Normal Pubertal Development

Excess adiposity can trend towards earlier puberty by about ___years in girls

0.5

If puberty does not begin in a boy by ___ years old, it is pathologic

18.0

Because of the immaturity of the HPG axis, about 50% of menstrual cycles are anovulatory or attenuated ovulation during the first __ years after menarche

2

A __% decline in the intensity of deep sleep occurs during adolescence, and 50% of this change occurs between 12-14 years old. Possibly due to widespread synaptic pruning that is related to the emergence of the adult cognitive capacity

50

What labs are required for delayed puberty?

CBC CMP ESR Thyroid Gonadotropin concentrations (FSH, LH)

___ results from early activation of the HPG axis, with gonadotropins stimulating sex hormone production 5x more common in girls and 90% of cases are idiopathic In boys, only 50% are idiopathic and organic CNS disorders are more prevalent in precocious boys than in precocious girls Rule out obesity/overweight as a cause Rapidly progressive precocity, particularly before 6 years of age or if associated with vaginal bleeding at breast stage 2, requires investigation and treatment DDX: Nonclassic congenital adrenal hyperplasia McCune-Albright syndrome Testotoxicosis Neoplasms Vaginal bleeding in the absence of breast development is likely to not be hormonally mediated (foreign body, abuse, or genital tract neoplasm) should be explored

Complete precocious puberty

How does female puberty typically progress?

Female puberty typically begins with thelarche (breast enlargement) (can be asymmetric) and precedes the onset of sexual hair by 1.0 to 1.5 years, although pubarche may occur first or simultaneously. Menarche occurs approximately 2.5 years after thelarche, with an average age of 12.6 years in white girls and 12.1 years in African American girls

What typically happens to early maturing girls and boys?

Girls maturing early in western countries have lower self-image, esteem, more emotional problems, and higher rates of depression, anxiety, and disordered eating Early maturing boys are at higher risk for engaging in antisocial behaviors and precocious sexual activities, particularly when they become friends with older peers at school

How is height more accurately interpreted from bone age?

Height is more accurately determined from bone age The degree of bone maturation is inversely proportional to the amount of epiphyseal cartilage growth remaining and the fraction of final height achieved at each level of bone age is known Interpretation of bone age for height prediction purposes is best performed by a pediatric endocrinologist

What are some effects of puberty on blood labs?

Hemoglobin concentration usually increases from about 12.5 to about 13.5 in late childhood to about 17.5 during puberty (only boys) Alkaline phosphatase can go as high as 525 in females/585 in males to show osteoblastic activity Total cholesterol peaks in early puberty LDL cholesterol peaks in later puberty HDL stays relatively the same Blood pressure gradually rises Triglycerides rise slightly

_____, excessive sexual hair development in a female (as distinguished from a generalized excess of body hair or hypertrichosis), is a normal variant when mild and isolated

Hirsutism

__ is suspected when the patient experiences too little pubertal development, micropenis, or cryptorchidism. Obesity could play a role and result in "pseduomicropenis"; palpation of the penile corpus is required.

Hypogonadism

What is this condition: Characterized by slowly progressive pubic or axillary hair development and may be accompanied by increased body odor or mild acne Typically not accompanied by breast or testicular enlargement or a pubertal growth spurt Causes: Idiopathic Apparent sexual hair follicle hypersensitivity to normal traces of androgen of early adrenarche Due to premature adrenarche or anabolic steroid exposure Diagnosed when premature pubarche is accompanied by a mild elevation of plasma DHEA (40-130), which is above the upper limit for normal preadrenarchal children and in the range for normal early pubertal children DHEA elevation is due to early maturation of the zona reticularis of the adrenal cortex Could be an early sign of PCOS or a virilizing disorder Close observation of pubertal development, height velocity, and bone age is indicated

Idiopathic premature pubarche/adrenarche

What condition is this: May begin unilaterally or bilaterally This change occurs in siolation - affected girls do not have pubic hair development or a pubertal growth spurt Breast development may regress in a few months or persist in proportion to somatic growth until true puberty begins at a normal age Usually is due to the HPOvarian axis with predominant FSH secretion, but estrogen formation in excess adipose tissue and exogenous estrogen exposure (topical tea tree) should be considered Occasionally the first sign of progressive true sexual precocity, indicating need for close observation of pubertal development, height velocity, and bone age Breast development in a boy before puberty (unaccompanied by pubertal genital and pubic hair development) is abnormal, and a feminizing disorder such as a neoplasm must be ruled out

Idiopathic premature thelarche

Two extreme variants of normal are common, particularly in girls: What are they?

Idiopathic premature thelarche Idiopathic premature pubarche/adrenarche

How does male puberty typically progress?

Male puberty typically begins with testicular enlargement, pubarche characteristically follows with most boys attaining SMR 3 pubic hair within 1.0 to 1.5 years after testicular enlargement. The pubertal growth spurt occurs during genital stages 3 and 4, during which time spermarche occurs. Further masculinization, including facial hair appearance and voice change, occur during genital stage 4

Primary hypogonadism usually has FSH elevated Usually is due to gonadal dysgenesis that results form sex chromosomal errosr in cell dvision What is the male and female examples?

