1.Effect of gonadotropin-releasing hormone analogue treatment in improving final adult height of children with central precocious puberty or early and fast puberty: a Meta analysis.
Zhao-Le CHU ; Hui JIANG ; Qian WU
Chinese Journal of Contemporary Pediatrics 2021;23(11):1161-1168
OBJECTIVES:
To systematically evaluate the effect of gonadotropin-releasing hormone analogue (GnRHa) treatment on the final adult height of children over 6 years of age with central precocious puberty (CPP) or early and fast puberty (EFP).
METHODS:
PubMed, MEDLINE, Embase, Cochrane Library, CNKI, and Wanfang Data were searched for related articles on GnRHa treatment for children with CPP or EFP. Stata 12.0 software was used to perform a Meta analysis of related data.
RESULTS:
A total of 10 studies were included, and the total sample size was 720 children, with 475 children in the GnRHa treatment group and 245 children in the control group. The Meta analysis showed that compared with the control group, the GnRHa treatment group had significantly better final adult height (
CONCLUSIONS
GnRHa treatment is safe and effective in improving the final adult height of children over 6 years of age with CPP or EFP.
Adult
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Body Height
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Child
;
Gonadotropin-Releasing Hormone
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Humans
;
Puberty
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Puberty, Precocious/drug therapy*
2.Familial male-limited precocious puberty due to Asp578His mutations in the LHCGR gene: clinical characteristics and gene analysis in an infant.
Min WANG ; Min LI ; Yue-Sheng LIU ; Si-Min LEI ; Yan-Feng XIAO
Chinese Journal of Contemporary Pediatrics 2017;19(11):1159-1164
The aim of the study was to provide a descriptive analysis of familial male-limited precocious puberty (FMPP), which is a rare inherited disease caused by heterozygous constitutively activating mutations of the luteinizing hormone/choriogonadotropin receptor gene (LHCGR). The patient was a ten-month-old boy, presenting with penile enlargement, pubic hair formation, and spontaneous erections. Based on the clinical manifestations and laboratory data, including sexual characteristics, serum testosterone levels, GnRH stimulation test, and bone age, this boy was diagnosed with peripheral precocious puberty. Subsequently the precocious puberty-related genes were analyzed by direct DNA sequencing of amplified PCR products from the patient and his parents. Genetic analysis revealed a novel heterozygous missense mutation c.1732G>C (Asp578His) of the LHCGR gene exon11 in the patient, which had never been reported. His parents had no mutations. After combined treatment with aromatase inhibitor letrozole and anti-androgen spironolactone for six months, the patient's symptoms were controlled. The findings in this study expand the mutation spectrum of the LHCGR gene, and provide molecular evidence for the etiologic diagnosis as well as for the genetic counseling and prenatal diagnosis in the family.
Heterozygote
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Humans
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Infant
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Male
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Mutation
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Puberty, Precocious
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drug therapy
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genetics
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Receptors, LH
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chemistry
;
genetics
4.Efficacy of letrozole in treatment of children with congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency.
Qian WANG ; Shule ZHANG ; Xue MA ; Guimei LI ; Zengmin WANG ; Fengxue WANG
Journal of Zhejiang University. Medical sciences 2020;49(3):302-307
OBJECTIVE:
To assess the efficacy of letrozole in treatment of children with congenital adrenal hyperplasia (CAH) due to steroid 21-hydroxylase deficiency (21-OHD).
METHODS:
Twenty eight children, including 19 boys and 9 girls aged 4-10y, with CAH due to 21-OHD were enrolled in the study. At the first six months of study, all children received conventional treatment with hydrocortisone or fludrocortisone, then letrozole was added to original regimen. The height velocity (HV), difference between bone age and chronological age (BA-CA), height standard diviation score based on bone age (HtSDS ), predicted adult height (PAH), Tanner phase, sex hormone, and possible adverse reaction were evaluated and compared between those before and after letrozole treatment.
