Leptin Levels in Children with Central Precocious Puberty (CPP): The Impact of Reversible Gonadal Sex Steroid Suppression † 465

Abstract Objective: We assessed in a retrospective unicenter study the impact of treatment with GnRH analogs (GnRHa) on adult height (AH), body mass index (BMI), bone mineral density (BMD), and reproductive function in girls with idiopathic central precocious puberty (ICPP). Patients: Eighty-seven ICPP patients were treated with GnRHa for 4.2 ± 1.6 yr (range 3–7.9) and observed for 9.9 ± 2.0 yr (range 4–10.6 yr) after discontinuation of treatment; to estimate the efficacy better, 32 comparable ICPP untreated girls were analyzed. Results: AH was 159.8 ± 5.3 cm, significantly higher than pretreatment predicted AH (PAH) either for accelerated or for average tables of Bayley and Pinneau. The gain in centimeters between pretreatment PAH and AH was 5.1 ± 4.5 and 9.5 ± 4.6 cm, respectively. Hormonal values and ovarian and uterine dimensions, reduced during treatment, increased to normal after 1 yr without therapy. Age of menarche was 13.6 ± 1.1 yr with an interval of 0.9 ± 0.4 yr after therapy. Menstrual pattern was normal. Six girls became pregnant and delivered normal offspring. BMI sd score for chronological age increased, but not significantly, before, during, and after therapy. BMD at discontinuation of treatment was significantly lower and increased to control values after gonadal activity resumption. Conclusions: GnRHa treatment in ICPP is safe for the reproductive system, BMD, and BMI and helpful in reaching AH close to target height; however, the variability of individual responses suggests that one choose more parameters than increment in height, especially in girls with pubertal onset over 8 yr of age.

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Precocious Puberty- A Case Report

TAJ Journal of Teachers Association ◽

10.3329/taj.v21i1.3227 ◽

1970 ◽

Vol 21 (1) ◽

pp. 83-86

Author(s):

K Kabiruzzaman Shah ◽

N Begum Laz ◽

AB Siddique ◽

FU Ahmed

Keyword(s):

Case Report ◽

Precocious Puberty ◽

Sexual Development ◽

Central Precocious Puberty ◽

Female Child ◽

Male Child ◽

Sex Steroid ◽

Steroid Secretion ◽

Gonadotrophin Secretion ◽

Hypothalamic Pituitary Axis

Appearance of secondary sexual development before the age of 9 in a male child and before the age of 7 in a female child is called precocious puberty. When the cause of precocious puberty is premature activation of the hypothalamic-pituitary axis, it is called central or complete precocious puberty, if ectopic gonadotrophin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex it is called incomplete precocious puberty. |Here we are reporting a 5 year old girl with central precocious puberty. doi: 10.3329/taj.v21i1.3227 TAJ 2008; 21(1): 83-86

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Low Frequency of MKRN3 and DLK1 Variants in Chinese Children with Central Precocious Puberty

International Journal of Endocrinology ◽

10.1155/2019/9879367 ◽

2019 ◽

Vol 2019 ◽

pp. 1-6

Author(s):

Ting Chen ◽

Linqi Chen ◽

Haiying Wu ◽

Rongrong Xie ◽

Fengyun Wang ◽

...

Keyword(s):

Precocious Puberty ◽

Protein Structures ◽

Ring Finger ◽

Low Frequency ◽

Central Precocious Puberty ◽

Bioinformatic Analysis ◽

Chinese Patients ◽

Early Puberty ◽

Hydrogen Bond Formation ◽

The Impact

Background. Central precocious puberty (CPP) is defined by gonadotropin-dependent development of secondary sexual characteristics before the age of 8 years in girls and 9 years in boys. MKRN3 and DLK1 are two genes, disease-causing variants of which have recently been discovered to cause idiopathic CPP. Methods. We screened 173 Chinese patients (9 males and 164 females; 9 familial and 164 sporadic) with ICPP and 43 patients (9 males and 34 females; 3 familial and 40 sporadic) with early puberty for variants in MKRN3. We also screened 19 patients with ICPP and early puberty for variants of DLK1 (17 males and 2 females; 5 familial and 14 sporadic). Results. We identified four novel missense variants of MKRN3, c.1138G > A (p.Glu380Lys), c.1420T > A (p.Leu474Met), c.673C > G (p.Leu225Val), and c.1071C > G (p.Ile357Met) in two sporadic cases and three familial cases. According to ACMG standards, two MKRN3 variant (p.Glu380Lys and p.Ile357Met) are likely pathogenic, and two others are of uncertain significance. We also performed bioinformatic analysis to evaluate the impact of variants on MKRN3 protein structures, which showed that Ile357Met locates at the zinc-binding region (C3HC4 RING finger motif), while Glu380Lys is spatially extremely close to the C3HC4 RING finger, MKRN-specific Cys-His domain, and the third C3H1 zinc-finger motif region. Per Glu380Lys, Glu with negative charges has been changed into Lys with positive charges, which may affect the hydrogen bond formation between amino acids and the stability of the local structure, thus affecting the binding of zinc iron to MKRN3 protein. Besides, we did not identify any variants of DLK1 gene in our patients. Conclusions. In this study, we report four novel MKRN3 variants in patients with ICPP. Moreover, we did not find any variants of DLK1 gene. Variants of MKRN3 are relatively uncommon in Chinese ICPP patients.

