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Adrenarche

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https://read.qxmd.com/read/30709755/iugr-genetic-influences-metabolic-problems-environmental-associations-triggers-current-and-future-management
#1
REVIEW
Feyza Darendeliler
The children with intrauterine growth restriction (IUGR) especially if they make a catch-up growth in early life have a higher risk for long term problems including short stature and also developing metabolic syndrome, Type 2 diabetes, insulin resistance and cardiovascular diseases. The studies also support that these children may have abnormalities in pubertal timing, adrenarche and reproductive function. The aim of this review was to summarize the published reports mainly on puberty and reproductive functions in children born IUGR at older ages in association with metabolic problems that they encounter...
January 22, 2019: Best Practice & Research. Clinical Endocrinology & Metabolism
https://read.qxmd.com/read/30703436/in-silico-and-functional-studies-reveal-novel-loss-of-function-variants-of-srd5a2-but-no-variants-explaining-excess-5%C3%AE-reductase-activity
#2
Efstathios Katharopoulos, Kay Sauter, Amit V Pandey, Christa E Flück
Androgens are steroid hormones essential for human male and female development. Steroid reductases 5α (SRD5 As) are key enzymes in androgen biosynthesis. Mutations in the human SRD5 A2 are known to cause loss-of-function and severe 46,XY undervirilization. Gain-of-function variants have been suggested in androgen excess syndromes, but have not been found so far. Therefore we searched for gain-of-function mutations in the human SRD5 A2 gene which might explain hyperandrogenic disorders such as the polycystic ovary syndrome, premature adrenarche and prostate cancer...
January 28, 2019: Journal of Steroid Biochemistry and Molecular Biology
https://read.qxmd.com/read/30658614/adrenarche-and-pubarche-in-girls-with-turner-syndrome-during-growth-promoting-therapy-with-human-growth-hormone
#3
Helmuth G Dörr, Theresa Penger, Michaela Marx, Manfred Rauh, Patricia G Oppelt, Thomas K M Völkl
BACKGROUND: Data on adrenarche and pubarche in girls with Turner syndrome (TS) are inconsistent in the literature. METHODS: The cohort consisted of 94 girls and young women with TS born between 1971 and 2001 (age range: 3.1-23.2 yrs.), who were treated with human growth hormone and regularly presented at our outpatient clinic every 4 to 6 months.The longitudinal data of all patients were ascertained retrospectively from patient charts. The data collection ended in January 2016...
January 18, 2019: BMC Endocrine Disorders
https://read.qxmd.com/read/30599482/interpretation-of-androgen-and-anti-mullerian-hormone-profiles-in-a-hispanic-cohort-of-5-to-8-year-old-girls-with-premature-adrenarche
#4
Preneet Cheema Brar, Elena Dingle, Daniela Ovadia, Sarah Pivo, Veeramac Prasad, Raphael David
PURPOSE: Premature adrenarche (PA) often leads to polycystic ovary syndrome (PCOS). Higher anti-mullerian hormone (AMH) levels are reported in PCOS. We studied the androgen profile and AMH profiles in Hispanic girls with PA (aged 5-8 years) and age and body mass index (BMI) matched controls. METHODS: Retrospective review of electronic medical records of girls who met the inclusion criteria for premature adrenarche were done. RESULTS: PA girls (n=76) were matched to control girls (n=12) for age (mean±standard deviation) (6...
December 2018: Annals of Pediatric Endocrinology & Metabolism
https://read.qxmd.com/read/30566905/causes-patterns-and-severity-of-androgen-excess-in-487-consecutively-recruited-pre-and-post-pubertal-children
#5
Jan Idkowiak, Yasir S Elhassan, Pascoe Mannion, Karen Smith, Rachel Webster, Vrinda Saraff, Timothy G Barrett, Nicholas G Shaw, Nils Krone, Renuka P Dias, Melanie Kershaw, Jeremy M Kirk, Wolfgang Högler, Ruth E Krone, Michael W O'Reilly, Wiebke Arlt
OBJECTIVE: Androgen excess in childhood is a common presentation and may signify sinister underlying pathology. Data describing its patterns and severity are scarce, limiting the information available for clinical decision processes. Here, we examined the differential diagnostic value of serum dehydroepiandrosterone sulfate (DHEAS), androstenedione (A4), and testosterone (T) in childhood androgen excess. DESIGN: Retrospective review of all children undergoing serum androgen measurement at a single center over 5 years...
