Rebecca Schneider Aguirre, Erica A Eugster
Central precocious puberty (CPP) results from early activation of the hypothalamic - pituitary -gonadal (HPG) axis and follows the same sequence as normal puberty. While many factors involved in pubertal initiation remain poorly understood, the kisspeptin system is known to play a key role. Currently, mutations in the kisspeptin system, MKRN3, and DLK1 have been identified in sporadic and familial cases of CPP. The diagnosis is based on physical exam findings indicating advancing puberty and on laboratory tests confirming central HPG axis activation...
August 2018: Best Practice & Research. Clinical Endocrinology & Metabolism
Mateus Cavarzan Lopes, Carolina Oliveira Ramos, Ana Claudia Latronico, Berenice B Mendonça, Vinicius N Brito
OBJECTIVES: Unfavorable predicted adult height and psychosocial inadequacy represent parameters used to guide therapeutic intervention in girls with central precocious puberty. Gonadotropin-releasing hormone analog is the first-line treatment. The aim of this study was to compare two methods used to predict adult height and assess a validated tool for predicting the age at menarche in girls with central precocious puberty. METHODS: The predicted adult height of 48 girls with central precocious puberty was calculated at diagnosis using the Bayley-Pinneau method based on average and advanced bone age tables and compared with the predicted adult height calculated using a mathematical model...
July 26, 2018: Clinics
Paul B Kaplowitz, Philippe F Backeljauw, David B Allen
The use of gonadotropin-releasing hormone analogs (GnRHa) for the treatment of central precocious puberty (CPP), especially in girls, has increased rapidly in recent years. In the context of a secular trend towards earlier puberty onset, many girls now treated for CPP are healthy children experiencing puberty onset within the early end of the normal range. Justifications for GnRHa treatment include the preservation of adult height (AH) potential and the alleviation of presumed distress of early maturation and menarche...
2018: Hormone Research in Pædiatrics
Jinshui He, Yueya Kang, Lixia Zheng
BACKGROUND: This paper aims to investigate the correlation between serum levels of luteinizing hormone (LH), insulin-like growth factor-1 (IGF-1) and leptin and the incidence of idiopathic central precocious puberty (ICPP) in girls, and to explore the clinical values in the diagnosis of ICPP. METHODS: A total of 48 girls with ICPP were selected in our hospital from March 2014 to March 2015 to serve as ICPP group. At the same time, 48 girls with the same age distribution were selected as control group...
July 24, 2018: Minerva Pediatrica
Audrey Vincent, Jean-Claude Souberbielle, Raja Brauner
BACKGROUND: The bone markers bone alkaline phosphatase (BAP) and C-terminal telopeptide of type I collagen crosslinks (CTX) are correlated with growth rate during normal puberty. The objective of this study was to evaluate the relationship between the serum concentrations of BAP and CTX and growth evolution in girls with idiopathic central precocious puberty (CPP) to help predict adult height. METHODS: A retrospective single-center study was conducted in 74 girls with CPP for whom a serum sample at initial evaluation was available to retrospectively measure BAP and CTX concentrations; 66...
July 9, 2018: BMC Pediatrics
Rosita A Condorelli, Rossella Cannarella, Aldo E Calogero, Sandro La Vignera
INTRODUCTION: The assessment of testicular function is not currently performed in childhood. The aim of this review was to address the usefulness of serum Anti-Müllerian Hormone (AMH), inhibin B, and testicular volume (TV) evaluation in children. REVIEW: Serum AMH and inhibin B levels reflect Sertoli cells (SCs) health and number, SCs maturation degree and their exposure to FSH and to intra-tubular testosterone (T). These hormones might be helpful in discriminating between congenital central hypogonadism (cHH) and constitutional delay in growth and puberty (CDGP) and in case of clinical suspicious of precocious puberty...
November 2018: Endocrine
Dong Wook Kim, Junghwan Suh, Ah Reum Kwon, Hyun Wook Chae, Choon Sik Yoon, Ho-Seong Kim, Duk Hee Kim
PURPOSE: This study aimed to investigate associations of central obesity with sexual maturation and metabolic parameters in Korean girls with precocious puberty. METHODS: This retrospective study evaluated data from 72 girls under 8 years of age with a chief complaint of early breast development. The patients were categorized as central precocious puberty (CPP) subjects or non-CPP subjects based on their gonadotropin-releasing hormone stimulation test results. Visceral fat thickness (VFT) was measured using ultrasonography and defined as the distance from the linea alba to the aorta...
