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Journal of Pediatric Endocrinology &... Mar 2016Current clinical practice is to evaluate children presenting with premature adrenarche (PA) for non-classical congenital adrenal hyperplasia (NC-CAH). Our main objective...
BACKGROUND
Current clinical practice is to evaluate children presenting with premature adrenarche (PA) for non-classical congenital adrenal hyperplasia (NC-CAH). Our main objective was to assess the prevalence of NC-CAH among children presented with PA. Additional objectives were to ascertain whether subpopulations were prone to NC-CAH, and therefore justified to be tested, and if obesity is a factor that can exclude the need for CAH testing.
METHODS
A retrospective chart review of all children ≤11 years, who presented to our clinic with PA between January 2012 and May 2015 (n=103) was conducted. PA was defined based on commonly accepted clinical criteria.
RESULTS
We did not identify any subjects with NC-CAH but one was affected with previously undiagnosed classical simple virilizing CAH (SV-CAH). The subject was born prior to the implementation of CAH newborn screening in the state of birth. The affected subject was of Middle Eastern origin and also obese (BMI >95 percentile for age and sex).
CONCLUSIONS
Undiagnosed CAH is an uncommon cause of PA, and therefore routine screening for NC-CAH in every case of PA may not be justified, although, perhaps, should still be considered in high risk ethnicities. Obesity does not appear to exclude the possibility of being affected with mild or NC-CAH.
Topics: 17-alpha-Hydroxyprogesterone; Adrenal Hyperplasia, Congenital; Adrenarche; Child; Child, Preschool; Female; Follow-Up Studies; Humans; Male; Prognosis; Puberty, Precocious; Retrospective Studies; Steroid 21-Hydroxylase
PubMed: 26641961
DOI: 10.1515/jpem-2015-0252 -
Clinical Pediatric Endocrinology : Case... 2020
PubMed: 32694892
DOI: 10.1297/cpe.29.131 -
Pediatrics Sep 1998Premature adrenarche refers to the early maturation of the adrenal zona reticularis such that the resultant modest hyperandrogenism causes the early appearance of pubic...
BACKGROUND
Premature adrenarche refers to the early maturation of the adrenal zona reticularis such that the resultant modest hyperandrogenism causes the early appearance of pubic hair before the age of 8 years in girls and 9 years in boys. The precise etiology of premature adrenarche is not known. However, recent studies indicate that certain girls with premature adrenarche are at risk of developing functional ovarian hyperandrogenism, polycystic ovarian syndrome, and hyperinsulinism. Caribbean Hispanic women in general are at increased risk of developing polycystic ovarian syndrome, and African-Americans are at increased risk of developing the complications of hyperinsulinism. Previously, girls with premature adrenarche were reported to have androgens in the range found in normal children in the early stages of puberty. We noted that many of our African-American and Caribbean Hispanic patients with premature adrenarche had androgens that were much higher than what has been reported previously.
OBJECTIVE
This retrospective study was performed to characterize the adrenocorticotropin-stimulated androgen response in an African-American and Caribbean Hispanic population of girls with premature adrenarche.
METHODOLOGY
The androgen response to adrenocorticotropin stimulation in 72 African-American and Caribbean Hispanic girls with premature adrenarche was compared with those reported for normal girls in early puberty (Tanner stages II and III). The mean age was 6.8 +/- 0.8 years, bone age was 8 +/- 1.5 years, pubic hair was Tanner stages II and III, and body mass index was 18.6 +/- 4.
RESULTS
Of the girls, 28% were found to have elevated stimulated 17OHPregnenolone (17OHPreg) levels that were >2 SD units above the mean for normal early pubertal children. The stimulated ratio of 17OHPreg/17OHProgesterone also was elevated in 18% of the girls and showed a modest correlation with body mass index.
