1 - Precocious puberty . 2 - Delayed puberty .
-Definition: Secondary Sexual Characters do not develop by the age of 14
year or no menstruation till age of 16year
It is either :
-It means menarche or appearance of any of the secondary sexual charactersbefore the
definition age of 8 years
1) Delayed onset: Breast bud does not appear till 13 years or
menarche does notoccur till 16 years
2)Delayed progression : Menarche doesnot occur within 5 years after breast
bud
Adolescence
🔸
•Is the period of life during which the childbecomes an adult personi.e.
1 - True precocious puberty .True (central ,cerebral) It is due to increased production the physical , sexual and psychologicaldevelopment are complete .
of pituitary gonadotrophins
• Puberty represents the first part of adolescence .
🔸 2 - False (pseudo-precocious puberty)
-It is of peripheral origin.
-It is due to secretion of sex hormones; (estrogen or androgen) which is not dependent on
pituitary gonadotrophins as in case of estrogenic or androgenic ovarian tumors. PUBERTY:
-False precocious puberty may be isosexual or heterosexual.
It is a physiological phase lasting 2 to 5 years, during which the genital
🔸
-A girl who feminizes early is defined as having isosexual precocious puberty.
-A girl who virilize early is defined as having heterosexual precocious puberty.(female --------------- organs mature The first sign of pubertal development is usually breast growth
Types pseudohermaphrodite) (thelarche),followed by appearance of pubic hair (pubarche), then
🔸 3 - Incomplete precocious puberty .
-In this case only one pubertal change as breast development is present before the age of 8
Manifestations of puberty in the female include
1. Menarche,
🔸
(axillary hair), then (menarche).
The mean interval between breast budding and menarche is 2.5 years
with a standard deviation of about oneyear
years without the presence of any other pubertal changes and in absence of increased
🔸
2.Appearance of secondary sex characters,
estrogen production. Adrenarche:
-The other pubertal changes occur at the normal age. 3. Physical development
means increased activity of the suprarenal cortex at puberty with
-Incomplete forms of precocious puberty include premature thelarche (unilateral or 4. Psychological development
increased production of adrenal androgens which lead to appearance of
bilateral), premature pubarche and premature adrenarche with appearance of pubic and pubic and axillary hair
axillary hair
🔸
Secondary sex characters: include
🔸
development of the breast
1.Constitutional or idiopathic: appearance of pubicand axillary hair.
-In most cases of precocious puberty (90%) , no cause is found.
-For some unknown reason the hypothalamus stimulates the pituitary gland to secrete its
gonadotrophic hormones.
Adolescence and
🔸
-There is normal menstruation and ovulation. Cause of puberty
-Pregnancy can occur at young age.
During childhood
2. Organic lesions of the brain: puberty the hypothalamus is extremely sensitive to the negative feed back
-The next common cause. exerted by the small quantities of estradiol & testosterone produced by
-Organic lesions affecting the midbrain, hypothalamus, pineal body, or pituitary gland may
🔸
lead to premature release of pituitary gonadotrophins. 1 -Constitutional with +ve family history , short stature &normal fertility . the child's ovaries .
-Examples include traumatic braininjury, meningitis, encephalitis, brain abscess, brain tumor 2 –Hypergonadotropic hypogonadism(FSH > 40) = ovarian causes of As puberty approaches
as glioma, craniopharyngioma, and hamartomas. primary amenorrhea = primary ovarian failure &secondary ovarian failure (if
occurs before puberty).
-the sensitivity of the hypothalamus is decreased and subsequently , it
3. Juvenile hypothyroidism: 3 –Hypogonadtropic hypogonadism = hypothalamic & pituitary causes of increase the pulsatile GnRH secretion .
Lack of thyroxine leads to increased production of thyroid stimulating hormone and the primary amenorrhea e.g. Kallman'ssyndrome , Anorexia nervosa . -The anterior pituitary responds by progressive secretion of FSH and LH
etiology secretion of pituitary gonadotrophins may also be increased. 4 –Normogonadtropic hypogonadism = end organ defects = uterine causes
associated with increased secretion of growth hormone .
