Central precocious puberty

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Puberty is a crucial biological process normally occurring at a specific time during the lifespan, during which sexual and somatic maturation are completed, and reproductive capacity is reached. Pubertal timing is not only determined by genetics, but also by endogenous and environmental cues, including nutritional and metabolic signals. During the last decade, we have learned much regarding the essential roles of kisspeptins and the neuropeptide pathways that converge on these neurones to modulate kisspeptin signalling, as well as neurokinin B and dynorphin, the co-transmitters of Kiss1 neurones in the arcuate nucleus, and the effects of melanocortins on puberty...

With improved survival of childhood, adolescent, and young adult (CAYA) cancer, the European survivor population of 300,000-500,000 continues to expand. Most survivors will experience at least one and often multiple cancer- and treatment-related late effects throughout their lives, including endocrine toxicities. Besides affecting their physical and psychosocial health status, these might reduce life expectancy and quality of life. Prevalent endocrine complications include hypothalamic-pituitary dysfunction, central precocious puberty, primary thyroid, male or female gonadal dysfunction, metabolic syndrome, and low bone mineral density...

There has been a global increase in pediatric endocrinology referrals for the concerns of early puberty. The objective of this study was to determine the reasons behind this increase. A retrospective cross-sectional study was designed to analyze the clinical characteristics of patients seen for the concerns of early puberty in pediatric endocrinology clinic of a tertiary care center (Study A). Additionally, a prospective questionnaire study was designed to assess the knowledge and concerns of the mothers regarding the timing of puberty in girls (Study B)...

BACKGROUND/AIMS: Kisspeptin (KP) is a key player in the regulation of the release of gonadotropin-releasing hormone (GnRH), which increases the secretion of gonadotropin during puberty to establish reproductive function and regulate the hypothalamic-pituitary-gonadal axis. Premature activation of GnRH secretion leads to idiopathic/central gonadotropin-dependent precocious puberty (CPP). We aimed to compare the blood KP concentrations in girls with CPP and healthy controls. METHODS: A systematic review and meta-analysis was performed...

BACKGROUND: The gold standard for the diagnosis of central precocious puberty (CPP) is gonadotropin-releasing hormone (GnRH) or GnRH analogs (GnRHa) stimulation test. But the stimulation test is time-consuming and costly. Our objective was to develop a risk score model readily adoptable by clinicians and patients. METHODS: A cross-sectional study based on the electronic medical record system was conducted in the Children's Hospital, Fudan University, Shanghai, China from January 2010 to August 2016...

Research suggests a role for cannabidiol oil in managing certain forms of paediatric onset epilepsy. However, studies on the impact of cannabis on the hypothalamo-pituitary-gonadal (HPG) axis have conflicting results. Delta-9-tetrahydrocannabinol (Δ9-THC) acutely inhibits gonadotropin-releasing hormone in the hypothalamus, reducing testosterone levels by 65% in rhesus monkeys. Additionally, there have been reports of pubertal arrest and delayed puberty in male cannabis users. In contrast, other studies have reported higher testosterone levels following long-term cannabis use...

OBJECTIVES: Gonadotropin-releasing hormone agonist treatment is important for optimal growth in girls with central precocious puberty (CPP). Data are lacking regarding benefit to height outcome when treatment is started after chronological age (CA) of 7 years, and if continued beyond CA of 10 years or bone age (BA) of 12 years. METHODS: Forty-eight girls with CPP were treated with monthly leuprolide depot. Change in predicted adult height (PAH) during treatment was assessed...

Objective: The present study was designed to compare the changes in ovarian and uterine parameters in girls with idiopathic central precocious puberty (ICPP) before and after gonadotropin-releasing hormone analogues (GnRHa) treatment to determine which sensitive indexes effectively reflect the therapeutic effect. Methods: Sixty girls diagnosed with ICPP were enrolled in the present study. Relevant data were recorded before treatment. Leuprorelin acetate microspheres were injected at a dose of 30-180 μg/(kg four weeks)...

BACKGROUND: Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is one of the most common autoimmune encephalitis in children. Most children recovered well after anti-NMDA receptor encephalitis. However, the NMDA receptor network functions are critical for the developing brain in children. The long-term consequences in pediatric patients of anti-NMDA receptor encephalitis are very infrequently reported. METHODS: This case series study retrospectively enrolled 10 children aged below 18 years old with antibody-proved anti-NMDA receptor encephalitis in a tertiary medical center from 2010 to 2019...

