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Benign Premature Adrenarche

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1. Benign Premature Adrenarche

Benign Premature Adrenarche Benign Premature Adrenarche Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Benign Premature Adrenarche (...) Benign Premature Adrenarche Aka: Benign Premature Adrenarche , Premature Adrenarche From Related Chapters II. Pathophysiology Benign self-limited cause of Results from adrenal androgen release as opposed to the pubertal -Pituitary-Gonadal activation III. Epidemiology Onset before age 6 years IV. Signs Early secondary sexual characteristics in children Early pubic hair and axillary hair development Increased sebaceous activity Adult-type body odor Mild acne No No penis or enlargement No s, clitoris

2018 FP Notebook

2. Trajectories of Growth and Serum DHEAS and IGF-1 Concentrations in Girls With a History of Premature Adrenarche: Attenuation of the Phenotype by Adulthood (PubMed)

Trajectories of Growth and Serum DHEAS and IGF-1 Concentrations in Girls With a History of Premature Adrenarche: Attenuation of the Phenotype by Adulthood 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 (...) 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.

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2018 Frontiers in endocrinology

3. Benign Premature Adrenarche

Benign Premature Adrenarche Benign Premature Adrenarche Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Benign Premature Adrenarche (...) Benign Premature Adrenarche Aka: Benign Premature Adrenarche , Premature Adrenarche From Related Chapters II. Pathophysiology Benign self-limited cause of Results from adrenal androgen release as opposed to the pubertal -Pituitary-Gonadal activation III. Epidemiology Onset before age 6 years IV. Signs Early secondary sexual characteristics in children Early pubic hair and axillary hair development Increased sebaceous activity Adult-type body odor Mild acne No No penis or enlargement No s, clitoris

2015 FP Notebook

4. Premature adrenarche. (PubMed)

Premature adrenarche. Premature adrenarche refers to the presence of secondary sexual hair in girls younger than 8 years old and boys younger than 9 years old. It is a relatively common presentation to paediatricians and is more frequent in girls than boys. It is a benign diagnosis, but other causes of androgen excess such as congenital adrenal hyperplasia or adrenal tumours should be excluded first. In conjunction with history and clinical examination, first line investigations should include (...) determination of serum androgen concentrations, along with bone age, proceeding to synacthen stimulation test (for 17OHP levels) and adrenal ultrasound if indicated. The phenotype of premature adrenarche varies considerably between populations but may be associated with low birth weight, insulin resistance, adverse cardio-metabolic risk and progression to polycystic ovarian syndrome in some populations. In the majority of cases, no specific treatment is recommended, but where there is a history of low birth

2011 Archives of Disease in Childhood

5. Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency

are brought to prompt medical attention. Third, premature, sick, or stressed infants have higher levels of 17OHP than do term infants, generating many false positives. For example, in 26 years of operation of the Swedish screening program, the positive predictive value was 25% for full-term infants but only 1.4% for preterm infants, and the predictive value correlated very strongly with gestational age ( ). Finally, immunoassays may lack specificity. There are no universally accepted standards (...) -pregnenolone sulfate ( ). Immunoassay specificity may be improved with organic extraction to remove cross-reacting substances, such as steroid sulfates. The dissociation-enhanced lanthanide fluoroimmunoassay was reformulated in late 2009 to reduce its sensitivity to cross-reacting compounds in premature infants ( ). This change improved the positive predictive value from 0.4% to 3.7% for the first screen alone ( ). Finally, antenatal corticosteroids may reduce 17OHP levels, potentially increasing

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2018 The Endocrine Society

6. Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency

all females with salt-wasting CAH are virilized, and thus are brought to prompt medical attention. Third, premature, sick, or stressed infants have higher levels of 17OHP than do term infants, generating many false positives. For example, in 26 years of operation of the Swedish screening program, the positive predictive value was 25% for full-term infants but only 1.4% for preterm infants, and the predictive value correlated very strongly with gestational age ( ). Finally, immunoassays may lack (...) results due to cross-reactivity with other steroids, for example, 17-OH-pregnenolone sulfate ( ). Immunoassay specificity may be improved with organic extraction to remove cross-reacting substances, such as steroid sulfates. The dissociation-enhanced lanthanide fluoroimmunoassay was reformulated in late 2009 to reduce its sensitivity to cross-reacting compounds in premature infants ( ). This change improved the positive predictive value from 0.4% to 3.7% for the first screen alone ( ). Finally