RESULTS:
After 6 months of letrozole treatment, there was significant deceleration of HV, but it would recover soon. There was significant increase of HtSDS after 12 months of letrozole treatment ( < 0.05 or < 0.01), and significant changes in BA-CA after 18 months of letrozole treatment ( < 0.05). PAH of female children was significantly increased during letrozole treatment ( < 0.05), whereas PAH of male children was significantly increased 18 months after letrozole treatment ( < 0.05). Follicle-stimulating hormone (FSH) and luteinizing hormone (LH) levels were significantly increased, but did not meet the diagnostic criteria of central precocious puberty. Estradiol was significantly decreased ( < 0.01), but no changes in testosterone level was observed. During 24 months letrozole treatment, no hirsutism, severe acne, headache, bone pain, obesity, hypertension, rash and other adverse reactions were observed.
CONCLUSIONS
Letrozole can delay bone maturation and improve PAH, which can be used with conventional treatment for children with CAH due to 21-OHD, especially for those with high BA and low PAH.
Adrenal Hyperplasia, Congenital
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drug therapy
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Body Height
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Child
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Child, Preschool
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Female
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Humans
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Letrozole
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therapeutic use
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Male
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Puberty, Precocious
5.Etiological trends in male central precocious puberty.
Jisun LEE ; Jinsup KIM ; Aram YANG ; Sung Yoon CHO ; Dong Kyu JIN
Annals of Pediatric Endocrinology & Metabolism 2018;23(2):75-80
PURPOSE: In the present study, the etiological trends in male central precocious puberty (CPP) were examined, and annual distribution was evaluated. METHODS: Seventy-one male CPP subjects who started puberty before 9 years of age were included in this study. All individuals were diagnosed as having CPP at Samsung Medical Center between 2001 and 2016. Chronological age at puberty onset, diagnosis of CPP, bone age, weight (kg), height (cm), puberty stage, brain magnetic resonance imaging findings, testosterone level, basal gonadotropin level, and gonadotropin level after gonadotropin releasing hormone stimulation were analyzed. RESULTS: The 71 patients were divided into 2 groups: idiopathic (group I) and organic (group II) when the lesion was identified as associated with the central nervous system (CNS) or when the patient received chemotherapy for non-CNS tumors before CPP diagnosis, respectively. Forty-four cases (62%) were idiopathic, and 27 (38%) were organic. The proportion of idiopathic CPP was higher than that of organic CPP during the study period. In 51.9% of organic cases, puberty started before 8 years of age, whereas it started after that age in 93.2% of the idiopathic cases. CONCLUSIONS: In the present study, among all male CPP cases, 62% were idiopathic. The probability of idiopathic CPP prevalence was higher in males when the puberty onset was after 8 years of age with no history of cranial radiotherapy or chemotherapy.
Adolescent
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Brain
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Central Nervous System
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Diagnosis
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Drug Therapy
;
Gonadotropin-Releasing Hormone
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Gonadotropins
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Humans
;
Magnetic Resonance Imaging
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Male*
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Prevalence
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Puberty
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Puberty, Precocious*
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Radiotherapy
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Testosterone
6.A new method for adult height prediction in girls with idiopathic central precocious puberty treated with gonadotropin releasing hormone agonist.
Yan LIANG ; Hong WEI ; Xiao YU ; Ningyi SONG ; Xiaoping LUO
Chinese Journal of Pediatrics 2015;53(11):840-844
OBJECTIVETo develop a new method for predicting adult height (PAH) based on the theory of Bayley-Pinneau and to evaluate the feasibility of this method in predicting adult height of girls with idiopathic central precocious puberty (ICPP) who were treated with gonadotropin releasing hormone agonist (GnRHa).
METHOD(1) The new method for PAH, i.e. PAH = Height/percentage of adult height for bone age, was established according to the theory of Bayley-Pinneau and the data from the national growth survey of children in the nine cities of China in the year 2005. (2) Data from seventeen female patients with ICPP received GnRHa treatment and achieved final adult height (FAH) were collected. Before and during the treatment, PAH was calculated by the method of Bayley-Pinneau and the new method.
