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Hormone and Metabolic Research =... Mar 2020If circulating adrenal androgens levels rise before the age of 8 years in girls, this phenomenon is termed premature adrenarche (PA), while the concomitant appearance of... (Comparative Study)
Comparative Study
If circulating adrenal androgens levels rise before the age of 8 years in girls, this phenomenon is termed premature adrenarche (PA), while the concomitant appearance of pubic hair is called premature pubarche (PP). Girls with PA-PP display an unfavorable hormonal profile compared to their normal peers and have an increased risk of developing polycystic ovary syndrome (PCOS) features peripubertally. However, the sequelae of premature adrenarche remains unclear. We assessed metabolic, hormonal, psychologic profiles, and ovarian morphology in 21 women of mean age (±SD) 21.3±3.3 years, BMI: 23.6±4.4 kg/m with PA-PP, 45 women with PCOS and 26 controls, matched for age and BMI. PA-PP women displayed a favorable lipid profile compared to PCOS and controls. Insulin resistance index (HOMA-IR), however, were similar in PA-PP and PCOS women (2.09±1.42, 2.08±0.83) and higher than controls (1.13±0.49, p <0.05). Circulating androstenedione levels did not differ between PA-PP and PCOS women (0.11±0.05 vs. 0.12±0.03), but was higher than that of controls (0.02±0.0 nmol/l, p <0,05). Ovarian volume was increased in PA-PP and PCOS (11.14±3.3 vs. 10.99±4.61) compared to controls (6.74±1.83 cm). PA-PP women had a higher score of state/trait anxiety and depressive and eating disorder symptoms than controls, with a pattern that matched that of PCOS women. Only 14% of the PA-PP group fulfilled the Rotterdam PCOS criteria. Some women with a history of PA-PP displayed hormonal and psychologic profile similar to PCOS, and accordingly a regular monitoring of these girls during adulthood is advised.
Topics: Adolescent; Adrenarche; Androgens; Body Mass Index; Cross-Sectional Studies; Female; Hormones; Humans; Ovary; Polycystic Ovary Syndrome; Puberty, Precocious; Ultrasonography; Young Adult
PubMed: 32074632
DOI: 10.1055/a-1109-2630 -
Problemy Endokrinologii Nov 2022Adrenocortical adenomas are often followed with steroid hormones hyperproduction, and therefore determination of their concentration plays an important role in the...
BACKGROUND
Adrenocortical adenomas are often followed with steroid hormones hyperproduction, and therefore determination of their concentration plays an important role in the differential diagnosis of adrenal diseases. Steroid profiling by tandem mass spectrometry is one of the main diagnostic methods in steroidogenesis characterization. Currently plasma and urinary steroid profiling is of particular interest in differential diagnosis and subtyping patients with adrenocortical adenomas.
AIM
Steroid profiling of pediatric patients with adrenal diseases (incidentalomas, ACTH-secreting pituitary adenoma, ACTH-independent Cushing syndrome, premature adrenarche).
MATERIALS AND METHODS
We conducted a retrospective analysis of steroid profile of 41 pediatric patients with adrenal diseases who were observed between 2005 and 2020 at the Endocrinology Research Centre.
RESULTS
All patients were divided into groups due to diagnosis: with ACTH-secreting pituitary adenoma [n=7], ACTH-independent Cushing syndrome (autonomous cortisol secretion by an adrenal adenoma) [n=4], with incidentaloma [n=7] and premature adrenarche [n=23]. In group of patients with ACTH-independent Cushing syndrome identified statistically significant higher levels of 11-deoxycortisol (р=0, 0035) and significant lower levels of 17-hydroxypregnenolone (р=0, 0026) and DHEA (р=0, 0047) compared to other groups. Statistically significant differences in steroid profiles between other groups were not identified.
CONCLUSION
Results of our study steroid profiling can be used as additional differential diagnosis method in patients with adrenocortical adenomas with or without hormonal hyperproduction (ACTH-independent Cushing syndrome and incidentaloma). Further studies are needed to identify steroid markers for subtyping pediatric adrenal diseases.
Topics: Humans; Child; Adrenocortical Adenoma; ACTH-Secreting Pituitary Adenoma; Hydrocortisone; Retrospective Studies; Adenoma
PubMed: 36689716
DOI: 10.14341/probl13166 -
The Journal of Clinical Endocrinology... Oct 2014Adrenarche refers to the onset of increased production of adrenal androgens in childhood leading variably to clinical signs of androgen action. The prevalence and...
CONTEXT
Adrenarche refers to the onset of increased production of adrenal androgens in childhood leading variably to clinical signs of androgen action. The prevalence and presentation of adrenarche in prepubertal girls and boys is not well known.
OBJECTIVE
Our objective was to examine the prevalence and clinical presentation of adrenarche in a population sample of prepubertal children aged less than 9 years.
