Board Review 3/7/2013
Board Review 3/7/2013 Who remembered that daylight savings is this weekend?? A. I did! B. Nooooooooo!!! Developmental stage characterized by: Maturation of gametogenesis Secretion of gonadal hormones Development of secondary sexual characteristics and reproductive functions Pediatricians are constantly faced with questions about what is “normal” during puberty Changes in GnRH secretion (increased pulsatility) result in puberty Age of onset: Girls: 8-13 years Thelarche can occur at age 7 in African-American and Mexican American girls Boys: 9-14 years Sexual Maturity Rating (SMR) Used to document a child’s development through puberty Consists of inspection of breast, genital, and pubic hair development Limitation: does not have specific measurements Stage 1: prepubertal Stage 2: girls: thelarche boys: testicular enlargement (≥4mL) Stage 3: ongoing Stage 4: nearly complete Stage 5: adultlike Pubertal progression from stage 2 to stage 5 can take between 2.5-5 years to complete Which is the correct sequence of pubertal events in a female? A. Menarche thelarche peak height velocity pubarche B. Thelarche pubarche peak height velocity menarche C. Pubarche thelarche menarche peak height velocity D. Thelarche pubarche menarche peak height velocity Males: Testicular growth pubarche penile growth peak height velocity Testicular volume < 4mL (2.5cm) is prepubertal Reach SMR4 prior to attainment of peak height velocity Females: Thelarche pubarche peak height velocity menarche Peak height velocity is typically 1 year before menarche Girls height will typically increase ~6-8cm after menarche has occurred A mother brings in her 13 year old son at his request due to his concerns about breast tissue. He has noticed he has a small amount of breast tissue and wants to know if it can be removed. He is otherwise developing appropriately and does well in school. On exam, he is SMR3 for genital growth and pubic hair. You also notice a small amount of breast tissue bilaterally under his nipples. What is the most appropriate course of action? A. Refer the patient to a plastic surgeon B. Refer the patient to an endocrinologist for hormone therapy C. Order a brain MRI to rule out malignancy D. Reassure the patient that this is a normal part of puberty and follow up in 6 months E. Order FSH and LH levels Asymmetric breast or testicular development Can see up to 1 stage advance of unilateral development at onset of puberty Gynecomastia 50% of boys will have some degree of breast tissue during puberty Typically during pubic hair stage 3 or 4 Gynecomastia without onset of puberty is concerning Lasts less than 1 year Can just be observed for resolution Peak height velocity in girls occurs earlier chronologically and in pubertal staging than in boys Boys growth spurt is typically 2 years after girls Peak height velocity is more closely correlated with SMR than chronologic age Average prepubertal height velocity 5-6cm/yr Average pubertal height velocity: Boys = 9-10cm/yr Girls = 8-9cm/yr Completion of this growth spurt takes 2-4 years Longer period of prepubertal growth and greater pubertal height velocity account for the typical height discrepancy between males and females Longitudinal growth chart is much more useful in determining abnormality of growth versus a single point in time A boy comes in with his parents for his 12 year old well-child check and wants to know how tall he is going to be. His mother reports that he has been growing but that he is shorter than most of his peers. His mother is 160 cm (63 in) tall and his father is 172 cm (68 in) tall. With the exception of his relative short stature, his medical history is unremarkable. You advise him that the best way to estimate his potential for growth is to calculate his midparental target height and compare it to his current height and his skeletal maturity. Of the following, what is the BEST estimate of his midparental target height? A. 160 cm (63in) B. 165.5cm (65in) C. 172.5cm (68in) D. 177.5cm (70in) E. 182.5cm (72in) Height is largely determined genetically Target height can be crudely estimated: Average of parents heights in cm +6.5cm for boys -6.5cm for girls Bone age can also be used predict height Best performed by an endocrinologist Keep in mind a bone age can be off from chronologic age by as much as 2 years and still be normal (ie constitutional growth delay) There is a genetic influence on the timing of puberty ?autosomal dominant Earlier puberty: will have tall stature during puberty compared to peers Will complete their growth prematurely and have a lower peak adult height than expected Rapid fusion of their growth plates Later puberty: short stature in youth but an adult height that is slightly above expectations Slow but constant prepubertal growth of long bones without rapid maturation of the growth plate Timing of puberty affects both linear growth rate and skeletal maturity A mother brings in her 9 year old daughter because she saw some blood in her underwear and is concerned that her daughter has started her period. Menarche for mom was achieved at age 11. On exam, the girl is well appearing with no dysmorphic features or rash. She has SMR stage 1 breast development and no pubic hair development. Of the following, which is