RESULTThe mean FAH(cm) of the 17 patients with ICPP was 159.81 ± 4.95. The PAH (cm), before and after treatment for 1, 2 and 3 years, were 156.53 ± 3.63, 157.71 ± 3.62, 158.60 ± 3.50, 161.46 ± 4.50 and 161.56 ± 3.77, 161.68 ± 3.44, 162.04 ± 4.42, 163.13 ± 2.36 respectively by using the new method (PAH-D) and Bayley-Pinneau method(PAH-BP). The mean value of (PAH-D-FAH) and (PAH-BP-FAH) were -1.96 cm and 1.48 cm. However, the 95% confidence interval was (-3.82 cm to -0.11 cm), (-1.60 cm to 4.55 cm) for (PAH-D-FAH) and (PAH-BP -FAH). There was no significant difference between the values obtained before and after treatment in terms of PAH by use of Bayley-Pinneau method. By the new method, however, the results showed that the PAH increased and improved further with prolonged treatment periods. And at the end of treatment, there was no significant difference between PAH and FAH. The correlation coefficient was 0.93. Regression analysis showed that the trend line was in parallel with baseline data.
CONCLUSIONThe new method we established could predict better the final heights of girls with CPP who were treated with GnRHa.
Adult ; Body Height ; Child ; China ; Drug Therapy, Combination ; Female ; Gonadotropin-Releasing Hormone ; agonists ; Human Growth Hormone ; Humans ; Puberty, Precocious ; drug therapy ; Reference Values
7.Effects of gonadotropin releasing hormone analog and growth hormone on height in girls with idiopathic central precocious puberty.
Chun-Lin WANG ; Li LIANG ; Pei-Ning LIU ; Xian-Jiang JIN ; Lin-Qi CHEN ; Fang YANG ; Qun LIAN ; Rui-Min CHEN
Chinese Journal of Contemporary Pediatrics 2014;16(1):25-30
OBJECTIVETo determine the effect of gonadotropin releasing hormone agonist (GnRHa), by itself alone or in combination with recombinant human growth hormone (rhGH), on height in young girls (bone age≥10 years) with idiopathic central precocious puberty (ICPP).
METHODSEighty girls with ICPP (9.0±0.7 years old) from six medical centers across Southeast and Southwest China participated in this study. They were allocated to treatment with GnRHa+rhGH (n=31) and GnRHa (n=49) respectively. Girls in the GnRHa+rhGH group (bone age 11.18 ±0.53 years) were treated with GnRHa for 25.29±6.92 months and rhGH for 12.87±7.02 months. Girls in the GnRHa group (bone age 11.03 ±0.50 years) were treated with GnRHa for 25.96±8.95 months. The height standard deviation for bone age (HtSDS-BA), predicted adult height, near-adult height and net height increase before and after treatment were recorded for girls in both groups.
RESULTSHtSDS-BA was significantly improved after treatment for both groups (P<0.01) and the HtSDS-BA value was superior in the GnRHa+rhGH group over the GnRHa group (P<0.01). Values in near adult height (157±6 cm vs 157±4 cm), net height increase after treatment (4.68 cm vs 3.89 cm), and predicted adult height after drug withdrawal (161±5 cm vs 158±5 cm) were higher in the GnRHa+rhGH group than the GnRHa group, but the differences were not significant.
CONCLUSIONSBoth GnRHa plus rhGH and GnRHa alone can improve the near adult height in girls with ICPP with a bone age ≥10 years to a similar extent. Adult height predicted based on bone age in ICPP girls following drug withdrawal is usually overestimated and precautions should be taken when this parameter is used.
