Precocious puberty

Precocious puberty

Q. 1

Precocious puberty may be seen in all of the following conditions except :

 A

Granulosa – cell tumour

 B

Head – injury

 C

Corticosteroid intake

 D

Hyperthyroidism

Q. 1

Precocious puberty may be seen in all of the following conditions except :

 A

Granulosa – cell tumour

 B

Head – injury

 C

Corticosteroid intake

 D

Hyperthyroidism

Ans. D

Explanation:

Hyperthyroidism

2.  Puberty is the state of becoming functionally capable of procreation.

Age group : • Males 14 years°

  • Females– 12 years°

In females :

  • The definate signs of puberty are present by the age of 9 or 10 years when breasts begin to bud.°
  • The first menstrual period usually occurs between the age of 10 – 16 years. (Average age in India = 13.5 years).°
  • Precocious puberty is defined as puberty before the age of 8 years° in female or menarche before the age of 10 years°
  • In males puberty before the aqe of 9 years is precocious.°

Central (True) precocious puberty°Peripheral (Pseudo)rep puberty°

So, from the exhaustive list we can see hypothyroidism and not hyperthyroidism causes precocious puberty. Reason :

If precocious puberty is associated with delayed bone age it suggests primary hypothyroidism.°



Q. 2

The sexual development is considered is precocious if there is breast and public hair growth before the age of :

 A

8 years

 B

9 years

 C

10 years

 D

11 years

Q. 2

The sexual development is considered is precocious if there is breast and public hair growth before the age of :

 A

8 years

 B

9 years

 C

10 years

 D

11 years

Ans. A

Explanation:

8 years


Q. 3

GnRH analogue may be given in all of the following except

 A

Prostate Ca

 B

Endometrium Ca

 C

Fibromyoma – uterus

 D

Precocious puberty

Q. 3

GnRH analogue may be given in all of the following except

 A

Prostate Ca

 B

Endometrium Ca

 C

Fibromyoma – uterus

 D

Precocious puberty

Ans. B

Explanation:

Ans. is b i.e Endometrial Ca

GnRH agonist :

Mechanism of action : GnRH agonist bind to GnRH receptors in the pituitary, and cause competitive inhibition of endogenous GnRH. Initially there is a supraphysiological release of FSH and LH from pituitary (Flare) followed by decrease in the synthesis and release of both FSH and LH.

Preparations of GnRH :               • Buserelin                               • Leuprorelin

  • Nafarelin                         • Gosserelin
  • Triptorelin                       • Historelin

Method of administration : Subcutaneous/intramuscular injections or as nasal sprays.

Indications :

In females :          (In all those conditions where there is increased estrogen, GnRH analogues are useful). Mnemonic : A – F and HIP.

A = Adenomyosis

B = Irritable Bowel Syndrome (under Trial)

C = Ca Breast (Tamoxifen + GnRH agonist give good result)

D = DUB

E = Endometriosis

F = Fibromyoma uterus.

H = Hirsutism

I    = Infertility

P = Precocious Puberty.

In males :

  • Cryptorchidism : short term intranasal nafarelin is used.
  • Carcinoma prostrate : GnRH agonist are as effective as orchidectomy in Ca prostrate but if there are neurological symptoms or life threatening metastatic disease – androgens should first be started followed by GnRH agonist.

BPH : Daily administration of leuprorelin or nafarelin decreases obstructive urinary symptoms in 1-6 months time.

Adverse effects of GnRH :

  • Local allergic reaction may occur at injection site.
  • They cause menopause like Symptoms — Hot flushes, vaginal dryness, headache, joint and muscle stiffness, depression, osteoporosis and irregular vaginal bleeding.

GnRH antagonist : Acts immediately to stop Gonadotropin secretion (without any flare) eg : Cetrirelix, Ganirelix, Nal-glu.

