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OBJECTIVE: To analyse the clinical characteristics,aetiology distribution,and metabolic risk parameters of patients with with premature pubarche(PP). METHODS: A retrospective study was performed in 55 patients affected by PP,who received ACTH stimulation test,and the data were evaluated. Among them 17 cases were detected CYP21 A2 gene,and indicators of lipid metabolism of idiopathic premature adrenarche(IPA,15 cases)and idiopathic premature pubarche(IPP,14 cases)were analyzed. RESULTS: The sample included 55 patients with PP(53 female and 2 male),with a mean pubarche age of(7.2±0.9)years for girls and(7±0.8)years for boys. There was an accelerated bone age(BA/CA ratio>1)(1.20 ± 1.5)in girls. A total of 9 patients were classified as non-classic congenital adrenal hyperplasia(NCCAH,16.4%),15 as IPA(27.3%),14 as IPP(25.4%),16 as HPGA function launch(29.1%)and 1 with exaggerated adrenarche(EA,1.8%). There was no significant difference in the diagnostic rate of NCCAH between ACTH stimulation test and CYP21 A2 gene detection(P=0.596). The sex hormone binding protein(SHBP)of IPA was reduced(P=0.007). CONCLUSION: The etiology of premature pubarche-non-classic congenital adrenal hyperplasia(16.4%)is not uncommon. The ACTH stimulation test is useful for the diagnosis of NCCAH,but the sensitivity is not 100%.
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[Objective] To investigate the effect of letrozole on the reproductive function and linear growth in the early and mid pubertal boys.[Methods] 43 early and middle pubertal boy with seriously damaged predict adult height,treated with letrozole 1.5 mg/m2/d Po ((>)2.5mg/d) were enrolled as treatment group.48 cases of healthy pubertal boys were enrolled as control.Growth parameters,sex hormone profiles,IGF-1,AMH and Inhibin B (INHB) were elevated at the beginning and after letrozole treatment.[Results] At baseline,no significant differences appeared in age,bone age,observation time,height for chronological age,height for bone age,midparental target height,BMI,or testis volume between two groups.After intervention,treatment group of bone age delayed,predict adult height increased,testicular volume increased and BMI increased compared with the control group (P=0.001,0.018,0.002,and 0.027,respectively).The serum FSH,△FSH,LH,△LH,LH/FSH,T,and △T in the treatment groups were much higher (all P<0.001),while the serum E2 and △ E2 levels were obviously lower than the control group (P=0.043 and P=0.033,respectively).17 cases of control group and 13 cases of treatment group had serum AMH,INHB level tested before and after letrozole treatment.Serum AMH level in the control group appeared with a decreasing trend with the progress of puberty,while the treatment group showed the opposite tendency.And the △ AMH was significant difference between control group and treatment group (P<0.001).The serum INHB in the two groups increased in varying degrees after the intervention,the INHB level in control group increased more than the treatment group,but the difference was not statistically significant (P=0.517).[Conclusion] Letrozole treatment can elevated levels of serum T with E2 reduce,bone age delay,predict adult height improved,and can obviously promote the secondary sex characters development in adolescent boys.And the longer letrozole treatment time,the more obvious growth effect.As to the reproductive function,letrozole may have inhibitory effect on testis maturity and cannot deny testis sertoli cells function affected with letrozole exposure.