(Mullerian agenesis and testicular feminization syndrome), imperforate hymen
(c/o =delayed menarche + normal other aspects of puberty), PCOD and -The ovaries respond to the increase Gonadotrophin secretion by
4. Ovarian causes :
(a) Estrogen producing tumors as granulosa and theca cell tumor; Virilizing ovarian adrenal tumors . follicular development & estrogensecretion .
5 - General causes of amenorrhea (endocrinal or non-endocrinal especially
Abnormalities -Estrogen causes development of the genital organs and the
(b) Androgen producing tumors as androblastoma;
(c) Choriocarcinoma because it secretes human chorionic malnutrition) if occurred before puberty &↓GH & steroid synthesis defects .
appearance of the secondary sexual characters .
gonadotrophin (HCG) which may stimulate the ovaries to secrete estrogen;
of puberty (d) Dysgerminoma if it secretes HCG. - With increased estrogen secretion, menarche and cyclic estrogen
(e) McCune-Albright syndrome. secretion occurs .
5. Adrenal causes:
(a) Hyperplasia, adenoma, or carcinomaof suprarenal cortex. Congenital adrenal
hyperplasia and Cushing syndrome lead to precocious puberty in the male direction, i.e.
heterosexual precocious puberty;
(b) Estrogen secreting adrenal tumor which is very rare 🔸
Genital organs changes
🔸
Mons pubes, labia majora & minora: increase in size.
Vagina:
1. length: increase, appearance of the rugae
1. History: 2. Epithelium: thick, stratified squamous., containing glycogen
-It excludes iatrogenic source of estrogen or androgen. History :
🔸
-It differentiates between isosexual and heterosexual precocious puberty 1-Family history , nutritional history , any systemic diseases (e.g. history of 3. pH: acidic.
🔸
endocrinal disturbance). Uterus: enlarge, Uterus / Cervix :
2. Physical examination: 2 - Clinical picture of space occupying lesion in the ovary , adrenal, pituitary & Ovaries:1.Increase in size, almond shape2.300 thousands primary
-It diagnoses McCune-Albrightsyndrome. hypothalamus.
-Neurologic and ophthalmologicexaminations exclude organic lesions of the brain. 3 - Periodic pain and +ve 2ry sexual characteristics in imperforate hymen
follicle at menarche ( 2 million at birth)
3. Special investigations:
🔸
These are done according to the historyand clinical findings and include:
a. Hormonal assay: including serum FSH, LH, prolactin, estradiol, testosterone, 17α
hydroxy progesterone, TSH, and human chorionic gonadotrophin to diagnose
Examination
(A) Body measurement for causes of amenorrhea + ↑or ↓weight, short or tall
stature , proportions (upper / lower segment ratio & arm span / height ratio).
diagnosis
🔸
Choriocarcinoma.
b. X-ray examination of the hand and wrist to determine bone age.
-Estrogen stimulates growth of bone but causes early fusion of the epiphysis.
(B) Tanner staging of breast, pubic & axillary hair if present.
(C) Clinical picture of Turner ,Mullerian agenesis & imperforate hymen . McCune-Albright Syndrome
(D) Neurological examination for smell sense(Kallman's syndrome), visual field The disease is found more frequently in girls.
🔸
-So the child is taller than her peers during childhood, but she is short during adult life
🔸 c. Ultrasonography to diagnose ovarian or adrenal tumor.
d. CT or MRI : to diagnose an organic lesion of the brain, or adrenal tumor.
& other cranial nerve lesions .
-It consists of a triad of :
1. Precocious puberty
Investigations
-Hypothyroidism retards bone age, and is the only condition of precocious puberty in which 1 - FSH & LH assay important to differentiate level of the lesion & 2. Cystic changes in bones
bone age is retarded progesterone assay in 17 OH deficiency . 3. Cafe-au lait patches of the skin.