They have been superceeded by GnRH agonist in clinical practice except for their potential role in Contraception. Nal-glu given for 3 weeks inhibits spermatogenesis



Q. 4

Precocious puberty is seen with which ovarian tumour :

 A

Dermoid

 B

Gynandroblastoma

 C

Granulosa cell tumour

 D

Arrhenoblastoma

Q. 4

Precocious puberty is seen with which ovarian tumour :

 A

Dermoid

 B

Gynandroblastoma

 C

Granulosa cell tumour

 D

Arrhenoblastoma

Ans. C

Explanation:

Granulosa cell tumour


Q. 5

Which of the following is true regarding precocious puberty :

 A

Sexual maturity is attained early

 B

Mental function is increased

 C

No reproductive function

 D

Body proportions are enlarged

Q. 5

Which of the following is true regarding precocious puberty :

 A

Sexual maturity is attained early

 B

Mental function is increased

 C

No reproductive function

 D

Body proportions are enlarged

Ans. A

Explanation:

Ans. is a i.e. Sexual maturity is attained early

Precocious puberty is the appearance of appropriate secondary sexual characters before the age of 8 years and occurrence of menstruation before 10 years of chronological age.

Also know : Delayed puberty : is considered delayed when the secondary sexual characters do not appear by the age of 14, and menarche is not established by 16 years of age.


Q. 6

Menstruation is defined as precocious if it starts before the child reaches the age of :

 A

8 years

 B

10 years

 C

14 years

 D

20 years

Q. 6

Menstruation is defined as precocious if it starts before the child reaches the age of :

 A

8 years

 B

10 years

 C

14 years

 D

20 years

Ans. B

Explanation:

Ans. is b i.e. 10 years


Q. 7

An 8 year old girl is brought to the outpatient department for having early periods. Assertion: Precocious puberty can be heterosexual. Reason: It is due to excess production of progesterone.

 A

Both Assertion and Reason are true, and Reason is the correct explanation for Assertion

 B

Both Assertion and Reason are true, and Reason is not the correct explanation for Assertion

 C

Assertion is true, but Reason is false

 D

Assertion is false, but Reason is true

Q. 7

An 8 year old girl is brought to the outpatient department for having early periods. Assertion: Precocious puberty can be heterosexual. Reason: It is due to excess production of progesterone.

 A

Both Assertion and Reason are true, and Reason is the correct explanation for Assertion

 B

Both Assertion and Reason are true, and Reason is not the correct explanation for Assertion

 C

Assertion is true, but Reason is false

 D

Assertion is false, but Reason is true

Ans. C

Explanation:

Precocious puberty is heterosexual due to excess production of androgen, whereas isosexual due to excess production of estrogen. Ref: Textbook of Gynecology by D.C.Dutta, 4th Edition, Page 48.


Q. 8

Which of the following statements about 21 alpha hydroxylase deficiency is false?

 A

Most common cause of Congenital Adrenal Hyperplasia (CAH) in children

 B

Affected females present with ambiguous genitalia

 C

Affected males present with precocious puberty

 D

Hypokalemic alkalosis is seen

Q. 8

Which of the following statements about 21 alpha hydroxylase deficiency is false?

 A

Most common cause of Congenital Adrenal Hyperplasia (CAH) in children

 B

Affected females present with ambiguous genitalia

 C

Affected males present with precocious puberty

 D

Hypokalemic alkalosis is seen

Ans. D

Explanation:

Hyperkalemia is seen in this case.Deficiency of 21 hydroxylase results in manifestations of aldosterone deficiency (salt wasting), loss of sodium (hyponatremia), increase in potassium (hyperkalemia).

Ref: OP Ghai 6th Edition, Page 490;Harrison 17th Edition, Page 2267; Nelson 18th Edition, Page 2360- 2365


Q. 9

A five year old boy presents with Precocious Puberty and a Blood Pressure of 130/80 mm Hg. Estimation of which of the following will help in diagnosis:

 A

↑17 Hydroxy progesterone

 B

↑ Cortisol

 C

↑ Deoxycortisol

 D

↑ Aldosterone

Q. 9

A five year old boy presents with Precocious Puberty and a Blood Pressure of 130/80 mm Hg. Estimation of which of the following will help in diagnosis:

 A

↑17 Hydroxy progesterone

 B

↑ Cortisol

 C

↑ Deoxycortisol

 D

↑ Aldosterone

Ans. C

Explanation:

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.

