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Puberty precocis

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https://www.readbyqxmd.com/read/28101370/recurrent-ovarian-sertoli-leydig-cell-tumor-in-a-child-with-peutz-jeghers-syndrome
#1
Edward J Bellfield, Ramin Alemzadeh
We present a female child with Peutz-Jeghers syndrome (PJS) with a recurrent ovarian Sertoli-Leydig cell tumor (SLCT). SLCTs are relatively rare sex cord neoplasms that can occur in PJS. The patient was an African-American female who first presented at the age of 3 years with precocious puberty, and then at the age of 17 years with abdominal pain and irregular menses. In each case, she had resection of the mass, which included oophorectomy. To our knowledge, this is the first reported case in a child with PJS to have a recurrent ovarian SLCT...
August 2016: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/28091641/effects-of-pomegranate-extract-in-supplementing-gonadotropin-releasing-hormone-therapy-on-idiopathic-central-precocious-puberty-in-chinese-girls-a-randomized-placebo-controlled-double-blind-clinical-trial
#2
Jinsheng Liu, Jiulai Tang
Central precocious puberty (CPP) without organic abnormality is called idiopathic CPP (ICPP). The objective of this trial was to evaluate the effects of pomegranate extract in supplementing gonadotropin-releasing hormone (GnRH) analog therapy on ICPP-affected girls in the Chinese population. 286 girls, diagnosed with ICPP were initially enrolled into this trial, and among them 225 eligible patients were randomized to receive a combinational GnRH analog treatment supplemented with either a placebo or pomegranate extract on a daily basis for a period of 3 months...
January 16, 2017: Food & Function
https://www.readbyqxmd.com/read/28088414/social-dominance-in-prepubertal-dairy-heifers-allocated-in-continuous-competitive-dyads-effects-on-body-growth-metabolic-status-and-reproductive-development
#3
C Fiol, M Carriquiry, R Ungerfeld
The objective of this study was to compare the body weight (BW) and size, metabolic status, and reproductive development of dominant and subordinate prepubertal dairy heifers allocated in competitive dyads. Sixteen Holstein and Jersey × Holstein prepubertal heifers (means ± SEM; 250.8 ± 9.8 d; 208.5 ± 13.9 kg of BW) were assigned to 8 homogeneous dyads according to breed, age, and BW. Dyads were housed in pens separated 1 m from each other during 120 d, receiving a total mixed ration on a 5% restriction of their potential dry matter intake, and had access to the same feeder (60 cm) throughout the experiment...
January 11, 2017: Journal of Dairy Science
https://www.readbyqxmd.com/read/28071630/ovarian-tumors-in-children-10-year-experience-from-a-tertiary-care-center-in-south-india
#4
B Rajeswari, M Nair, A Ninan, K Parukuttyamma
BACKGROUND: Ovarian tumors are uncommon in childhood and constitute around 1% of childhood malignancies. Two thirds of pediatric ovarian tumors are germ cell tumors. Epithelial ovarian tumors and stromal tumors are less frequent. We share our experience in childhood ovarian cancers, analyzing a series of cases with respect to the clinical profile, treatment and survival. METHODS: All newly diagnosed ovarian tumors in children up to 14 years of age, registered in our Pediatric Oncology Division between January 2000 and December 2009 were retrospectively reviewed...
April 2016: Indian Journal of Cancer
https://www.readbyqxmd.com/read/28070054/-van-wyk-grumbach-syndrome-a-case-report-and-%C3%A2-literature-review
#5
Ping Jin, Qin Zhang, Zhaohui Mo, Fan Yang, Yanhong Xie
Van Wyk-Grumbach syndrome (VWGS) is a rare complication of prolonged untreated juvenile hypothyroidism characterized by precocious puberty and enlarged multicystic ovaries. A 13-year-old girl visited our outpatient clinic due to menstrual irregularities. She had precocious puberty, pituitary hyperplasia and multiple cystic ovaries in addition to clinical signs of severe congenital hypothyroidism. After the initiation of L-thyroxine therapy, the symptoms were alleviated in a short time. This rare syndrome is easy to be misdiagnosed as pituitary and ovarian tumor...
