Endocrinology Book

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Precocious Puberty

Aka: Precocious Puberty, Premature Sexual Development
  1. See Also
    1. Precocious Puberty Causes
    2. Benign Premature Adrenarche
    3. Benign Premature Thelarche
    4. Sexual Development
    5. Male Tanner Stage
    6. Female Tanner Stage
  2. Epidemiology
    1. Girls: Benign central cause in 50 to 90%
    2. Boys: Pathologic peripheral cause in 50%
  3. Definition: Precocious Puberty
    1. Girls
      1. Secondary sexual characteristics before age 8 years in girls
      2. Breast gland development
        1. Abnormal if before age 7-8 years
        2. Most will have onset after age 8 years, but occurs age 7-8 years in 20% of black, 5-10% of white girls
        3. Early Breast gland development (at age 7-8 years) also occurs with Obesity
      3. Menarche before age 10 years
      4. Pubic Hair (modified 1999)
        1. White: Before age 7 years
        2. Black: Before age 6 years
      5. Referral recommended if 2 signs at age <8 years
      6. References
        1. Kaplowitz (1999) Pediatrics 104:936-41 [PubMed]
        2. Midyett (2003) Pediatrics 111:47-51 [PubMed]
    2. Boys
      1. Testes > 2.5 cm (>3 ml vol) before age 9 years
      2. Pubic hair before age 9 years
  4. History
    1. Timing of secondary sexual characteristics
      1. Body odor
      2. Breast Development or Testicular Development
      3. Pubic and axillary hair
      4. Acne
      5. Exposures
      6. Exogenous sex steroids
      7. Head Trauma
      8. Radiation Therapy or exposure
    2. Other history
      1. Family History of Precocious Puberty
      2. Brain malignancy
    3. Symptoms
      1. Hypothyroidism or Hyperthyroidism symptoms
      2. Abdominal Pain (malignancy)
      3. Vaginal Bleeding
        1. Genital Trauma or sexual abuse
        2. McCune-Albright Syndrome
  5. Exam
    1. Constitutional
      1. Plot height, weight and Body Mass Index on growth curves
        1. Calculate Growth Velocity
        2. Calculate Midparental Height
        3. Compare Midparental Height with projected height from growth curve
          1. Abnormal if difference >10 cm
      2. Findings
        1. Body mass increased (associated with Precocious Puberty)
        2. Pubertal growth spurt (greater than the 5 cm basal rate)
        3. Short Stature (Thyroid disease)
    2. Head and Neck
      1. Thyromegaly
    3. Genitourinary
      1. Sexual maturity staging (Tanner Stage)
      2. Asymmetric Testes (gonadal mass)
      3. Clitoromegaly (Hyperandrogenism)
      4. Vagina
        1. Dull pink instead of red due to Estrogen exposure
    4. Neurologic
      1. Focal neurologic deficits (intracranial lesion)
    5. Skin
      1. Hirsutism
        1. Hyperandrogenism (androgen-secreting tumor, Congenital Adrenal Hyperplasia)
      2. Cafe Au Lait spots
        1. McCune-Albright Syndrome
        2. Neurofibromatosis
  6. Findings: Red Flags suggesting pathologic cause
    1. Premature Puberty in very young children
    2. Contrasexual development
      1. Feminization in boys
      2. Virilization in girls
    3. Peripheral cause (often asynchronous development)
      1. Penis enlarges without scrotal enlargement
      2. Extensive pubic Hair Growth
      3. Menarche without Breast bud development in girls
    4. Precocious Puberty in boys (50% pathologic)
    5. Visual field deficit suggests pituitary mass
  7. Causes
    1. See Precocious Puberty Causes
  8. Labs (See Evaluation below)
    1. Follicle Stimulating Hormone (FSH)
    2. Luteinizing hormone (LH)
    3. Estradiol Level (in girls)
    4. Testosterone Level (in boys)
    5. Thyroid Stimulating Hormone (TSH)
    6. Serum Human chorionic gonadotropin (HCG)
      1. Screen for gonadotropin secreting tumor
    7. Consider GnRH Stimulation Test
    8. See additional evaluation for Step 2c below
      1. 17-Hydroxyprogesterone
      2. Serum Dehydroepiandrosterone (Serum DHEA)
  9. Imaging (See Evaluation below)
    1. Left wrist radiograph for Bone Age
    2. Consider Head MRI
      1. Screen for pituitary or other CNS Lesion
    3. See additional evaluation for Step 2c below
  10. Evaluation: Step 1 - Initial Evaluation
    1. Clinical history and physical
    2. Exogenous Sex Hormone sources
      1. Androgens and Anabolic Steroids in boys
      2. Oral Contraceptive use in girls
      3. Estrogen or placental containing hair products
        1. Common use in African American girls
        2. Associated with Breast or pubic hair development
    3. Evaluate Pubertal Milestones (See Tanner Staging)
    4. Evaluate growth chart
    5. Obtain Left Wrist XRay for Bone Age
  11. Evaluation: Step 2a - Unremarkable Evaluation in Step 1
    1. Findings
      1. Early, but normal Puberty