MOST likely going to elicit a cause for her vaginal bleeding? A. Obtaining a bone age radiograph B. Plotting the child’s height and weight on a growth chart C. Obtaining serum LH and FSH levels D. Obtaining a serum estradiol concentration E. Examination of the genetalia for a foreign body Genetic influence on age of menarche Typically occurs at SMR 4 breast development Vaginal bleeding at SMR 1-2 is not likely to represent menarche or be hormonally mediated Average age of menarche: 12.6 yrs Range: 11-14 yrs Average time of menarche after thelarche: 2 yrs Range 0.5 – 5 yrs No menarche by age 16 warrants investigation Physiologic leukorrhea precedes menses by 3-6 months Immature hypothalamic-pituitary-gonadal axis at the beginning of menstruation 50% of menstrual cycles are anovulatory in first 2 years after menarche Can cause menstrual irregularity that is normal A 15 yo boy comes to the ER because of crampy abdominal pain, diarrhea, and body aches. His siblings also have diarrhea. Exam reveals no icterus or organomegaly, although he has increased bowel sounds and mild diffuse abdominal tenderness. His genetalia are at SMR4. Amount the results of his lab tests are the following: Total bilirubin: 0.6mg/dL ALT: 18U/L Alkaline phosphatase: 460IU/L AST: 22U/L Of the following, what is the MOST likely explanation for these lab results? A. Physiologic growth spurt B. Bone malignancy C. Infectious hepatitis D. Inflammatory bowel disease E. Viral gastroenteritis Hematocrit increases in males when the growth spurt beings After puberty, males normal Hgb = 14-18g/dL Females remain lower: 12-15g/dL Alkaline phosphatase can increased during a growth spurt Result of rapid bone growth (high osteoblastic activity) Can be up to 500IU/L Cholesterol concentrations peak in early puberty Blood pressure gradually increases Based on height, sex, age <95% requires evaluation Sleep: 50% decrease in intensity of deep sleep Difficulty initiating and maintaining sleep Environmental factors play a role as well Television, computer, texting in bedroom Require 9-9.5 hrs of sleep per night Girls: Thelarche or pubarche before 8yrs Thelarche before 7 years for African-American or Mexican- American Boys: Pubarche or genital development before 9yrs Clinically significant precocious puberty is suggested if puberty advances rapidly or if it is accompanied by a growth spurt Most sexual precocity is not serious and does not need to be treated Precocity in the 6-8 year range Usually not rapidly progressive May not require treatment May be due to obesity Idiopathic premature thelarche Unilateral or bilateral NO pubic hair; NO growth spurt Caused by early activation of the hypothalamicpituitary-ovarian axis (FSH secretion) Exogenous estrogen source should be considered You are examining at 6 year old boy during a health supervision visit. Exam reveals SMR 3 pubic hair. Of the following, what is the MOST important initial step to guide your management? A. Investigate possible exposure to androgens B. Inquire about the age of puberty in family members C. Order an FSH and LH D. Order a testosterone level E. Examine the boy’s testicular size Idiopathic premature pubarche Slowly progressive pubic or axillary hair development NO breast or testicular enlargement Can be idiopathic (hypersensitivity of hair follicles to circulating androgens) or due to premature adrenarche or anabolic steroid exposure Premature adrenarche: elevated DHEAS Occasionally precursor for PCOS or evidence of virilizing disorder Premature thelarche or pubarche could be first sign of progressive true sexual precocity Must monitor pubertal development, height velocity, bone age If rapidly progressive, associated with a growth spurt, or significantly increased bone age more concerning Complete precocious puberty: Early activation of the HPG axis 5 times more common in girls 90% of cases are idiopathic In boys, only 50% are idiopathic – more often have organic cause CNS disorders , testicular tumors, adrenal hyperplasia/tumor Examining testicles can lead the evaluation: if large – they are likely the source of androgen Rapidly progressive precocity: Especially before age 6 years Always requires investigation Intrinsic adrenal or gonadal disorders CAH, McCune-Albright, testotoxicosis, neoplasms Exogenous hormones Precocious puberty risk for premature epiphyseal fusion Short adult stature despite tall stature in childhood A 7 year old girl is being treated for precocious puberty by an endocrinologist. On exam she is at SMR 3. Her parents are pleased with her medical treatment but are concerned because she does not fit in with her 7-year-old peers that she used to play with and seems withdrawn around them. She is now gravitating towards playing with older children in the neighborhood. The parents ask for advice regarding her behavior. What is the BEST response to their concerns: A. Explain that having older friends is beneficial to her selfesteem, has no risks, and should be encouraged B. Explain that their daughter should be treated more maturely because her body is maturing faster C. Recommend counseling to help the girl deal with changes in her body and to help her learn positive social interactions with her peers D. Suggest the parents call her peers and scold them for teasing