2 - Chromosomal study if short stature or hypergonadotropic type . -The cause of precocious puberty is autonomous production of estrogen
Idiopathic precocious puberty 3 - Radiological bone age study & radiologic study for pituitary adenoma by the ovaries.
is diagnosed after excluding all other causes.
-FSH and LH levels are low.
-The treatment is testolactone oral tabletswhich inhibit ovarian
steroidogenesis.
Treatment The drug inhibits the formation of estrogen from its precursors, so reduces
1. Arrest maturation until normal pubertal age.
🔸
estrogen level.
2. Attenuate & diminish established precocious characteristics. The dose is 20 mg/kg body weight in 4 divided doses and increased to
3. Maximize adult height.
40 mg/kgbody weight during a 3 week interval.
4. Avoid abuse, reduce emotional & social problems
🔸 1. Treatment of the cause, e.g., thyroxin for hypothyroidism, removal of ovarian and
🔸
adrenal tumors.
2. Incomplete forms of precocious puberty do not require treatment, as estrogen
production is not increased.
Treatment
*Constitutional : Reassurance .
*Treatment of the cause (if treatable) or cyclic estrogen-progesterone
treatment Idiopathic type .4
-is treated by explanation and reassuranceand by giving one of the following drugs which
hormone replacement therapy if the cause is not treatable , for 3 cycles:
Norethistrone acetate 5 mg twice daily for 21 d or OCP
inhibit the secretion of gonadotrophins: * Patient with Y chromosome cell line :Gonadectomy + hormone replacement
(a)Gonadotrophin releasing hormone analogues which are given as daily nasal spray, therapy
intramuscular, or subcutaneous injections every 4 weeks.
(b)Medroxyprogesterone acetate tablets(Provera tablets) or intramuscular injection
(Depo-Provera);
(c) Danazol capsules;
🔸
(d)Cyproterone acetate tablets (Androcur).
idiopathic type Treatment is given till the age of 12 years (mean age of pubertal
development)
by fatema okoff
-Definition: Secondary Sexual Characters do not develop by the age of 14
year or no menstruation till age of 16year
It is either :
-It means menarche or appearance of any of the secondary sexual charactersbefore the
definition age of 8 years
1) Delayed onset: Breast bud does not appear till 13 years or
menarche does notoccur till 16 years
2)Delayed progression : Menarche doesnot occur within 5 years after breast
bud
Adolescence
🔸
•Is the period of life during which the childbecomes an adult personi.e.
1 - True precocious puberty .True (central ,cerebral) It is due to increased production the physical , sexual and psychologicaldevelopment are complete .
of pituitary gonadotrophins
• Puberty represents the first part of adolescence .
🔸 2 - False (pseudo-precocious puberty)
-It is of peripheral origin.
-It is due to secretion of sex hormones; (estrogen or androgen) which is not dependent on
pituitary gonadotrophins as in case of estrogenic or androgenic ovarian tumors. PUBERTY:
-False precocious puberty may be isosexual or heterosexual.
It is a physiological phase lasting 2 to 5 years, during which the genital
🔸
-A girl who feminizes early is defined as having isosexual precocious puberty.
-A girl who virilize early is defined as having heterosexual precocious puberty.(female --------------- organs mature The first sign of pubertal development is usually breast growth
Types pseudohermaphrodite) (thelarche),followed by appearance of pubic hair (pubarche), then
🔸 3 - Incomplete precocious puberty .
-In this case only one pubertal change as breast development is present before the age of 8
Manifestations of puberty in the female include
1. Menarche,
🔸
(axillary hair), then (menarche).
