Precocious and delayed puberty

puberty;delayed;precocious puberty

1. Precocious and D elayed puberty ANDREA.R.S

2. introduction Puberty in girls is the period which links childhood to adulthood It is the period of gradual development of secondary sexual characters Common order is Beginning of growth spurt Breast budding( thelarche ) Pubic and axillary hair growth( adrenarche ) Peak growth in height Menstruation(menarche) All these changes are completed by 10-16 yrs

3. Precocious puberty This term is reserved for girls who exhibit any sexual characters before the age of 8 OR menstruate before age of 10 TYPES -true or complete or central pseudo or incomplete or peripheral

4. Central -more common Due to premature activation of HPO axis The pituitary is involved Peripheral -rarer Here pituitary is not involved Local problem in ovaries or adrenals

5. causes Complete - I diopathic,familial Intracranial lesion Incomplete -Premature thelarche Premature puberache Premature menarche Precocious pseudopuberty -Feminising ovary tumors Adrenal hyperplasia Iatrogenic Hypothyroidism McCune -Albright syndrome

6. Aetiopathology Constitutional -commonest Due to premature activation of HPO axis Secretion of gonadotrophins and gonadal steroids due to premature GnRH release Bone maturation is accelerated McCune Albright syndrome characterised by sexual precocity,multiple cystic bone lesion,endocrinopathies and café au- lait spots

7. Diagnosis True precocious -familial history tanner stages no cause could be detected Basic investigations- X-ray wrist for bone age Pelvic sonography to exclude ovarian pathology CT brain and MRI brain Serum FSH and LH

8. Premature thelarche - isolated breast development B one age not advanced Nipple devt . Absent Premature puberche -isolated devt.of axillary and or pubic hair prior to 8yrs Estimation of serum 17 alpha hydroxyprogesterone,DHEA -S and serum testosterone –in suspected case of adrenal hyperplasia Premature menarche -rule out other cause of vaginal bleeding like foreign body or trauma If bleeding is cyclic then diagnosis is confirmed

9. Treatment Goals - reduce gonadotrophin secretion decrease growth rate to normal and slowing skeletal maturation DRUGS - GnRH agonist Medroxy progesterone acetate Cyproterone acetate Danazol

10. GnRH agonist-arrest pre pubertal precocity and growth velocity Supress premature HPO axis activation and thus diminish estrogen secretion GnRH agonist supresses FSH,LH secretion,reverses ovarian cycle,establishes amenorrhea,causes regression of breast,pubic hair change and other sexual characters Buserelin nasal spray 100 micro gram/day Leuprolide depot once a month

11. Medroxy progesterone acetate-30mg/day oral or 100-200mg IM weekly Danazol Duration of treatment- upto 11 yrs usually

12. Delayed puberty Pubert y is said to be delayed when the breast tissue and or pubic hair have not appeared by 13-14 years or menarche appears as late as 16 years

13. Causes Hypergonadotrophic hypogonadism Gonadal agenesis,45XO Pure gonadal dysgenesis 46XX,46XY Ovarian failure 46XX Hypogonadotrophic hypogonadism Constitutional delay Chronic illness Primary hypothyroidism Kallmann syndrome Intracranial lesion Eugonadism Anatomical- mullerian agenesis imperforate hymen Androgen insensitivity syndrome

14. Treatment Hypogonadism Cyclic estrogen Unopposed estrogen 0.3mg/day for first 6 months Then combined estrogen and progestin,sequential regimen is started Hypergonadism Chromosomal study