        1. Girls: Breasts enlarge early
        2. Boys: Testicles enlarge early
      2. Bone Age exceeds Chronological age
        1. Early growth spurt and initially taller than peers
        2. Early epiphyseal closure and short in adulthood
    2. Diagnosis
      1. Constitutional or Idiopathic Precocious Puberty
    3. Further evaluation
      1. Observation
      2. Consider further diagnostic testing (see above)
        1. All labs at pubertal levels
        2. All imaging studies normal
    4. Management
      1. Counseling and reassurance
      2. Consider GnRH analog to suppress FSH and LH
        1. Leuprolide (Lupron) long acting injectable
        2. Nafarelin (Synarel) short acting intranasal
  12. Evaluation: Step 2b - Normal Variation in Step 1
    1. Findings
      1. Early, but normal Puberty
      2. Bone Age consistent with Chronological age
    2. Diagnosis: Benign Premature Adrenarche
      1. Girls
        1. Benign Premature Thelarche
          1. Glandular Breast tissue
        2. Benign premature Menarche
          1. Prepubertal Vaginal Bleeding
        3. Benign Premature Adrenarche
          1. Pubic and axillary hair, body odor or acne
          2. Distinguish from Congenital Adrenal Hyperplasia, cortisol excess, adrenal tumor (see step 2c)
        4. Fatty Breast tissue (Lipomastia)
      2. Boys
        1. Benign Gynecomastia of Adolescence
        2. Familial Gynecomastia
        3. Consider evaluation for alternative causes of persistent Gynecomastia for >18-24 months
          1. Testicular Cancer
          2. Adrenal adenoma
          3. Performance enhancing drugs
          4. Hypogonadism (e.g. Klinefelter Syndrome)
    3. Further evaluation
      1. Observation over 3-6 months
      2. Consider further laboratory testing for progressive symptoms (see diagnostics above and to Step 2c below)
    4. Management
      1. Counseling and reassurance
  13. Evaluation: Step 2c - Abnormal Evaluation in Step 1
    1. Findings
      1. Abnormal Pubertal Milestone sequence
      2. Bone Age variable
        1. May be consistent with Chronological age
    2. Differential Diagnosis (pathologic cause suspected)
      1. See Precocious Puberty Causes
      2. Central Precocious Puberty due to idiopathic or CNS Lesion (pubertal LH and gonad size)
        1. Features
          1. Despite Precocious Puberty, otherwise normal development
          2. More common in girls than boys (by 10 fold)
          3. Typically idiopathic in girls, but more likely to be pathologic in boys (e.g. Head Trauma, Brain Tumor)
        2. Consider Gonadotropin releasing hormone (GnRH) therapy (e.g. Lupron)
          1. Early initiation before epiphyseal closure preserves height potential
        3. Brain MRI indications
          1. Boys with Precocious Puberty
          2. Girls under age 6 years old
          3. Neurologic findings (Headache, Seizure, vision changes)
      3. Peripheral Precocious Puberty (prepubertal LH and gonad size)
        1. Congenital Adrenal Hyperplasia, cortisol excess, adrenal tumor
          1. Consider Corticotropin Stimulation Test, adrenal imaging, Cushing Syndrome
          2. Endocrinology referral
        2. Exogenous sex steroid exposure
        3. Hypothyroidism
        4. Ovarian tumor
          1. Elevated estradiol level and low LH level seen with Estrogen-secreting tumors
        5. Testicular tumor
        6. McCune-Albright Syndrome
        7. Neurofibromatosis
    3. Further evaluation
      1. Further laboratory testing (see above)
      2. Additional lab testing (esp. Virilization, hyperandrogenic effects in girls)
        1. 17-Hydroxyprogesterone
        2. Serum Dehydroepiandrosterone (Serum DHEA)
      3. Additional imaging (suspect peripheral cause)
        1. Pelvic Ultrasound of Ovaries
        2. Consider Adrenal MRI
    4. Management
      1. Assess for exogenous sex steroid exposure
      2. Treat based on underlying cause
  14. References
    1. Blondell (1999) Am Fam Physician 60:209-24 [PubMed]
    2. Fahmy (2000) Br J Radiol 73(869):560-7 [PubMed]
    3. Foster (1992) Obstet Gynecol Clin North Am 19:59-70 [PubMed]
    4. Klein (2017) Am Fam Physician 96(9): 590-99 [PubMed]
    5. Styne (1997) Pediatr Clin North Am 44(2):505-29 [PubMed]
    6. Tiwary (1998) Clin Pediatr 37(12):733-9 [PubMed]
    7. Walvoord (1999) Pediatrics 104(4):1010-4 [PubMed]

Precocious Puberty (C0034013)

Definition (NCI) Unusually early sexual development or maturity; premature puberty.
Definition (NCI_CTCAE) A disorder characterized by unusually early development of secondary sexual features; the onset of sexual maturation begins usually before age 8 for girls and before age 9 for boys.
Definition (NCI) Unusually early sexual maturity.
Definition (CSP) unusually early sexual maturity.
