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    by: khaleeq

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    1 : PUBERTY 3/14/2011 1 Arshiya Sultana Lecturer, Dept. of Obstetrics & Gynaecology NIUM, Bangalore, Karnataka.
    2 : Puber- marriageable age Pubertus – adulthood The period of transition between sexual immaturity and maturity Puberty is first phase of adolescence. How puberty occurs ? 3/14/2011 2
    3 : 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. 3/14/2011 3
    4 : 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 3/14/2011 4
    5 : 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 3/14/2011 5
    6 : 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 amount 3/14/2011 6
    7 : 3/14/2011 7
    8 : 3/14/2011 8
    9 : STAGES OF PUBERTY Growth spurt Breast development (thelarche) Pubic hair growth (Adrenarche) Menstruation (menarche) Axillary hair growth 70% of girls, variation often occur in Tanner Definite signs of puberty are usually present by the age 9 or 10 years 3/14/2011 9
    10 : Growth 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 3/14/2011 10
    11 : Tanner staging of breast development – Marshall and Tanner (1969) Elevation of papilla Elevation of papilla & breast on a small mount, increased in areola Further 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 3/14/2011 11
    12 : 3/14/2011 12
    13 : Menarche occurs at any between 9 to 17 yrs In India -13.5 yrs Age of menarche varies family Race Social class Family size, birth order Environment Diet General health 3/14/2011 13
    14 : 3/14/2011 14
    15 : Axillary hair Appears later During the 2yrs before the menarche the genital tract develops Menstrual phase itself often preceded by mucoid vaginal discharge 3/14/2011 15
    16 : Other Changes during puberty Apart from development of secondary sexual characters and growth spurt other changes are Gonads Sex organs Pelvis Skin changes Psychological changes Hormonal 3/14/2011 16
    17 : Factors Geographical Genetic Body weight Health Socioeconomic Family background 3/14/2011 17
    18 : Puberty Precocious puberty Delayed puberty 3/14/2011 18
    19 : Precocious puberty Tanner 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 3/14/2011 19
    20 : 3/14/2011 20
    21 : Precocious Puberty Isosexual Heterosexual Complete Incomplete Central Peripheral Combined Premature thelarche Premature adrenarche 3/14/2011 21
    22 : Precocious Puberty Isosexual Complete Central 3/14/2011 22
    23 : Complete Central isosexual puberty Systemic estrogen effect True or gonadotrophin dependent 90% Cyclic release of gonadotrophin Classification: Idiopathic or organic brain disease Idiopathic : Most common 70% Underlying etiology unknown 3/14/2011 23
    24 : Growth spurt is rapid with short duration Rate of progression vary General health is not impaired USG- functional follicular ovarian cyst Incidence of POF and infertility is not increased Other causes are to be excluded before diagnosis – MRI, CT scan, etc 30% cases may have organic brain disease 3/14/2011 24
    25 : Precocious Puberty Isosexual Complete Central Peripheral 3/14/2011 25
    26 : Peripheral precocious puberty Pseudoprecocious puberty Gonadotrophin independent Classification: Ovarian tumour Adrenal tumour – estrogen secreting - rare Iatrogenic- exogenous administration of sex steroids Primary hypothyroidism MC Cune Albright syndrome Ovarian cyst- estrogen secreting can cause PPP 3/14/2011 26
    27 : Ovarian tumour It is common cause for PPP Granulosa theca cell tumour – benign, estrogen secreting, confined to one ovary, Palpable rectal abdominal examination or USG Treatment : unilateral salpingoopherectomy 3/14/2011 27
    28 : Mc Cune Albright syndrome Rare – girls Triad Precocious puberty multiple area of fibrous dysplasia of bone café au lait spots of the skin facial asymmetry or skeletal deformities X-ray shows dysplastic lesions Fluctuation of estrogen levels and low gonadotrophin- independent of GnRH stimulation 3/14/2011 28
    29 : Café au lait skin pigmentation 3/14/2011 29
    30 : Facial asymmetry 3/14/2011 30
    31 : X ray showing dysplastic lesion 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 3/14/2011 31
    32 : Precocious Puberty Isosexual Complete Central Peripheral combined 3/14/2011 32
    33 : Combined CAH Virilizing adrenal tumours 3/14/2011 33
    34 : Precocious Puberty Isosexual Complete Incomplete Central Peripheral Combined Premature thelarche Premature adrenarche 3/14/2011 34
    35 : Incomplete precocious puberty No systemic estrogen effect One pubertal change is clinically apparent Absence of superficial cell desquamated from vaginal mucosa or bone age 3/14/2011 35
    36 : Premature thelarche – development of breast < 8yrs in white and <7yrs in black 3/14/2011 36 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. 
