No abstract available.
Diagnosis*

No abstract available.
Child* ; Humans ; Male*

No abstract available.
Child* ; Humans

No abstract available.
Diabetes Mellitus* ; Insulin*

No abstract available.
Animals ; Growth Hormone* ; Hypothyroidism* ; Rats*

No abstract available.

No abstract available.
Silver-Russell Syndrome*

The final adult height in the children with true precocious puberty are destined to be short due to excessive bone maturation, compared to the growth velocity, regardless of its etiologies. To improve this final shortness, long-acting GnRH analog have been tried to the children with true precocious puberty. We evaluated the parameters of the growth. including the growth velocity, height SDS, predicted final adult height obtained by Bayley-Pinneau method in the 12 children with true precocious puberty after treatment of long-acting GnRH analog, Decapeptyl, The results were as belows; 1) The mean age of pubertal onset was 5.0 +/-2.9 year of age (1~8.6 years of age). The bone age (10.2+/-3.5 years of age) at diagnosis were significantly higher than the chronological age (7.2 +/-3.0)(Fig. 1,p<0.001). 2) During treatment with Decapeptyl, the progression of bone maturation seemed to be reduced, compared to the progression of chronological age, but there was no statistically significant difference (p>0.05). 3) The responses of LH and FSH to GnRH administration at 6 months of treatment with Decapeptyl were significantly reduced to prepubertal level, compared to those before the initiation of Decapeptyl treatment. 4) The height SDS before and at the first year of treatment with Decapeptyl were 1.5+/-0.3 and 1.4 +/-0.2, which had no significant change during treatment (Fig, 3, p>0.05). But the height velocity during the first year of treatment (4.9+/-1.7 cm/year) was significantly reduced, compared to the height velocity during the one year before treatment (10.1+/-1.5 cm/year)(Fig, 4, p=0.01). 5) The predicted final adult height, obtained by Bayley-Pinneau method, at second year of treatment (174.4 +/-1.8 cm) were significantly improved, compared to those at initial treatment (151.7 +/-2.3 cm) and 6 months of treatment (156.9+/-2.5 cm)(Fig, 5, p<0.05). 6) The predicted final adult height, obtained at the first year of treatment had significant inverse correlation with the bone age at the initiation of treatment with Decapeptyl (Fig. 6, p<0.05,r=-0.84), but had no corrleation with the chronological age at the initiation of treatment. 7) During this study, we could not find any adverse reaction, which could come with the therapy of Decapeptyl, such as facial flushing and hypotension. With these result, we can conclude that the final adult height can be improved if true precocious puberty could be diagnosed early and treatment with long-acting GnRH analog be given early.
Adult* ; Child* ; Diagnosis ; Flushing ; Gonadotropin-Releasing Hormone ; Humans ; Hypotension ; Puberty, Precocious* ; Triptorelin Pamoate

We studied the correlation between the bone age and the predicted adult height, final adult height in the 69 children (30 salt losing form and 39 non-salt losing form) diagnosed as 21-hydroxylase deficiency, retrospectively. The results were as belows; 1) The bone age was similar to the chronological age in the children with salt-losing form (5.3+/-2.3 and 6.3+/-3.5 years, respectively), but the bone age was much more advanced, compared to the chronological age in the children with non-salt losing from (7.8+/- 2.9 and 12.0 +/-3.5 years, respectively)(Table 1.p<0.001) 2) In the children with salt-losing form, the height SDS for the chronological age and bone age, were -0.3 +/-1.3 and -1.1+/-2.6, respectively, which has no difference. In the children with non-salt losing form, the height SDS for the bone age, were much lower than the height SDS for the chronological age (-2.1+/-1.3 and 1.5+/-1.5, respectively)(Table 2.p<0.001). 3) The incidence of true precocious puberty is significantly higher in the children with non-salt losing from (26 children) than in the children with salt losing from (6 children)(p<0.05). 4) All children with salt losing form received hydrocortisone and mineralocorticoid within the first month of life, but the average age at the first hydrocortisone therapy in the children with non-salt losing form was 5.0 3.3(0.1~13.1) years of age. The dosages of hydrocortisone were similar in two forms (24.3 +/-7.6 mg/m2 in non-salt losing form). 5) The predicted adult height, obtained by BP method, was shortest among three methods predicting adult height (RWT; 181.5 +/-14.6 cm, TW 169.2 13.2 cm, BP; 151.6 +/-9.3 cm)(Fig.1.p<0.001). 6) Seventeen children with 21-hydroxylase deficiency attained 152.1+/-8.8 cm of final adult height (152.2+/-1.5 cm in 12 salt losing form and 152+/-1.4 cm in 5 non-salt losing form). These final adult heights were closest to the predicted adult height, obtained by BP method, compared to other two methods (Fig. 2.p<0.05). 7) Among 7 children, whose mid-parental height could be obtained, one child could reach within the target height and other 6 children reached far below the target height (Fig. 3.p<0.005). In conclusion, to attain normal growth and normal final adult height, in is suggested that meticulous control should be needed for adequate suppression of adrenal androgen and mineralocorticoid should be given to all children who have high level of plasma renin activity in the children with 21-hydroxylase deficiency, with regular follow-up of laboratory tests and growth parameter. Additionally, even if patient has non-salt losing form, the diagnosis should be made and adequate hormonal therapy should be given as soon as after birth.
Adrenal Hyperplasia, Congenital* ; Adult* ; Child* ; Diagnosis ; Follow-Up Studies ; Humans ; Hydrocortisone ; Incidence ; Parturition ; Plasma ; Puberty, Precocious ; Renin ; Retrospective Studies ; Steroid 21-Hydroxylase*

The diagnostic value of GHRH in assessing GH secretion in biochemical GH sufficient short children was examined. GHRH (1microgram/kg i.v bolus) was given to three groups (upslope, trough, downslope) arbitrarily classified according to the basal pulsatile GH secretory pattern before GHRH administration. Cmax following GHRH administration were variable and overlapping. Two children in downslope group, three children in trough group, and one child in upslope group showed subnormal GH responses to GHRH administration despite of normal GH response to more than two classical GH provocative tests (Fig.1). The time of maximal GH response after GHRH administration (Tmax) in upslope group was significantly faster than those in other two groups (Fig.2). There was a highly significant correlation between AUC and Cmax (p<0.001) after GHRH administration (Fig.3) which suggests that AUC or Cmax can be used for parameters of GH response to GHRH each other. The AUC and Cmax after GHRH administration between three groups were significantly different (2764+/-579.1ng/ml min, 52.6 ng/ml, respectively in upslope group; 1645+/-383.9ng/ml min, 37.7+/-9.4ng/ml, respectively in downslope group; 1098+/-150.2ng/ml min, 26.3+/-4.5ng/ml, respectively in trough group)(p<0.005) (Fig.4, Table 1). In conclusion, GH responses to GHRH adminstration could be variable according to the basal GH secretory rhythm. Therefore, we should be cautious in interpreting the GH response to GHRH to evaluate the GH secretory capacity because subnormal GH response to GHRH administration could be observed even if normal GH response to classical GH provocative tests. In addition, the classification of these arbitary three groups (upslope, trough, and downslope) is remained to defined so as to promote the diagnostic value of GHRH in GH deficiency.
Area Under Curve ; Child* ; Classification ; Growth Hormone* ; Humans