Primary amenorrhoea
-
Upload
drmcbansal -
Category
Documents
-
view
4.158 -
download
5
description
Transcript of Primary amenorrhoea
PRIMARY AMENORRHOEA
Prof. M.C.BansalMBBS., MS., FICOG., MICOG.
Founder Principal & Controller,Jhalawar Medical College & Hospital Jjalawar.
MGMC & Hospital , sitapura ., Jaipur
DIFFERENTIAL DIAGNOSIS OF PRIMARY AMENORRHOEA
A. Anatomic abnormalities of the genital outflow tract1. Müllerian dysgenesis (Rokitansky–Küster–Hauser syndrome)2. Distal genital tract obstruction
a. Imperforate hymenb. Transverse vaginal septum
B. Hypergonadotropic (follicle–stimulating hormone >30 mIU/mL) hypogonadism (gonadal “failure”)
1. Gonadal dysgenesis with stigmata of Turner syndrome2. Pure gonadal dysgenesis
a. 46,XXb. 46,XY3. Early gonadal “failure” with apparent normal ovarian
development
• C. Hypogonadotropic (luteinizing hormone and follicle–stimulating hormone <10 mIU/mL) hypogonadism
• 1. Constitutional delay• 2. Isolated gonadotropin deficiency
• a. Associated with midline defects (Kallmann syndrome)
• b. Independent of associated disorders• c. Prader–Labhart–Willi syndrome• d. Laurence–Moon–Bardet–Biedl syndrome• e. Many other rare syndromes
• 3. Associated with multiple hormone deficiencies• 4. Neoplasms of the hypothalamic–pituitary area
• a. Craniopharyngiomas• b. Pituitary adenomas• c. Other
• 5. Infiltrative processes (Langerhans cell–type
histiocytosis)
• 6. After irradiation of the central nervous system
• 7. Severe chronic illnesses with malnutrition
• 8. Anorexia nervosa and related disorders
• 9. Severe hypothalamic amenorrhea (rare)
• 10. Antidopaminergic and gonadotropin–releasing
hormone–inhibiting drugs(especially psychotropic
agents, opiates)
• 11. Primary hypothyroidism• 12. Cushing syndrome• 13. Use of chemotherapeutic (especially alkylating)
agents• II. Asynchronous pubertal development• A. Complete androgen insensitivity syndrome (testicular
feminization)• B. Incomplete androgen insensitivity syndrome
DISCUSSION
FSH
Peripheral signals
Leptin, Ghrelin
HYPOTHALAMUS
Central signalsGABA,
NPY,GLUTAMATE
ANT. PITUITARY
GnRH
Kisspeptin-GPR54 system
LH
FSH LH
ESTRADIOLINHIBIN
GRAN THE
Follicular growthMid cycle LH peak
Androgen production
Aromatisation of androgens
OVARY
• WHO divides patients into groups based on endogenous oestrogen production, follicle-stimulating hormone (FSH) levels, prolactin levels, and hypothalamic-pituitary dysfunction.
• This classification is a guide that eliminates several diagnoses based on initial information. However, further work-up is still required.
• Group I: low oestrogen, low FSH, and no hypothalamic-pituitary pathology, leading to a diagnosis of hypogonadotrophic hypogonadism.
• Group II: normal oestrogen, normal FSH, and normal prolactin, leading to a diagnosis of polycystic ovary syndrome.
• Group III: low oestrogen and high FSH, leading to a diagnosis of gonadal failure.
APPROACH TO A CASE OF PRIMARY AMENORRHOEA
HISTORY & CLINICAL EXAM
ASYNCHRONOUS DEVELOPMENTBREAST > PUBIC HAIR
ANDROGEN INSENSITIVITY
IMMATURE SECONDARY SEXUAL CHARACTERISTICS
FSH , PROLACTIN
MATURE SECONDARY SEXUAL CHARACTERISTICS
DISTAL GENITAL TRACT OBSTRUCTION,MULLERIAN AGENESIS
FSH , PROLACTIN
HIGH FSH LOW OR NORMAL FSH HIGH PROLACTIN
KARYOTYPE
NORMAL ABNORMAL
• 46, XX GONADAL DYSGENESIS
• PREMATURE OVARIAN FAILURE
• 45,XX OR 46,XY• MOSAIC
GONADAL DYSGENESIS
PITUITARY FUNCTION TESTINGSELLAR X-RAY
NORMAL ABNORMAL
•CONSTITUIONAL DELAY•ISOLATED GONADOTROPIN DEFICIENCY•MALNUTRITION•CHRONIC ILLNESS
• HYPOPITUITARISM• CNS TUMOR
CHECK T4, TSH
NORMAL TSH
HIGH TSH
MRI OR CT
HYPOTHYROIDISM
Thank you…