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Precocious puberty ppt

Definition and classification, etiology and pathogenesis, diagnosis and treatment....

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Precocious puberty ppt

  1. 1. By Umoh Emmanuel
  2. 3. WHAT IS PRECOCIOUS PUBERTY? <ul><li>This is the onset of sexual maturation at any age that is 2.5 SD earlier than the normal age for the population. </li></ul><ul><li>In other words: development of sexual maturation before the ages of 8-9 years in girls and boys. </li></ul>
  3. 4. CLASSIFICATION <ul><li>TRUE PRECOCIOUS PUBERTY (GnRH-Dependant ): </li></ul><ul><li>Idiopathic </li></ul><ul><li>CNS lesions: Hamartomas, Craniopharyngioma, etc </li></ul><ul><li>Primary hypothyroidism  </li></ul><ul><li>PSEUDOPRECOCIOUS PUBERTY ( GnRH-independent): </li></ul><ul><li>Isolated precocious thelarche </li></ul><ul><li>Isolated precocious menarche </li></ul><ul><li>Estrogen-secreting tumors of the ovary or adrenals in girls  </li></ul><ul><li>  Ovarian cysts </li></ul><ul><li>McCune-Albright syndrome    </li></ul><ul><li>Peutz-Jeghers syndrome    </li></ul><ul><li>Iatrogenic </li></ul><ul><li>III.   CONTRASEXUAL PRECOCITY (ISOLATED VIRILIZATION): </li></ul><ul><li>Isolated precocious adrenarche </li></ul><ul><li>Congenital adrenal hyperplasia    </li></ul><ul><li>Androgen-secreting ovarian or adrenal neoplasm    </li></ul><ul><li>Iatrogenic </li></ul>
  4. 5. NORMAL PUBERTAL DEV. CHART
  5. 6. True Precocious Puberty  <ul><li>This results from early maturation of the hypothalamic- pituitary-gonadal axis. </li></ul><ul><li>Serum gonadotropins, gonadal pulsitality and sex steroid concentrations are in the normal postpubertal range. </li></ul><ul><li>idiopathic precocious puberty seems to be the most common cause of CPP. </li></ul><ul><li>Neurogenic TPP seems to be found more frequently in extremely young girls with the earliest onset of puberty. </li></ul>
  6. 7. Etiology <ul><li>CNS lesions identified include neoplasms, trauma, hydrocephalus, postinfectious encephalitis, congenital brain defects, and such genetic disorders as neurofibromatosis type 1 and tuberous sclerosis. </li></ul><ul><li>The most commonly identified neurogenic neoplasms found in TPP include hamartomas, astrocytomas, and pituitary microadenomas </li></ul><ul><li>Hamartomas are congenital hypothalamic malformations that histologically contain fiber bundles, glial cells and GnRH- secreting neurons and often act as a mini-hypothalamus. </li></ul>
  7. 8. Contd. <ul><li>Girls with severe primary hypothyroidism can develop true precocious puberty. </li></ul><ul><li>These girls have elevated gonadotropins in addition to high TSH levels. </li></ul><ul><li>The associated precocity may result from cross-activation of the FSH receptor by the high circulating TSH or from direct stimulation of the ovary by the gonadotropins. </li></ul>
  8. 9. MANAGEMENT <ul><li>DIAGNOSIS: </li></ul><ul><li>The management of true precocious puberty requires identification of underlying CNS lesions, if present, or in other children identification of a pubertal gonadotropin response to GnRH that is usually associated with idiopathic true precocious puberty and occasionally with a hamartoma. </li></ul><ul><li>Hence we do: Imaging of the CNS and a GnRH challenge test. </li></ul>
  9. 10. Contd. <ul><li>bone age X-rays are helpful to identify the advance physiologic age associated with true precocious puberty. </li></ul><ul><li>Ovarian imaging , thyroid and hCG testing may also compliment the evaluation. </li></ul><ul><li>FSH AND LH LEVELS. </li></ul><ul><li>ULTRASOUND OF THE ADRENAL GLANDS. </li></ul>
  10. 11. TANNER STAGING
  11. 12. TANNER STAGING 2
  12. 13. ORCHIDOMETER
  13. 14. TREATMENT <ul><li>Administer GnRH analogues : they are modifications of the native hormones which have greater resistance to degradation and increased affinity for the pituitary GnRH receptors. </li></ul><ul><li>They induce down-regulation of receptor function, resulting in temporary, reversible inhibition of the hypothalamic-pituitary-ovarian axis as reflected by minimal or no response to GnRH stimulation and regressionof the manifestation of puberty. </li></ul>
  14. 15. Gonadotropic Independent Preococious Puberty (GIPP) <ul><li>GIPP can originate from the gonads, the adrenals, from extragonadal or intragonadal sources of human chorionic gonadotropin, or from exogenous sources. </li></ul><ul><li>In girls, functionally autonomous ovarian cysts are the most common cause of GIPP. </li></ul><ul><li>Ovarian follicles up to 8mm in diameter are common in normal prepubertal girls and may appear or regress spontaneously, but rarely secrete significant amounts of estrogen </li></ul>
