Adrenal insufficiency(AI) is a disorder that can result from primary adrenal failure(primary type) or secondary adrenal disease due to impairment of the hypothalamic-pituitary axis (central type).It is characterized by glucocorticoid deficiency,with or without inappropriate mineralocorticoid or androgen production.The diagnosis and management of children with AI remain challenging as clinical characteristics of AI in children maybe non-specific and acute adrenal crisis is life-threatening,needing life-long glucocorticoid replacement treatment and education of the patient and the family.New formulations of hydrocortisone which should be able to simulate the circadian rhythm of steroid secretion are supposed to improve outcome in patients with adrenal insufficiency in the near future.

46,XX primary ovarian insufficiency(POI)is a clinical syndrome defined by loss of ovarian activity before the age of 40 years old with a karyotype 46,XX,characterized by menstrual disturbance(amenorrhea or oligomenorrhea)in association with hypergonadotropic hypogonadism.46,XX POI is a rare disease with the prevalence lower than 1％,of whom 2.5％are adolescents.Potential etiologies for 46,XX POI can be divided into genetic,autoimmune,and iatrogenic categories.Unfortunately,for most patients presenting with POI,the cause will remain unexplained.Once,POI is diagnosed,clinical indicated tests are needed to identify the mechanism causing POI.POI is a complex condition appearing with a strong genetic basis.Large-scale genomic sequencing had recently identified new mechanisms of POI.The management of the condition should address both of physical and emotional well-being health,including health education,hormone develoment treatment,prevention and treament of lower-estrogen associated diseases,with the support from a multidisciplinary team.

Along with the rapid development of global medical technology, great progress has been made in clinical diagnosis and treatment of childhood cancer,hence childhood cancer survival rate is increasing markedly. The clinicians have become concerned about life quality of childhood cancer survivors. A number of studies reported that long-term childhood cancer survivors are at increased risk of developing metabolic syn-drome,especially after cranial irradiation,abdomal irradiation,or total body irradiation. Metabolic syndrome is a variety of metabolic abnormalities commonly clustered together in a condition of the same individual,which sig-nificantly increases risk of cardiovascular diseases. Though the etiology of the metabolic syndrome in cohorts of childhood cancer survivors has not been elucidated,the predisposing factors have been identified as the lack of hormones after cancer treatment,damage from medicine or radiation therapy,endothelial dysfunction and so on. Accordingly,early diagnosis of metabolic syndrome is of great importance with medical interventions,such as encouraging cancer survivors to improve dietary habit and enhance exercise to achieve ideal weight,and to subse-quently decrease the risk of metabolic syndrome and cardiovascular events.

Congenital adrenal hyperplasia (CAH) owing to steroid 21-hydroxylase deficiency (21-OHD) was a relatively frequent of autosomal recessive disorders characterized by the inactivation of the steroid-synthesizing enzyme in the adrenocortex.Corticosteroids (glucocorticoids and mineralocorticoid) replacement therapy was the primary treatment of 21-OHD.The main objective of 21-OHD treatment in children was to maintain normal growth.Inadequate or excessive treatment was commonly observed.A number of studies reported that 21-OHD adult were at increasing risk of developing metabolic syndrome and cardiovascular events.However,there was few researches on 21-OHD children with metabolic disorders,and no domestic reports.The article summarized recent clinical research progresses in research on the alterations of lipid and carbohydrate metabolism in children with classic 21-OHD.

Objective To recognize the changes in the somatotropin axis function in girls with idiopathic central precocious puberty (ICPP) treated with GnRH analogue (GnRHa) and to probe into the cause of growth velocity reduction during GnRHa treatment. Methods Fourteen girls with ICPP were studied. Their growth velocities at the beginning and the end of 6th month of GnRHa treatment were observed. Maturation indexes (MI) were got from vaginal smears, and serum E 2, insulin like growth factor (IGF Ⅰ), IGF binding protein 3 (IGFBP 3) concentrations were determined and IGF Ⅰ/IGFBP 3 calculated at the beginning and the end of 6th month of GnRHa treatment. The controls were 13 age matched healthy prepubertal girls. Results After 6 month GnRHa treatment, the growth velocity reduced significantly from (8.23?1.67)cm/y to (6.27?1.54)cm/y (P

Objective To investigate the long-term outcome in the girls with central precocious puberty (CPP) treated by gonadotropin-releasing hormone analogue (GnRHa). Methods Thirty girls with idiopathic CPP treated with GnRHa for (23.0?7.6)months achieved their near final heights after (3.2?0.8)years follow-up. Comparisons were made among their final adult height (FAH), target height (THt), predicted adult height(PAH)at the onset and the end of GnRHa treatment (PAH 1 and PAH 2) . Factors affecting the height gain were also analysed. Results PAH increased after the GnRHa treatment [ PAH 2(155.2?5.7)cm vs PAH 1 (150.7?5.4)cm,P