June 2018: Annals of Pediatric Endocrinology & Metabolism
Jisun Lee, Jinsup Kim, Aram Yang, Sung Yoon Cho, Dong-Kyu Jin
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...
June 2018: Annals of Pediatric Endocrinology & Metabolism
Silvano Bertelloni, Cristina Mucaria, Giampiero I Baroncelli, Diego Peroni
Triptorelin depot is largely used to treat central precocious puberty (CPP) in children. Areas covered: This review updates triptorelin depot treatment of CPP, focusing on trials that compared 3.75 mg/28 day treated and untreated children till the adult height (AH). Efficacy of the new 11.25 mg/90 days or 22.5 mg/6 month formulations in suppressing pituitary-gonadal axis in short-term trials is also addressed. Short- and long-term safety was summarized. Expert commentary: Long experience on triptorelin depot use in children with CPP is available...
July 2018: Expert Review of Clinical Pharmacology
Lisa Swartz Topor, Kimberly Bowerman, Jason T Machan, Courtney L Gilbert, Tairmae Kangarloo, Natalie D Shaw
OBJECTIVE: Recent studies in the US and abroad suggest that boys are undergoing puberty at a younger age. It is unknown if this secular trend extends to boys with central precocious puberty (CPP), who sit at the extreme end of the pubertal spectrum, and if neuroimaging should remain a standard diagnostic tool. STUDY DESIGN: Retrospective chart review of all boys with CPP seen by Endocrinology at a US pediatric hospital from 2001-2010. RESULTS: Fifty boys had pubertal onset at an average age of 7...
2018: PloS One
Sezer Acar, Nur Arslan, Ahu Paketçi, Tuncay Derya Okur, Korcan Demir, Ece Böber, Ayhan Abacı
Tay-Sachs disease is an autosomal recessive type of lysosomal storage disorder. The disease is very rare in Turkey, with an incidence of 0.54/100,000. The clinical manifestations of Tay-Sachs disease include progressive developmental delay, seizures, deafness, blindness, spasticity, and dystonia, which are caused by the accumulation of gangliosides in the central nervous system. To date, only one case indicating the association between Tay-Sachs disease and central precocious puberty has been reported. Although the mechanism of this association is not clear, it is thought to be due to ganglioside accumulation in the central nervous system or the inhibition of the hypothalamic inhibiting pathway...
September 2018: Hormones: International Journal of Endocrinology and Metabolism
Sena Cantas-Orsdemir, Jane L Garb, Holley F Allen
Background Some pediatric endocrinologists recommend that girls with central precocious puberty (CPP) have cranial magnetic resonance imaging (MRI) performed only if they are younger than 6 years of age. However, no practice guidelines exist. The objective of this review was to assess the frequency of intracranial lesions in girls with CPP. Content We searched six electronic databases (PubMed, Cochrane, Web of Science, SCOPUS, ProQuest, and Dissertation & Theses) from 1990 through December 2015. We included studies on girls with CPP and MRI data...
July 26, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
Yılmaz Kor
Familial testotoxicosis is a disease with autosomal dominant inheritance that only affects men and which causes gonadotropin-independent precocious puberty. Although basal levels of luteinizing hormone and follicle-stimulating hormone are low, similar to what is expected in the pre-pubertal period, testosterone levels are high. Bicalutamide as an anti-androgen medication and anastrozole as an aromatase inhibitor have been proposed as agents that can be safely used in children. In the present study, we present the case of coexistent familial testotoxicosis and central precocious puberty induced by long-term androgen exposure in a patient aged 7...
June 2018: Hormones: International Journal of Endocrinology and Metabolism
Karen O Klein, Peter A Lee
This review suggests a central theme: that the treatment of each patient presenting with evidence consistent with central precocious puberty (CPP) needs to be individualized. This pertains to multiple factors relating to growth and growth potential, monitoring patients on treatment with gonadotropin-releasing hormone analogue (GnRHa), evaluating psychological issues with CPP and therapy, and concerns about weight gain during GnRHa therapy. Individual cases are presented. New data on adult height and rate of bone age advance are included...