    37 : 3/14/2011 37 Benign and needs no therapy Commonly occurs between 1and 4 yrs of age. No progression 1/3th regression 1/10 progression Estradiol level < 20 ng/ml GnRH stimulation: FSH increases and LH no response
    38 : 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. 3/14/2011 38 Premature Adrenarche
    39 : 3/14/2011 39
    40 : Premature Adrenarche Adrenal androgen increases increase 17 hydroxyprogesterone –ACTH stimulation Shows 21 hydroxylase deficiency 3/14/2011 40
    41 : Diagnosis To distinguished heterosexual and isosexual puberty- History Physical examination –identify Tanner staging Height Incomplete precocious puberty-serial observation for at least 6 months 3/14/2011 41
    42 : Diagnosis contd 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 3/14/2011 42
    43 : 3/14/2011 43 Diagnosis contd
    44 : To distinguish incomplete (Premature thelarche) from complete precocious puberty Serum estradiol Prolactin LH GnRH stimulation test 3/14/2011 44
    45 : 3/14/2011 45
    46 : Incomplete precocious puberty - Premature adrenarche Cranial CT scan, 17 alpha hydroxyprogesterone level at baseline and following intravenous ACTH stimulation 3/14/2011 46
    47 : To distinguish Peripheral PP from central precocious puberty 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 3/14/2011 47
    48 : 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. 3/14/2011 48
    49 : Treatment Incomplete forms – self limiting Hypothyroid –thyroid replacement therapy Iatrogenic Ovarian and adrenal tumours – removed 3/14/2011 49
    50 : 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, abdominalcramping 3/14/2011 50
    51 : Idiopathic – GnRH analogs are reported as being sucessful in the treatment of IPP and central nervous system . Therapy – early – increase the height Long acting GnRH agonist Deslorelin 4-8 ug/kg Leuprolide acetate 20-60ug/kg Buserelin 20-30 ug/kg Leuprolide acetate IM 60ug/kg every 4 weeks Buserelin 1200-1800 ug/kg intranasally 3/14/2011 51 Once daily SC injection
    52 : 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. 3/14/2011 52
    53 : 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. 3/14/2011 53
    54 : 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. 3/14/2011 54
    55 : Delayed 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. 3/14/2011 55
    56 : Causes 3/14/2011 56 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
    57 : Diagnosis Thorough history Previous illness Physical examination: Height and weight secondary sexual characters growth pattern 3/14/2011 57
    58 : height Short stature (<147 cm) – chronic illness turner syndrome Hypothalamic or pituitary lesions hypothyroidism laurence moon biedl syndrome 3/14/2011 58
    59 : Weight: Underweight : malnutrition malabsorption syndrome aneroxia nervosa Excessive dieting other psychiatric diseases Normal weight or obese : constitutional delay, XY gonadal dysgenesis Kallman syndrome pituitary tumours, PCOS, Adrenal abnormalities and other causes of secondary amenorrhoea. 3/14/2011 59
    60 : Investigations Physical examination karyotyping FSH level – Increased in ovarian failure Decreased in hypopituitarism Thyroid and prolactin Ultrasound X ray pituitary MRI CT scan Laparoscopy 3/14/2011 60
    61 : 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 3/14/2011 61
    62 : combined estrogen and progestin sequential regimen is started cases of hypergonadotrophic hypogonadism should have chromosomal study to exclude intersexuality. 3/14/2011 62
    63 : 3/14/2011 63 Thank you
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