puberty dr-arshiyasultana-110312042731-phpapp02
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PUBERTYPUBERTY
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Arshiya Sultana Lecturer, Dept. of Obstetrics & GynaecologyNIUM, Bangalore, Karnataka.
Puber- marriageable age
Pubertus – adulthood
The period of transition between sexual immaturity and maturity
Puberty is first phase of adolescence.
How puberty occurs ?
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Fetal and Infancy Fetal and Infancy During the latter half of fetal life,
the hypothalamus pituitary ovarian axis is functional completely.
FSH levels are suppressed from 20 weeks gestation by the production of estrogen by the placenta and by the fetus itself.
At birth, the fetus is separated from its placenta and therefore the major source of estrogen is removed.
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Hypoestrogenic state of the
fetus
FSH level rises and remains
elevated for 6-10 months
After birth
But FSH is suppressed by Central inhibition of production of GnRH
Controlled by gene in the GnRH cell
nucleus in the hypothalamus
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FSH pulses are undetectable -8-9
yrs
1-2 yrs – spike of FSH increases
in frequency
Childhood
4-5 yrs – frequency of the FSH pulses increases in day
light hours
Fully functional production of GnRH
with N adult frequency,
amplitude and pulse s
5-10yrs ovulatory
menstrual cycle
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Pituitary glands
All activities increases
At puberty – increase secretion of releasing factors by the hypothalamus
Manifested by sudden spurt in height, enlargement of thyroid, adrenal cortex activity, skin pigmentation
Cyclical production of gonadotrophin and estrogen in
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STAGES OF PUBERTYSTAGES OF PUBERTY
Growth spurt Breast development (thelarche) Pubic hair growth (Adrenarche)Menstruation (menarche)Axillary hair growth70% of girls, variation often
occur in TannerDefinite signs of puberty are
usually present by the age 9 or 10 years 04/08/23 9
Growth spurtGrowth spurt
It begins around the age of 11yrs in girls
6 to 10cms per year for around 2 years
Effect of estrogen – fusion of end plate of the femur and growth ceases by the age of 15 yrs
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Tanner staging of breast Tanner staging of breast development – development – Marshall and Marshall and
Tanner (1969)Tanner (1969)
Elevation of papilla
Elevation of papilla & breast on a small mount, increased in areolaFurther enlargement
Secondary mound of areola and papilla
Recession of areola to contour of breast
prepubertal
9-13 yrs
10-14 yrs
11-15 yrs
12-17 yrs
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MenarcheMenarche
occurs at any between 9 to 17 yrsIn India -13.5 yrs Age of menarche varies familyRaceSocial classFamily size, birth orderEnvironmentDietGeneral health
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Axillary hairAxillary hairAppears laterDuring the 2yrs before the
menarche the genital tract develops
Menstrual phase itself often preceded by mucoid vaginal discharge
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Other Changes during Other Changes during pubertypubertyApart from development of
secondary sexual characters and growth spurt other changes are
GonadsSex organsPelvisSkin changesPsychological changesHormonal
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Factors Factors Geographical GeneticBody weightHealth Socioeconomic Family background
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PubertyPuberty
Precocious puberty
Delayed puberty
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Precocious pubertyPrecocious pubertyTanner stage 2 of
breast development prior the age of 8 yrs in white and 7 yrs in black
Elevation of papilla & breast on a small mount, increased in areola
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Precocious Precocious PubertyPuberty
Isosexual Heterosexual
Complete Incomplete
Central
Peripheral
Combined
Premature thelarche
Premature adrenarche
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Precocious Precocious PubertyPuberty
Isosexual
Complete
Central
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Complete Complete Central isosexual pubertyCentral isosexual puberty
Systemic estrogen effectTrue or gonadotrophin dependent90% Cyclic release of gonadotrophinClassification: Idiopathic or organic
brain diseaseIdiopathic : Most common70% Underlying etiology unknown
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Growth spurt is rapid with short duration
Rate of progression varyGeneral health is not impaired USG- functional follicular ovarian cystIncidence of POF and infertility is not
increasedOther causes are to be excluded
before diagnosis – MRI, CT scan, etc30% cases may have organic brain
disease
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Precocious Precocious PubertyPuberty
Isosexual
Complete
Central Peripheral
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Peripheral precocious Peripheral precocious pubertypuberty