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2018 Pediatric Endocrine Society

7. An update to the Greig Health Record: Preventive health care visits for children and adolescents aged 6 to 17 years ? Technical report

status is associated with higher rates of infant mortality, childhood asthma, overweight and obesity, injuries and deaths from injury, and mental health issues, including learning and emotional disorders. Children growing up in low-income families experience poorer adult health including physical disability, clinical depression and premature death. While government subsidies are available to help alleviate poverty, many families are unaware of benefits that may be available to them by applying (...) evidence of no benefit and good evidence of harm in the form of increased physician visits and benign biopsy results. For women under 40 years of age, there is little evidence on which to base a recommendation; however the very low incidence of breast cancer in this age group makes the net risk of harm more likely. There is evidence to recommend against counselling for testicular self-examination or routine clinical examination in individuals of average risk, in light of the low incidence of testicular

2016 Canadian Paediatric Society

8. Review of Prader-Willi syndrome: the endocrine approach

( ). Adrenarche may be associated with advanced bone age in some cases, although presence of obesity may also be causative of skeletal advancement ( ). Premature adrenarche in PWS is typically not rapidly progressive or associated with other signs of central puberty, and is generally felt to be benign. Reassurance should be provided to families that further investigation or treatment is not usually warranted. Hypothyroidism Similar to other endocrinopathies in PWS, hypothalamic dysfunction may place patients (...) at graduated doses are typically used for initiation or continuation of stalled puberty, with combined oral contraceptive pills (OCPs) used after the first menstrual bleed has occurred. Premature adrenarche There is a high rate of premature adrenarche in both males and females with PWS, with a prevalence of 14–30% ( , ). Androgens levels may be slightly elevated during childhood, but these typically normalize as adults ( , ). GH treatment generally has no effect on adrenarche or DHEA-S levels

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2017 Pediatric Endocrine Society

9. Congenital Adrenal Hyperplasia Due to Steroid 21-hydroxylase Deficiency

, 17-OHP levels increase with time in infants affected with CAH. Thus, diagnostic accuracy is poor in the first 2 d, which can be problematic if newborns are discharged early. Additionally, premature, sick, or stressed infants typically have higher levels of 17-OHP than term infants and generate many false positives unless higher cutoffs are used. There are no universally accepted standards for stratifying infants, but most U.S. laboratories use a series of birth weight-adjusted cutoffs (...) ( , , ). Specificity of newborn screening might be improved by using actual gestational age to stratify subjects, rather than birth weight, because 17-OHP levels are much better correlated with gestational age ( ). Indeed, in The Netherlands and Switzerland, adopting gestational age criteria improved the positive predictive value of screening ( , ). Finally, antenatal corticosteroid treatment (as used to induce lung maturation in fetuses at risk for premature birth) might reduce 17-OHP levels, but inconsistent

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2010 The Endocrine Society

10. Disorders of the Breast (Diagnosis)

involves antibiotics, needle aspiration, or surgical drainage. The decision for surgical drainage should be carefully made because future breast deformation may occur. For this reason, needle aspiration is the preferred treatment. [ ] The literature suggests that drainage alone, without adjunctive antibiotics, may be effective in skin and soft-tissue abscesses caused by MRSA; no definitive antibiotic recommendation regarding MRSA breast abscesses in particular is recognized. [ ] Benign premature (...) thelarche Benign premature thelarche is defined as isolated breast development in females aged 6 months to 9 years. Physical examination for this entity should carefully seek out other signs of puberty, such as development of pubic hair, thickening of the vaginal mucosa, and accelerated bone growth. If no other signs of puberty are present, reassure the patient and family that this is a benign finding. Examine the child every 6-12 months. If other signs of puberty are evident, precocious puberty should