her E. Suggest the parents buy her a pet to keep her happy Girls: emotional problems, low self-image, higher rate of depression, anxiety, disordered eating Shy, withdrawn with same-age peers Prefer to be with older individuals Risks of premature sexual activity, drug use, etc Parents should encourage positive self-image, social interactions with same-age peers, may need counseling Boys: higher risk for antisocial/aggressive behaviors, precocious sexual activity; but high self-esteem Hirsutism – excessive hair development in a female Normal variant when mild and isolated When accompanied by menstrual abnormality or when severe consider hyperandrogenism PCOS accounts for 85% of cases Gynecomastia Transient gynecomastia is normal during puberty Breast development in a boy BEFORE puberty is abnormal Must rule out feminizing disorder: ie. neoplasm Degree of gynecomastia matters Mid-adolescent degree of breast tissue can persist (SMR 3-4) Can indicate estrogen excess, androgen deficiency, or liver dysfunction Definition: Girls: lack of breast development by age 13 Boys: lack of testicular development by age 14 Prepubertal testicular volume is < 4mL Prepubertal penile length is < 7cm Typically accompanied by slowed growth velocity and short stature Pubic/axillary hair and body odor are due to increases in adrenal androgens (not the HPG axis) So can still have pubertal delay with the presence of pubic hair A 13 yo boy presents for his annual well child visit. His father is concerned about his son’s stature and pubertal development. The boy is doing well in school, plays soccer competitively, and is not concerned about his growth or development. The boy’s father did not start shaving until he was a senior in high school and recalls that he continued to get taller during his first 2 years of college. On exam, you note SMR 2 pubic hair and testicles 3 cm in length. The remainder of his exam is normal. A bone age radiograph demonstrates skeletal maturity of 11 years. What is the MOST likely diagnosis? A. Klinefelter syndrome B. Constitutional growth delay C. Primary gonadal failure D. Isolated gonadotropin deficiency E. Anorexia nervosa Most delayed puberty is NOT serious Majority is constitutional delay of pubertal growth and development (CDP) Especially in BOYS Boys : 63% of those with delayed puberty Girls :30% Strong genetic component Mother with menarche at <14yrs old Father with growth spurt at 15-16 yrs Variation of normal growth Normal or near normal growth rate during prepubertal years Then reduced tempo of development Height and weight both cross percentiles Caused by a delay in the onset of HPG activation delayed puberty Once puberty begins, its course and tempo are normal Bone age is delayed Catch-up growth to target height occurs (might be slightly lower than MPH) A 15yo girl presents for her annual health supervision visit. She has no complaints, and her review of systems is negative. Her mother is concerned that her daughter has not yet had menarche. The girl’s height is at the 5th percentile and her weight is at the 25th percentile. On physical examination, she has SMR stage 1 breast development and SMR stage 3 pubic hair. Examination of the external genitalia reveals a patent vagina and pink mucosa. Of the following, the BEST next step in evaluation of this patient is to: A. Measure serum estradiol B. Obtain a karyotype C. Obtain a pelvic ultrasound D. Reassure the family and see them again in 1 year E. Obtain more information about the mother’s age of menarche Primary hypogonadism (gonadal failure) “Hypergonadotropic hypogonadism” Elevated gonadotropins Particularly FSH Secondary or teritiatry hypogonadism Hypogonadotropic hypogonadism Low gonadotropins (FSH, LH) Or “normal” but inappropriately low for patient age Use a bone age to determine if patient has prepubertal or pubertal bone age Helps determine if FSH, LH levels are appropriate for that bone age Girls: Turner syndrome (gonadal dysgenesis) Can present for the first time as pubertal delay Ovarian damage radiation, chemo, autoimmune Boys: Klinefelter syndrome Small testes with normal pubic hair/penile length Testicular damage radiation, torsion, mumps You are following a 14 year old boy who has prepubertal testicular volume and penile length. All of the following would distinguish an isolated gonadotropin deficiency from constitutional growth delay, EXCEPT A. Pubertal development that has not started by age 17 B. Penile length ≤ 5cm C. Small or difficult to palpate testicles D. Delayed bone age E. Anosmia Isolated gonadotropin deficiency Complete or partial deficiency of GnRH Leads to decreased or absent secretion of LH and FSH Can resemble CDP except: Puberty does not start by age 17 May have micropenis (≤ 5cm) Testes are small and difficult to palpate Kallman syndrome (anosmia) Relatively rare in both males and females Functional gonadotropin deficiency More common in females Unusually thin Under-nutrition: Anorexia, chronic illness, excessive exercise Mechanism: Excessive exercise: decreased body fat decreased leptin concentration reversible gonadotropin deficiency Also at risk for low bone mass due to chronic low estrogen In males: more often due to chronic illness Functional gonadotropin deficiency Chronic illnesses: sickle