Definition (MSH) Development of SEXUAL MATURATION in boys and girls at a chronological age that is 2.5 standard deviations below the mean age at onset of PUBERTY in the population. This early maturation of the hypothalamic-pituitary-gonadal axis results in sexual precocity, elevated serum levels of GONADOTROPINS and GONADAL STEROID HORMONES such as ESTRADIOL and TESTOSTERONE.
Concepts Disease or Syndrome (T047)
MSH D011629
ICD10 E30.1
SnomedCT 267488008, 190576002, 154716006, 190575003, 400179000, 123527003, 70387004
English Puberty, Precocious, PUBERTY PRECOCIOUS, Sexual Precocity, Precocious Puberty, precocious puberty (diagnosis), precocious puberty, Maturation sex accelerated, Accelerated sexual maturation, Accelerated sexual maturity, Early puberty, Precocious puberty NOS, Puberty precocious, Puberty, Precocious [Disease/Finding], premature puberty, early puberty, precocious sexual development, puberty precocious, Puberty;precocious, sexual precocity, true precocious puberty, puberty early, True precocious puberty, Premature puberty, (Sexual precocity NOS) or (puberty - precocious), (Sexual precocity NOS) or (puberty - precocious) (disorder), Puberty - precocious, Precocious puberty, Sexual precocity, Precocious sexual development, Precocious puberty (disorder), Precocious sexual development (disorder), precocious; puberty, premature; puberty, pubertas praecox, puberty; precocious, puberty; premature, Pubertas praecox, Precocious true puberty, Sexual precocity, NOS, Sexual precocity NOS
French PUBERTE PRECOCE, Puberté précoce SAI, Maturation sexuelle accélérée, Maturité sexuelle accélérée, Puberté précoce vraie, Puberté précoce
Portuguese PUBERDADE PRECOCE, Maturação sexual acelerada, Maturidade sexual acelerada, Puberdade precoce NE, Puberdade precoce verdadeira, Puberdade precoce, Puberdade Precoce
Spanish PUBERTAD PRECOZ, Pubertad temprana, Maduración sexual acelerada, Pubertad precoz NEOM, Madurez sexual acelerada, pubertad precoz verdadera, Pubertad precoz verdadera, desarrollo sexual Y/O puberal precoz, desarrollo sexual precoz (trastorno), desarrollo sexual precoz, precocidad sexual, pubertad precoz (trastorno), pubertad precoz verdadera (trastorno), pubertad precoz, pubertas praecox, Pubertad precoz, Pubertad Precoz
Dutch versnelde seksuele ontwikkeling, versnelde seksuele rijpheid, vroege puberteit, pubertas praecox NAO, seksuele ontwikkeling versneld, echte pubertas praecox, prematuur; puberteit, puberteit; prematuur, puberteit; vroegtijdigheid, vroegtijdigheid; puberteit, Pubertas praecox, pubertas praecox, Puberteit (ontwikkelingstoestand); vroegtijdig, Vroegtijdige puberteit
German beschleunigte sexuelle Reife, fruehe Pubertaet, Geschlechtsreife beschleunigt, Pubertas praecox NNB, PUBERTAS PRAECOX, Vorzeitige Pubertaet [Pubertas praecox], Pubertas praecox vera, Pubertas praecox, Pubertät, verfrühte
Italian Pubertà precoce NAS, Pubertà anticipata, Maturità sessuale accelerata, Sviluppo sessuale precoce, Precocità puberale, Maturazione sessuale accelerata, Pubertà precoce vera, Pubertà precoce
Japanese 思春期早発, 性成熟亢進, 思春期早発症NOS, セイセイジュクコウシン, シシュンキソウハツ, シシュンキソウハツショウNOS, シシュンキソウハツショウ, 思春期早発症, シンセイシシュンキソウハツショウ, 中枢性思春期早発症, 中枢性早熟症, 性早熟症, 性的早熟, 早期破瓜, 早熟, 早熟症, 早発思春期, 早発性大性器症, 早発青春期, 真性思春期早発症, 真性性早熟症, 性的早熟症, 早発症-思春期
Swedish Pubertet, för tidig
Czech puberta předčasná, Urychlená pohlavní zralost, Urychlené pohlavní dospívání, Pubertas praecox, Předčasná puberta, Pubertas praecox NOS, Pravá předčasná puberta
Finnish Ennenaikainen murrosikä
Russian POLOVOE RAZVITIE PREZHDEVREMENNOE, ПОЛОВОЕ РАЗВИТИЕ ПРЕЖДЕВРЕМЕННОЕ
Korean 사춘기 조숙
Croatian PUBERTET, PRIJEVREMENI
Polish Dojrzewanie płciowe przedwczesne, Pokwitanie przedwczesne
Hungarian Accelerált sexualis érettség, Pubertas praecox, Gyorsult nemi érés, Accelerált sexualis érés, Korai pubertas, Praecox pubertas k.m.n., Praecox pubertas, Valódi pubertas praecox
Norwegian For tidlig Pubertet, Pubertas praecox, For tidlig kjønnsutvikling
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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