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The Impact of Reversible Gonadal Sex Steroid Suppression on Serum Leptin Concentrations in Children with Central Precocious Puberty1

The Journal of Clinical Endocrinology & Metabolism ◽

10.1210/jcem.83.4.4700 ◽

1998 ◽

Vol 83 (4) ◽

pp. 1091-1096

Author(s):

Mark R. Palmert ◽

Sally Radovick ◽

Paul A. Boepple

Keyword(s):

Body Composition ◽

Longitudinal Study ◽

Sex Steroids ◽

Central Precocious Puberty ◽

Sex Steroid ◽

Serum Leptin ◽

Reproductive Axis ◽

Discernible Effect ◽

The Impact ◽

The Relationship

Serum leptin concentrations increase during childhood in both sexes. During sexual maturation, levels rise further in girls, but decrease in boys. These data suggest that testosterone either directly suppresses leptin levels or induces changes in body composition that result in lower leptin concentrations. To examine further the relationship between sex steroids and leptin, we performed a longitudinal study in children with central precocious puberty (28 girls and 12 boys) before, during, and after discontinuation of GnRH agonist-induced pituitary-gonadal suppression. Nighttime and daytime leptin levels were measured to determine whether the activity of the pituitary-gonadal axis affects their diurnal variation. In the boys, suppression of testosterone increased leptin levels, whereas resumption of puberty was associated with decreased leptin levels [3.5 ± 0.8 vs. 9.5 ± 3.1 ng/dL (P = 0.005) and 12.2 ± 4.5 vs. 7.0 ± 2.6 ng/dL (P = 0.012), respectively]. Serum leptin levels did not change in the girls with alteration of the pituitary-ovarian axis and consistently exceeded those in boys. Nighttime levels were consistently greater than daytime values by an average of 38.3% in the girls and 29.4% in the boys. These serial observations during reversible pituitary-gonadal suppression suggest that testosterone decreases leptin concentrations, but that estrogen, at least in this childhood model, has no discernible effect. In addition, our data indicate that the presence of the diurnal rhythm in leptin concentrations is independent of the state of the reproductive axis.

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Growth, Growth Hormone and Sex Steroid Secretion in Girls with Central Precocious Puberty Treated with a Gonadotrophin Releasing Hormone (GnRH) Analogue

Acta Paediatrica ◽

10.1111/j.1651-2227.1988.tb10694.x ◽

1988 ◽

Vol 77 (4) ◽

pp. 525-530 ◽

Cited By ~ 34

Author(s):

R. STANHOPE ◽

P. J. PRINGLE ◽

C. G. D. BROOK

Keyword(s):

Growth Hormone ◽

Precocious Puberty ◽

Central Precocious Puberty ◽

Sex Steroid ◽

Gnrh Analogue ◽

Steroid Secretion ◽

Releasing Hormone ◽

Gonadotrophin Releasing Hormone

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Central precocious puberty in spina bifida children: Guidelines for the care of people with spina bifida

Journal of Pediatric Rehabilitation Medicine ◽

10.3233/prm-200728 ◽

2020 ◽

Vol 13 (4) ◽

pp. 557-563

Author(s):

Nourah Almutlaq ◽

Joseph O’Neil ◽

John S. Fuqua

Keyword(s):

Spina Bifida ◽

Precocious Puberty ◽

Early Recognition ◽

Central Precocious Puberty ◽

Fourth Edition ◽

Diagnosis And Management ◽

Pediatric Endocrinologist ◽

The Impact ◽

Timely Referral

Children with spina bifida are at greater risk of developing central precocious puberty (CPP) compared to others. Therefore, early recognition and timely referral for further evaluation by a pediatric endocrinologist allows appropriate management that reduces the impact of CPP. This article discusses the diagnosis and management of CPP in children with spina bifida. This guideline was developed for SB Transition Healthcare Guidelines from the 2018 Spina Bifida Association’s Fourth Edition of the Guidelines for the Care of People with Spina Bifida.

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