CONCLUSION
In contrast to previous studies of girls of mixed ethnic backgrounds with premature adrenarche, 28% of the 72 African-American and Caribbean Hispanic girls with premature adrenarche had adrenocorticotropin-stimulated 17OHPreg levels that were significantly higher than those seen in early pubertal girls. Because 17OHPreg hyperresponsiveness has been described previously in women with hirsutism or polycystic ovarian syndrome, the similar finding in many African-American and Caribbean Hispanic girls with premature adrenarche suggests that the two conditions may share a common mechanism for their hyperandrogenism. Therefore, the hyperandrogenism in certain African-American and Caribbean Hispanic girls with premature adrenarche may not be benign and may be the first presentation of polycystic ovarian syndrome.
Topics: Black or African American; Black People; Case-Control Studies; Child; Female; Hispanic or Latino; Humans; Indiana; Multivariate Analysis; Polycystic Ovary Syndrome; Pregnenolone; Progesterone; Puberty, Precocious; Retrospective Studies; Risk Assessment; White People
PubMed: 9724684
DOI: 10.1542/peds.102.3.e36 -
Endocrinology and Metabolism Clinics of... Dec 2012Bone age (BA) indicates more clearly than chronologic age how far an individual has progressed toward full maturity, and predicts the potential for further growth.... (Review)
Review
Bone age (BA) indicates more clearly than chronologic age how far an individual has progressed toward full maturity, and predicts the potential for further growth. Single or serial skeletal age estimations help to confirm the diagnosis of normal puberty and normal pubertal variants such as constitutional delay of growth and puberty, premature therlache, and precocious adrenarche. BA can aid in the clinical workup of children whose sexual maturation is early or delayed. Although BA is considered a qualitative rather than quantitative measure, it serves to round out the clinical picture, providing information without which diagnosis could not be achieved.
Topics: Adolescent; Adolescent Development; Age Determination by Skeleton; Body Height; Bone Development; Child; Child Development; Epiphyses; Female; Gonadotropin-Releasing Hormone; Growth Disorders; Growth Hormone; Humans; Male; Puberty; Puberty, Delayed; Puberty, Precocious
PubMed: 23099272
DOI: 10.1016/j.ecl.2012.08.003 -
Current Diabetes Reports Aug 2001The emerging epidemic of type 2 diabetes (T2DM) in young people reflects increasing rates of obesity and parallels the increasing frequency of T2DM in adults. As in... (Review)
Review
The emerging epidemic of type 2 diabetes (T2DM) in young people reflects increasing rates of obesity and parallels the increasing frequency of T2DM in adults. As in adults, T2DM in children is part of the insulin resistance syndrome that includes hyperandrogenism seen as premature adrenarche and polycystic ovary syndrome, hypertension, dyslipidemia, and other atherosclerosis risk factors. Recent studies in children document risk factors for T2DM, and associated cardiovascular risk factors, including obesity, family history, diabetic gestation, and underweight or overweight for gestational age. Genetically determined insulin resistance or limited beta-cell reserve has been demonstrated in high-risk individuals, including first-degree relatives of girls with premature adrenarche. This genetic background, considered advantageous in a feast-and-famine existence (the thrifty genotype), is rendered detrimental with abundant food and physical inactivity, a lifestyle demonstrated to be typical of families of children with T2DM. The increasing incidence of T2DM in children and adolescents threatens to become a major public health problem. Risk factors for cardiovascular disease, hypertension, hyperlipidemia, and microalbuminuria are present at diagnosis of T2DM in Native American adolescents, indicating that insulin resistance has been present for some time before the diagnosis of diabetes was made. Case finding is likely to be beneficial in high-risk youths. Treatment is the same as that of adults. The only data on use of oral hypoglycemic agents in children have been with metformin. Community and governmental efforts to educate all children and their parents about the need for physical activity and dietary modification are essential to control this epidemic.
Topics: Child; Diabetes Mellitus, Type 2; Disease Outbreaks; Humans; Risk Reduction Behavior
PubMed: 12762953
DOI: 10.1007/s11892-001-0006-x -
Clinical Endocrinology May 2009Premature adrenarche (PA) is associated with unfavourable metabolic characteristics. We hypothesized that genetic variation in low density lipoprotein (LDL)...