[Objective] To observe the effect of stanozolol on proliferation and differentiation of cultured growth plate chondrocytes in vitro.[Methods] At 3 week of age,Sprague–Dawley rats received 2.5 mg/kg in slow-released GnRHa (triptorelin) which was repeated every 2 weeks for 2 times,at 7-week old.The tibial growth plate cartilage were aseptically dissected and tripsin and EDTA digested for 0.5 h,then collagenase digested for 3 h at 37 ℃.Chondrocytes were cultured in DMEM:F12 medium for 48 h,the cells were starved for 24 h in serum-free DMEM:F12 medium before stanozolol treatment.In dose-effect groups,chondrocytes were incubated in serum-free media in various concentrations of stanozolol for 48 h.In time-course groups,chondrocytes were incubated in serum-free media in various times of stanozolol (10-8 mol/L).immunohistochemical staining of collagen Ⅱ,Ⅹ,PCNA,and MTT were conducted.[Results] The results of MTT,PCNA,and typeⅡcollagen synthesization demonstrated stanozolol enhanced the proliferation of the chondrocytes,time-course studies had shown that the proliferation were maximally stimulated by stanozolol after 2 or 3 days of incubation and decreased again after longer periods of incubation.Stanozolol stimulated the proliferation of the chondrocytes dose-dependently at 10-11 mol/L and 10-8 mol/L,maximally stimulatory concentrations of Stanozolol was 10-9 ～ 10-8 mol/L,and decreased again after higher concentration of stanozolol.Stanozolol did not stimulated type X collagen synthesization from 10-11 mol/L ～ 10-8 mol/L,but experiments showed that type X collagen was already stimulated after incubation in Stanozolol (10-7 ～ 10-5 mol/L).Time-course studies had shown those typeⅩcollagen synthesizations were stimulated by stanozolol after 4 ～ 5 days of incubation.[Conclusion] Stanozolol enhances the proliferation of chondrocytes of pubertal female rat treated with GnRHa in vitro (time-course- dependent and concentration -dependent).

0.05).(2) Expression levels of Akt : At baseline,Akt was already activated in NCU-SGA rats compared to no Akt activation in normal control rats.However,post-stimulating of insulin,the increase level of phosphate Akt in NCU-SGA rats was remarkably lower than that in control rats(P

Objective To understand how serum DHEAS levels change with sex,age and stage of sexual maturation in children and adolescents and explore the relationship between adrenarche and pubertal maturatiotL Methods Serum samples from 120 healthy boys,198 healthy girls and 152 girls with idiopathic central precocious puberty (ICPP) were examined for DHEAS.Referenee ranges for healthy children and adolescents and statistical difierences between heahhy girls and ICPP girls were analyzed with respect to sex,age and stage of sexual maturation.Results Both healthy children and ICPP girls showed extremely low levels of serum DHEAS and they were not related to sex.age or tanner stages in the individuals below age of 6 years.Serum DHEAS levels were positively related to both age (above age of 6 years)and tanner stage in healthy groups(r=0.69 and 0.71 respectively,P＜0.01).After the onset of puberty,serum DHEAS levels appeared to be higher in boys than that in girls within the same tanner stage(P＜0.05).Within the individusis in the same age group with same sex.serum DHEAS levels increased along with pubertal development.While within the individuals in same tanner stage group with salne sex after puberty onset.serum DHEAS levels showed no significant difference among different age groups.For example.there was no difference in serum DHEAS levels of healthy girls in tanner stage Ⅲ among different age subgroups(age of8-9;age of 10-11,age of 12-13)and the mean vallie of serum DHEAS was 532.0-557.8μg/L(F=0.21,P=0.98).In different age subgroups above age of 6 years,Z scores for serum DHEAS in ICPP girls were highher than them healthy ones with advanced tanner stages(0.97us-0.1 and 1.39us-0.08,JP≤0.01)In different tanner stage subgroups.Z scores for serum DHEAS showed no difierence between healthy and ICPP girls despite apparent different age ranges(0.00 us-0.31-0.18,P＞0.05).Conclusions Serum DHEAS level increased along with both age (above 6 years) and tanner stage in healthy children and adolescents.There was no gender difference until the onset of puberty.It was demonstrated that adrenache and gonadarche were related to each other.Reference ranges for adolescents should be interpreted according to sex.age and tanner stage simultaneously.

Objective To analyze the clinical characters of childhood adrenocortical tumors, and to enhance the knowledge of diagnosis of this disease. Methods A retrospective analysis of clinical characters,laboratory tests,and imaging findings in 31 cases of childhood adrenocortical tumors was carried out. Results 16 cases of adenoma and 15 cases of carcinoma were included. The average age was (4.49±3.51) years old, and 67.7% of the patients were younger than 5 years old. The ratio of male to female was 1.0: 1.4. 12 patients presented only precocious sexual development, 4 patients presented only Cushing syndrome, 10 patients showed sexual precocity combined with Cushing syndrome, and 5 patients did not have any endocrine abnormalities. Raised testosterone level in 92.3% of these patients was the most common finding in laboratory tests. Only 12.5% of ultrasound images and 20.8% of CT images were consistent with pathologic diagnosis. Conclusions The clinical manifestations of adrenocortical tumors in childhood are precocious sexual development, Cushing's syndrome, or nonfunctional. The common laboratory findings are elevation of sex hormone and disorder of cortisol circadian rhythm. Precocious sexual development and elevation of androgens are more common in childhood adrenocortical tumor than those in adults. Imaging usually cannot give proper diagnosis. Final diagnosis should be established by clinical features, laboratory tests, imaging, and pathologic results.