The mean interval between breast budding and menarche is 2.5 years
with a standard deviation of about oneyear
years without the presence of any other pubertal changes and in absence of increased
🔸
2.Appearance of secondary sex characters,
estrogen production. Adrenarche:
-The other pubertal changes occur at the normal age. 3. Physical development
means increased activity of the suprarenal cortex at puberty with
-Incomplete forms of precocious puberty include premature thelarche (unilateral or 4. Psychological development
increased production of adrenal androgens which lead to appearance of
bilateral), premature pubarche and premature adrenarche with appearance of pubic and pubic and axillary hair
axillary hair
🔸
Secondary sex characters: include
🔸
development of the breast
1.Constitutional or idiopathic: appearance of pubicand axillary hair.
-In most cases of precocious puberty (90%) , no cause is found.
-For some unknown reason the hypothalamus stimulates the pituitary gland to secrete its
gonadotrophic hormones.
Adolescence and
🔸
-There is normal menstruation and ovulation. Cause of puberty
-Pregnancy can occur at young age.
During childhood
2. Organic lesions of the brain: puberty the hypothalamus is extremely sensitive to the negative feed back
-The next common cause. exerted by the small quantities of estradiol & testosterone produced by
-Organic lesions affecting the midbrain, hypothalamus, pineal body, or pituitary gland may
🔸
lead to premature release of pituitary gonadotrophins. 1 -Constitutional with +ve family history , short stature &normal fertility . the child's ovaries .
-Examples include traumatic braininjury, meningitis, encephalitis, brain abscess, brain tumor 2 –Hypergonadotropic hypogonadism(FSH > 40) = ovarian causes of As puberty approaches
as glioma, craniopharyngioma, and hamartomas. primary amenorrhea = primary ovarian failure &secondary ovarian failure (if
occurs before puberty).
-the sensitivity of the hypothalamus is decreased and subsequently , it
3. Juvenile hypothyroidism: 3 –Hypogonadtropic hypogonadism = hypothalamic & pituitary causes of increase the pulsatile GnRH secretion .
Lack of thyroxine leads to increased production of thyroid stimulating hormone and the primary amenorrhea e.g. Kallman'ssyndrome , Anorexia nervosa . -The anterior pituitary responds by progressive secretion of FSH and LH
etiology secretion of pituitary gonadotrophins may also be increased. 4 –Normogonadtropic hypogonadism = end organ defects = uterine causes
associated with increased secretion of growth hormone .
(Mullerian agenesis and testicular feminization syndrome), imperforate hymen
(c/o =delayed menarche + normal other aspects of puberty), PCOD and -The ovaries respond to the increase Gonadotrophin secretion by
4. Ovarian causes :
(a) Estrogen producing tumors as granulosa and theca cell tumor; Virilizing ovarian adrenal tumors . follicular development & estrogensecretion .
5 - General causes of amenorrhea (endocrinal or non-endocrinal especially
Abnormalities -Estrogen causes development of the genital organs and the
(b) Androgen producing tumors as androblastoma;
(c) Choriocarcinoma because it secretes human chorionic malnutrition) if occurred before puberty &↓GH & steroid synthesis defects .
appearance of the secondary sexual characters .
gonadotrophin (HCG) which may stimulate the ovaries to secrete estrogen;
of puberty (d) Dysgerminoma if it secretes HCG. - With increased estrogen secretion, menarche and cyclic estrogen
(e) McCune-Albright syndrome. secretion occurs .
5. Adrenal causes:
(a) Hyperplasia, adenoma, or carcinomaof suprarenal cortex. Congenital adrenal
hyperplasia and Cushing syndrome lead to precocious puberty in the male direction, i.e.
heterosexual precocious puberty;
(b) Estrogen secreting adrenal tumor which is very rare 🔸
Genital organs changes
🔸
Mons pubes, labia majora & minora: increase in size.
Vagina:
1. length: increase, appearance of the rugae
1. History: 2. Epithelium: thick, stratified squamous., containing glycogen
-It excludes iatrogenic source of estrogen or androgen. History :
🔸
-It differentiates between isosexual and heterosexual precocious puberty 1-Family history , nutritional history , any systemic diseases (e.g. history of 3. pH: acidic.