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[Objective] To analyze blood lipid and its related factors in Chinese children and adolescents with Turner syndrome.[Methods] The untreated TS patients were divided into two groups according to age (<11 years old and 11~15 years old) and enrolled two groups of age-matched control girls,blood lipid and the incidence of dyslipidemia were compared between the four groups,the related factors of blood lipid were also analyzed.Moreover,TS patients were divided into two groups according to karyotype,including 45,XO karyotype (55 cases) and other karyotypes (53 cases),blood lipid and the incidence of dyslipidemia in two groups were compared.[Result] Compared to age-matched control girls,TS patients of age 11~15 years group had higher TG levels and higher incidence of hypertriglyceridemia and borderline-hypertriglyceridemia (P<0.05) and the incidence of borderline-hypercholesterolemia was also significantly higher (P<0.01).But there were no differences in blood lipid level,incidence of dyslipidemia and the incidence of borerline-dyslipidemia between TS patients who were less than 11 years old and age-matched control girls.Total cholesterol of TS patients was negatively related to bone age (P<0.05).Triglyceride of TS patients was positively related to waist circumference (P<0.01).TS patients of 45,XO karyotype had lower TG levels,higher HDL levels and lower incidence of low HDL,borderline-high non-HDL and borderline-hypertriglyceridemia compared with those of other karyotypes (P<0.05).[Conclusions] Triglyceride in TS patients of age 11-15 years were higher than the control subjects,which may be related to estrogen deficiency and chromosome karyotype.
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<p><b>OBJECTIVE</b>To evaluate the effect of combined use of stanazolol (ST) on the final adult height (FAH) in girls with idiopathic central precocious puberty (ICPP) and apparently decreased linear growth during gonadotropin-releasing hormone analog (GnRHa) therapy.</p><p><b>METHOD</b>Sixty-three girls with ICPP and decreased velocity of growth of height (HV<4 cm/yr) during GnRHa therapy were divided into 3 groups based on the following types of interventions:group 1 (n = 20), GnRHa+ST [25-30 µg/(kg·d) every 3-month followed by 3-month discontinuation], group 2 (n = 21), GnRHa+recombinant human growth hormone [rhGH, 1-1.1 U/(kg·w)], group 3 (n = 22), GnRHa alone.HV, the advancement of bone age (BA) for chronological age (CA) (ΔBA/ΔCA) and FAH were compared among groups.</p><p><b>RESULT</b>(1)Total duration of ST combination therapy was (12.22 ± 3.62) months, while total duration of combination of rhGH was (13.22 ± 6.80) months. (2)HV increased significantly in both group 1 [ (2.79 ± 0.60) cm/yr vs. (6.27 ± 1.98) cm/yr, P < 0.01] and in group 2 [(2.80 ± 0.50) cm/yr vs. (6.25 ± 1.98) cm/yr, P < 0.01] during combined therapy, but maintained at low levels in group 3 [(3.95 ± 1.10) cm/yr vs. (3.34 ± 0.95) cm/yr, P > 0.05].No significant differences of ΔBA/ΔCA were found among the three groups [0.25(0.11∼0.28), 0.22(0.15∼0.31),0.19(0.10∼0.32), P > 0.05]. (3)FAH was significantly higher than predicted adult height (PAH) before combined therapy, as well as higher than target height (THt) in both group 1 [(156.25 ± 2.90) cm vs. (150.78 ± 3.70) cm, P < 0.01, (156.25 ± 2.90) cm vs. (153.94 ± 2.62) cm, P < 0.01], and in group2 [ (157.33 ± 4.69) cm vs. (152.61 ± 3.92) cm, P < 0.01, (157.33 ± 4.69) cm vs. (154.39 ± 4.72) cm, P = 0.01].In group 3, FAH was similar to PAH [(153.88 ± 2.6) cm vs. (152.54 ± 5.86) cm, P > 0.05], and was less than THt [(153.88 ± 2.6) cm vs. (155.60 ± 4.52) cm, P = 0.02]. (4)In girls treated with ST, no hirsutism, clitorism or hoarse voice was recorded.No polycystic ovary syndrome was found by B-mode ultrasound.</p><p><b>CONCLUSION</b>Intermittent combined use of low dose ST therapy can increase HV and thus improve FAH in girls with ICPP and apparently decreased linear growth during GnRHa therapy.</p>