Males - Klinefelter Females - Turner

When hirsutism + menstrual abnormalities >> hyperandrogenism must be considered (___ is the cause of 80% of these cases)

PCOS

__ is a disorder of otherwise unexplained hyperandrogenic anovulation that manifests typically in the perimenarchal stages of development Usually due to functional ovarian hyperandrogenism 50% of these girls are obese and 50% have metabolic syndrome

PCOS

What is stage 1 on the SMR chart?

Prepubertal

___ occurs in approximately 50% of boys, begins after the onset of genital development (most often at pubic hair stage 3-4) and usually lasts < 1 year

Pubertal gynecomastia

T/F After 18.0 years of age, hypogonadotropic hypogonadism is indicated by a lack of elevated gonadotropin values, not necessarily by low values

T

T/F Anorexia nervosa leading to anovulation - will need to restore 10-15% of body weight in order to recover menses

T

T/F Complete hypogonadism that is present prepubertally causes sexual infantilism in both sexes and puberty will never occur

T

T/F Congenital hypogonadism may cause disorders of sexual differentiation in genetic males

T

T/F Delayed puberty is defined as lack of breast development by age 13.0 years in girls and lack of pubertal testicular developmental (genital stage 2) by 14.0 years in boys Delay is accompanied by slowed linear growth velocity, and sometimes the accompanying short stature is the primary complaint

T

T/F During puberty, most boys develop transient gynecomastia Macromastia is a mid-adolescent female degree of breast development in a boy and reasons for estrogen excess, androgen deficiency, or liver dysfunction needs to be ruled out

T

T/F Height should be plotted on a longitudinal growth chart for interpreting growth after 9 years in relation to sexual maturity The arrest of previously normal growth rate should be looked into for endocrine, metabolic, and systemic disorders

T

T/F Normal age of puberty is between 8-13 years old for girls and 9-14 years old for boys

T

T/F Occasional experimentation and risk taking are normal, as are withdrawal and conflict with parents. Adolescent behavior must be understood in the context of individual susceptibility, family upbringing and interactions, peer group interactions, changes in brain maturation, and adolescent's reaction to their perceptions of bodily changes and sexual urges

T

T/F Primary amenorrhea - failure of menses to begin by age 15 or within 3 years of thelarche Secondary amenorrhea - cessation of menstrual periods for 90 days or more after initially menstruating Oligomenorrhea or amenorrhea - infrequent periods or no periods within any year after initially menstruating Dysfunctional uterine bleeding - anovulatory bleeding that is excessive in amount or frequency

T

T/F Primary hypogonadism usually has FSH elevated Secondary (pituitary) or tertiary (hypothalamic) hypogodnadism is either indicated by none or low LH Bone age is important to determine if gonadotropin levels are appropriate Neuroendocrine puberty may not begin until the bone age reaches a pubertal level Prepubertal gonadotropin levels do not necessarily rule out primary hypogonadism before this stage of bone age After a pubertal bone age is achieved, prepubertal gonadotropin concentrations rule out primary hypogonadism

T

T/F Pubertal disorders occur when puberty (or menses) is too early, too much, too little, or too late

T

T/F Pubertal growth spurt corresponds more closely to pubertal stage rather than to chronological age per se Pubertal growth spurt in girls begins at breast and pubic hair stage 2 Linear growth slows for girls after menarche, and growth is about 99% complete at age 15 Linear growth accelerates in boys beginning at genital and pubic stage 2 Growth is approximately 99% complete at age 17

T

T/F Sexual (stage 3) pubic hair occurs in fewer than 5% of girls younger than 8 years old, regardless of ethnicity

T

T/F Thelarche in normal-weight girls occurs about 1 year earlier in non-Hispanic African American and Mexican American girls than in non-Hispanic white girls

T

How do you roughly calculate expected height?

Target height = average of the parents heights + 6.5 cm for boys and - 6.5 cm for girls

Why is there a height discrepancy between boys and girls?

The height discrepancy between males and females is that there is a longer period of prepubertal growth and greater pubertal peak height velocity

When is precocious puberty considered in boys and girls? When is it normal?

Thelarche or pubarche before age 8.0 years of age in girls and pubarche or genital development before age 9.0 years in boys is considered premature, except in the non-Hispanic African American and Mexican American girls, in whom thelarche is normal in their 7th year

These menstrual irregularities are considered ABNORMAL: Even within the first year after menarche, evaluation should be considered for an unusual degree of menstrual irregularity Missed period for 90 days for even one cycle Bleeding more frequently than a 21 day interval Bleeding more than 7 days Requiring a pad or tampon to be changed more than every 1-2 hours

True

___ underlies the delayed puberty of disadvantaged populations

Undernutrition

Most delayed puberty does not have a serious cause and is an extreme variant of normal, called __. What sex is it more common in?

constitutional growth delay of puberty and development Boys

Sexually precocious children are at risk for ___ that leads to the paradox of short adult stature despite tall stature in childhood

premature epiphyseal fusion

A clinically significant excess of sex hormone output is suggested if ___

puberty advances rapidly or is accompanied by a growth spurt

What stage of the Sexual Maturity Rating Chart is puberty?

stage 2 - breast development in girls and testicular enlargement in boys

Failure of the ___ is a risk factor for infertility and testicular malignancy

testes to descend