2014 eMedicine Pediatrics

11. Precocious Puberty (Overview)

common, benign, normal variant conditions that can resemble precocious puberty but are nonprogressive or very slowly progressive. Premature thelarche refers to the isolated appearance of breast development, usually in girls younger than 3 years; premature pubarche refers to appearance of pubic hair without other signs of puberty in girls or boys younger than 7-8 years. A thorough history, physical examination, and growth curve review can help distinguish these normal variants from true sexual (...) of the normal distribution. Age If precocious puberty in females (mostly central) is defined as the early onset of breast development, then the data of Herman-Giddens et al can be used to estimate frequency of central precocious puberty at different ages in both white girls and black girls. Be cautious, however, in equating breast development in 3-year-olds with central precocious puberty because most such girls actually have premature thelarche, a benign normal variant (see Differentials). The younger

2014 eMedicine Pediatrics

12. Precocious Puberty (Diagnosis)

common, benign, normal variant conditions that can resemble precocious puberty but are nonprogressive or very slowly progressive. Premature thelarche refers to the isolated appearance of breast development, usually in girls younger than 3 years; premature pubarche refers to appearance of pubic hair without other signs of puberty in girls or boys younger than 7-8 years. A thorough history, physical examination, and growth curve review can help distinguish these normal variants from true sexual (...) of the normal distribution. Age If precocious puberty in females (mostly central) is defined as the early onset of breast development, then the data of Herman-Giddens et al can be used to estimate frequency of central precocious puberty at different ages in both white girls and black girls. Be cautious, however, in equating breast development in 3-year-olds with central precocious puberty because most such girls actually have premature thelarche, a benign normal variant (see Differentials). The younger

2014 eMedicine Pediatrics

13. Disorders of the Breast (Treatment)

involves antibiotics, needle aspiration, or surgical drainage. The decision for surgical drainage should be carefully made because future breast deformation may occur. For this reason, needle aspiration is the preferred treatment. [ ] The literature suggests that drainage alone, without adjunctive antibiotics, may be effective in skin and soft-tissue abscesses caused by MRSA; no definitive antibiotic recommendation regarding MRSA breast abscesses in particular is recognized. [ ] Benign premature (...) thelarche Benign premature thelarche is defined as isolated breast development in females aged 6 months to 9 years. Physical examination for this entity should carefully seek out other signs of puberty, such as development of pubic hair, thickening of the vaginal mucosa, and accelerated bone growth. If no other signs of puberty are present, reassure the patient and family that this is a benign finding. Examine the child every 6-12 months. If other signs of puberty are evident, precocious puberty should

2014 eMedicine Pediatrics

14. Disorders of the Breast (Overview)

involves antibiotics, needle aspiration, or surgical drainage. The decision for surgical drainage should be carefully made because future breast deformation may occur. For this reason, needle aspiration is the preferred treatment. [ ] The literature suggests that drainage alone, without adjunctive antibiotics, may be effective in skin and soft-tissue abscesses caused by MRSA; no definitive antibiotic recommendation regarding MRSA breast abscesses in particular is recognized. [ ] Benign premature (...) thelarche Benign premature thelarche is defined as isolated breast development in females aged 6 months to 9 years. Physical examination for this entity should carefully seek out other signs of puberty, such as development of pubic hair, thickening of the vaginal mucosa, and accelerated bone growth. If no other signs of puberty are present, reassure the patient and family that this is a benign finding. Examine the child every 6-12 months. If other signs of puberty are evident, precocious puberty should

2014 eMedicine Pediatrics

15. Disorders of the Breast (Follow-up)

involves antibiotics, needle aspiration, or surgical drainage. The decision for surgical drainage should be carefully made because future breast deformation may occur. For this reason, needle aspiration is the preferred treatment. [ ] The literature suggests that drainage alone, without adjunctive antibiotics, may be effective in skin and soft-tissue abscesses caused by MRSA; no definitive antibiotic recommendation regarding MRSA breast abscesses in particular is recognized. [ ] Benign premature (...) thelarche Benign premature thelarche is defined as isolated breast development in females aged 6 months to 9 years. Physical examination for this entity should carefully seek out other signs of puberty, such as development of pubic hair, thickening of the vaginal mucosa, and accelerated bone growth. If no other signs of puberty are present, reassure the patient and family that this is a benign finding. Examine the child every 6-12 months. If other signs of puberty are evident, precocious puberty should