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Niño , Femenino , Humanos , Estatura , Desarrollo Óseo , Desarrollo Infantil , Quimioterapia Combinada , Hormona Liberadora de Gonadotropina , Usos Terapéuticos , Trastornos del Crecimiento , Quimioterapia , Hormona de Crecimiento Humana , Usos Terapéuticos , Pubertad Precoz , Quimioterapia , Estanozolol , Usos Terapéuticos , Resultado del TratamientoRESUMEN
<p><b>OBJECTIVE</b>To assess the utility of serum steroids measurement in monitoring the treatment of children with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21OHD).</p><p><b>METHOD</b>Nineteen Patients with CAH 21OHD aged (3.67±1.54) years treated with hydrocortisone and fluorocortisone replacement were followed up at an intervals of 0.33 - 1.0 years over a period of (1.47±0.7) years. At each visit, roentgenograms of the hands and wrists were taken, fasting peripheral blood were collected to test serum dehydroepiandrosterone sulfate, progesterone, 17-hydroxyprogesterone (17-OHP), androstenedione (Δ4-A), testosterone, free testosterone, estrone, and estradiol concentrations at 8 AM in the morning before the first dose of glucocorticoid. Then the patients were classified as being in "Good Control" or in "Poor Control" based on clinical criteria including signs of androgen excess, growth velocity and bone age increment at each interval. Comparisons were carried out between the serum steroid concentrations of the two groups. The receiver operating characteristic (ROC) curves were used to determine the cut-off values for diagnosing "Poor Control".</p><p><b>RESULT</b>Both of serum Δ4-A and 17-OHP concentrations were higher in "Poor Control" group than those in "Good Control" group [5.95 (2.23-11.2) nmol/L versus 1.05 (1.05-9.89) nmol/L, t=2.19; 13.85 (6.06-20) µg/L versus 3.67 (0.42-21.1) µg/L, t=2.17; P<0.05, respectively]. The ROC curves for serum Δ4-A concentrations, serum 17-OHP concentrations, serum Δ4-A in combination with 17-OHP concentrations were constructed with areas under the ROC curves (95%CI) of 0.76 (0.62, 0.90), 0.75 (0.62, 0.88), 0.69 (0.54, 0.84), P<0.05, respectively. Serum Δ4-A of 3.9 nmol/L had 0.78 of sensitivity and 0.75 of specificity in diagnosing "Poor Control". Serum 17-OHP of 7.1 µg/L has 0.67 of sensitivity and 0.71 of specificity in diagnosing "Poor Control".</p><p><b>CONCLUSION</b>Each of serum 17-OHP or/and Δ4-A concentration was of significance in diagnosing "Poor Control" during the glucocorticoid replacement treatment of CAH 21OHD, with the diagnostic efficacy being serum Δ4-A concentration, serum 17-OHP concentration and serum Δ4-A in combination with 17-OHP concentration in descending order. Serum Δ4-A and 17-OHP concentrations may be used as the biochemical indicators to monitor the therapy of CAH 21OHD.</p>
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Preescolar , Femenino , Humanos , Masculino , 17-alfa-Hidroxiprogesterona , Sangre , Hiperplasia Suprarrenal Congénita , Sangre , Diagnóstico , Terapéutica , Androstenodiona , Sangre , Sulfato de Deshidroepiandrosterona , Sangre , Hidrocortisona , Sangre , Progesterona , Sangre , Esteroide 21-Hidroxilasa , Sangre , Testosterona , SangreRESUMEN
<p><b>OBJECTIVE</b>To provide rapid and accurate prenatal genetic diagnosis for a fetus with high risk of Morquio A syndrome.</p><p><b>METHODS</b>Based on ascertained etiology of the proband and genotypes of the parents, particular mutations of the GALNS gene were screened at 10th gestational week with amplification refractory mutation system (ARMS), denaturing high performance liquid chromatography (DHPLC), and direct DNA sequencing.</p><p><b>RESULTS</b>DHPLC screening has identified abnormal double peaks in the PCR products of exons 1 and 10, whilst only a single peak was detected in normal controls. Amplification of ARMS specific primers derived a specific product for the fetus's gene, whilst no similar product was detected in normal controls. Sequencing of PCR products confirmed that exons 1 and 10 of the GALNS gene from the fetus contained a heterozygous paternal c.106-111 del (p.L36-L37 del) deletion and a heterozygous maternal c.1097 T>C (p.L366P) missense mutation, which resulted in a compound heterozygote status.</p><p><b>CONCLUSION</b>The fetus was diagnosed with Morquio A syndrome and a genotype similar to the proband. Termination of the pregnancy was recommended. Combined ARMS, DHPLC and DNA sequencing are effective for rapid and accurate prenatal diagnosis for fetus with a high risk for Morquio A syndrome. Such methods are particularly suitable for early diagnosis when pathogenesis is clear. Furthermore, combined ARMS and DHPLC are suitable for rapid processing of large numbers of samples for the identification of new mutations.</p>
