So today we are going to discuss about the pathological real type Primary Amenorrhoea. So what is Primary Amenorrhoea:-
Definition:- A young girl who has not yet menstruated by her 16years of age is having primary amenorrhoea rather than delayed menarche.The normal upper age limit for menarche is 15years. So what are the reasons of Primary Amenorrhoea –
(A) Hypogonadotrophic hypogonadism :-
(1) Delayed puberty:- delayed GnRH (Gonadotropin releasing hormone) pulse reactivation.
(2) Hypothalamic and pituitary dysfunction:- Gonadotropin deficiency due to stress;weight loss; excessive exercise, anorexia nervosa; chronic disease(tuberculosis ).
(3) Kallmann’s syndrome:-inadequate GnRH pulse secretion-reduced FSH (Follicle stimulating hormone ) & LH (Luteinizing hormone )
(4) Central nervous system tumours
[B] Hypogonadotrophic hypogonadism :-
(1) Primary ovarian failure:- It’s also know as Primary ovarian insufficiency or Premature ovarian failure or premature ovarian insufficiency:-It is defined when ovarian failure occurs before the age of forty. It occurs in about 1% of female population. During intrauterine life either there is failure of germ cell migration or there may be normal germ cell migration but an accelerated rate of germ cell depletion(apoptosis )due to various reasons. This results in either no follicle or only few follicles left behind in the ovary by the time they reach puberty.(2) Resistant ovarian syndrome.(3) Galactosemia:-due to premature ovarian failure. (4) Enzyme deficiency.
[C] Abnormal chromosomal pattern:-
(1) Turner’s syndrome (45X)
(2) Various mosaic states 45X/45XX
(3) Pure gonadal dysgenesis (46XX or 46XY)- Phenotypically female with streak gonads. Stature is average with some secondary sexual characters.
(4) Androgen insensitivity syndrome(Testicular feminisation syndrome) 46XY (5)Partial deletions of the X chromosome.
[D] Developmental defect of genital tract(discuss at cryptomenorrhoea) two points want to include here; (1) Complete absence of vagina & (2)Absence of uterus in MRKH (Mayer-Rokitansky-Kuster-Hauser syndrome) [E] Dysfunction of thyroid and adrenal cortex:- (1) Adrenogenital syndrome (2) Cretinism. [F] Metabolic disorders:- Juvenile diabetes. [G] Systemic illness:- Malnutrition,anaemia,weight loss,tuberculosis. [H] Congenital:- uterine synechiae.
Dr Priyanka Baisya
