Pubertal Disorders - Precocious Puberty

Question 1

What is Precocious Puberty?

Answer 1

Development of secondary sexual characteristics before 8 in girls and 9 in boys (commoner in girls).

Question 2

What are the types of Precocious Puberty? (2).

Answer 2

1. Gonadotrophin Dependent (Central/True).

2. Gonadotrophin Independent (Pseudo/False).

Question 3

Aetiology of Gonadotrophin-Dependent PP (2).

Answer 3

Premature Activation of HPG Axis (High LH, FSH) :

1. Idiopathic.
2. Cranial Causes e.g. Radiotherapy, Disability, Injury, Neoplasms.

Question 4

Aetiology of Gonadotrophin-Independent PP.

Answer 4

Excess sex hormones (Low LH, FSH) :

1. Ovarian Causes : Follicular Cyst, Tumours.
2. Testicular Causes : Tumours, LH Receptor Defect.
3. Adrenal Causes : CAH.
4. McCune Albright Syndrome.

Question 5

What is McCune Albright Syndrome?

Answer 5

Random somatic mutation in GNAS gene.

Question 6

Clinical Features of McCune Albright Syndrome (4).

Answer 6

1. Precocious Puberty.
2. Cafe-au-Lait Spots.
3. Polyostotic Fibrous Dysplasia.
4. Short Stature.