Table II-12-1. Precocious Puberty



Diagnosis

Female secondary sexual characteristics

 

Accelerated growth <8 years of age in girls

     
Normal pubertal Thelarche 9–10 years
landmarks Breast development  
     
  Adrenarche 10–11 years
  Pubic and axillary hair  
     
  Maximal growth 11–12 years
  Growth spurt  
     
  Menarche 12–13 years
  Onset of first menses  
     

 

 

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Growth spurt

Pubarche

Breast

Age

               
                                                                                                                             
9           10         11         12         13         14         15  

Menstruation

 

Figure II-12-1. Overview of Puberty


 

GYN Triad

Idiopathic or Constitutional

 

• Precocious complete isosexual puberty

• 6-year-old girl

 

• Normal head MRI

 

GYN Triad

CNS Lesions

 

• Precocious complete isosexual puberty

• 4-year-old girl

 

• Abnormal head MRI

 

GYN Triad

McCune-Albright Syndrome

 

• Precocious complete isosexual puberty

• 6-year-old girl

 

• Café-au-lait skin lesions

 

 

GYN Triad

Granulosa Cell Ovarian

 

Tumor

 

• Precocious complete isosexual puberty

• 6-year-old girl

 

• Pelvic mass

 

 

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Classification of precocious puberty

 

Incomplete Isosexual Precocious Puberty. This involves only one change—either thelarche,adrenarche, or menarche. This condition is the result of either transient hormone elevation or unusual end-organ sensitivity. Management is conservative.

 

Complete Isosexual Precocious Puberty. All changes of puberty are seen including breastdevelopment, growth spurt, and menstrual bleeding. The primary concern is premature closure of the distal epiphyses of the long bones, resulting in short stature. Fertility and sexual response are not impaired.

 

Gonadotropin-dependent. This occurs because of increased secretion of estrogensthat are dependent on premature release of gonadotropins from the hypothalamus and pituitary.

 

Idiopathic. The most common explanation is constitutional without a pathologic process present, accounting for 80% of precocious puberty. The age of the patient is usually 6 or 7 years. The diagnosis is usually one of exclusion after CNS imaging is shown to be normal. Management is GnRH agonist suppression (leuprolide or Lupron) of gonadotropins until appropriate maturity or height has been reached.

 

CNS pathology. This is a rare cause of precocious puberty. A CNS pathologic process stimulates hypothalamic release of GnRH, which leads to FSH release and ovarian follicle stimulation of estrogen production. This may include hydrocephalus, von Recklinghausen disease, meningitis, sarcoid, and encephalitis. CNS imaging is abnormal. The age of the patient is usually <6 years. Management is directed at the specific pathologic process.

 

Gonadotropin-independent. This occurs when estrogen production is independent ofgonadotropin secretion from the hypothalamus and pituitary.

 

McCune-Albright syndrome. Also known as polyostotic fibrous dysplasia, this dis-order is characterized by autonomous stimulation of aromatase enzyme production of estrogen by the ovaries. The syndrome includes multiple cystic bone lesions and café au lait skin spots. This accounts for 5% of precocious puberty. Management isadministration of an aromatase enzyme inhibitor.

 

Granulosa cell tumor. A rare cause of precocious puberty is a gonadal-stromal cell ovarian tumor that autonomously produces estrogen. A pelvic mass will be iden-tified on examination or pelvic imaging. Management is surgical removal of the tumor.

Follow-Up. Patients with idiopathic precocious puberty should be maintained with inhibition ofthe hypothalamic–pituitary–ovarian axis until the chronologic age catches up with the bone age.

 

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Symptoms. The symptoms may be of varied descriptions, including fluid retention(bloating, edema, breast tenderness), autonomic changes (insomnia, fatigue, heart pounding), emotional symptoms (crying, anxiety, depression, mood swings), or mus-culoskeletal complaints (headache, muscle aches, joint aches).


 

 

Fluid Retention

 

• Breast tenderness

 

• Extremity edema

 

• Weight gain

 

• Bloating

 

Emotional

 

• Nervous tension

 

• Mood swings

 

• Depression

 

• Irritability

 

• Anxiety, crying


 

 

Automomic

 

• Heart pounding

 

• Confusion

 

• Dizziness

 

• Insomnia

 

• Fatigue

 

Musculoskeletal

 

• Muscle aches

 

• Joint aches

 

• Headaches

 

• Cramps


 

 

Figure II-12-4. PMS Symptoms

 

Management of PMS and PMDD

 

Proven treatments include the following:

 

Selective serotonin reuptake inhibitors (SSRIs). Fluoxetine hydrochloride (Prozac);

 

natural progesterone vaginal suppositories; MPA (Depo-Provera), spironolactone, and vitamin B6 (pyridoxine). All of these options have been proposed for the treatment of

 

PMS, but only fluoxetine, alprazolam (Xanax), and GnRH agonists have been shown in controlled, double-blind trials to be superior to placebo for the more severe symptoms of PDD. Recently reported double-blind trials of fluoxetine have shown reductions of

40–75% in troublesome behavioral and emotional symptoms. Similar outcomes have been reported for buspirone hydrochloride (BuSpar) and meclofenamate sodium in descriptive studies. SSRIs are the treatment of choice for emotional symptoms of PMS.

