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 |
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Complete precocious puberty |
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Incomplete precocious puberty: |
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Pseudoprecocious puberty: (GnRH independent) |
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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 Important
- 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
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