DESIGN AND PARTICIPANTS
This cross-sectional study included prepubertal children (209 girls and 228 boys; median age 7.6 [range 6.8-8.9] years) taking part in The Physical Activity and Nutrition in Children (PANIC) Study.
MAIN OUTCOME MEASURE
The prevalence of adrenarche was assessed.
RESULTS
The prevalence of any clinical sign of androgen action was higher in girls than in boys (26.1% vs 10.0%; P < .001) and biochemical adrenarche without any clinical sign was less common in girls than in boys (8.1% vs 16.7%; P = .007). When premature adrenarche was defined by serum dehydroepiandrosterone sulfate concentration ≥1 μmol/L (≥37 μg/dL) and any clinical sign before the age of 8 years in girls and 9 years in boys, its total prevalence was 8.6% in girls and 1.8% in boys. The risk of having any clinical sign increased with higher body fat percentage in boys and with higher serum dehydroepiandrosterone sulfate concentration in girls.
CONCLUSIONS
Clinical signs of androgen action are more common, but biochemical adrenarche without any clinical sign is less common in prepubertal girls than boys. This sexual dimorphism of adrenarche might be explained by sex-dependent differences in peripheral androgen metabolism or action that are modified by body fat.
Topics: Adipose Tissue; Adiposity; Adrenal Cortex; Adrenarche; Androgens; Body Composition; Child; Cross-Sectional Studies; Dehydroepiandrosterone Sulfate; Female; Finland; Humans; Male; Obesity; Prevalence; Puberty, Precocious; Risk Factors; Sex Characteristics
PubMed: 25029425
DOI: 10.1210/jc.2014-2049 -
The Journal of Clinical Endocrinology... Aug 2021Premature adrenarche (PA) may increase the risk for polycystic ovary syndrome (PCOS).
CONTEXT
Premature adrenarche (PA) may increase the risk for polycystic ovary syndrome (PCOS).
OBJECTIVE
To study features of PCOS in young adult women with a history of PA.
METHODS
Thirty PA and 42 control females were followed from prepuberty to young adulthood (median age 18.1 years). The main outcome measures were ovarian function, the use of contraceptives, and clinical and biochemical indicators of hyperandrogenism.
RESULTS
We found no differences in the use of hormonal contraceptives (50 vs 50%, PA vs controls, respectively; P > .999), indication for using contraceptives (P = .193), or in the history of oligo- (17 vs 26%, P = .392) and amenorrhea (0 vs 0%, P > .999). Among women not using hormonal contraceptives, those with a history of PA had a higher prevalence of hirsutism (27 vs 0%, P = .023) but not acne (87 vs 67%, P = .252). Steroid profiles were broadly comparable between the groups, but PA women had lower sex hormone-binding globulin (SHBG) concentrations (30.1 vs 62.4 nmol/L, P < .001) resulting in higher free androgen index (3.94 vs 2.14, P < .001). The difference in SHBG levels persisted through body mass index adjustment. SHBG correlated negatively with the homeostasis model assessment for insulin resistance (r -0.498, P = .003). Anti-Müllerian hormone concentrations were comparable between the groups (39.3 vs 32.1 pmol/L, P = .619).
CONCLUSION
PA was not associated with evident ovarian dysfunction in young adult women. However, women with a history of PA had decreased SHBG levels and thus, increased bioavailability of circulating androgens.
Topics: Acne Vulgaris; Adolescent; Adrenarche; Amenorrhea; Androgens; Anti-Mullerian Hormone; Body Mass Index; Case-Control Studies; Contraceptives, Oral, Hormonal; Female; Follow-Up Studies; Hirsutism; Humans; Hyperandrogenism; Insulin Resistance; Ovarian Function Tests; Polycystic Ovary Syndrome; Prevalence; Sex Hormone-Binding Globulin; Steroids; Young Adult
PubMed: 34060603
DOI: 10.1210/clinem/dgab385 -
Hormone Research in Paediatrics 2020The term premature pubarche (PP) refers to the appearance of pubic hair before age 8 in girls and before age 9 in boys. Although idiopathic PP (often associated with...
BACKGROUND
The term premature pubarche (PP) refers to the appearance of pubic hair before age 8 in girls and before age 9 in boys. Although idiopathic PP (often associated with premature adrenarche) is considered an extreme variation from the norm, it may be an initial sign of persistent hyperandrogenism. Factors contributing to PP onset and progression have not been identified to date.
AIMS
The objectives of this study are to describe a group of Italian children with PP, to identify potential factors for its onset, and to define its clinical and biochemical progression.
METHODS
We retrospectively enrolled all infants born between 2001 and 2014 with PP. Children with advanced bone age (BA) underwent functional tests to determine the cause of PP. Hormonal analysis and BA determination were performed annually during a 4-year follow-up period.