December 1, 2018: European Journal of Endocrinology
https://read.qxmd.com/read/30559721/genotype-is-associated-to-the-degree-of-virilization-in-patients-with-classic-congenital-adrenal-hyperplasia
#6
Vassos Neocleous, Pavlos Fanis, Leonidas A Phylactou, Nicos Skordis
Background: Molecular defects of CYP21A2 consistently decrease 21-hydroxylase activity and result in a variable expression of disease severity in patients with congenital adrenal hyperplasia (CAH). Aim: The genotype and biochemical findings were examined in an attempt to reveal any association to the degree of virilization in classic CAH patients. Methods: The study included 18 CAH patients with complete characterization of CYP21A2 mutations and were sorted based on the severity of the inherited mutations and the expected percentage of 21-hydroxylase enzyme activity...
2018: Frontiers in Endocrinology
https://read.qxmd.com/read/30557606/a-tale-of-two-steroids-the-importance-of-the-androgens-dhea-and-dheas-for-early-neurodevelopment
#7
REVIEW
Ronda Greaves, Stefan A Wudy, Emilio Badoer, Margaret Zacharin, Jonathan J Hirst, Tracey Quinn, David W Walker
DHEA and DHEAS are neuroactive neurosteroids that interact with several major receptor system in the brain, including sigma (σ), glutamate, and GABA-A receptors. It has been recognized as early as 1952, that the loss of DHEA/DHEAS in adult life is associated with neuropsychiatric disorders (eg schizophrenia, depression). However, the mechanistic role for DHEA/DHEAS in any of these domains remains speculative, not the least because the presence of these androgens in the adrenal gland and brain is largely confined to humans and only some non-human primates...
December 14, 2018: Journal of Steroid Biochemistry and Molecular Biology
https://read.qxmd.com/read/30529136/anti-m%C3%A3-llerian-hormone-in-girls-with-premature-adrenarche-the-impact-of-polycystic-ovary-syndrome-history-in-their-mothers
#8
Alexandra Efthymiadou, Maria Bogiatzidou, Dimitra Kritikou, Dionisios Chrysis
OBJECTIVES: To assess whether the serum levels of anti-Müllerian hormone (AMH) are increased in girls with premature adrenarche because they are at a higher risk of developing polycystic ovary syndrome (PCOS) later in life. STUDY DESIGN: We measured serum levels of AMH, dehydroepiandrosterone sulfate (DHEAS), testosterone, sex hormone binding globulin, androstenedione, and 17-hyroxyprogesterone in 89 girls with premature adrenarche aged 6.98 ± 1.60 years, and in 55 prepubertal normal girls aged 6...
October 24, 2018: Journal of Pediatrics
https://read.qxmd.com/read/30286573/compound-heterozygosity-for-a-whole-gene-deletion-and-p-r124c-mutation-in-cyp21a2-causing-nonclassic-congenital-adrenal-hyperplasia
#9
Hamza Nasir, Syed Ibaad Ali, Naeem Haque, Stefan K Grebe, Salman Kirmani
We present a family with 2 members who received long-term steroid treatment for presumed classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency, until molecular testing revealed nonclassic CAH, not necessarily requiring treatment. A 17-year-old male presented to our clinic on glucocorticoid and mineralocorticoid treatment for classic CAH. He was diagnosed at 4 years of age based on mild-moderate elevations of 17-hydroxyprogesterone (17-OHP) and adrenocorticotropic hormone (ACTH), but without evidence of precocious adrenarche/puberty...
September 2018: Annals of Pediatric Endocrinology & Metabolism
https://read.qxmd.com/read/30223866/non-classical-11%C3%AE-hydroxylase-deficiency-caused-by-compound-heterozygous-mutations-a-case-study-and-literature-review
#10
Dongdong Wang, Jiahui Wang, Tong Tong, Qing Yang
BACKGROUND: 11β-hydroxylase deficiency (11OHD) is extremely rare, and reports of non-classical 11OHD are even rarer. Non-classical 11OHD usually presents as premature adrenarche, hyperandrogenism, menstrual disorders, and hypertension. Because the symptoms of non-classical 11OHD are mild, delayed diagnosis or misdiagnosis as polycystic ovary syndrome or primary hypertension is common. CASE PRESENTATION: This paper introduces a case of a young female patient presenting hypertension and menstrual disorders...