OBJECTIVE
Premature adrenarche (PA) is associated with unfavourable metabolic characteristics. We hypothesized that genetic variation in low density lipoprotein (LDL) receptor-related protein 5 (LRP5), which is involved in Wnt signalling in the adrenal cortex and in cholesterol metabolism, plays a role in the pathogenesis of PA.
DESIGN AND PATIENTS
We performed a cross-sectional association study in 73 Finnish children with PA and 97 age- and gender-matched healthy controls.
MEASUREMENTS
LRP5 genotypes were determined by direct sequencing. Single-marker associations with clinical-metabolic characteristics, including adrenocortical function, glucose tolerance and lipid profile, were examined with age and gender as covariates.
RESULTS
Nineteen single nucleotide polymorphisms (SNPs) in LRP5 were found in the 170 children. No significant differences in the genotype distributions were observed between the PA and control groups. SNPs A1330V and N740N were associated with higher serum dehydroepiandrosterone sulphate (DHEAS) levels in the control subjects (A/A vs. A/a; mean 0.8 vs. 1.4 micromol/l, P = 0.01). They were also associated with higher plasma levels of total (4.2 vs. 4.7 mmol/l, P = 0.02) and LDL cholesterol (2.4 vs. 2.9 mmol/l, P = 0.02) in the control group, as was SNP V1119V (P = 0.04 and P = 0.03, respectively). SNPs F549F and V1119V were associated with higher systolic blood pressure (P = 0.04 and P = 0.02, respectively). There were no differences in the parameters of glucose metabolism between the genotype groups.
CONCLUSIONS
Genetic variation in LRP5 did not predispose to PA but was associated with metabolic characteristics, especially lipid profile, in healthy prepubertal children.
Topics: Adrenarche; Alleles; Case-Control Studies; Child; Cross-Sectional Studies; Female; Finland; Gene Frequency; Genetic Predisposition to Disease; Genetic Variation; Genotype; Humans; LDL-Receptor Related Proteins; Lipids; Low Density Lipoprotein Receptor-Related Protein-5; Male; Metabolic Syndrome; Phenotype; Polymorphism, Single Nucleotide; Signal Transduction; Wnt Proteins
PubMed: 18721193
DOI: 10.1111/j.1365-2265.2008.03388.x -
Archives of Pediatrics & Adolescent... Feb 1999Puberty consists of 2 components: gonadarche and adrenarche. Both components have distinct endocrine changes. Adrenarche has virtually been ignored with respect to...
BACKGROUND
Puberty consists of 2 components: gonadarche and adrenarche. Both components have distinct endocrine changes. Adrenarche has virtually been ignored with respect to examining hormone-behavior relations.
OBJECTIVES
To provide descriptive biological and behavioral information on children with premature adrenarche (PA) and to examine differences in biological, psychological, and cognitive variables of children with PA and a healthy comparison group of children with on-time adrenarche.
DESIGN
Descriptive pilot study.
SETTING
A consecutive sample of patients was recruited from pediatric endocrine clinics; comparison children were recruited from the community.
PARTICIPANTS
Children aged 6 to 9 years. Mean (+/-SD) age of children with PA (n = 9) was 7.8 (+/-1.3) years; of children with on-time adrenarche (n = 20), 8.0 (+/-1.2) years.
METHODS AND MEASURES
Serum and saliva samples were collected for measurement of hormone concentrations. Questionnaires, tests, and interviews were completed by children and parents.
RESULTS
Compared with the on-time group, the PA group had significantly higher concentrations of adrenal androgens, estradiol, thyrotropin, and cortisol. By parent report on the Diagnostic Interview Schedule for Children, 4 children (44%) met diagnostic criteria for psychological disorders (primarily anxiety disorders). The PA group also had more self-reported depression and parent-reported behavior problems and lower scores on various intelligence tests.
CONCLUSIONS
Although PA is considered a normal variation of pubertal development that warrants no medical intervention, PA presents with significant psychosocial problems. Children with PA may need psychological evaluation and follow-up. Future studies should confirm these findings with a larger sample and examine the long-term ramifications of this early presenting abnormality.