Body Height ; drug effects ; Child ; Female ; Gonadotropin-Releasing Hormone ; analogs & derivatives ; pharmacology ; Human Growth Hormone ; pharmacology ; Humans ; Puberty, Precocious ; drug therapy ; physiopathology
8.Impact of gonadotropin-releasing hormone analogs on body mass index in girls with idiopathic central precocious puberty: a long-term follow-up study.
Jin-Na YUAN ; Li LIANG ; Xi-Ding CAI ; Zhen LI ; Min BAI ; Cheng-Ping GU
Chinese Journal of Contemporary Pediatrics 2011;13(11):896-899
OBJECTIVETo study the impact of gonadotropin-releasing hormone analogs (GnRHa) on body mass index (BMI) in girls with idiopathic central precocious puberty (ICPP).
METHODSOne hundred and thirty-four girls with ICPP were enrolled. Fifty-seven out of the 134 girls were treated with GnRHa for 1.69±0.43 years. The height, weight, bone age and BMI were measured before treatment, at the end of the treatment and after reaching near adult height and compared with those in the untreated 77 girls.
RESULTSThe adult predicted height standard deviation score (SDS) at the end of treatment was significantly higher than that before treatment (P<0.01) and was similar to the target height SDS in the GnRHa treatment group (P>0.05). With GnRHa treatment, the near-adult height SDS was higher than the target height SDS (P<0.01). At the end of treatment, the BMI SDS slightly increased compared with pretreatment level (P>0.05). A significant reduction in the BMI was observed when reaching the near-adult height in the GnRHa treatment group compared to the level of pretreatment and the untreated group (P<0.01). However, the BMI in the GnRHa treatment group before treatment, at the end of the treatment and after reaching near adult height remained in the normal range (±1 SD).
CONCLUSIONSGnRHa may improve the final height in girls with ICPP. The alterations of BMI after GnRHa therapy fluctuate in a normal range.
Body Height ; drug effects ; Body Mass Index ; Child ; Female ; Follow-Up Studies ; Gonadotropin-Releasing Hormone ; analogs & derivatives ; therapeutic use ; Humans ; Puberty, Precocious ; drug therapy ; physiopathology
10.A Case of Hepatoblastoma with Precocious Puberty.
Chang Won CHOI ; Hyun Jin PARK ; Ki Woong SUNG ; Eun Sun YOO ; Hee Young SHIN ; Sei Won YANG ; Hyo Seop AHN
Journal of the Korean Pediatric Society 2000;43(2):288-293
Hepatoblastoma is the most common primary malignant liver tumor in childhood. Most cases are boys generally below three years of age. Hepatoblastoma are associated with various non-metastatic syndromes. Rarely, human chorionic gonadotropin may be produced by the tumor. Ectopic gonadotropin production from this source is a rare cause of precocious puberty in boys. Since a report by Behrendt in 1931, about 25 cases of hepatoblastoma with precocious puberty have been reported in the literature. We describe here a new case of hepatoblastoma with precocious puberty in a 2-year-9-month-old boy. He presented with precocious puberty and abdominal mass. He had an enlarged penis, pubic hair and deep voice. Laboratory examination revealed that serum alpha-fetoprotein(AFP) was above 70,000ng/mL, and serum beta-human chorionic gonadotropin(beta-HCG) 360mIU/mL. Abdominal CT revealed a huge tumor occupying the entire right lobe of the liver. A diagnosis of hepatoblastoma was made by percutaneous needle biopsy. After completing four cycles of chemotherapy, a complete tumor resection was undertaker. Postoperative level of the serum AFP was below 5ng/mL, and serum beta-HCG below 3mIU/mL. He received two additional cycles of treatment. He is alive and in a disease-free state for two years after cessation of the treatment.
Biopsy, Needle
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Chorion
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Chorionic Gonadotropin
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Diagnosis
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Drug Therapy
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Gonadotropins
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Hair
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Hepatoblastoma*
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Humans
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Liver
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Male
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Penis
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Puberty, Precocious*
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Tomography, X-Ray Computed
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Virilism
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Voice