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Femenino , Humanos , Embarazo , Secuencia de Bases , Condroitinsulfatasas , Genética , Pruebas Genéticas , Métodos , Datos de Secuencia Molecular , Mucopolisacaridosis IV , Genética , Linaje , Complicaciones del Embarazo , Genética , Diagnóstico Prenatal , Métodos , Factores de RiesgoRESUMEN
<p><b>OBJECTIVE</b>To study the clinical manifestations of germinoma in children with precocious puberty and to evaluate the diagnostic value of serum levels of β-human chorionic gonadotropin (β-hcG) combined with detections of β-hcG in cerebrospinal fluid (CSF).</p><p><b>METHOD</b>Twelve male children with germinomas confirmed by pathology from Jan. 2005 to Dec. 2009, aged from 4.2 to 10.2 years, were enrolled in this study. Patients were classified into two groups according to tumor locations: intracranial group and non-intracranial group. Levels of β-hcG in serum as well as in CSF were detected before the initiation of therapy. Age and gender matched 5 children undergoing lumbar puncture for other diseases were set as control group for the determinations of β-hcG in CSF. Levels of β-hcG and testosterone in serum and CSF were compared between intracranial group and non-intracranial group, and levels of β-hcG in CSF were compared between non-intracranial group and control group.</p><p><b>RESULT</b>The 12 children showed elevated serum levels of testosterone: 10.43 (1.70-254.00) µg/L, 11 children had testicular volume > 4 ml, while response to LHRH stimulation tests were low; 6 children had gynecomastia. Serum levels of β-hcG were elevated in both intracranial and non-intracranial group and no significant differences were found between groups 63.75 (8.50-309.50) IU/L vs. 59.00 (25.10-71.77) IU/L, P = 0.644. No correlations were found between serum levels of β-hcG and ages, tumor locations, and courses of the patients. Levels of β-hcG in CSF were significantly higher in intracranial group than that in non-intracranial group 488.99 (17.30-1048.53) IU/L vs. 1.20 (1.20-1.50) IU/L, P = 0.009. Children with non-intracranial germinomas had similar levels of β-hcG in CSF as that in control group (P = 0.571).</p><p><b>CONCLUSION</b>The main clinical manifestations in boys suffered from germinoma included pseudo-precocious puberty, disproportionate testicular volume and gynecomastia. Detection of serum levels of β-hcG combined with β-hcG levels in CSF may be useful for determination of the locations of germinomas in children with precocious puberty.</p>
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Niño , Preescolar , Humanos , Masculino , Neoplasias Encefálicas , Diagnóstico , Estudios de Casos y Controles , Gonadotropina Coriónica Humana de Subunidad beta , Sangre , Líquido Cefalorraquídeo , Germinoma , Diagnóstico , Neoplasias del Mediastino , Diagnóstico , Pubertad PrecozRESUMEN
<p><b>OBJECTIVE</b>To investigate the pattern of pubertal development in healthy Cantonese schoolgirls.</p><p><b>METHOD</b>From 1992 to 2001, 311 normal Cantonese schoolgirls, ages from 6.25 to 8.83 yrs (7.24 +/- 0.38) at baseline, were followed up until they reached their final adult height (age 15.72 +/- 0.84 yrs, n = 238). Annual physical examinations including height and weight measurement were performed. From the 3rd visit, pubertal maturations (breast and pubic hair development) were also assessed annually until they were 14.5 years. Age of menarche was recorded.