cell, celiac, JIA, CF, Chron’s, CRF, severe asthma Other causes: Hypothyroidism Panhypopituitarism Midline defects, trauma, tumor Absence of any signs of puberty after the age of 13 years in girls or 14 years in boys merits investigation Premature arrest of previously normal growth rate in an adolescent demands thorough evaluation Bone age Typically delayed 2 years in CDP Can help predict adult height LH and FSH Elevated in primary hypogonadism Girls order karyotype If not elevated, consider CDP or gonadotropin deficiency Can measure after GnRH stimulation Total testosterone (boys) Delayed puberty <40ng/dL Puberty underway >50ng/dL Estradiol (girls) Other: thyroid studies, IGF-1, head imaging Referral to endocrinologist Parents of a 15yo boy diagnosed with constitutional growth delay are concerned that their son is being bullied by his 10th grade classmates. His grades have declined from As to Bs, and he says he dislikes school. He is in good health; he exercises and eats in moderation. On exam his height and weight are at the 10th percentile, and his genitalia are SMR 1. When you speak to him in private he becomes tearful and explains that he is afraid to change for gym and that his friends wont talk to him. He and his parents ask for advice. Of the following, the BEST response is to: A. Offer reassurance and arrange for 6 month follow up B. Recommend caloric supplements C. Refer the family for behavioral counseling D. Refer the boy for a psychoeducational evaluation E. Refer the boy to an endocrinologist for re-evaluation and possible hormonal therapy Boys: concern about being short and underdeveloped Report teasing, low self-esteem May drop out of sports Occasionally see declining academic performance and school avoidance Girls: fewer concerns than boys Report feeling “different” Some report feeling the delay affected their success at school, work, or socially But typically not a problem Referral to endocrinologist CDP: Short physiologic courses of androgen replacement therapy may help the self-image of delayed boys Injections preferred Brief therapy (4-5 months), then stop Re-assess linear growth, testicular size For girls: oral estrogens for 4-6 months Permanent hypogonadism Boys: testosterone injections/patch/gel Girls: estrogens (oral/patch) You are seeing a 14 yo female for her annual well child exam. She is there with her mother who is concerned that her daughter has not yet had her menses. The mother’s menarche was at age 13. The patient began breast development at age 12. On exam, the patient is well appearing and has SMR stage 4 breast development and SMR stage 5 pubic hair. You consult her growth chart and note that she has been growing appropriately along the 60th percentile for height and weight. Of the following, what is the next best step in management? A. Obtain a urine pregnancy test B. Draw serum LH and FSH levels C. Obtain a bone age radiograph D. Reassure the mother and follow up in 1 year E. Obtain a karyotype Primary amenorrhea: lack of menses by 15-16 years; or within 3 years of thelarche Pregnancy!, anatomic abnormalities,delayed puberty, ovarian pathology, adrenal disease, hypothalamic or pituitary disorders, disorders of sexual development Secondary amenorrhea: cessation of menstrual periods for 90 days or more Pregnancy!, anovulatory disorders: PCOS, nutritional disturbance, hyperprolactinemia Oligomenorrhea: infrequent periods DUB: bleeding excessive in amount or frequency Irregularity is common in first year of menses but should still investigate any unusual degree of irregularity: Missing a period for 90 days Bleeding more often than at 21day intervals Bleeding for more than 7 days or very heavy bleeding Bleeding for more than 10 days is NOT physiologic Failure to establish a regular period by 2 years Don’t forget complete androgen insensitivity Genetic male with normal breast development, scanty pubic hair, primary amenorrhea (We are sticking to the content specs ONLY) Definition of adolescence is not based on age norms, but by the developmental tasks that are achieved during this stage That is a lot to expect from a 12 year old… We just spent an hour talking about this but… Rapid body changes can effect an adolescents sense of self Variations in the timing of puberty can have a psychological impact Early puberty Males: greater self-confidence; greater academic/athletic success Females: lower self-confidence; concern about body image Late puberty – opposite generally true Rate of emotional/cognitive development does NOT always parallel the rate of physical maturation A single father of one of your 13 year old female patients calls you because he is concerned about something he found in his daughter’s bedroom. She is an avid soccer player and he states that he found a box of pictures of her soccer coach under her bed with hearts drawn on them. He is very concerned because his daughter has come home late from soccer practice multiple times over the past week. He seems upset and asks you if his daughter is pregnant. What is the MOST appropriate response? A. Advise him to bring the girl in to the office for a pregnancy test and STD screening B. Advise him to bring the girl in to the office to examine her for signs of abuse C. Reassure the father that crushes are a