An African American female with irregular menses and weight gain.
History of Present Illness
Kendra and her room state that she started noticing excessive hair around 6 months ago. She informs the medical staff at the Endocrinology clinic that she has been shaving with a razor but that this only makes the hair return even thicker than before. She reports that she started her menstrual cycle at the age of 9 and that it is very irregular, lasting sometimes 2 to 3 weeks, with associated heavy bleeding. She has also experienced amenorrhea for 2 or 3 months. She was started on Yasmin[R] 6 months ago by her primary care physician to control the cycle irregularities. Kendra and her mom report that the birth control pills (BCPs) have been of some help, taking notice that the cycles were a little more regular as far as duration but that the bleeding remained heavy. Patient's mother noticed that Kendra's weight gain has been an ongoing problem since Kendra was a toddler. Kendra's mom states, "I didn't think anything of it since both sides of the family are big." In the last month, Kendra has noticed frequent urination, as well as excessive thirst, both of which occur during the night.
Kendra was born at 40 weeks gestation after a normal spontaneous vaginal delivery. She had a 2-day length of stay after birth without complications. Kendra's birth weight was 6 pounds and her height was 20 inches, with apgar scores of 8 and 10. Kendra was breastfed as an infant for 11 months and weaned to whole milk and table foods at the age of 12 months without any difficulty. Kendra's developmental milestones were reached at the appropriate age and she continues to excel in academics while in her current grade at school.
Kendra lives with both parents, who have been married for 28 years, in a middle class neighborhood on the south side of town. She has two male siblings who are ages 16 and 27. Kendra's oldest brother has a significant history of hypertension, while her younger brother has no significant health problems. Kendra's father is a 44 year-old foreman at the local steel plant and has hypertension as well as Type II Diabetes. Her mother is a 40 year-old bank teller and has no chronic medical problems. Kendra's mother remembers having heavy menstrual cycles and having facial hair when she was growing up. Kendra's maternal grandmother has hypertension and her paternal grandmother has a history of Type II Diabetes and hypertension. Neither of Kendra's grandfathers has any significant health problems. No one in Kendra's household smokes nor is she exposed to smoking on a regular basis. There are no pets in the home except tropical fish. Kendra is enrolled in public school and usually goes home after school or to her maternal grandmother's house until one of her parents arrives to pick her up. Both of Kendra's grandparents as well as her extended family are of great support to the family and all live in the locale.
Kendra is a popular girl at school and is a flute player in her high school band. She makes A's and B's in her advanced academic classes in the 10th grade and hopes to go to college someday to become a doctor. She gets along well with her two brothers and her parents.
Kendra has never had a steady boyfriend and she attributes this to her being overweight and her facial hair. She says she is very motivated to lose weight and seeks medical attention for these reasons.
General appearance: Kendra is a well-groomed, obese African American female who is very pleasant throughout the exam.
Vital signs: T-97.4 degrees F; HR-67; RR-28; B/P 121/63
Height: 166 cm (73rd percentile)
Weight: 102 kg (greater than 97th percentile)
BMI: 37 (greater than 97th percentile)
HEENT: Normocephalic. Marked hirsutism noted to face. PERRLA. Vision normal. Tympanic membranes clear with cone of light visualized. Nares patent bilaterally with no drainage noted. Tonsil 1+ with normal oropharynx. Mucous membranes pink and moist. No dental caries. Normal dentition.
Neck: Supple, no lymphadenopathy.
Lungs: Clear and equal bilateral breath sounds with no cough noted, equal chest expansion.
Abdomen: Soft, non-distended and non-tender, no masses. No hepatosplenomegaly. Bowel sounds in four quadrants.
Cardiovascular: Regular rate and rhythm, no murmur, pulses equal bilaterally.
Skin, hail, nails: Acanthosis nigricans to neck and arm pits (3 on a scale of 1 to 5), no rash or lesions noted. Hair healthy with new growth noted at the hairline.
Genitalia: Tanner Stage 5 Breasts, with normal genitalia.
Musculoskeletal: Full range of motion of all extremities. Spine straight; gait steady.
Fasting lab data: Hematocrit-34 (normal range 32-44); Cholesterol-129 (normal range: less than 200 mg/dl); Magnesium 1.9 (normal range1.3-2.1); Phosphate-3.7 (normal range 3.0-4.5 mg/dl); Glucose-83 (normal range 80-110 mg/dl); Insulin-29 (Normal lab values 6-26); Testosterone-136 (normal range less than 60 ng/dl); LH-12.78 (Normal range 1.68-15); FSH 1.42 (Normal range 1.37-9.9); prolactin level 11 (normal range 0-25); Hemoglobin A1-C 5.2 (normal 2.2 -4.8); adrenocorticotropic hormones (ACTH)--2 (normal range less than 80 pg/ml).