December 28, 2016: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/28049241/predictive-value-of-dental-maturity-for-a-positive-gonadotropin-releasing-hormone-stimulation-test-result-in-girls-with-precocious-puberty
#6
Jee Seon Baik, Jin Woo Choi, Su Jin Kim, Ji Hyun Kim, Sollip Kim, Jae Hyun Kim
Dental maturity is associated with skeletal maturity, which is advanced in girls with central precocious puberty (CPP). We investigated the performance of dental maturity as a screening method for CPP using mandibular second premolar and molar calcification stages, assessed the associated anthropometric and laboratory factors, and evaluated pubertal response predictors using the gonadotropin-releasing hormone stimulation test (GnRHST) in prepubertal and pubertal girls. A prospective case-control study was conducted in girls, aged 7...
February 2017: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/28008860/basal-serum-neurokinin-b-level-can-be-used-to-differentiate-idiopathic-central-precocious-puberty-from-premature-thelarche-in-girls
#7
Mesut Parlak, Doğa Türkkahraman, Hamit Yaşar Ellidağ, Gamze Çelmeli, Ayşe Eda Parlak, Necat Yılmaz
OBJECTIVE: The aim of the study is to investigate the diagnostic role of kisspeptin and neurokinin B in idiopathic central precocious puberty (ICPP) and premature thelarche (PT). METHODS: The girls who presented with early breast development before the age of 8 years were evaluated. Basal and stimulated FSH, LH levels and basal neurokinin B and kisspeptin levels were measured. Patients who had peak LH > 5mIU/mL, and a bone age (BA) /chronological age (CA) ratio >1...
December 23, 2016: Journal of Clinical Research in Pediatric Endocrinology
https://www.readbyqxmd.com/read/27998218/prevalence-and-risk-factors-of-early-endocrine-disorders-in-childhood-brain-tumor-survivors-a-nationwide-multicenter-study
#8
Sarah C Clement, Antoinette Y N Schouten-van Meeteren, Annemieke M Boot, Hedy L Claahsen-van der Grinten, Bernd Granzen, K Sen Han, Geert O Janssens, Erna M Michiels, A S Paul van Trotsenburg, W Peter Vandertop, Dannis G van Vuurden, Leontien C M Kremer, Hubert N Caron, Hanneke M van Santen
Purpose To evaluate the prevalence of, and risk factors for, early endocrine disorders in childhood brain tumor survivors (CBTS). Patients and Methods This nationwide study cohort consisted of 718 CBTS who were diagnosed between 2002 and 2012, and who survived ≥ 2 years after diagnosis. Patients with craniopharyngeoma or a pituitary gland tumor were excluded. Results of all endocrine investigations, which were performed at diagnosis and during follow-up, were collected from patient charts. Multivariable logistic regression was used to study associations between demographic and tumor- and treatment-related variables and the prevalence of early endocrine disorders...
December 20, 2016: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/27990598/disorders-of-sex-development-dsd-not-only-babies-with-ambiguous-genitalia-a-practical-guide-for-surgeons
#9
Irene Kearsey, John M Hutson
INTRODUCTION AND METHODS: In this review, we describe the common clinical scenarios that may be present to a paediatric surgeon when a patient has a disorder of sex development (DSD). Our aim was to prepare surgeons so that they can respond with correct approaches to diagnose and manage the given situations. RESULTS: DSD present in three distinct clinical situations: in the neonate with some abnormality of the external genitalia; in the child undergoing surgical treatment for inguinal hernia or during open or laparoscopic orchidopexy or during hypospadias correction; and at or after puberty, which may be precocious or delayed or in an adolescent girl with masculinisation at puberty...
December 18, 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27988506/plasma-concentrations-of-osteocalcin-are-associated-with-the-timing-of-pubertal-progress-in-boys
#10
Michael M Schündeln, Lena Bäder, Cordula Kiewert, Ralf Herrmann, Dagmar Führer, Berthold P Hauffa, Corinna Grasemann
BACKGROUND: Animal models have shown that the skeletal hormone osteocalcin stimulates testicular testosterone synthesis. To assess whether osteocalcin might be a useful marker to detect pubertal development disorders, we examined osteocalcin plasma concentrations in children and adolescents with and without disorders of pubertal development. METHODS: Osteocalcin concentrations were investigated in a total of 244 patients with endocrine disorders (122 males, mean age: 11...