</p><p><b>RESULT</b>(1) Median age at the entry of puberty (age at reaching B2) was 9.83 years (9.33-10.33). Median age at initiation of pubic hair development (PH2) was 10.67 (9.92-11.38) years. Menarche occurred at (12.35 +/- 1.30) years. The age at reaching B2, age at reaching PH2 and age of menarche were all later than that observed in the cross-section study performed in 2003, Guangzhou, China. Peak height velocity (PHV) was reached at (10.52 +/- 1.07) years, 1.00 (0.50-1.50) years after B2 was reached. Interval between "age at onset of breast development" and "age at menarche" was 2.92 (2.08-3.67) years. Duration of pubertal growth (defined as the time from age at B2 to age at which adult height was attained) was (4.80 +/- 0.85) years. (2) Average final adult height (FAH) was (158.74 +/- 5.74) cm. As compared with the cross-section studies held in Guangzhou, China, the FAH in our study was higher than that observed in 1985 but was lower than that observed in 2003. (3) Multiple linear regression analyses showed that the age reaching B2 was an independent factor associated with the age of menarche. (4) Durations of breast stages, interval between B2 and menarche and duration of pubertal growth were similar to that reported in the longitudinal studies in the United Kingdom (1969), Senegal (1995-2000), the United States (1986-1996).</p><p><b>CONCLUSION</b>In healthy Cantonese schoolgirls, the timing of sexual maturation was in a trend of decline in the past 20 years, however it may have no significant impacts on the tempo of pubertal development and FAH.</p>
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Adolescente , Niño , Femenino , Humanos , Desarrollo del Adolescente , Estatura , Peso Corporal , China , Estudios Longitudinales , Pubertad , Maduración Sexual , EstudiantesRESUMEN
<p><b>OBJECTIVE</b>To investigate the effects and the mechanisms of stanozolol (ST) on the proliferation, maturation and differentiation of in vitro cultured growth plate chondrocyte isolated from gonadotropin releasing hormone analogue (GnRHa)-treated adolescent rats, to study if ST mediates the proliferation of chondrocytes via the estrogen receptor alpha (ERalpha), androgen receptor (AR) and/or insulin-like growth factor-1 receptor (IGF-1R) and interactions of the two receptor and IGF-1R receptor signaling pathway, to investigate the mechanism of the biological effects in ST promoting bone growth/maturity at molecular level.</p><p><b>METHOD</b>The rats were weaned at the end of 3 weeks and intramuscular injection of triptorelin of GnRHa preparations, qow x 2 was started. The rats were sacrificed at the end of 7 weeks, and then the tibiae growth plates were taken out with sterile procedure. The chondrocytes were obtained by two-time enzyme digestion method, and the experiments were carried out with the primary chondrocytes. Immunohistochemical staining of proliferating cell nuclear antigen (PCNA) and Western blot analysis were applied.</p><p><b>RESULT</b>The results of PCNA demonstrated that stanozolol enhanced the proliferation of the chondrocytes, time-course studies showed that the proliferation were maximally stimulated by stanozolol after 2 days of incubation and decreased again after longer periods of incubation. The expression of p-ERalpha, p-IGF-1R and p-extracellular-signal regulated kinase 1/2 (ERK1/2) increased with the incubation period of ST treatment, and reached the peak value at a certain time, and then gradually decreased. The expression of p-ERalpha, p-IGF-1R and p-ERK1/2 increased with the elevation of ST concentration, and reached the peak value at 10(-9) - 10(-8) mol/L, then gradually decreased. ST induced-p-ERalpha expression was partially blocked by ERalpha and mitogen-activated protein kinase kinase inhibitors. ST induced-p-IGF-1R expression was partially blocked by ERalpha and IGF-1R inhibitors. ST induced-p-ERK1/2 expression was partially blocked by mitogen-activated protein kinase kinase and IGF-1R inhibitors.</p><p><b>CONCLUSION</b>As an androgen derivation, ST exerts its biological effects of promoting proliferation of the long bone growth plate chondrocytes via activating the classic ERalpha receptor pathway and mitogen-activated protein kinase pathway, and at the same time, by activation of IGF-1R. Both IGF-1R and ERalpha can promote "cross-talk" of two systems' receptor signal through mitogen-activated protein kinase signal pathway.</p>