CT of the abdomen and pelvis showed that the adrenal gland, pelvic, bladder and distal ureters were normal and without mass or tumors. Ovaries appear to be normal in size and without evidence of cysts or tumors.
What is Your Differential Diagnosis?
The differential diagnosis for Kendra include: 1) ovarian hyperthecosis, 2) congenital adrenal hyperplasia, 3) Cushing syndrome, 4) androgen-producing neoplasms/adrenal carcinoma, and 5) polycystic ovarian syndrome. Characteristics of each diagnosis are shown in Table 1.
Ovarian hyperthecosis. Ovarian hyperthecosis is the unusual proliferation of the ovaries that causes nests of luteinizing theca cells throughout the stroma (Chang, 2004). The ovaries are enlarged with fibroblastic growth with the absence of follicle formation, which distinguishes from polycystic ovary. Physical findings include severe hirsutism, clitoromegaly, temperal balding, a male body habitus, and a deepening of the voice. Patients with hyperthecosis have marked insulin resistance with elevated insulin levels and are often obese and have acanthosis nigricans.
Congenital adrenal hyperplasia (CAH). CAH involves several autosomal recessive disorders that are in common. There is a deficiency of an enzyme involved in the adrenal gland that causes a complete or partial deficiency of an enzyme involved in adrenal synthesis, the most common of which is 21-hydroxylase deficiency (CAH-21). CAH-21 is normally recognized at birth (classical CAH); however, non-classical or adult onset has similar symptoms of increased androgen and testosterone (Chang, 2004; Wilson, 2005). Clinical presentation that distinguishes it from other diagnosis includes severe hirsutism, clitormegaly, familial tendency, and short stature.
Cushing Syndrome. Cushing syndrome is a manifestation of symptoms caused by prolonged exposure to elevated levels of glucocorticoids (ACTH) or overproduction of cortisol (Chang, 2004). Individuals diagnosed with Cushing syndrome have complaints of obesity, moonlike facial appearance, muscle wasting, abdominal striae, irregular menses, hirsutism, and hypertension. In most cases, the over-production of ACTH is caused by a pituitary tumor.
Adrenal carcinoma. Adrenal cortical carcinoma is a rare tumor in the pediatric population (age 0-21). Examination would include a palpable abdominal or pelvic mass that is hard and nonmovable. Also premature eruption of pubic and axillary hair, lack of breast development, signs of Cushing syndrome, severe hirsutism, a male body habitus, and clitoromegaly are indicative of Adrenal carcinoma. The early stages of the development of these tumors can mimic other illnesses; however, the rapid onset of symptoms provide an important clue in this diagnosis (Chang, 2004).
Polycystic Ovarian Syndrome (PCOS). PCOS affects 5% to 10% of women of reproductive age and is the most common endocrine disorder among women of this age group (Marx & Mehta, 2003). Presentation may include amenorrhea, menstrual irregularities, signs of androgen excess (hirsutism, acne and male pattern balding, alopecia). Half of the patients with PCOS are obese (Chang, 2004). Metabolic problems usually develop with age and include Type 2 Diabetes Mellitus, dyslipidemia, hypertension, cardiovascular disease, and high risk for endometrial or ovarian cancer as well as infertility (Salmi, Zisser, & Jovanovic, 2004).
Laboratory findings for PCOS may include an elevated luteinizing hormone (LH) with a normal or low follicle-stimulating hormone (FSH); however the LH/FSH ratio is often greater than 3 (Silverman, 2005). Testosterone and prolactin may also be elevated. Fasting cholesterol, high-density lipoprotein (HDL), low-density lipoprotein (LDL), triglycerides, and hemoglobin A1C may also be elevated and show signs of insulin resistance Ultrasound is used to detect the existence of polycystic ovaries and CT scan or MRI is only indicated if the presence of an adrenal tumor is possible (Silverman, 2005).
What is Your Assessment?
Kendra showed little physical signs of Cushing syndrome, however evaluation of lab specimens to check ACTH or cortisol levels were done to rule out this diagnosis and were found to be within normal limits. Ovarian hyperthecosis was ruled out due to the fact that there were no signs of enlarged ovaries and her clinical findings did not suggest ovarian hyperthecosis. Kendra's gastrointestinal exam was normal without masses and was confirmed by ultrasound, which ruled out adrenal carcinoma. Kendra's physical findings were not consistent with CAH, but to rule out the diagnosis, a CAH-21 level was drawn and was within normal limits. A quick review of Kendra's labs reveals her LH/FSH ratio was 12, which is a classic symptom of inappropriate gonadotropin secretion, causing her ovarian dysfunction. Her testosterone and insulin levels were elevated; however, her prolactin and fasting glucose levels were all normal. Kendra presents with the other classic symptoms of hirsutism and obesity, but without lipid abnormalities, or cardiovascular involvement. Based on the findings, Kendra was diagnosed with polycystic ovarian syndrome, although she did not have the classical polycystic ovaries. Only 20% of the adolescents and women who are diagnosed with PCOS have polycystic ovaries (Franks, 1997).