Topics: Adrenal Hyperplasia, Congenital; Anxiety; Child; Child Behavior Disorders; Depression; Female; Gender Identity; Gonadal Steroid Hormones; Humans; Intelligence; Internal-External Control; Learning Disabilities; Male; Personality Assessment; Puberty, Precocious; Reference Values; Sexual Maturation; Social Adjustment; Wechsler Scales
PubMed: 9988243
DOI: 10.1001/archpedi.153.2.137 -
Clinical Pediatric Endocrinology : Case... 2020
Response to the Letter to the Editor of entitled "Nutritional thrift can be associated with precocious puberty and premature adrenarche in children born small for gestational age" by Hüseyin Anıl Korkmaz, Clin Pediatr Endocri (2020) 29: 131-132.
PubMed: 32694893
DOI: 10.1297/cpe.29.133 -
Journal of Pediatric Endocrinology &... Apr 2009Premature adrenarche (PA) is recognized to be a possible precursor of polycystic ovarian syndrome, type 2 diabetes mellitus and cardiovascular disease. Visceral...
BACKGROUND
Premature adrenarche (PA) is recognized to be a possible precursor of polycystic ovarian syndrome, type 2 diabetes mellitus and cardiovascular disease. Visceral adiposity and increased intramyocellular lipid (IMCL) are associated with insulin resistance and increased risk of cardiovascular disease.
AIM
To determine whether prepubertal girls with PA have altered visceral adiposity and/or increased muscle lipid content compared to prepubertal girls without PA using proton magnetic resonance imaging (MRI) and spectroscopy (1H MRS).
PATIENTS AND METHODS
We performed total body dual energy X-ray absorptiometry (DXA) scans, MRI of the trunk, and MRS of the tibialis anterior muscle in the right calf on six girls with PA and eight prepubertal controls.
RESULTS
Amount of visceral adipose tissue (VAT), abdominal subcutaneous adipose tissue (SAT), and VAT to SAT ratio did not differ significantly between the PA and control girls. Those with PA, however, had significantly greater IMCL than controls (p = 0.004).
CONCLUSIONS
This study adds further evidence that PA is not a benign condition, and future studies investigating early intervention with dietary and exercise counseling may help diminish potential risk for diabetes mellitus and/or cardiovascular disease.
Topics: Absorptiometry, Photon; Adrenarche; Body Composition; Child; Female; Humans; Intra-Abdominal Fat; Lipid Metabolism; Magnetic Resonance Imaging; Magnetic Resonance Spectroscopy; Muscle, Skeletal; Puberty, Precocious; Subcutaneous Fat
PubMed: 19554803
DOI: 10.1515/jpem.2009.22.4.301 -
Journal of Pediatric Endocrinology &... Jan 2014Premature adrenarche (PA) may be associated with obesity and advanced bone age. Our objective was to determine if children with PA had a predicted adult height less than...
OBJECTIVE
Premature adrenarche (PA) may be associated with obesity and advanced bone age. Our objective was to determine if children with PA had a predicted adult height less than target height.
METHODS
A retrospective chart review identified 100 children originating predominantly from South and Central America and the Caribbean region previously diagnosed with PA from one pediatric endocrinology clinic.
RESULTS
The majority (89.2%) of the sample had advanced bone age, while 43.4% were obese and 41.8% had a predicted height below target. There was a significant correlation between body mass index Z-score and bone age advancement among boys (r=0.67, p<0.001). The majority (72.7%) of those with a predicted height below target had a bone age advancement of two standard deviations or more.
CONCLUSIONS
These findings suggest that PA and obesity are associated with rapid skeletal maturation and that significant bone age advancement in PA is associated with a sub-optimal adult height.
Topics: Adrenarche; Adult; Body Height; Bone Development; Child; Female; Humans; Male; Retrospective Studies
PubMed: 23959660
DOI: 10.1515/jpem-2013-0199