Pseudoprecocious puberty Gonadotrophin independentClassification:Ovarian tumour Adrenal tumour – estrogen secreting
- rareIatrogenic- exogenous
administration of sex steroidsPrimary hypothyroidismMC Cune Albright syndromeOvarian cyst- estrogen secreting can
cause PPP04/08/23 26
Ovarian tumourOvarian tumourIt is common cause for PPPGranulosa theca cell tumour –
benign, estrogen secreting, confined to one ovary,
Palpable rectal abdominal examination or USG
Treatment : unilateral salpingoopherectomy
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Mc Cune Albright Mc Cune Albright syndromesyndromeRare – girlsTriad1. Precocious puberty2. multiple area of fibrous dysplasia of
bone3. café au lait spots of the skin facial asymmetry or skeletal deformitiesX-ray shows dysplastic lesionsFluctuation of estrogen levels and low
gonadotrophin- independent of GnRH stimulation
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Café au lait skin Café au lait skin pigmentationpigmentation
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Facial asymmetryFacial asymmetry
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X ray showing dysplastic X ray showing dysplastic lesionlesion
Single view of the left hand demonstrates multiple large expansile "bubbly" lytic lesions with sharp transition zones and without an associated periosteal reaction (arrows). The lesions are located in the phalanges, carpels, metacarpals, distal ulna and radial bones. The cortex is very thin in many areas overlying the expansile lytic lesion, making it difficult to determine if a fracture has occurred
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Precocious Precocious PubertyPuberty
Isosexual
Complete
Central Peripheral
combined
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CombinedCombinedCAHVirilizing adrenal tumours
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Precocious Precocious PubertyPuberty
Isosexual
Complete Incomplete
Central
Peripheral
Combined
Premature thelarche
Premature adrenarche
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Incomplete precocious Incomplete precocious pubertypubertyNo systemic estrogen effectOne pubertal change is clinically
apparentAbsence of superficial cell
desquamated from vaginal mucosa or bone age
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Premature thelarche – Premature thelarche – development of breast < 8yrs in development of breast < 8yrs in white and <7yrs in blackwhite and <7yrs in black
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This is a bilateral enlargement of breasts in 1-2 yr olds that is common. There are no other signs of puberty development and the growth is normal. As long as the vulva, labia, vagina are normal infantile, and there is no pubic hair, then nothing is done.
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Benign and needs no therapyCommonly occurs between 1and
4 yrs of age.No progression1/3th regression1/10 progressionEstradiol level < 20 ng/mlGnRH stimulation: FSH increases
and LH no response
Appearance of pubic hair <8 yrs
No other pubertal changes
No evidence of systemic estrogen
Other androgen mediated clinical findings- axillary hair growth, oily skin, and acne
One half children have organic brain disease.
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Premature AdrenarchePremature Adrenarche
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Premature AdrenarchePremature Adrenarche Adrenal androgen increases increase 17
hydroxyprogesterone –ACTH stimulation
Shows 21 hydroxylase deficiency
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DiagnosisDiagnosisTo distinguished heterosexual
and isosexual puberty- HistoryPhysical examination –identify Tanner stagingHeight Incomplete precocious puberty-
serial observation for at least 6 months
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Diagnosis Diagnosis contdcontd
Thyroid dysfunction can be evaluated by thyroid profile.
Serum HCG concentrations are elevated in the presence of trophoblastic disease.
Iatrogenic sources of estrogen – medical history
Mc Cune Albright – clinical features
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Diagnosis Diagnosis contdcontd
To distinguish incomplete To distinguish incomplete (Premature thelarche) from (Premature thelarche) from
complete precocious pubertycomplete precocious puberty
Serum estradiolProlactinLHGnRH stimulation test
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Incomplete precocious puberty - Incomplete precocious puberty - Premature adrenarchePremature adrenarche
Cranial CT scan, 17 alpha hydroxyprogesterone
level at baseline and following intravenous ACTH stimulation
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To distinguish Peripheral PP To distinguish Peripheral PP from central precocious from central precocious
puberpubertyty
GnRH stimulation test - In PPP no change in gonadotrophin levels whereas True PP FSH increases more than LH
advanced bone age in both Increase in ovarian volume and
uterine size in TPP
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A rectal abdominal examination and pelvic USG – identify ovarian tumours and ovarian cysts.