 

Yaz (drospirenone/ethinyl estradiol), with the unique progestin, drospirinon (DRSP),has been approved by the FDA for the treatment of PMS. Yaz is a low-dose, monophasic combination oral contraceptive with 24 hormone days with only a 4 day hormone-free interval. Studies show that the symptoms of PMS are decreased with a shorter hormone-free time period. DRSP is an analogue of spironolactone which differs from other OCP progestins by exhibiting both antimineralocorticoid and antiandrogenic effects.

 

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Unproven treatments include the following:

Progesterone therapy has a long history in the treatment of PMS, but neither naturalprogesterone (vaginal suppositories) nor progestin therapy has been shown to be any more effective than placebo. Because of both a lack of efficacy and the possibility of inducing menstrual irregularities, these agents should not be used.

 

Diuretics. Because of the common complaint of “bloating” voiced by many patientswith PMS, diuretics such as spironolactone have been advocated. Spironolactone has been studied in double-blind, randomized trials, and the results have been mixed.

Although spironolactone may relieve some symptoms for some patients, the lack of con-sistent response across the studies in the literature suggests that other therapy is more effective.

 

Pyridoxine. Vitamin B6in doses of 50-200 mg/d has been suggested as a treatmentfor PMS. A number of randomized, blinded studies have been performed, but no

 

conclusive findings have emerged. Because of the lack of demonstrated efficacy and the possibility of permanent sensory neuropathy associated with high-dose vitamin B6 consumptions, the use of vitamin B6 should be discouraged.


 

Nutritional

 

• Balanced diet

 

• caffeine

 

• sugar

 

• salt


 

Lifestyle

 

• Relaxation techniques

 

• Regular exercise

 

• Support groups


 

 

Medications    
• Progesterone   SSRIs
• Spironolactone   • Fluoxetine
• Pyridoxine (B6)   OCPs
    • Yaz
     

 

Figure II-12-5. PMS Treatment

 

 

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HIRSUTISM

A 28-year-old woman complains of increased hair growth on the face and on the chest. She states that this has been going on for the past 10 years; however, she is more conscious of it at the present time. Her menses are irregular and unpredictable. Even though she has been married for 8 years and never used contraception, she has never been pregnant. On pelvic examination the ovaries bilaterally are slightly enlarged, but there are no other abnormalities noted.

 

 

Definition. Hirsutism is excessive male-pattern hair growth in a woman on the upper lip, chin,chest, abdomen, back, and proximal extremities. Virilization is excessive male-pattern hair growth in a woman plus other masculinizing signs such as clitorimegaly, baldness, lowering of voice, increasing muscle mass, and loss of female body contours.

 

Pathophysiology. Hirsutism involves the conversion of vellus hair (fine, nonpigmented hair)to terminal hair (coarse, dark hair) within the hair follicle. This conversion is under the influ-ence of androgens. In women, androgens are generally produced in only 3 body locations: the ovaries, the adrenal glands, and within the hair follicle. The workup of hirsutism will seek to identify which of these body locations is producing the androgens that are responsible for the excess terminal hair.

 

Clinical Approach

History. Is there a positive family history? What was the age of onset? Was onset grad-ual or abrupt? Have menstrual periods been irregular or regular? Is medication history positive for androgenic steroids?

 

Examination. What is body-mass index? Location of excess hair? Evidence of virilization

 

(frontal balding, loss of female body contour, clitorimegaly)? Presence of adnexal masses?

 

Laboratory Tests. The primary purpose of these tests is to identify elevated free androgens.

 

Dehydroepiandrosterone sulfate (DHEAS) is produced only in the adrenal glands. Amarkedly elevated DHEAS is consistent with an adrenal tumor.

 

17-OH progesterone is a precursor in the biosynthesis pathway of cortisol. It is elevat-ed in late-onset congenital adrenal hyperplasia (CAH), with 21-hydroxylase deficiency.

It is converted peripherally into androgens.

 

Testosterone is produced by both the ovary and the adrenal glands. A mildly elevatedlevel is suggestive of PCO syndrome. A markedly elevated level is consistent with an ovarian tumor.

 

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Clinical entities

Adrenal Tumor

 

History. Typically the onset has been rapid without positive family history.

 

Examination. Physical examination will show evidence of virilization. Pelvic exami-nation is unremarkable.

 

Laboratory tests. DHEAS level is markedly elevated.

 

Imaging. CT or MRI scan will show an abdominal-flank mass.

 

Management. Treatment involves surgical removal of tumor.


 

l Hormonal Disorders

 

GYN Triad

Adrenal Tumor

 

• Abrupt-onset virilization

 

• Abdominal/flank mass

 

• ↑↑ DHEAS levels


 

Ovarian Tumor

 

History. Typically the onset has been rapid without positive family history.

 

Examination. Physical examination will show evidence of virilization. An adnexalmass will be palpated on pelvic examination.

 

Laboratory tests. Testosterone level is markedly elevated.

 

Imaging. Pelvic ultrasound will show an adnexal mass.

 

Management. Surgical removal of the mass, usually either a Sertoli-Leydig cell tumoror hilus cell tumor.

 


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