🔸
endocrinal disturbance). Uterus: enlarge, Uterus / Cervix :
2. Physical examination: 2 - Clinical picture of space occupying lesion in the ovary , adrenal, pituitary & Ovaries:1.Increase in size, almond shape2.300 thousands primary
-It diagnoses McCune-Albrightsyndrome. hypothalamus.
-Neurologic and ophthalmologicexaminations exclude organic lesions of the brain. 3 - Periodic pain and +ve 2ry sexual characteristics in imperforate hymen
follicle at menarche ( 2 million at birth)
3. Special investigations:
🔸
These are done according to the historyand clinical findings and include:
a. Hormonal assay: including serum FSH, LH, prolactin, estradiol, testosterone, 17α
hydroxy progesterone, TSH, and human chorionic gonadotrophin to diagnose
Examination
(A) Body measurement for causes of amenorrhea + ↑or ↓weight, short or tall
stature , proportions (upper / lower segment ratio & arm span / height ratio).
diagnosis
🔸
Choriocarcinoma.
b. X-ray examination of the hand and wrist to determine bone age.
-Estrogen stimulates growth of bone but causes early fusion of the epiphysis.
(B) Tanner staging of breast, pubic & axillary hair if present.
(C) Clinical picture of Turner ,Mullerian agenesis & imperforate hymen . McCune-Albright Syndrome
(D) Neurological examination for smell sense(Kallman's syndrome), visual field The disease is found more frequently in girls.
🔸
-So the child is taller than her peers during childhood, but she is short during adult life
🔸 c. Ultrasonography to diagnose ovarian or adrenal tumor.
d. CT or MRI : to diagnose an organic lesion of the brain, or adrenal tumor.
& other cranial nerve lesions .
-It consists of a triad of :
1. Precocious puberty
Investigations
-Hypothyroidism retards bone age, and is the only condition of precocious puberty in which 1 - FSH & LH assay important to differentiate level of the lesion & 2. Cystic changes in bones
bone age is retarded progesterone assay in 17 OH deficiency . 3. Cafe-au lait patches of the skin.
2 - Chromosomal study if short stature or hypergonadotropic type . -The cause of precocious puberty is autonomous production of estrogen
Idiopathic precocious puberty 3 - Radiological bone age study & radiologic study for pituitary adenoma by the ovaries.
is diagnosed after excluding all other causes.
-FSH and LH levels are low.
-The treatment is testolactone oral tabletswhich inhibit ovarian
steroidogenesis.
Treatment The drug inhibits the formation of estrogen from its precursors, so reduces
1. Arrest maturation until normal pubertal age.
🔸
estrogen level.
2. Attenuate & diminish established precocious characteristics. The dose is 20 mg/kg body weight in 4 divided doses and increased to
3. Maximize adult height.
40 mg/kgbody weight during a 3 week interval.
4. Avoid abuse, reduce emotional & social problems
🔸 1. Treatment of the cause, e.g., thyroxin for hypothyroidism, removal of ovarian and
🔸
adrenal tumors.
2. Incomplete forms of precocious puberty do not require treatment, as estrogen
production is not increased.
Treatment
*Constitutional : Reassurance .
*Treatment of the cause (if treatable) or cyclic estrogen-progesterone
treatment Idiopathic type .4
-is treated by explanation and reassuranceand by giving one of the following drugs which
hormone replacement therapy if the cause is not treatable , for 3 cycles:
Norethistrone acetate 5 mg twice daily for 21 d or OCP
inhibit the secretion of gonadotrophins: * Patient with Y chromosome cell line :Gonadectomy + hormone replacement
(a)Gonadotrophin releasing hormone analogues which are given as daily nasal spray, therapy
intramuscular, or subcutaneous injections every 4 weeks.
(b)Medroxyprogesterone acetate tablets(Provera tablets) or intramuscular injection
(Depo-Provera);
(c) Danazol capsules;
🔸
(d)Cyproterone acetate tablets (Androcur).
idiopathic type Treatment is given till the age of 12 years (mean age of pubertal
development)
by fatema okoff