normal part of adolescent behavior and advise him to set reasonable limits about her after practice activities D. Immediately report the coach to the police E. Refer the girl for psychologic counseling Four key components Separation from parents Identification with a peer group Creating a sense of personal identity Exploration of sexuality Separation from parents Begins early Parental acceptance of an adolescent’s separation often enables the adolescent to psychologically return later in life Adolescents often look to other adults as role models “Crushes” on idealized adults are common Can see periods of regression and dependency on parents during this stage as well Not a linear process Identification with peer group Typically happens earlier in adolescence Important means of beginning the separation from family Younger peer group: same sex, similar dress/grooming, behavior “loners” who do not identify with any peers may have psychologic difficulties Peer groups have a powerful influence on healthy and unhealthy behaviors Smoking, alcohol, drugs, sex, risk-taking, school attitude Sense of personal identity Some degree of rebellion, risk-taking, testing of limits is part of the search for identity Can involve sexual behavior, alcohol, drugs, thoughtless or dangerous behavior Require clear, firm, caring limits from parents and physicians Repeated high-risk behavior or testing limits of authorities could indicate turmoil in the family Important to obtain more information about family/social background Common risk factors for delinquent behavior Parental psychiatric illness, learning disabilities, history of serious head trauma, severe behavioral problems in childhood Sense of personal identity Despite risks, experimentation is essential to adolescent development and when safe, should be encouraged Experimenting with various styles of dress, hair, etc is part of the search for identity Important for family to encourage/foster a positive selfimage Modeling of healthy behaviors, conflict resolution Demonstrate acceptance of the adolescent; praise them Poor self-image correlates with many adolescent problems Adjustment issues, school, substance abuse, sexual acting out, etc) All of the following are true statements about the impact of illness on the self-esteem for adolescents, EXCEPT: A. A medical illness can make an adolescent feel flawed and alienated from his/her peers B. Illness can lead to greater dependence on parents C. Support groups or camps can help reduce the sense of isolation that illness can bring D. Non-visible illnesses such as diabetes do not have a significant effect on adolecents’ self-esteem E. Psychotherapy is a strategy used to help adolescents cope with illness Physical illness Chronic illness may adversely affect an adolescents achievement of independence and positive self-image Illnesses that affect appearance (acne, physical deformity) AND Non-visible health conditions (DM, epilepsy, learning disabilities) can have the same emotional problems Rejection of authority and risk-taking tendencies may also include rejection of medical advice/treatment Exploration of sexuality Transient homosexual experimentation is not uncommon in early and mid-adolescence Experimentation is normal and to be expected Sexual orientation is consolidated during late adolescence Physician’s role: to help adolescents understand the risks of their behavior and to be a nonjudgemental source of information Which type of thinking is characteristic of childhood and early adolescence? A. Concrete B. Pretend/imaginary C. Symbolic D. Abstract E. Object permanence Cognition evolves from concrete thinking in childhood/early adolescence to abstract reasoning during late adolescence Concrete thinking deals largely with physically present or real objects in problem solving Consequences? Limited ability of early adolescents to imagine consequences of risky behavior Cannot link cause and effect with health behavior (smoking, overeating, drugs, alcohol, reckless driving) Abstract thinking allows to think hypothetically and develop abstract concepts to guide decision making Changes happening in the brain that affect decision making Some of the last areas to develop are the capacity to evaluate risk and reward in decision making, emotion regulation, long-term planning ?more reckless behavior Role of physician and parent Health advice more effective if adapted for the cognitive developmental level of the patient Discussion of difficult decisions that adolescents are likely to face BEFORE they occur So decisions are less based on the emotional pressure of the moment Upper-to-lower (U:L) segment ratio can be used to detect subtle abnormalities of growth and development Lower segment: distance from the pubic symphysis to the floor (in a standing patient) Subtract from the patient’s height to obtain the upper segment value The U:L ratio is then calculated using the two values Infants: legs are relatively shorter than the rest of the body Typical U:L ratio of 1.7 As children grow, the U:L ratio decreases U:L of 1.0 at 10 years of age After puberty, the U:L ratio is 0.85 - 0.95. Can be used to determine mildly precocious puberty: Tall stature, advanced bone age U:L ratio < 1
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