Ref: Essential Pediatrics By O P Ghai, 6th Edition, Page 490; Nelson’s Textbook of Pediatrics, 18th Edition, Page 2362


Q. 10

Which of the following statement regarding precocious puberty is true?

 A

Mental retardation is present

 B

Absent reproductive dysfunction

 C

Sexual maturity attained early

 D

Enlarged body proportions

Q. 10

Which of the following statement regarding precocious puberty is true?

 A

Mental retardation is present

 B

Absent reproductive dysfunction

 C

Sexual maturity attained early

 D

Enlarged body proportions

Ans. C

Explanation:

Precocious puberty refers to the onset of secondary sexual characteristics below the age limit set for normal onset of puberty, i.e onset before the age of 8 years in girls and 9 years in boys.


Q. 11

A neurosurgeon saw a child with precocious puberty and uncontrollable laughing. He suggested the parent of child to get a MRI done to evaluate for the possibility of?

 A

Craniopharyngioma

 B

Hypothalamic Hamartoma

 C

Pheochromocytoma

 D

Pituitary adenoma

Q. 11

A neurosurgeon saw a child with precocious puberty and uncontrollable laughing. He suggested the parent of child to get a MRI done to evaluate for the possibility of?

 A

Craniopharyngioma

 B

Hypothalamic Hamartoma

 C

Pheochromocytoma

 D

Pituitary adenoma

Ans. B

Explanation:

Hypothalamic hamartomas and gangliocytoma may arise from astrocytes, oligodendrocytes, and neurons with varying degrees of differentiation. These tumors may overexpress hypothalamic neuropeptides, including GnRH, GHRH, and CRH.

 With GnRH-producing tumors, children present with precocious puberty, psychomotor delay, and laughing-associated seizures. 

Medical treatment of GnRH-producing hamartomas with long-acting GnRH analogues effectively suppresses gonadotropin secretion and controls premature pubertal development. 

Ref:Melmed S., Jameson J.L. (2012). Chapter 339. Disorders of the Anterior Pituitary and Hypothalamus. In D.L. Longo, A.S. Fauci, D.L. Kasper, S.L. Hauser, J.L. Jameson, J. Loscalzo (Eds), Harrison’s Principles of Internal Medicine, 18e.


Q. 12

All are true about familial male limited precocious puberty, EXCEPT:

 A

It is also called Testotoxicosis

 B

Autosomal dominant condition

 C

Testosterone and LH levels are elevated

 D

Flutamide can be used for treatment

Q. 12

All are true about familial male limited precocious puberty, EXCEPT:

 A

It is also called Testotoxicosis

 B

Autosomal dominant condition

 C

Testosterone and LH levels are elevated

 D

Flutamide can be used for treatment

Ans. C

Explanation:

Familial male limited precocious puberty is also called testotoxicosis.

FACTS :

  • It is an autosomal dominant disorder
  • It presents as premature androgenization of boys 
  • Testosterone is elevated, LH is suppressed in the condition

Treatment options :

1. Ketoconazole – Inhibits testosterone synthesis.

2. Flutamide – Used as an androgen receptor antagonist.

3. Anastrazole – Is a useful aromatase inhibitor.

Ref: Harrison, Edition – 18, Page – 3015


Q. 13

All of the following can cause precocious puberty in males except –

 A

17 alpha hydroxylase deficiency

 B

21 alpha hydroxylase deficiency

 C

11β hydroxylase deficiency

 D

None of the above

Q. 13

All of the following can cause precocious puberty in males except –

 A

17 alpha hydroxylase deficiency

 B

21 alpha hydroxylase deficiency

 C

11β hydroxylase deficiency

 D

None of the above

Ans. A

Explanation:

Ans. is ‘a’ i.e., 17 a hydroxylase


Q. 14

Precocious puberty is seen in –

 A

Hypothyroidism

 B

CNS irridation

 C

Mc cune-Albright syndrome

 D

All

Q. 14

Precocious puberty is seen in –

 A

Hypothyroidism

 B

CNS irridation

 C

Mc cune-Albright syndrome

 D

All

Ans. D

Explanation:

Ans. is ‘a’ i.e., Hypothyroidism; ‘b’ i.e., CNS irradiation; ‘c’ i.e., Mc Cune Albright syndrome

Precocious puberty

o Puberty before the age of 8 years in girls or 9 years in boys is considered precocious puberty. o Menarche before the age of 10 years in girls is also considered as precocious.

o Precocious puberty is of two types

1. Central or true precocious puberty

           Results from excessive GnRH, gonadotropins and target sex hormone elaborated by premature activation of hypothlamic pituitary-gonadal (HPG) axis.

2. Peripheral or pseudo-precocious puberty

            Due to increased sex steroid secretion from either the adrenal gland or the gonads.

              It is independent of HPG axis activation

Causes of Precocious puberty

A. Central precocious puberty

1.  Idiopathic : Sporadic or familial.

2.  Central nervous system abnormalities

i)    Congenital anomalies of CNS: Hypothalamic hamartoma, hydrocephalus, porencephaly, arachnoid cysts.

ii)     Acquired lesions of CNS : Inflammation, granuloma, trauma, surgery, radiation, chemotherapy.

iii)      Tumors of CNS : Pineal tumors, optic glioma, ependymoma, camiopharyngioma.

iv)   Hypothyroidism

B. Peripheral precocious puberty : Isosexual

Girls

1. Ovarian causes : McCune-Albright syndrome, benign follicular cysts, granulosa-theca cell tumors; Gonadoblastoma

2. Adrenal causes : Feminizing adrenal neoplasia

3. Exogenous estrogen administration

Boys

1. Testis : Leydig cell tumor, adrenal rest tumor, testotoxicosis.

2. Adrenal: CAH (21 or 11-(3 hydroxylase deficiency), virilizing tumors.

3. hCG secreting tumors : Hepatoma, hepatoblastoma, choriocarcinoma, chorionepithelioma, teratoma, dysgerminoma.

Exogenous testosterone

C. Heterosexual precocity

1.   Girls : Virilization in girls due to virilizing CAH, ovarian or adrenal neoplasia, polycystic ovarian disease.

2.   Boys : Feminization due to estogen producing adrenal tumors, exogenous estrogen, marijuana smoking.

Note – Hypothyroidism usually causes delayed puberty, but juvenile hypothyroidism some times can cause precocious puberty.


Q. 15

All of following may be causes of precocious puberty in girls except –

 A

Hypothalamic hamartoma

 B

Mc Cuone Albright syndrome

 C

Granulosa cell tumor of human ovary

 D

Congential 21-a hydroxylase deficiency

Q. 15

All of following may be causes of precocious puberty in girls except –

 A

Hypothalamic hamartoma

 B

Mc Cuone Albright syndrome

 C

Granulosa cell tumor of human ovary

 D

Congential 21-a hydroxylase deficiency

Ans. D

Explanation:

Ans. is ‘d’ i.e., Congenital 21-hydroxylase

o Congenital 21 hydroxylase deficiency causes precocious puberty in male due to excess of androgens.

o In female, it results in virilization.


Q. 16

The diagnosis of a patient presenting with familial Polyostosis, Precocious puberty and Pigmentation is:

 A

Tuberous sclerosis

 B

McCune Albright syndrome

 C

Klinefelter syndrome

 D

SLE

Q. 16

The diagnosis of a patient presenting with familial Polyostosis, Precocious puberty and Pigmentation is:

 A

Tuberous sclerosis

 B

McCune Albright syndrome

 C

Klinefelter syndrome

 D

SLE

Ans. B

Explanation:

Answer is B (McCune Albright syndrome);

McCune Albright syndrome’

  • Polyostotic form of fibrous dysplasia
  • Cutaneous pigmentation
  • Endocrine dysfunction, especially Precocious puberty in girls.

The triad of symptoms in the question thus characterizes McCune Albright syndrome and hence is the answer.



Leave a Reply

%d bloggers like this:
Malcare WordPress Security