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Animales , Femenino , Ratas , Andrógenos , Farmacología , Células Cultivadas , Condrocitos , Biología Celular , Metabolismo , Receptor alfa de Estrógeno , Metabolismo , Placa de Crecimiento , Metabolismo , Proteína Quinasa 3 Activada por Mitógenos , Metabolismo , Receptor Cross-Talk , Receptor IGF Tipo 1 , Metabolismo , Transducción de Señal , Estanozolol , FarmacologíaRESUMEN
<p><b>OBJECTIVE</b>Human growth hormone (hGH) is an essential therapeutic drug for the treatment of growth hormone (GH) deficiency (GHD). However, the process of dissolving hGH of the powder form is complicated and potentially hazardous. In the present study, we evaluated the efficacy and safety of preparation in the replacement therapy for children with GH deficiency.</p><p><b>METHODS</b>A 12-month randomized, open-label, multicenter trial was conducted in 31 previously untreated children with growth failure secondary to GH deficiency [20 boys and 11 girls, mean age (10.5 +/- 4.1) years]. An recombined human growth hormone (rhGH) solution (Iintropin AQ) was given via subcutaneous injection daily in every evening at a weekly dose of 0.25 mg/kg. The patients were followed up at 3, 6, 9, and 12 months of the treatment, and the course of treatment was 12 months. Body height was measured 3-monthly and height velocity (HV) and mean height standard deviation score (HT SDS) were calculated. Serum Insulin-like growth factor I (IGF-1), Insulin-like growth factor binding protein 3 (IGFBP-3), GH antibodies and safety parameters were assessed at the baseline and at 3-month intervals. Bone age (BA) was assessed at the baseline and the rate of skeletal maturation (DeltaBA/DeltaCA) was calculated after 6 and 12 months of rhGH treatment by a central bone age reader. Moreover, the safety of rhGH solution treatment was assessed.</p><p><b>RESULTS</b>After 12 months of liquid rhGH therapy, growth parameters were significantly increased over baseline. (1) The mean (+/- SD) height increment DeltaHT (cm) was 4.0 +/- 1.3, 7.0 +/- 2.0, 10.3 +/- 2.6 and 12.9 +/- 3.3 after 3, 6, 9, and 12 months of treatment, respectively (P < 0.01), which indicated linear growth after treatment. The GV (cm/years) was 2.7 +/- 0.9 before treatment and increased to 16.0 +/- 5.1, 14.1 +/- 4.0, 13.7 +/- 3.5, and 12.9 +/- 3.3 after treatment, suggesting that catch-up growth was significant after treatment as compared to the pre-treatment status (P < 0.01). Accordingly, post-treatment catch-up growth was obvious, significant differences were observed in HT SDS, which was -4.62 +/- 1.46 at the onset of therapy and increased significantly after the treatment to -3.80 +/- 1.53, -3.28 +/- 1.60, -2.86 +/- 1.75 and -2.47 +/- 1.86, respectively (P < 0.01). The height difference between GH deficient children and unimpaired children of the same age and gender gradually decreased after treatment, which was significantly different from that seen before treatment (P < 0.01). (2) The levels of serum IGF-1 and IGFBP-3 were increased comparably for the treatment. IGF-1 level (microg/L) was 41 +/- 64 at baseline and increased to 179 +/- 155, 202 +/- 141, 156 +/- 155 and 159 +/- 167 after 3, 6, 9, 12 months of treatment. IGFBP-3 level (mg/L) was 1540 +/- 1325 at baseline, and increased to 3891 +/- 1815, 4051 +/- 1308, 3408 +/- 1435 and 3533 +/- 1413, respectively, suggesting that with the increases in height, IGF-1, and IGFBP-3 were significantly activated to relatively high levels by the medication and reached peak values between 3 and 6 months of treatment. The levels of IGF-1 and IGFBP-3 were significantly different before and after treatment (P < 0.01). The IGF-1/IGFBP-3 molar ratio significantly increased during GH therapy (0.143 +/- 0.013 pre-therapy up to 0.240 +/- 0.055 post-therapy, P < 0.01). The IGF-1/IGFBP-3 molar ratio tended to stabilize after 3-month GH therapy. (3) The bone age assessment carried out 6 and 12 months after treatment showed that the bone maturity (DeltaBA/DeltaCA) was 1.01 +/- 0.57 and 1.07 +/- 0.75, respectively, suggesting that there was no speed-up development in the bone age. No severe adverse events were observed during the trial and the most frequent accompanying event was mild hypothyroidism.</p><p><b>CONCLUSIONS</b>rhGH solution (Iintropin AQ) is a safe and effective preparation in the replacement therapy for children with GH deficiency.</p>