September 17, 2018: Journal of Ovarian Research
https://read.qxmd.com/read/30196871/interaction-between-parenting-styles-and-adrenarcheal-timing-associated-with-affective-brain-function-in-late-childhood
#11
Carolina Barbosa, Julian G Simmons, Nandita Vijayakumar, Paul Dudgeon, George C Patton, Lisa K Mundy, Nicholas B Allen, Sarah Whittle
OBJECTIVE: Parenting and pubertal timing have consistently been associated with internalizing and externalizing symptoms in childhood and adolescence, and there is some evidence that the interaction between these factors may be important in conferring risk. However, few studies have investigated whether neurobiological factors mediate these relationships. The current study examined whether interactions between adrenarcheal timing and parenting styles were associated with affective brain function and, in turn, mental health difficulties...
September 2018: Journal of the American Academy of Child and Adolescent Psychiatry
https://read.qxmd.com/read/30137510/11-ketotestosterone-is-the-dominant-circulating-bioactive-androgen-during-normal-and-premature-adrenarche
#12
Juilee Rege, Adina F Turcu, Josephine Z Kasa-Vubu, Antonio M Lerario, Gabriela C Auchus, Richard J Auchus, Joshua M Smith, Perrin C White, William E Rainey
Context: Adrenarche refers to the rise of dehydroepiandrosterone sulfate (DHEA-S) associated with the development of a functional adrenal zona reticularis. Clinical features of adrenarche include onset of body odor, axillary hair, and pubic hair, which reflect increased androgen action. An early rise in adrenal androgens, or premature adrenarche (PremA), is a risk factor for adverse metabolic profiles in adolescence and adulthood. The bioactive androgens associated with adrenarche and PremA remain poorly understood...
December 1, 2018: Journal of Clinical Endocrinology and Metabolism
https://read.qxmd.com/read/30129969/vitamin-d-status-and-premature-adrenarche
#13
Onur Akın, Aysun Bideci, Esra Döğer, Emine Demet Akbaş, Aylin Kilinç Uğurlu, Süleyman Tolga Yavuz, Şehri Elbeğ, Orhun Çamurdan, Peyami Cinaz
BACKGROUND: Vitamin D, an important factor in calcium-phosphate homeostasis, has recently been suggested to play an important role in the pathogenesis of numerous chronic conditions such as hyperandrogenism. The aim of this study was to investigate the relationship between vitamin D status and premature adrenarche (PA). METHODS: A total of 71 girls with PA and 52 healthy girls, as the control group, were recruited. Axillary and/or pubic hair development before the age of 8 years was defined as PA...
October 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
https://read.qxmd.com/read/30120063/body-image-dissatisfaction-and-the-adrenarchal-transition
#14
Elizabeth K Hughes, Lisa K Mundy, Helena Romaniuk, Susan M Sawyer, Melissa Wake, Joanne Williams, Timothy Olds, Nicholas B Allen, George C Patton
BACKGROUND: Puberty marks a transition in risk for body image disturbance and disordered eating. Yet few studies have examined these symptoms across puberty and none have examined links with adrenarche, the earliest phase in the pubertal hormonal cascade. METHOD: Levels of adrenal androgens (dehydroepiandrosterone, dehydroepiandrosterone sulphate, and testosterone) were measured in a population-based study of 8- to 9-year-old children (516 males and 621 females)...
August 11, 2018: Journal of Adolescent Health: Official Publication of the Society for Adolescent Medicine
https://read.qxmd.com/read/30099236/changes-to-sleep-wake-behaviors-are-associated-with-trajectories-of-pubertal-timing-and-tempo-of-secondary-sex-characteristics
#15
Joan E Foley, Nilam Ram, Elizabeth J Susman, Marsha Weinraub
We examined relations between sleep-wake behaviors and pubertal development from age 8.5 through 15.5 years in a US-based sample of 488 boys (75% White) and 478 girls (78% White). Applying conditional nonlinear growth models to 7-waves of longitudinal data, we examined how sleep-wake behaviors are related to individual differences in the developmental timing and tempo of secondary sex characteristics. For girls, results supported the hypothesis that circadian changes in bedtimes, wake times, sleep duration, and eveningness preference were uniquely related to development of discrete aspects of secondary sex characteristics...