  15. 16. McCune-Albright syndrome <ul><li>classically includes the triad </li></ul><ul><li>of hyperpigmented caf?au-lait spots </li></ul><ul><li>progressive polystotic fibrous dysplasia of the bones and </li></ul><ul><li>GnRH-independent sexual precocity. </li></ul><ul><li>At least 2 of these features must be present to consider the diagnosis. </li></ul><ul><li>The sexual precocity of McCune Albright syndrome is due to autonomously functioning follicular cysts </li></ul><ul><li>Testolactone, an aromatase inhibitor , has been shown to be effective treatment for the GnRH independent phase of this condition. </li></ul><ul><li>When the shift from gonadotropin independent to gonadotropin dependent puberty takes place, GnRH analog therapy then becomes effective. </li></ul>
  16. 17. Image of McCune-Albright syndrome
  17. 18. PREMATURE THELARCHE <ul><li>Isolated development of the breast tissue prior to age 8 yrs, most commonly occurring between 1 and 3 years of age. It may affect 1 or both breasts. </li></ul><ul><li>On examination, the somatic growth pattern is not accelerated, bone age is not advanced and smear of vaginal secretion fails to show estrogen effect. </li></ul><ul><li>Occurs on exposure to exogenous estrogen, as happened in Puerto Rico in the 1970’s. </li></ul>
  18. 19. Image of premature thelarche
  19. 20. PREMATURE PUBARCHE <ul><li>Defined as the appearance of pubic or axillary hair prior to age 7 years in white girls and 6 years in black girls. Such hair growth may be idiopathic and of clinical significance. </li></ul><ul><li>It usually results from an earlier than-usual increase in the secretion of androgens by adrenal glands. </li></ul><ul><li>Thorough evaluation of the gonadal and adrenal function should be made to exclude such abnormalities. </li></ul><ul><li>Signs of sever androgen excess( clitoral enlargement, growth acceleration, acne) should prompt further investigation for rare virilazation tumor. </li></ul>
  20. 21. Pathogenesis of pp.
  21. 23. PREMATURE MENARCHE <ul><li>Denotes the appearance of cyclic vaginal bleeding in children in the absence of other signs of secondary sexual development. </li></ul><ul><li>It could be related to increased end-organ sensitivity of the endometrium to low prepubertal levels of estrogens. </li></ul><ul><li>Diagnosis is formulated by exclusion following investigation of other causes of vaginal bleeding and confirmed when the cyclic nature of the bleeding becomes apparent. </li></ul>
  22. 24. Contrasexual precocity <ul><li>Most girls with contrasexual precocious puberty present with early appearance of pubic hair or hirsuitism. </li></ul><ul><li>The most common cause is a mild form of 21-hydroxylase deficiency , which is present in 0.1-1.0% of the population. </li></ul><ul><li>Other more rare forms of congenital adrenal hyperplasia have also been identified in these patients. </li></ul><ul><li>Virilizing adrenal (occasionally malignant) and ovarian tumors (e.g., Leydig or Sertoli cell tumors) in young girls can similarly present with virilizing precocious puberty. </li></ul>
  23. 25. CAH
  24. 26. EVALUATION OF PATIENTS WITH PRECOCIOUS PUBERTY <ul><li>GENERAL CHANGES : </li></ul><ul><li>Enhancement of general growth is coincident with the onset of estrogen-stimulated change. The child often exhibits accelerated growth velocity, tall stature for age, and advanced skeletal maturation. </li></ul><ul><li>SKIN : </li></ul><ul><li>Additional androgen-dependent findings include, acne and adult-type body odor. </li></ul><ul><li>BREAST : </li></ul><ul><li>According to TANNER, it is at stage II with areolae having a broadened, darkened appearance. </li></ul><ul><li>GENITALIA : </li></ul><ul><li>Genital changes reflect estrogen-induced thickening of the genital tissues. Increased vaginal secretions may result in leukorrhea. Dark, coarse pubic hair may be present. </li></ul>
  25. 27. How an Individual Can Cope with Precocious Puberty  <ul><li>Educate Yourself About the Changes </li></ul><ul><li>Realize that there are a variety of body types — big, small, and everything in between. </li></ul><ul><li>Try not to compare yourself with those around you. </li></ul><ul><li>Avoid those with negative outlooks; surround yourself with those who care about you </li></ul><ul><li>Talk to someone you trust, they could offer suggestions and make you feel a little less alone </li></ul><ul><li>Avoid those with negative outlooks; surround yourself with those who care about you. </li></ul><ul><li>Talk to someone you trust, they could offer suggestions and make you feel a little less alone. </li></ul>
  26. 28. Warning Signs of Effects on Emotional Development <ul><li>poor grades problems at school loss of interest in daily activities and depression </li></ul>
  27. 29. THE END <ul><li>Give your child with precocious puberty OR your friend or Patient love and support! </li></ul>

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