PRECOCIOUS AND DELAYED PUBERTY

Question 1

What is the definition of precocious puberty in females?

Answer 1

Sexual maturation takes place before the age of 8.

9 in males

Question 2

What does the term thenarche mean?

Answer 2

First stage of breast development

Question 3

What does the term adrenarche mean?

Answer 3

First stage of pubic hair development

Question 4

What is the most common cause of precocious puberty in females?

Answer 4

Idiopathic in 74% of cases

Question 5

What are the four organs/systems that may be affected where there is a non-idiopathic cause of precocious puberty?

Answer 5

CNS
Thyroid
Adrenals
Ovary

Question 6

What are the CNS causes of precocious puberty?

Answer 6

Infection - meningitis, encephalitis, abscess

Tumours - gliomas, neurofibromas, ependymoma, hamartoma

Head trauma

Hydrocephaly

Question 7

Does hyper or hypothyroidism cause precocious puberty?

Answer 7

Hypothyroidism

Question 8

What are the adrenal causes of precocious puberty?

Answer 8

Congenital adrenal hyperplasia

Adrenal tumours

Question 9

What are the ovary related causes of precocious puberty?

Answer 9

Oestrogen secreting tumours especially granulose cell tumours

Premature oestrogen secretion eg McCune-Albright syndrome

Question 10

What is McCune-Albright syndrome?

Answer 10

A complex genetic disorder affecting the bone, skin, and endocrine systems that is associated with hyperfunctioning endocrine disease, fibrous dysplasia and café au lait macules.

Question 11

What are the management options for someone with precocious puberty?

Answer 11

Endocrinologist referral - suppression of oestrogen and androgen production by GnRH analogues.

Psychological support

Surgical removal of tumour

Question 12

What is the definition of delayed puberty in females?

Answer 12

No signs of secondary sexual development by the age of 13.

Question 13

What is the most common cause of delayed puberty in females?

Answer 13

Gonadal failure

Question 14

What are the categories of causes of delayed puberty?

Answer 14

Idiopathic
CNS
Thyroid
Adrenal
Ovary
Genetic
Chronic illness

Question 15

What are the CNS causes of delayed puberty?

Answer 15

Infection - meningitis, encephalitis, abscess

Tumours - pituitary, destructive

Head trauma

Question 16

Does hyper or hypothyroidism cause delayed puberty?

Answer 16

Hypothyroidism

Question 17

What are the adrenal causes of delayed puberty?

Answer 17

Congenital adrenal hyperplasia

Question 18

What are the ovary related causes of delayed puberty?

Answer 18

Gonadal failure
PCOS
Resistant ovary syndrome

Question 19

What are the genetic diseases associated with delayed puberty?

Answer 19

Turner syndrome
Prader-Willi syndrome
Laurence-Moon-Biedl syndrome
Androgen insensitivity syndrome
Gonadal dysgenesis
Kalmann's syndrome

Question 20

What chronic illnesses can lead to delayed puberty?

Answer 20

Anorexia nervosa
Diabetes mellitus
Renal disease
Cystic fibrosis

Question 21

What investigations would you do in someone with delayed puberty?

Answer 21

Full endocrine profile - LH, FSH, Prolactin, TFT, 17-OH progesterone
Chromosomal analysis
Bone age studies
Imaging - CT or MRI head