2014 eMedicine Pediatrics

16. Dehydroepiandrosterone Sulphate

DHEAS, can result in some body hair and the change to adult body odour (sweat starts to smell). Premature (early) adrenarche is mostly benign and is more common in girls and children of South Asian and Afro-Caribbean origin. How is the sample collected for testing? A blood sample is obtained by inserting a needle into a vein in the arm. Is any test preparation needed to ensure the quality of the sample? No test preparation is needed. Accordion Title Common Questions DHEAS concentrations (...) but only in girls precocious puberty associated with signs of virilisation. DHEAS may also be used in the specialist investigation of disorders of sex development such as in babies with ambiguous genitalia (when difficult to determine whether baby is boy or girl) or at any point thereafter. A distinct period of puberty, which can occur before the other stages, is called adrenarche. This is activation of the adrenal gland before the ovaries or testicles where a surge of adrenal androgens, primarily

2012 Lab Tests Online UK

17. Etiology of precocious puberty, 10 years study in Endocrine Reserch Centre (Firouzgar), Tehran (PubMed)

hyperplasia (CAH) (42.8%), ovarian cysts (28.4%), McCune-Albright syndrome (14.2%) and adrenal carcinoma (14.2%). 34.1% of girls and 25% of boys had normal variant puberty including premature thelarche (57%), premature adrenarche (38%) as well as premature menarche (4.7%l).The most common etiology of precocious puberty in girls was idiopathic central precocious puberty and premature thelarche, while in boys they were neurogenic central precocious puberty and CAH. Therefore precocious puberty in girls (...) is usually benign. In boys, CNS anomalies should first be considered in the differential diagnosis of CPP. Therefore brain Magnetic Resonance Imaging (MRI) is mandatory in all cases.

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2012 Iranian Journal of Reproductive Medicine

18. Approach to the girl with early onset of pubic hair. (PubMed)

Approach to the girl with early onset of pubic hair. Premature pubarche, or the development of pubic hair before the age of 8 in girls or 9 in boys, is most commonly caused by premature adrenarche. Adrenarche is the maturation of the adrenal zona reticularis in both boys and girls, resulting in the development of pubic hair, axillary hair, and adult apocrine body odor. Although originally thought to be a benign variant of normal development, premature adrenarche has been associated with insulin

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2011 Journal of Clinical Endocrinology and Metabolism

19. Prader-Willi syndrome

behaviors. Endocrine [ ] Several aspects of PWS support the concept of a growth hormone deficiency. Specifically, individuals with PWS have short stature, are obese with abnormal body composition, have reduced fat-free mass, have reduced lean body mass and total energy expenditure, and have decreased bone density. PWS is characterized by hypogonadism. This is manifested as undescended testes in males and benign premature in females. Testes may descend with time or can be managed with surgery (...) or testosterone replacement. Adrenarche may be treated with hormone replacement therapy. Ophthalmologic [ ] PWS is commonly associated with development of . In one study, over 50% of patients had strabismus, mainly . Genetics [ ] PWS is caused by an epigenetic phenomenon known as , caused by the deletion of the paternal copies of the and genes along with clusters of : , SNORD107, SNORD108 and two copies of SNORD109, 29 copies of (HBII-85) and 48 copies of (HBII-52). These are on located in the region 15q11-13

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2012 Wikipedia

20. 9 year old girl with breast development and first period but no axillary or pubic hair. Is this consistent with normal puberty or does this require investigation.

, the 1st pubertal milestone is typically breast development (thelarche), followed soon after by appearance of pubic hair (pubarche) and axillary hair and later by the 1st menstrual period (menarche)…” It adds: “ Premature appearance of only one specific pubertal milestone is generally considered a benign variant of development. Examples are precocious appearance of pubic and axillary hair before age 8 in girls and age 9 in boys, and precocious onset of breast development before age 8 in girls (...) .” In the section on symptoms, signs and diagnosis, the authors list: “ In girls, breasts develop, and pubic hair, axillary hair, or both appear. Girls may begin to menstruate. Body odor, acne, and behavior changes may develop in either sex. Height growth is initially rapid in both sexes, but premature closure of the epiphyses results in short adult stature. Testicular or ovarian enlargement occurs in precocious puberty but is usually absent in isolated precocious adrenarche.” Concerning diagnosis: “ Diagnosis

2008 TRIP Answers

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