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Niño , Femenino , Humanos , Masculino , China , Enanismo Hipofisario , Sangre , Quimioterapia , Trastornos del Crecimiento , Sangre , Quimioterapia , Hormona de Crecimiento Humana , Usos Terapéuticos , Proteína 3 de Unión a Factor de Crecimiento Similar a la Insulina , Sangre , Factor I del Crecimiento Similar a la Insulina , Metabolismo , Estudios Prospectivos , Proteínas Recombinantes , Usos TerapéuticosRESUMEN
<p><b>UNLABELLED</b>It has been proved that to analyze the factors that determine responsiveness to rhGH and to develop growth prediction models can help doctors to individualize the treatment and maximize the effect.</p><p><b>OBJECTIVES</b>To set up and validate the predictive models of growth responses to rhGH treatment in the first year in prepubertal short stature children with various GH secretary statuses.</p><p><b>METHODS</b>Growth responses to rhGH treatment in the first year, height velocities (HV) and increases in height SDS (DeltaHtSDS), in 62 prepubertal short stature children with various GH secretary statuses were analyzed retrospectively. There were 27 patients with complete growth hormone deficiency (cGHD), 23 with partial GHD (pGHD) and 12 with idiopathic short stature (ISS) in the model group. According to the peak GH value in GH provocative test, the group of pGHD was divided into pGHD-1 (5 - 6.9 microg/L, 12 patients) and pGHD-2 (7 - 9.9 microg/L, 11 patients). All the cases in model group were used for setting up Model-total and the cases of growth hormone deficiency for Model-GHD. Predictive models, including Model-GHD and Model-total, to HV and DeltaHtSDS were set up by the way of multiple regression analysis, based on the results of simple correlation analysis. Other 14 children were included according to the same criteria with the model group, the validation group. The validation group was analyzed prospectively. The actual growth responses were compared with the predicted values calculated by different models so that the predictive models could be validated.</p><p><b>RESULTS</b>The simple correlation analysis showed that HV and DeltaHtSDS in the first year were negatively correlated with the same group factors at baseline: chronological age, bone age, height SDS, differences between the height SDS and the target height SDS, peak value in GH provocative test and IGF-1SDS. All the 4 predictive models were found to be significant at a level of P < 0.05, R(2) ranged from 0.244 to 0.519. The two models predicted HV and Model-GHD for DeltaHtSDS were proved to be validated. The observed and predicted responses positively and significantly correlated with each other, r value ranged from 0.753 to 0.996. And there was no significant difference between them when tested by paired t test.</p><p><b>CONCLUSIONS</b>The availability of the predictive model will help to individualize the growth hormone treatment in prepubertal short stature children with various growth hormone secretary status.</p>
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Adolescente , Niño , Preescolar , Femenino , Humanos , Masculino , Estatura , Trastornos del Crecimiento , Quimioterapia , Hormona del Crecimiento , Metabolismo , Hormona de Crecimiento Humana , Usos Terapéuticos , Modelos Estadísticos , Estudios Prospectivos , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Objective To observe the effect of stanozolol(ST) on long bone growth and maturation of pubertal female rats treated with gonadotropin releasing hormone agonist(GnRHa).Methods At 3 weeks of age,42 female Sprague-Dawley rats(brood) were divided into 7 groups(ST dosage groups,as 5 000 ?g/100 g group,200 ?g/100 g group,100 ?g/100 g group,50 ?g/100 g group,25 ?g/100 g group,solvent control group and blank control group)(n=6).Forty-eight female rats were divided into 8 groups(ST therapeutic duration)(n=6).Rats received 2.5 mg/kg im slow-released GnRHa(triptorelin,as 2 d group,3 d group,5 d group,7 d group,10 d group,13 d group,soluent control group and blank control group) which was repeated every 2 weeks for 2 times,3 days after the 2nd GnRHa(D1),ST dosage groups were subcutaneously administrated ST at the various dosage daily(D1-D13).ST therapeutic duration groups were subcutaneously administrated ST at the dosage of 100 ?g/100 g daily for different duration.All the rats were killed on the D14.On the day of sacrifice,body weight,body length and left tibial length were measured,plasma were taken for determining insulin-like growth factor-1(IGF-1),right tibia were fixed,demineralized and processed for paraffin-embedding.Paraff sections were HE stained for growth plate measurements.proliferating cell nuclear antigen(PCNA) on growth plate was analyzed with immunohistochemistry staining and image.Results 1.In the 5 000 ?g/100 g ST dosage group,the weight,Height and tibial length exceeded than those of the other dosage and control groups(Pa
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0.05).Conclusion GH improves FAH of children with GHD.Height at the initiation of puberty is the most significant determining factor for the long-term efficacy.Hence,it is important that the diagnosis should be made and treatment be initiated as early as possible to afford children with GHD the opportunity to make up much of their height deficit before puberty.Adequate dosage of GH should be used for the children taking initial treatment at puberty to attain satisfactory FAH.
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Objective To analyse the relative factors of the linear growth velocity(GV)in girls with central precocious puberty(CPP)during gonadotropin-releasing hormone analog(GnRHa)therapy,and to investigate the factors affecting the height gain during two-year GnRHa treatment.Methods In 86 girls aged (8.04?1.28)years with CPP treated with GnRHa for more than 2 years,the data including target height,age of onset,pubertal course,chronological age,bone age,linear GV,serum estradiol level and mature index of vaginal smear were analyzed,then the correlations and stepwise regression were performed.Results During GnRHa therapy,GV decreased year by year.The GV in the second year(GV_(2nd))was negatively correlated with the age of onset,bone age(BA_0,BA_2)and chronologic age(CA_0,CA_2)at the onset and by the end of the first year of GnRHa therapy(r=-0.37,-0.59,-0.57,-0.51 and-0.52,respectively,all P
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Objective To evaluate the effect of combined treatment with gonadotropin-releasing hormone analog(GnRHa)and recombinant human growth hormone(rhGH)on predicted adult height(PAH)in girls with central precocious puberty(CPP).Methods Fifteen girls with CPP,whose growth velocity during GnRHa treatment had been less than 4 cm/year,were given additional rhGH treatment at a dose of 1 U?kg~(-1)?w~(-1),sc, for 4-13 months.Comparisons of growth velocity,height SDS for bone age(HtSDS_(BA))and PAH were performed before and after the combined treatment.Results During rhGH combined with GnRHa therapy,growth velocity increased significantly[(7.4?1.7)cm/year vs (3.2?0.7)cm/year baseline,P<0.01].In 7 girls treated with rhGH and GnRHa for more than 9 months,growth velocity in the second 6 months[(6.5?1.0)cm/year]was slightly lower than that in the first 6 months[(8.8?1.1)cm/year],being both faster than that of baseline [(3.2?0.8)cm/year].There was a significant increase in rhGH-duration corrected change of HtSDS_(BA) [(0.35?0.15)/6 month vs (0.12?0.18 )/6 month baseline,P<0.01]and PAH[(3.2_+1.4)cm/ 6 month vs (1.4?1.1)cm/6 month baseline,P<0.01].Conclusion In girls with CPP showing a marked decrease in growth velocity during GnRHa treatment,the combined rhGH and GnRHa treatment remarkably improves growth velocity and PAH.