1.Etiology, diagnosis and management of adrenal insufficiency in children
Chinese Journal of Applied Clinical Pediatrics 2015;30(8):574-579
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.
2.Update on 46,XX primary ovarian insufficiency in adolescents
Chinese Journal of Applied Clinical Pediatrics 2017;32(8):570-573
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.
3.Effect of gonadotropin-releasing hormone analogue on levels of serum insulin-like growth factor (IGF)-Ⅰ and IGF binding protein-3 in girls with idiopathic central precocious puberty
Chinese Journal of Endocrinology and Metabolism 1986;0(03):-
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
4.Research progress of metabolic syndrome in children with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency
Chinese Journal of Applied Clinical Pediatrics 2016;31(8):631-634
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.
5.Long-term survival and metabolic syndrome in childhood cancer
International Journal of Pediatrics 2014;(5):496-499,500
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.
6.Improvement of final adult height in girls with central precocious puberty treated by gonadotropin-releasing hormone analogue
Chinese Journal of Endocrinology and Metabolism 1986;0(03):-
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
7.Post-receptor signaling crosstalk between GH and insulin in non-catch-up growth rats born small for gestational age
Tingting HUANG ; Minlian DU ; Yanhong LI ; Huamei MA
Chinese Journal of Pathophysiology 1986;0(02):-
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
8.Effects of Stanozolol on Proliferation and Differentiation of Cul tured Growth Plate Chondrocytes from Pubertal Female Rat Treated with GnRHa In Vitro
Shunye ZHU ; Huamei MA ; Yanhong LI ; Sinian PAN ; Minlian DU
Journal of Sun Yat-sen University(Medical Sciences) 2009;30(4):422-427
[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).
9.Prevalence and characterization of testicular adrenal rest tumors in children and adolescent males with congenital adrenal hyperplasia
Zhe SU ; Minlian DU ; Yanhong LI ; Huamei MA ; Hongshan CHEN ; Qiuli CHEN
Chinese Journal of Endocrinology and Metabolism 2013;29(8):648-652
Objective To summarize the prevalence,risk factors,and characteristics of testicular adrenal rest tumors(TART) in children and adolescent males with congenital adrenal hyperplasia(CAH) due to 21-hydroxylase deficiency (21-OHD).Methods During past 4 years,there were 44 males with CAH caused by 21-OHD undergone testicular ultrasound scan in our clinic.We have diagnosed 13 cases of TART and summarized the characterizations.Results The prevalence of TART in our group was 29.5% with 11 cases of salt wasters and 2 cases of simple virilizers.The median age of TART diagnosis was 10.2 years.The median follow-up period of TART was 3.0 years.History of poor control of CAH was found in every case before diagnosis of TART.The doses of hydrocortisone were increased in 5 cases of TART who refused operations or had small TART,resulting in tumor regression in two of those five patients.Testis-sparing surgeries were performed in 8 patients with symptoms relieved; 7 of those eight patients presented elevated FSH and LH after operations.Conclusions Prevalence of TART in our CAH children and adolescent males was 29.5%.Testicular ultrasound was useful in TART screening.TART should be considered when a CAH patient with poor control,especially in post-pubertal one.Delayed management of TART may lead to impaired testicular function.Increased doses of glucocorticoid may be effective in TART of small size.Testis-sparing surgery should be also considered if necessary.
10.Final height outcome of boys with idiopathic central precocious puberty treated with gonadotropin-releasing hormone analogue
Pimei ZHENG ; Zhe SU ; Huamei MA ; Minlian DU ; Qiuli CHEN ; Yanhong LI ; Hongshan CHEN
Chinese Journal of Endocrinology and Metabolism 2012;28(7):551-556
Objective To observe the final adult height of 20 boys with idiopathic central precocious puberty (ICPP) treated with slow-releasing gonadotropin-releasing hormone analogue(GnRHa).Methods Twenty boys with ICPP were treated with GnRHa for( 20.0 ± 6.1 ) months.At the beginning of therapy,mean chronological age and bone age was( 11.4 ± 1.0 ) years and ( 13.0 ± 0.4 ) years,respectively,GnRHa was discontinued when the boys reached the chronological age and bone age of( 13.2 ± 1.1 ) years and ( 13.7 ± 0.6 ) years,respectively.After the end of treatment,all the boys had been followed up for( 3.3 ± 1.5 ) years and had achieved adult height.Comparisons were made among their predicted adult height ( PAH ),final adult height ( FAH ),and target height ( THt ).The long-term outcome of final adult height in boys with ICPP was investigated after GnRHa treatment.Results All the boys reached target height range.Final height was similar to the target height [ ( 169.8 ± 5.8 vs 167.8 ± 4.6 ) cm,P>0.05 ].The height gain,defined as the difference between predicted adult height at the start of treatment using the height SDS for bone age and actual adult height was( 3.62 ± 3.57 ) cm with the residual growth capacity of ( 11.82 ±3.99)cm,PAH significantly improved after GnRHa treatment compared with before treatment [ ( 169.0 ± 5.0 vs166.2 ± 4.2 ) cm,P<0.01 ].There were no differences among PAH,FAH,and THt.Conclusion GnRHa treatment improves final height within the range of target height in boys with central precocious puberty.