +/- 1.5 to -0.8 +/- 1.3 in Group 2. Overall delta height SDS showed negative correlations with age (r = -0.32), height SDS (r = -0.41) and height--MPH SDS (r = -0.40) at onset of therapy (p < 0.001). CONCLUSIONS: These data show that in IGHD the slight increase (15-20%) in the dose of GH during puberty was not adequate to maintain height velocity at the same magnitude as in prepuberty, and thus was not cost effective.

Insulin secretion and insulin resistance determined by euglycemic clamp.

Obesity among children is increasingly recognized and linked to several metabolic problems. In this study, 47 children, aged 5-14 yr, with exogenous obesity were compared to 20 normal (non-obese) children to show alterations in glucose metabolism. All the obese children had body mass index > 95th percentile and weight for age > 120%. Basal and stimulated insulin and C-peptide levels were obtained during oral glucose tolerance test (OGTT). Seven children from the obese group had impaired OGTT according to WHO criteria. Mean fasting insulin levels were 26.7 +/- 14.6 microIU/ml in obese and 10.99 +/- 4.36 microIU/ml in controls; postprandial insulin levels were 70.4 +/- 56.4 microIU/ml and 22.23 +/- 6.55 microIU/ml, respectively (p < 0.001). The euglycemic glucose clamp technique was applied to 8 normal and 22 obese children. The amount of metabolized glucose (M) during clamp test is measured to identify glucose sensitivity. Mean M values were 3.24 +/- 1.35 mg/kg/min in obese and 6.525 +/- 0.770 mg/kg/min in control children (p < 0.001). As a result of this study, it seems reasonable to consider all obese children and adults as being at risk for hyperinsulinism and insulin resistance.

Steroid-free interval with anti-D in chronic idiopathic thrombocytopenic purpura.

Chronic idiopathic thrombocytopenic purpura (ITP) is an autoimmune disease characterized by the antibody-mediated destruction of platelets. To maintain the platelets above the symptomatic level we administered 100 micrograms of anti-D for 5 consecutive days to 19 children with ITP. Four patients did not respond to the treatment. Fifteen responded with an increase in the average platelet number to 76,000/microL 7 days postinjection. However, the platelet count dropped within 45 days to 27,000/microL. Three months after this study, two patients from the study group were then administered monthly anti-D after reinjecting anti-D daily for 5 consecutive days, as previously performed. Platelet levels in these two patients were maintained above 30,000/microL for 5 and 6 months respectively. We concluded that anti-D administration for 5 consecutive days can induce an increase in platelets followed by a decrease below 30,000/microL after 30-45 days. However, monthly administration of anti-D after daily injections for 5 consecutive days can keep platelets above the symptomatic level and may provide a corticosteroid-free safe interval for nearly 5 months.