December 19, 2016: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/27987429/a-reduction-in-long-term-spatial-memory-persists-after-discontinuation-of-peripubertal-gnrh-agonist-treatment-in-sheep
#11
D Hough, M Bellingham, I R Haraldsen, M McLaughlin, J E Robinson, A K Solbakk, N P Evans
Chronic gonadotropin-releasing hormone agonist (GnRHa) administration is used where suppression of hypothalamic-pituitary-gonadal axis activity is beneficial, such as steroid-dependent cancers, early onset gender dysphoria, central precocious puberty and as a reversible contraceptive in veterinary medicine. GnRH receptors, however, are expressed outside the reproductive axis, e.g. brain areas such as the hippocampus which is crucial for learning and memory processes. Previous work, using an ovine model, has demonstrated that long-term spatial memory is reduced in adult rams (45 weeks of age), following peripubertal blockade of GnRH signaling (GnRHa: goserelin acetate), and this was independent of the associated loss of gonadal steroid signaling...
November 30, 2016: Psychoneuroendocrinology
https://www.readbyqxmd.com/read/27982202/a-boy-with-prader-willi-syndrome-unmasking-precocious-puberty-during-growth-hormone-replacement-therapy
#12
Natasha G Ludwig, Rafael F Radaeli, Mariana M X Silva, Camila M Romero, Alexandre J F Carrilho, Danielle Bessa, Delanie B Macedo, Maria L Oliveira, Ana Claudia Latronico, Tânia L Mazzuco
Prader-Willi syndrome (PWS) is a genetic disorder frequently characterized by obesity, growth hormone deficiency, genital abnormalities, and hypogonadotropic hypogonadism. Incomplete or delayed pubertal development as well as premature adrenarche are usually found in PWS, whereas central precocious puberty (CPP) is very rare. This study aimed to report the clinical and biochemical follow-up of a PWS boy with CPP and to discuss the management of pubertal growth. By the age of 6, he had obesity, short stature, and many clinical criteria of PWS diagnosis, which was confirmed by DNA methylation test...
November 2016: Archives of Endocrinology and Metabolism
https://www.readbyqxmd.com/read/27941749/central-precocious-puberty-a-case-report
#13
T Haq, A M Taib, S Ikhtaire
Precocious puberty in a male child is uncommon. Moreover, a predominantly luteinising hormone secreting pituitary tumor is rare. We report a case of predominantly luteinising hormone secreting pituitary adenoma causing precocious puberty in a three year old boy. A thorough systematic investigation including serum gonadotropins and imaging should be done to search for an underlying cause, even in the absence of pressure symptoms. Surgery with long term follow up is the recommended treatment for functioning gonadotrophin tumors...
October 2016: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/27940757/atypical-leydig-cell-tumor-in-children-report-of-2-cases
#14
Chiara Mameli, Giorgio Selvaggio, Chiara Cerini, Gaetano Bulfamante, Cristina Madia, Giovanna Riccipetitoni, Gian Vincenzo Zuccotti
Leydig cell tumors (LCTs) are rare cord-stromal tumors that may occur in testis or ovaries and may produce androgens or estrogens. The majority has been found in men between the ages of 20 and 60 years. Adults with androgen-secreting LCTs are usually asymptomatic; feminizing syndromes may result from the production of estradiol or the peripheral aromatization of testosterone. In children, LCTs usually present between 5 and 10 years of age with isosexual precocious pseudopuberty or gynecomastia. We report 2 cases of LCT in prepubertal boys presenting with advanced unilateral pubarche and testicular volume asymmetry...
November 2016: Pediatrics
https://www.readbyqxmd.com/read/27940666/rasopathies-are-associated-with-delayed-puberty-are-they-associated-with-precocious-puberty-too
#15
Daniëlle C M van der Kaay, Bat-Sheva Levine, Daniel Doyle, Roberto Mendoza-Londono, Mark R Palmert
RASopathies, such as Noonan, Costello, and cardio-facio-cutaneous syndromes, are developmental disorders caused by mutations in rat sarcoma-mitogen-activated protein kinase pathway genes. Mutations that cause Noonan syndrome have been associated with delayed puberty. Here we report 4 patients with either Costello or cardio-facio-cutaneous syndrome who developed precocious puberty, suggesting complex regulation of the hypothalamic-pituitary-gonadal axis and the timing of puberty by the rat sarcoma-mitogen-activated protein kinase pathway...