October 2018: Journal of Adolescence
https://read.qxmd.com/read/30056577/body-mass-index-at-the-presentation-of-premature-adrenarche-is-associated-with-components-of-metabolic-syndrome-at-puberty
#16
Gamze Kaya, Zehra Yavas Abali, Firdevs Bas, Sukran Poyrazoglu, Feyza Darendeliler
The aim of this study was to analyze the relationship between premature adrenarche (PA) and metabolic syndrome (MeS) parameters at presentation and during puberty. This study comprised 47 girls with PA. Age- and puberty-matched 22 healthy girls without PA were the control group. Patients were evaluated at admission (first evaluation) and later in puberty (second evaluation). Anthropometric measurements, lipid levels, and hormonal parameters were studied and oral glucose tolerance test was performed. Indices for insulin resistance (IR) were calculated...
November 2018: European Journal of Pediatrics
https://read.qxmd.com/read/30042733/trajectories-of-growth-and-serum-dheas-and-igf-1-concentrations-in-girls-with-a-history-of-premature-adrenarche-attenuation-of-the-phenotype-by-adulthood
#17
Jani Liimatta, Pauliina Utriainen, Raimo Voutilainen, Jarmo Jääskeläinen
Background: It has been speculated that premature adrenarche (PA) could lead to unfavorable outcome, including shorter adult stature, but longitudinal follow-up data are insufficient. Methods: This prospective case-control study included 30 PA and 42 control females who were born mostly full-term and appropriate for gestational age. They were examined first at the median age of 7.6 years and now at 18.1 years. Main outcome measures were height, body mass index (BMI), age at menarche, and serum dehydroepiandrosterone sulfate (DHEAS) and insulin-like growth factor 1 (IGF-1) concentrations...
2018: Frontiers in Endocrinology
https://read.qxmd.com/read/30036793/associations-between-adrenarcheal-hormones-amygdala-functional-connectivity-and-anxiety-symptoms-in-children
#18
Marjolein E A Barendse, Julian G Simmons, Michelle L Byrne, George Patton, Lisa Mundy, Craig A Olsson, Marc L Seal, Nicholas B Allen, Sarah Whittle
OBJECTIVE: The transition from childhood to adolescence is a vulnerable period for the development of anxiety symptoms. There is some evidence that hormonal changes occurring during adrenarche, an early pubertal phase, might play a role in this increased vulnerability. Little is known about underlying brain mechanisms. Given the role of the amygdala-based fear circuit in anxiety, the current study aimed to investigate whether children's adrenarcheal hormone levels were associated with functional connectivity of the amygdala while processing fearful facial expressions, and how this in turn related to anxiety symptoms...
November 2018: Psychoneuroendocrinology
https://read.qxmd.com/read/30029738/mutation-of-hsd3b2-gene-and-fate-of-dehydroepiandrosterone
#19
REVIEW
Maria Sonia Baquedano, Gabriela Guercio, Mariana Costanzo, Roxana Marino, Marco A Rivarola, Alicia Belgorosky
3βHSD2 enzyme is crucial for adrenal and gonad steroid biosynthesis. In enzyme deficiency states, due to recessive loss-of-function HSD3B2 mutations, steroid flux is altered and clinical manifestations result. Deficiency of 3βHSD2 activity in the adrenals precludes normal aldosterone and cortisol synthesis and the alternative backdoor and 11-oxygenated C19 steroid pathways and the flooding of cortisol precursors along the Δ5 pathway with a marked rise in DHEA and DHEAS production. In gonads, it precludes normal T and estrogen synthesis...
2018: Vitamins and Hormones
https://read.qxmd.com/read/30029725/dhea-in-prenatal-and-postnatal-life-implications-for-brain-and-behavior
#20
REVIEW
Tracey Quinn, Ronda Greaves, Emilio Badoer, David Walker
Dehydroepiandrosterone (DHEA) and its sulfated congener (DHEAS) are the principal C19 steroid produced by the adrenal gland in many mammals, including humans. It is secreted in high concentrations during fetal life, but synthesis decreases after birth until, in humans and some other primates, there is a prepubertal surge of DHEA production by the adrenal gland-a phenomenon known as adrenarche. There remains considerable uncertainty about the physiological role of DHEA and DHEAS. Moreover, the origin of the trophic drives that determine the waxing and waning of DHEA synthesis are poorly understood...
2018: Vitamins and Hormones
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