RESULTS
A total of 334 children with PP were identified: idiopathic PP (92.5%, associated with premature adrenarche in some cases); related to precocious puberty (6.6%); late-onset 21-hydroxylase deficiency (0.9%). Low birth weight was associated with premature adrenal activation. Body mass index (BMI) was the only factor that influenced the progression of BA during follow-up.
CONCLUSIONS
Low birth weight is a predisposing factor for premature adrenal activation. The increase in BMI in patients with idiopathic PP during the 4-years of follow-up was responsible for BA acceleration. We recommend prevention of excessive weight gain in children with PP and strict adherence to follow-up in order to prevent serious metabolic consequences.
Topics: Birth Weight; Body Mass Index; Body Weight; Child; Child, Preschool; Disease Progression; Female; Follow-Up Studies; Humans; Infant; Italy; Male; Puberty, Precocious; Retrospective Studies; Risk Factors
PubMed: 33264767
DOI: 10.1159/000511873 -
Stress (Amsterdam, Netherlands) Nov 2018Concerns over anxiety and depressive symptoms in children with premature adrenarche (PA) have been recently raised. However, to date, most relevant studies are on a...
Increased symptoms of anxiety and depression in prepubertal girls, but not boys, with premature adrenarche: associations with serum DHEAS and daily salivary cortisol concentrations.
Concerns over anxiety and depressive symptoms in children with premature adrenarche (PA) have been recently raised. However, to date, most relevant studies are on a small number of girls. In this cross-sectional study, 82 pre-pubertal children (66 girls and 16 boys) diagnosed with PA, were compared to 63 control children regarding their psychological characteristics and hypothalamic-pituitary-adrenal (HPA) axis function, as assessed by salivary cortisol measurement. Symptoms of anxiety and depression were assessed by child self-report (Spence Children's Anxiety Scale (SCAS) and Depression self-rating scale for Children (DSRS)) and parent-report (Child Behaviour Checklist (CBCL)) tests validated for the Greek population. Salivary cortisol levels were determined directly after awakening (approximately 7am) and evening (8pm) of the same day. Morning serum DHEAS levels were assessed in PA children. Girls with PA scored significantly higher on anxiety (p = .016) and depression (p =.039) scales than controls. No group differences were noted for parent reports and children's salivary cortisol concentrations. Boys with PA did not demonstrate significant differences in any of the aforementioned parameters. Our findings suggest that girls with PA may be at higher risk for reporting symptoms of anxiety and depression than their non-PA peers. HPA axis dysregulation in this population was not documented.
Topics: Adrenarche; Anxiety; Child; Child, Preschool; Cross-Sectional Studies; Dehydroepiandrosterone Sulfate; Depression; Female; Humans; Hydrocortisone; Hypothalamo-Hypophyseal System; Male; Pituitary-Adrenal System; Puberty, Precocious; Saliva; Sex Factors
PubMed: 29916751
DOI: 10.1080/10253890.2018.1484446 -
Annals of Pediatric Endocrinology &... Dec 2018Premature adrenarche (PA) often leads to polycystic ovary syndrome (PCOS). Higher anti-mullerian hormone (AMH) levels are reported in PCOS. We studied the androgen...
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) (6.7±1 years vs. 6.2±1.3 years) and BMI (20±10 kg/m2 vs. 17.8±2.7 kg/m2). Dehydroepiandrostenedione sulfate (63.3±51.3 μg/dL vs. 29.8±17.3 μg/dL, P<0.001) and testosterone levels (11.4±4.8 ng/dL vs. 8.2±2.9 ng/dL, P=0.001) were significantly higher in the PA group than controls. AMH values (<14 years: reference range, 0.49-3.15 ng/mL) were 3.2±2.2 ng/mL vs. 4.6± 3.2 ng/mL respectively in the PA and control groups and were not different (P=0.4). AMH did not show a correlation with bone age (P=0.1), and testosterone (P=0.9) in the PA group. 17-hydroxyprogesterone levels (17-OHP ng/dL) were 39.5±30.5 ng/dL vs. 36.8±19.8 ng/dL in PA versus control girls. The concentration of 17-OHP was not statistically different between the control and PA groups.
CONCLUSION
Higher AMH was not observed in PA girls and no correlation with BA and androgen levels was observed.
PubMed: 30599482
DOI: 10.6065/apem.2018.23.4.210 -
Frontiers in Endocrinology 2018It has been speculated that premature adrenarche (PA) could lead to unfavorable outcome, including shorter adult stature, but longitudinal follow-up data are...
