Adolescent Case Presentation - NCC Pediatrics Residency

Adolescent Case Presentation - NCC Pediatrics Residency

Adolescent Case Presentation Kelli L. McDermott LT MC USNR Case history 14 y/o female CC: 3-6 months of irregular periods and unexplained weight gain In USOH, has not been ill in last few months PMH- not significant

Case History HEADS interview negative Home: lives with parents, no sibs, gets along fine Education: 9th grade, A-B student, has good group of friends Activities: rows for school crew team, movies & hanging out with friends

Case History Drugs: never smokes, drank, or tried any

drugs, no friends hace either Diet: parents MDs and help her eat a balanced diet, she reports no increased eating habits since weight gain Sex: never been active, never had a girl/boyfriend Suicide: no h/o depression Case History Menstrual hx menarche at age 12 Regular periods over past year and then irregular for about 6months; no

periods for about 3 months now Never been sexually active Case History FHx- NC, no female family member with abnormal periods, no problems with cycle, fertility. No cancers Physical Exam

VS: HR 65; RR18; BP112/80; wt 93.5kg (>99th%); ht 160cm (50th%); BMI= 36 HEENT: fat pad behind neck, thickening & slight hyperpigmentation of posterior neck skin, nl thyroid CV: S1+S2, no R/G/M, RR

Lungs: CTA bilat Abd: obese, soft, +BS, striae across abdomen and lower hips Physical Exam Ext: FROM, nl muscle tone, 2+ cap refill, pulses normal Skin- dry but no lesions, rashes, acne noted over face, chest, back, no excess hair. GU- no external abnormalities, Tanner 5, normal clitoris

QUESTIONS on H& P??? How about a differential for secondary amenorrhea? Differential Diagnosis Pregnancy PCO Hypothyroidism Ovarian tumor

Pituitary tumor Less likely differential CAH Female Athlete Triad (hypothalamic amenorrhea)

Turners syndrome Testicular Feminization Which labs would you think about at this initial presentation?

Laboratory Tests B-HCG Thyroid LH/FSH Prolactin Free/total testosterone Laboratory Tests Fasting glucose

Fasting Insulin level Fasting Lipid profile Androstenedione Fasting 17-OPH and cortisol DHEAS Karyotype Our patient

Nl TFTs Glucose 81 Lipid profile all WNL LH 4.17 FSH 6.8

PRL 5.75 Andro 181 17-OHPS 58 Insulin 5.1 Ttest 36 Free test 6.7 (only

abn lab) What is PCOS? Increased androgen production from ovaries and adrenal glands What does it mean to have PCOS? Well, unfortunately, it means a lot of difficult things for many women. I started to have facial hair growth in early highschool -- this was pretty embarassing, especially when I realized that it wasn't

"normal" compared to my other friends. Of course, I had lots of hair on my legs and arms too, at an even younger age -growing up in Southern California meant that I was doing a lot of hair removal all the time so as to not look like a freak in shorts or a bathing suit. My skin just didn't ever seem to clear up -- I spent many hours at the dermatologist. I also "learned" early on that I couldn't eat very much at all -- if I did, I immediately gained a lot of weight and it didn't want to come off. My cycles were horrible, when I had them, I understood why some women called it "the curse". I was diagnosed when I was 17 and immediately went on birth control pills to control my symptoms. This was the only

practical "treatment" known at that time. Later on, PCOS was the reason I couldn't easily conceive and then miscarried the 2 times I did conceive naturally. I think this is the most acutely painful aspect of this syndrome, and it is certainly the focus of many women's pain. Wanting a child and being unable to have one was one of the most difficult times of my life. Needing to take in order to conceive and carry a pregnancy can have some very subtle effects on how a woman thinks about herself, and when she has a condition that already makes her feel less attractive, less desirable and less feminine (at least by our culture's standards), she can end up seeing herself as pretty defective. Later in life, PCOS presents some serious health

problems. Women with PCOS are significantly more likely to have type II diabetes and heart disease and there appears to be a link to breast and colon cancer, so it isn't just a "cosmetic" or "infertility" condition -- it can be ugly. PCOS Spectrum of clinical d/os not diagnosed by lab Clinical presentation includes: Hirsuitism & acne

Obesity Oligomenorrhea Anovulation Infertility PCOS Pituitary gland is heightened to GnRH Exaggerated pulsatile LH release LH/FSH ratio may be elevated

LH stimulate ovary to secrete androgen Androgens are converted to estrone and estradiol Estrogens secreted tonically Augment pituitary sensitivity to GnRH And vicious cycle continues to LH

ovaries overproduce androstenedione and testosterone Other interesting findings Androgens SHBG; free testosterone Anovulation and insulin resistance- exact pathogenesis unclear

in basal insulin secretion in hepatic uptake B-cell dysfunction insulin has direct effect on pituitary in LH secretion and the ovary for androgen production Problems associated with high levels of sex hormones:

Anovulation results in amenorrhea & infertility

Hirsuitism, acne Male pattern baldness/thinning Obesity- android-type with waist-hip ratios Cancer- endometrial Problems associated with high levels of sex hormones: Insulin resistance

Hyperinsulinemia Diabetes Cardiovascular

disease Theories to etiology of PCOS Genetic predisposition is most likely although no gene isolated; believe in 2-hit hypothesis Premature adrenarche (<8 y/o) Heterozygosity for CAH IUGR Treatment

Cosmetic interventions OCPs: suppress LH androgens SHBG free testosterone adrenal production of androgen 5alpha-reductase

Spironolactone Treatment Cyclic progestins

GnRH agonists Weight control Low carb diets Exercise to reduce

weight and CV risk factors Treatment- controversial Insulin sensitizing drugs: biguanides & thiazolidinediones insulin R hirsuitism restore nl ovulatory patterns

Metformin Reduces hyperinsulinemia Decreases risk factors for CHD Improved weight-loss Normalization of circulating androgens Resumption of normal ovulatory menses and therefore reversal of infertility

Resources and Websites www.pcosupport/pcoteen

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