BACKGROUND: The Questionnaire for Triage and Assessment with 30 items (QTA30) is a standardized triage and assessment tool to assess pediatric psychosomatic disorders. It was estimated that one in ten children experience difficulties with regard to their school life in Japan. We evaluated mental health in children at an outpatient clinic in a local hospital with the QTA30. METHODS: All elementary and junior high school students 9 years of age or older who visited our institution between December 1, 2019 and March 31, 2020 were asked to complete the QTA30...

Introduction: Pediatric endocrinology rely greatly on hormone stimulation tests which demand time, money and effort. The knowledge of the pattern of pediatric endocrinology stimulation tests is therefore crucial to optimize resources and guide public health interventions. Aim of the study was to investigate the distribution of endocrine stimulation tests and the prevalence of pathological findings over a year and to explore whether single basal hormone concentrations could have saved unnecessary stimulation tests...

OBJECTIVES: The benefits of gonadotropin-releasing hormone analogues (GnRHa) in the treatment of central precocious puberty are well established, and their use is regarded as both safe and effective. Possible adverse effects on blood pressure (BP) and cardiac outcomes, body composition, bone health and brain development, however, continue to be of some concern. The aim of this study was to analyze BP changes in transgender female adolescents before and after receiving GnRHa and after adding estrogen treatment...

Central precocious puberty (CPP) results from early activation of the hypothalamic-pituitary-gonadal (HPG) axis. The current state of knowledge of the complex neural network acting at the level of the hypothalamus and the GnRH neuron to control puberty onset has expanded, particularly in the context of molecular interactions. Along with these advances, the knowledge of pubertal physiology and pathophysiology has also increased. This review focuses on regulatory abnormalities occurring at the hypothalamic level of the HPG axis to cause CPP...

BACKGROUND: Gonadotropin-releasing hormone analogues (GnRHa) administered as depot formulations are the standard of care for children with central precocious puberty (CPP). Puberty resumes after treatment discontinuation, but little is known concerning fertility in women who have been treated with GnRHa for CPP during childhood. METHODS: The PREFER (PREcocious puberty, FERtility) study prospectively analysed fertility, via a series of questionnaires, in women treated during childhood with triptorelin (depot formulation) for CPP...

BACKGROUND: A random ultrasensitive luteinizing hormone (LH) (LH-ICMA) ≥0.3 mIU/L is highly accurate in confirming a diagnosis of central precocious puberty (CPP). However, a prepubertal value does not exclude the diagnosis. The clinical differences between patients with CPP who have a pubertal versus prepubertal LH-ICMA have not been clearly defined. Furthermore, there is minimal information regarding the utility of this test in boys with CPP. The objective of this study was to analyze differences between patients diagnosed with CPP who had a pubertal versus prepubertal LH-ICMA, including a cohort of boys...

CONTEXT: Central precocious puberty(CPP) may arise from central nervous system(CNS) lesions in a few affected girls. Recently, the incidence of girls with CPP has increased mostly in 6-8 year-olds, in whom the necessity of magnetic resonance imaging(MRI) is debated. OBJECTIVE: To investigate the frequency, long-term outcome and potential predictors of CNS lesions in a large cohort of girls with CPP. DESIGN AND METHOD: A multi-center cohort of 770 Turkish girls with CPP who had systematic cranial MRI between 2005-2017...

BACKGROUND: Many central nervous system disorders result in hypothalamic-pituitary (HP) axis dysfunction. Alternating Hemiplegia of Childhood (AHC) is usually caused by mutations in the ATP1A3 subunit of the Na+ /K+  ATPase, predominantly affecting GABAergic interneurons. GABAergic interneurons and the ATP1A3 subunit are both important for function of the hypothalamus. However, whether HP dysfunction occurs in AHC and, if so, how such dysfunction manifests remains to be investigated...

The classic definition of precocious sexual maturation is the development of secondary sexual characteristics before 8 years of age in girls and before 9 years of age in boys. It is classified as central precocious puberty when premature maturation of the hypothalamic-pituitary-gonadal axis occurs, and as peripheral precocious puberty when there is excessive secretion of sex hormones, independent of gonadotropin secretion. Precocious sexual maturation is more common in girls, generally central precocious puberty of idiopathic origin...

The family of Poly(A)-binding proteins (PABPs) regulates the stability and translation of messenger RNAs (mRNAs). Here we reported that the three members of PABPs, including PABPC1, PABPC3 and PABPC4, were identified as novel substrates for MKRN3, whose deletion or loss-of-function mutations were genetically associated with human central precocious puberty (CPP). MKRN3-mediated ubiquitination was found to attenuate the binding of PABPs to the poly(A) tails of mRNA, which led to shortened poly(A) tail-length of GNRH1 mRNA and compromised the formation of translation initiation complex (TIC)...