It has been speculated that premature adrenarche (PA) could lead to unfavorable outcome, including shorter adult stature, but longitudinal follow-up data are insufficient. 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. The PA and control females had comparable mean (standard deviation) adult height [167.2 (6.8) vs. 164.5 (5.1) cm, = 0.059] and median (25th-75th percentiles) BMI [22.8 (21.1-28.9) vs. 21.6 (19.8-24.3) kg/m, = 0.068, respectively]. Adult heights were comparable with the mid-parental heights in both study groups. The PA females were taller than the controls until the age of 12 years and they lacked a distinct pubertal growth spurt. Serum DHEAS and IGF-1 concentrations did not differ between the PA and control groups at the age of 18 years. Median (range) age at menarche was significantly lower in the PA than control females [11.5 (9.5-15.0) vs. 13.0 (10.0-15.0), = 0.001]. Although PA girls have advanced growth and earlier pubertal development together with a tendency to be more overweight, their height, BMI, and serum DHEAS and IGF-1 concentrations are comparable to those of their peers at the age of 18 years. Our findings indicate a benign outcome of PA in appropriate for gestational age -born females concerning adult height and adrenal androgen secretion.
PubMed: 30042733
DOI: 10.3389/fendo.2018.00375 -
Frontiers in Endocrinology 2023Prader-Willi syndrome (PWS) is a rare genetic disorder resulting from lack of expression of the paternally derived chromosome 15q11-13, associated with several... (Review)
Review
A rare occurrence of non-classic congenital adrenal hyperplasia and type 1 diabetes mellitus in a girl with Prader-Willi Syndrome: Case report and review of the literature.
Prader-Willi syndrome (PWS) is a rare genetic disorder resulting from lack of expression of the paternally derived chromosome 15q11-13, associated with several complications, including pubertal disorders, short stature, hyperphagia, obesity, glucose metabolism abnormalities, scoliosis, obstructive sleep apnea syndrome (OSAS) and behavioral problems. We report the case of a girl affected by PWS who presented at the age of 5.9 with premature pubarche, accelerated linear growth and advanced bone age (BA). She was subsequently diagnosed with non-classic congenital adrenal hyperplasia (CAH) confirmed by genetic analysis. Considering the clinical, biochemical, and genetic findings, hydrocortisone therapy was started to prevent rapid BA acceleration and severe compromission of final height. During infancy, short stature and low levels of insulin-like growth factor-1 (IGF-1) for age and gender led to suspicion of growth hormone deficiency (GHD), confirmed by stimulation testing (arginine and clonidine). rhGH therapy was administered and continued until final height was reached. During endocrinological follow up she developed impaired glucose tolerance with positive markers of β-cell autoimmunity (anti-glutamic acid decarboxylase antibodies, GAD Ab), which evolved over time into type 1 diabetes mellitus and insulin therapy with a basal-bolus scheme and an appropriate diet were needed.
Topics: Female; Humans; Prader-Willi Syndrome; Diabetes Mellitus, Type 1; Adrenal Hyperplasia, Congenital; Human Growth Hormone; Obesity
PubMed: 37124733
DOI: 10.3389/fendo.2023.1148318 -
Journal of Pediatric Endocrinology &... May 2017The aim of this study was to determine the serum levels of kisspeptin and ghrelin (GAH), as well as the relationship of these two peptides with each other in premature...
BACKGROUND
The aim of this study was to determine the serum levels of kisspeptin and ghrelin (GAH), as well as the relationship of these two peptides with each other in premature thelarche (PT) and premature adrenarche (PA) cases and to investigate the possibility of using these peptides as markers in the differentiation of puberty disorders.
METHODS
A PT group aged 1-8 years (n = 40), a PA group aged 1-9 years (n = 23, female/male = 20/3) and control groups consistent with each of the previous groups in terms of age and gender were created for the study. Kisspeptin and ghrelin levels were measured with ELISA methods from blood samples drawn while fasting in the morning.
RESULTS
When the PT group was compared with the controls, the plasma kisspeptin levels of the cases were significantly higher than the control group (165.47 ± 15.45 pmol/L, 96.82 ± 12.33 pmol/L, p = 0.005, respectively). Kisspeptin levels in the PA group did not show a difference with the control group (121.36 ± 17.99 pmol/L, 95.52 ± 11.54 pmol/L, p = 0.249, respectively). No significant difference could be found when GAH levels in the PT and PA groups were compared with controls. No significant correlation was found between kisspeptin and GAH levels in the PT and PA groups.
CONCLUSIONS
Our results indicate that kisspeptin plays an important role in the PT, but GAH is not associated with puberty disorders.
Topics: Adrenarche; Biomarkers; Case-Control Studies; Child; Child, Preschool; Female; Ghrelin; Humans; Infant; Kisspeptins; Male; Puberty, Precocious; Sexual Maturation
PubMed: 28422705
DOI: 10.1515/jpem-2016-0127