December 2016: Pediatrics
https://www.readbyqxmd.com/read/27931036/clinical-exome-sequencing-reveals-mkrn3-pathogenic-variants-in-familial-and-nonfamilial-idiopathic-central-precocious-puberty
#16
Nelmar Valentina Ortiz-Cabrera, Rosa Riveiro-Álvarez, Miguel Ángel López-Martínez, Pilar Pérez-Segura, Isabel Aragón-Gómez, María José Trujillo-Tiebas, Leandro Soriano-Guillén
BACKGROUND/AIMS: Idiopathic central precocious puberty (ICPP) is the premature activation of the hypothalamic-pituitary-gonadal axis in the absence of organic disease. Up to now, just gain-of-function mutations of KISS1/KISS1R and loss-of-function mutations of the maternally imprinted gene MKRN3 are the known genetic causes of ICPP. Our intention is to evaluate variants present in genes related to the pubertal onset pathway that could act as disease-causing or predisposing variants. METHODS: We studied the clinical exome of 20 patients diagnosed with ICPP using the Illumina platform...
December 9, 2016: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/27928728/clinical-perspectives-in-congenital-adrenal-hyperplasia-due-to-11%C3%AE-hydroxylase-deficiency
#17
REVIEW
Krupali Bulsari, Henrik Falhammar
Congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency is a rare autosomal recessive genetic disorder. It is caused by reduced or absent activity of 11β-hydroxylase (CYP11B1) enzyme and the resultant defects in adrenal steroidogenesis. The most common clinical features of 11 beta-hydroxylase deficiency are ambiguous genitalia, accelerated skeletal maturation and resultant short stature, peripheral precocious puberty and hyporeninemic hypokalemic hypertension. The biochemical diagnosis is based on raised serum 11-deoxycortisol and 11-deoxycorticosterone levels together with increased adrenal androgens...
December 7, 2016: Endocrine
https://www.readbyqxmd.com/read/27914139/genetic-variations-of-the-kiss1r-gene-in-korean-girls-with-central-precocious-puberty
#18
Yeon Joung Oh, Young Jun Rhie, Hyo Kyoung Nam, Hye Ryun Kim, Kee Hyoung Lee
The timing of puberty onset varies greatly among individuals, and much of this variation is modulated by genetic factors. This study aimed to identify the kisspeptin receptor (KISS1R) gene variations and to investigate the associations between these variations and central precocious puberty (CPP). Korean girls with CPP (n = 194) and their healthy controls (n = 99) were included in this study. The entire coding region and the exon-intron boundaries (exon 1 through 5) of the KISS1R gene were directly sequenced...
January 2017: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/27903360/-update-on-pubertal-development-among-primary-school-students-in-shanghai-2014
#19
Y Chen, Y T Zhang, C Chen, Y R Jiang, Y J Song, S J Liu, F Jiang
Objective: To investigate the current prevalence of pubertal development in healthy Shanghai schoolchildren. Methods: This study was a cross-sectional investigation focused on current pubertal development conducted in healthy Shanghai schoolchildren by multi-stage cluster sampling. The sample included 17 571 children in grades 1-5 investigated in June 2014. The data were weighted by inverse probability weighting (IPW) to make them more representative. At examination, stages of breast and pubic hair development were rated according to the Tanner method...
November 6, 2016: Zhonghua Yu Fang Yi Xue za Zhi [Chinese Journal of Preventive Medicine]
https://www.readbyqxmd.com/read/27897024/-peripheral-precocious-puberty
#20
J Zvonařová Skalická, R Pilka
OBJECTIVE: To summarize literature data on peripheral precocious puberty. DESIGN: A literature review. SETTING: Středomoravská nemocniční a.s., hospital Šternberk, Department of Obstetrics and Gynaecology, University Hospital, Medical Faculty, Palacky University, Olomouc. METHODS AND RESULTS: We searched in PubMed using the key words stated below according to date and published since 1980.Peripheral precocious puberty occurs in girls with the frequency 1:400-1000...
2016: Ceská Gynekologie
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