What is Your Management Plan?
Weight loss is the recommended goal of treatment in PCOS. Although it may not be the simplest way, it is the non-pharmacological way to restore menstruation and ovulation in patients who have been diagnosed. Exercise and weight control will reduce the chances of Kendra developing metabolic syndromes later in life. A realistic weight management regimen consisting of diet and exercise must be devised to meet Kendra's individual needs and the goal must be attainable. A nutritional consultation can be made so that Kendra and her family can identify target areas of concern that may be helpful in facilitating weight loss. As for anyone who is considering weight loss, it should become a family affair to promote a healthy lifestyle that everyone will benefit from in the future.
Kendra's current lab results were slightly elevated (insulin, HgbA1C, LH) and need to be closely monitored in the future to detect any possibilities of complete insulin resistance. Also due to her familial tendencies for the development of hypertension and Type 2 Diabetes, Kendra must be closely monitored and followed by a physician. Kendra was prescribed an oral contraceptive which is used to treat amenorrhoea, anovulation and hirsutism. It can be used in conjunction with Metformin[R], an oral anti-diabetic drug, to treat PCOS and to further restore fertility and regular menstrual cycles if her symptoms do not reside after a 3-month period. Because hirsutism is a big issue for teenage and adolescent girls, a plan should be implemented to encourage them that this condition will improve with time. The effects of oral contraceptives may take 30 to 90 days and alternative methods of hair removal such as electrolysis or laser, waxing or shaving can be effective in Kendra's case. Spironolactone is another pharmacological method of effectively treating hirsutism; however, it has a potential teratogenic effects and is usually prescribed with an oral contraceptive to prevent this. Other side effects of spironalactone include hyperkalemia, gastrointestinal discomfort, and irregular menstrual bleeding which can be managed by taking an oral contraceptive as well.
Although polycystic ovarian syndrome (PCOS) and obesity are generally thought of as physical conditions, the potential psychosocial and emotional consequences of these issues cannot be ignored. In the case of Kendra, psychological effects seemed to be handled well due to her positive outlook on the manifestations of PCOS. However, women of childbearing age may be dealing with the long-term effects of infertility when a diagnosis of PCOS is made. Although not everyone living with PCOS, obesity, or any other metabolic disorder experiences psychosocial or emotional consequences, these health issues do place women at greater risk, and health care providers should be encouraged to screen for common mental health symptoms when treating patients who present with these health issues. Some studies suggest that certain factors, such as family connectedness, act as protectors against the negative effects of overweight status (Mellin, NeumarkSztainer, Story, Ireland, & Resnick, 2002), so PCOS and obesity should not be considered the direct cause of potential psychosocial consequences. As with most other conditions, it is likely a combination of severity of illness, personality characteristics, and availability of social support that determine if someone who is obese or has PCOS will experience the potential psychosocial consequences. One study with obese adolescent girls demonstrated that low self-esteem is a primary psychosocial correlate of obesity, with self-esteem particularly affected in three domains: athletic competence, physical appearance, and global self-worth (Burrows & Cooper, 2002). Health care professionals are urged to be sensitive to the psychosocial dimensions of these disorders and evaluate patients on a case by case basis, referring to mental health providers when appropriate (see Table 2 for resources).
This is a Pediatric Management Problem (PMP) designed to test your problem-solving and decision-making abilities
Instructions: Read the PMP below, Then outline how you would assess and manage the problem. Finally, compare your rationale and decision to that listed in the shaded area.
Please submit material to: Jean Ivey, DSN, CRNP; Pediatric Nursing Journal; East Holly Avenue/Box 56; Pitman, NJ 08071-0056; (856) 256-2345 (fax)
Tina Simpson, MD Jean Ivey, DSN, CRNP
Burrows, A., & Cooper, M. (2002). Possible risk factors in the development of eating disorders in overweight pre-adolescent girls. International Journal of Obesity and Related Metabolic Disorders, 26(9), 1268-1273.
Chang, R. (2004). A practical approach to the diagnosis of polycystic ovary syndrome. American Journal of Obstetrics and Gynecology, 191(3), 713-717.
Franks, S. (1997). Polycystic ovary syndrome. Archives of Disease in Children, 77(1), 89-90.
Marx, T., & Mehta, A. (2003). Polycystic ovary syndrome: Pathogenesis and treatment over the short and long term. Cleveland Clinical Journal of Medicine, 70(1), 31-45.