April 2018: Pediatric Endocrinology Reviews: PER
José Maria Soares-Jr, Felisbela Soares de Holanda, Cézar Noboru Matsuzaki, Isabel Cristina Esposito Sorpreso, Eduardo Carvalho de Arruda Veiga, Luiz Carlos de Abreu, Kátia Cândido Carvalho, Edmund Chada Baracat
BACKGROUND: Precocious puberty (PP) is defined as premature pubertal development. Its consequences surpass the physical evidence of sexual maturity with the premature epiphyseal closure of the long bones and the reduction of adult stature by varied degrees. Central PP is characteristically dependent on GnRH and most of its causes are not completely known. Altered estrogen action is also believed to be involved in the genesis of PP. In fact, estrogen receptor alpha (Rea) gene polymorphisms may be associated with early age at menarche...
May 25, 2018: BMC Medical Genetics
Delanie B Macedo, Monica M França, Luciana R Montenegro, Marina Cunha-Silva, Danielle S Best, Ana Paula Abreu, Ursula B Kaiser, Berenice B Mendonca, Alexander A L Jorge, Vinicius N Brito, Ana Claudia Latronico
CONTEXT: Loss-of-function mutations in the coding region of MKRN3, a maternally imprinted gene at chromosome 15q11.2, are a common cause of familial central precocious puberty (CPP). Whether MKRN3 alterations in regulatory regions can cause CPP has not been explored to date. We aimed to investigate potential pathogenic variants in the promoter region of MKRN3 in patients with idiopathic CPP. PATIENTS/METHODS: A cohort of 110 patients with idiopathic CPP was studied...
2018: Neuroendocrinology
Karen O Klein, Sanja Dragnic, Ahmed M Soliman, Peter Bacher
BACKGROUND: Children with central precocious puberty (CPP) are treated with gonadotropin-releasing hormone agonists (GnRHa) to suppress puberty. Optimizing treatment outcomes continues to be studied. The relationships between growth, rate of bone maturation (bone age/chronological age [ΔBA/ΔCA]), luteinizing hormone (LH), predicted adult stature (PAS), as well as variables influencing these outcomes, were studied in children treated with depot leuprolide (LA Depot) Methods: Subjects (64 girls, seven boys) with CPP received LA Depot every 3 months for up to 42 months...
June 27, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
Carla Marisa Lopez, Daniel Solomon, Susan D Boulware, Emily R Christison-Lagay
BACKGROUND: The objective of the study was to identify national trends in the utilization of histrelin acetate implants among transgender children in the United States. METHODS: We analyzed demographic, diagnostic and treatment data from 2004 to 2016 on the use of histrelin acetate reported to the Pediatric Health Information System (PHIS) to determine the temporal trends in its use for transgender-related billing diagnoses, e.g. "gender identity disorder"...
June 27, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
Hee-Kyung Lee, Sung-Hwan Choi, Dong Fan, Kyung-Mi Jang, Min-Seon Kim, Chung-Ju Hwang
OBJECTIVES: To identify unique characteristics of the craniofacial complex and dental maturity in girls with central precocious puberty (CPP). MATERIALS AND METHODS: This study included 34 Korean girls with idiopathic CPP (mean age, 8.6 ± .5 years) and 28 normal healthy girls of the same chronological age. An initial evaluation of the growth pattern of the craniofacial complex and dental maturity was conducted by analyzing lateral cephalometric and panoramic radiographs...
September 2018: Angle Orthodontist
Nursel Muratoğlu Şahin, Asiye Uğraş Dikmen, Semra Çetinkaya, Zehra Aycan
Objective: Data concerning subnormal growth velocity (GV) and factors that influence this during gonadotropin-releasing hormone analog (GnRHa) therapy for idiopathic central precocious puberty (ICPP) are scarce. We investigated the incidence of subnormal GV and associated factors in patients receiving GnRHa therapy for ICPP. Methods: In this retrospective cohort study, the records of 50 girls who had been diagnosed with ICPP and started on GnRHa treatment before the age of eight years were investigated...
July 31, 2018: Journal of Clinical Research in Pediatric Endocrinology
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