Adrenal tumours –adrenal sonograms
CNS diseases is confirmed with the use of neurologic and ophthalmologic examination, skull x – ray, EEG and CT cranial scan or MRI study of the brain.
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TreatmentTreatmentIncomplete forms – self limitingHypothyroid –thyroid
replacement therapyIatrogenic Ovarian and adrenal tumours –
removed
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Mc Cune Albright syndrome-Testolactone – total daily oral dose
of 20 mg/kg body in four divided doses-
over a 3 weeks interval the total daily dose is increased to 40 mg/kg body wt
Continue till the sign regress Side effects: diarrhoea,
abdominalcramping04/08/23 50
Idiopathic – GnRH analogs are reported as being sucessful in the treatment of IPP and central nervous system .
Therapy – early – increase the heightLong acting GnRH agonistDeslorelin 4-8 ug/kgLeuprolide acetate 20-60ug/kg Buserelin 20-30 ug/kg
Leuprolide acetate IM 60ug/kg every 4 weeks
Buserelin 1200-1800 ug/kg intranasally
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Once daily SC injection
GnRH agonist are not useful in PPP
Side effects: allergic reactions allergy symptoms of lungs with
intranasal GnRH should be continued TPP till
the mean age of pubertal development.
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Precocious puberty can be differentiated from premature adrenarche by the concomitant appearance of pubic hair with breast development in girls and with testicular enlargement in boys.
Other differential diagnoses include virilization caused by congenital adrenal hyperplasia and an adrenocortical or gonadal tumor. In premature adrenarche, serum concentrations of dehydroepiandrosterone, dehydroepiandrosterone sulfate (DHEAS), androstenedione, and testosterone and urinary 17-ketosteroids are usually increased for chronological age and in the range of those found in early puberty.
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The bone age is usually within 2 standard deviations of the chronological age. Moderately elevated levels of serum androgen other than DHEAS, bone age advancement, or signs of atypical premature pubarche (such as cystic acne or symptoms of systemic virilization) indicate the need for a corticotropin test to rule out late-onset congenital adrenal hyperplasia.
Marked elevation of serum androgen levels and advanced bone age suggest the possibility of an adrenocortical or gonadal tumor.
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Delayed pubertyDelayed puberty breast tissue and/or pubic hair
have not appeared by 13-14 yrs of age
Or menarche appears as late as 16 yrs
The normal upper age limit of menarche is 15 yrs.
15% cases – constitutional delay – PCOD, cryptamenorrhoea.
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Causes Causes
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Hypergonadotrophic hypogonadism
Hypogonadotrophic hypogonadism
Anatomic causes
• Gonadal dysgenesis• Pure gonadal dysgenesis (46xx, 46xy)• Ovarian failure
• constitutional delay• chronic illness • Malnutrition• primary hypothyoidism• isolated gonadotrophin deficiency • Intracranial lesions • Pure gonadal dysgenesis (46xx, 46xy)• Ovarian failure
• mullerian • imperforate hymen• transerve vaginal septum
DiagnosisDiagnosisThorough historyPrevious illnessPhysical examination:Height and weight secondary sexual characters growth pattern
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heightheight
Short stature (<147 cm) – chronic illness turner syndromeHypothalamic or pituitary lesions hypothyroidism laurence moon biedl syndrome
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Weight:Weight:Underweight :1. malnutrition2. malabsorption syndrome3. aneroxia nervosa4.Excessive dieting5. other psychiatric diseasesNormal weight or obese :1. constitutional delay, 2.XY gonadal dysgenesis3.Kallman syndrome4. pituitary tumours, PCOS, 5.Adrenal abnormalities and other causes
of secondary amenorrhoea.
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InvestigationsInvestigations Physical examination karyotyping FSH level – Increased in ovarian
failure Decreased in hypopituitarismThyroid and prolactinUltrasoundX ray pituitaryMRICT scanLaparoscopy
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Treatment Treatment Treatment is directed according to the
etiology Assurance, improvement of general
health and treatment of any illness may be of help in non endocrinal causes
cases with hypogonadism may be treated with cyclic estrogen
Unopposed estrogen 0.3 mg (conjugated estrogen) daily is given for first 6 months
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combined estrogen and progestin sequential regimen is started
cases of hypergonadotrophic hypogonadism should have chromosomal study to exclude intersexuality.
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