Mellin, A., Neumark-Sztainer, D., Story, M., Ireland, M., & Resnick, M. (2002). Unhealthy behaviors and psychosocial difficulties among overweight adolescents: The optimal impact of familial factors. The Journal of Adolescent Health, 31(2), 145-153.
Salmi, D., Zisser, H., & Jovanovic, L. (2004). Screening for and treatment of polycystic ovary syndrome in teenagers. Experimental Biology and Medicine, 229(5), 369-377.
Silverman, K. (2005). Polycystic ovarian syndrome. Retrieved March 28, 2006, from http://www.emedicine. com/ped/topic2155.htm
Wilson, T.A. (2005). Congenital adrenal hyperplasia. Retrieved March 28, 2006, from http://www.emedicine. com/PED/topic48.htm
Yolanda S. Harris, BSN, RN
Nurse Practitioner Fellow
University of Alabama
Department of Pediatrics
Sydney Brodeur McDonald, PhD
University of Alabama
Department of Pediatrics
Table 1. Differential Diagnosis Chart for Polycystic Ovarian Syndrome Elevated Obesity Glucocorticoids * Cushing Syndrome X X Ovarian Hyperthecosis Adrenal Carcinoma X X Congenital Adrenal Hyperplasia Polycystic Ovarian Syndrome X Pituitary Moon-like Muscle Tumor Facial Wasting Features Cushing Syndrome X X X Ovarian Hyperthecosis Adrenal Carcinoma X X Congenital Adrenal Hyperplasia Polycystic Ovarian Syndrome Abdominal Irregular Hirsutism Straie Menses Cushing Syndrome X X X Ovarian Hyperthecosis Adrenal Carcinoma X X Congenital Adrenal Hyperplasia X X Polycystic Ovarian Syndrome X X Hypertension Clitoromegaly Cushing Syndrome X X Ovarian Hyperthecosis X Adrenal Carcinoma X Congenital Adrenal Hyperplasia X Polycystic Ovarian Syndrome X Male Male Voice Pattern Body Deepening Baldness Habitus Cushing Syndrome Ovarian Hyperthecosis X X X Adrenal Carcinoma X Congenital Adrenal Hyperplasia Polycystic Ovarian Syndrome Rise in Acanthosis Palpable Insulin/ Nigricans Abdominal/ Insulin Pelvic Mass Resistance Cushing Syndrome Ovarian Hyperthecosis X X Adrenal Carcinoma X Congenital Adrenal Hyperplasia Polycystic Ovarian Syndrome X X Lack of Rapid Autosomal Breast Onset of Recessive Development Symptoms Disorder Cushing Syndrome Ovarian Hyperthecosis Adrenal Carcinoma X X Congenital Adrenal Hyperplasia X Polycystic Ovarian Syndrome 21-Hydroxylase Rise in deficiency Adrogens Cushing Syndrome Ovarian Hyperthecosis Adrenal Carcinoma Congenital Adrenal Hyperplasia X X Polycystic Ovarian Syndrome X Rise in Familial Short Testosterone Tendencies Stature Cushing Syndrome Ovarian Hyperthecosis Adrenal Carcinoma Congenital Adrenal Hyperplasia X X X Polycystic Ovarian Syndrome Ovarian Acne Alopecia Cysts/ Fibroids Cushing Syndrome Ovarian Hyperthecosis Adrenal Carcinoma Congenital Adrenal Hyperplasia Polycystic Ovarian Syndrome X X X Infertility Excess Facial Hair Cushing Syndrome Ovarian Hyperthecosis Adrenal Carcinoma Congenital Adrenal Hyperplasia Polycystic Ovarian Syndrome X X Note: * adrenocorticotropic hormone (ACTH) and cortisol Table 2. Resources for Healthcare Professionals, Patients and Family Dealing With PCOS The Hormone Foundation--Polycystic Ovary Syndrome http://www.hormo ne.org/learn/pcos.html International Council on Infertility Information Dissemination, Inc.--PCOS Frequently Asked Questions http://www.inciid.org/faq.ph p?cat=infertility 101&id=2 PCOS.net-Helping Women with Polycystic Ovarian Syndrome http://www.pcos.net/ ObGyn.net--PCOS Pavilion http://www.obgyn.net/pcos/pcos.asp Polycystic Ovarian Syndrome Association (PCOSA) http://www.pcossupport.org/ The University of Chicago Hospitals--Center for Polycystic Ovary Syndrome http://centerforpcos.bsd.uchicago.edu/ U.S. Department of Health and Human Services--Polycystic Ovarian Syndrome http://www.4woma n.gov/faq/pcos.htm
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|Author:||Simpson, Tina; Ivey, Jean|
|Date:||Jul 1, 2006|
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