Precocious puberty

Precocious puberty


DEFINITION:

  • Precocious puberty defined as the appearance of any of the secondary sexual characteristics before the age of 8 years or the occurrence of menarche before the age of 10 years
  • It is heterosexual
  • 21 alpha hydroxylase deficiency & 11β hydroxylase deficiency  can cause precocious puberty in males 

TYPES:

  • True, Complete or Isosexual precocious puberty:
  • Results from the premature activation of the endocrine pathway comprising the hypothalamic– pituitary–ovarian axis.
  • Total growth spurt and potential increase in height is not achieved

Pseudo or incomplete:

  • Result of sex steroid stimulation independent of the above axis.

ETIOLOGICAL CLASSIFICATION:

TYPES FEATURES

Complete precocious
puberty

  • Idiopathic, familial or sporadic, genetic (75%)
  • Congenital lesions of the hypothalamus–pituitary Acquired lesions—trauma, infection, neoplasm— tuberculosis (TB) meningitis in childhood
  • Part of a specific syndrome—McCune- Albright (5%), von Recklinghausen’s neurofibrobromatosis
  •  Other causes—endocrine/ metabolic disorders

Incomplete
precocious
puberty:

  • Premature thelarche
  • Premature adrenarche
  • Premature menarche

Pseudoprecocious
puberty: (GnRH
independent)

  • Feminizing ovarian tumours (10%) (hormone secreting)
  • Adrenal hyperplasia/neoplasm—20%
  •  Hypothyroidism
  •  Hepatoblastoma producing gonadotropins
  •  Iatrogenic–oestrogen administration

CLINICAL FEATURES:

  • Early growth spurt
  • Early fusion of epiphysis of long bones
  • Sexual maturity attained early
  • Difficult social and emotional situations.

Associated condition: 

  • McCune-Albright syndrome:
  • Multiple cystic bone lesions
  • Café-au-lait spots
  • Early Menstruation
  • Events of thelarche and adrenarche preceding menarche
  • Fertility remains unimpaired and the adult height attained
  • Granulosa cell tumour

INVESTIGATION:

  • Radiograph of the wrist to establish bone age
  • Thyroid function tests—T3, T4, and TSH. TSH stimulates FSH receptors.
  • EEG and CAT/MRI scan of the skull.
  • Adrenal function tests to exclude heterosexual precocity.
  • Pelvic sonography to exclude pelvic neoplasms.
  • FSH, LH, oestrogen levels.
  • GnRH test to exclude autonomous ovarian cysts from those secondary to gonadotropin stimulation.
  • GnRH test—IV 20 mcg/kg GnRH—estimate LH level 30 min later; level . 9.2 IU/L indicates true precocious puberty (GnRH related).

TREATMENT:

  • Proper treatment should be instituted for hypothyroidism, adrenal hyperplasia and surgical intervention for tumours of the ovary, adrenals or of neurological origin.

DRUGS:

  • Inj. depot medroxyprogesterone acetate (DMPA): To induce regression of these changes and cessation of menstruation
  • Cyproterone acetate exerts antiandrogenic and antigonadotropin effects.
  • GnRH agonists (Buserelin) form the mainstay of treatment in present day practice.

The monthly administration of depot preparations:

  • Buserline 100 mcg nasal spray daily.
  • Leuprotide 7.5 mg monthly. A single implant of histrelin—effect lasts for 1 year.
  •  Triptorelin 11.25 mg 3 monthly for 1 year with calcium and vitamin D to prevent osteoporosis 20 mcg.
Exam Question
 
  • Precocious puberty may be seen in  Granulosa cell tumour, Head injury & Corticosteroid intake
  • The sexual development is considered is precocious if there is breast and public hair growth before the age of 8 years
  • GnRH analogue may be given in Prostate Ca, Fibromyoma – uterus & Precocious puberty
  • Precocious puberty is seen with  Granulosa cell tumour
  • Sexual maturity is attained early in precocious puberty
  • Menstruation is defined as precocious if it starts before the child reaches the age of 10 years
  •  Precocious puberty can be heterosexual
  • 21 alpha hydroxylase deficiency males present with precocious puberty
  • A male child presenting with early onset puberty and hypertension is suggestive of congenital adrenal hyperplasia due to the deficiency of 11-β hydroxylase deficiency.
  • Sexual maturity attained early in  precocious puberty
  • Familial male limited precocious puberty  is also called Testotoxicosis
  • Familial male limited precocious puberty  is Autosomal dominant condition
  • Flutamide can be used for treatment of Familial male limited precocious puberty
  • 21 alpha hydroxylase deficiency & 11β hydroxylase deficiency  can cause precocious puberty in males 
  • Precocious puberty is seen in  Hypothyroidism, CNS irridation & Mc cune-Albright syndrome
  •  Congenital 21 hydroxylase deficiency causes precocious puberty in male due to excess of androgens.
  • The diagnosis of a patient presenting with familial Polyostosis, Precocious puberty and Pigmentation is McCune Albright syndrome
Don’t Forget to Solve all the previous Year Question asked on Precocious puberty

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