2. - Is an intermingling of characters of both sexes
in one individual in varying degrees,including physical form,
reproductive organs,harmonal profile and sexual behaviour.
* INCIDENCE =1.7%
3. TYPES OF INTERSEX
• 1. Gonadal agenesis - gonads completely absent.
• 2. Gonadal dysgenesis - gonads of only 1 sex present, but fail to
develop at puberty.
• 3. True hermaphrodite - gonads of both sexes present.
• 4. Psuedohermaphrodite - gonads of one sex present , but external
sexual characteristics are of opposite sex.
4. 1.GONADAL AGENESIS
• In gonadal agenesis, testes and ovaries are completely absent.
• sex chromatin is absent (46,XY).
2.GONADAL DYSGENESIS
* Testes and ovaries are present , but they fail to develop normally.
* Main features - Streak gonads(gonads composed of fibrous tissue)
* 2 TYPES - a) KLINEFELTER'S SYNDROME.
b) TURNER'S SYNDROME.
5. a)KLINEFELTER'S SYNDROME
2 X chromosomes and a Y chromosome.
• Genotype = 47,XXY
(48XXXY,49XXXXY,48XXYY- also seen)
• Phenotype = male.
• Incidence = 1:600 males.
SIGNS & SYMPTOMS
*DIAGNOSIS - usually diagnosed when there is
delay in onset of puberty, behavioural disorders ,
mental retardation.
*BODY DIMENSION - increased height , narrow
shoulders , broad hips.
6. *SEX RELATED - microorchidism(small testicles),low testosterone
levels, normal to moderately reduced leydig cell function,Azospermia ,
sterility, Gynecomastia.
* HAIR - hair on chest and chin reduced, Axillary & pubic hair absent
*SERUM & URINARY GONADOTROPHINS - Increased.(primary
hypogonadism leads to decreased testosterone levels which causes
increase in levels of FSH and LH)
*OTHER - D.M ,Osteoporosis , thromboembolic disease , varicose
veins.
*HISTOLOGY - testicular atrophy with hyalinization of seminiferous
tubules.
7.
8. b)TURNER'S SYNDROME
*Affected individual has only one X
chromosome & no Y chromosome.
*Phenotype - Female
*Genotype - 45,XO
*INCIDENCE = 1:2500 girls
>SIGNS & SYMPTOMS
*Diagnosis - at birth by low birth weight,
edema of dorsum of hands & feet, loose
skin folds in the nape of neck(webbed
neck/pterygium colli).
*Stature - short stature, slow growth,micro-
gnathia,shortened 4th metacarpal.
9. *Sex related - premature ovarian
failure,rudimentary ovaries gonadal
streak,sterility,lack of primary &
secondary sexual characteristics,
increased serum and urinary
gonadotrophins.
*Face - ptosis,visual & auditory
impairment,ear infections,high arch
palate, low set ears,low hairline.
*Chest - broad/shield chest,poor breast
development, widely placed nipples.
*Other - cubitus valgus(increased carrying angle),high waist hip ratio,
obesity,pigmented moles,Heart anomalies, renal defects,spina bifida,
D.M, cushings sydrome.
10. *Intelligence - most patients have normal intelligence.
*Histology - Ovaries donot contain primordial follicles.
COMPARISION
11. 3.TRUE HERMAPHRODITISM
*condition in which the affected
individual has both ovarian and
testicular tissue.
*very rare condition
*commonly gonads are fused in the
form of ovotestis,but may also present
as ovary on one side and testis on the
other side.
*position-abdominal/inguinal/ing-
scrotal.
*Neither gonad is completely functional.
*Karyotype -46XX/46XY or
46XX/47XXY.
12.
13. 4.PSEUDOHERMAPHRODITISM
*Condition in which the affected individual has gonads of one sex , but
external genitalia of opposite sex.
*2 types - Male pseudohermaphroditism
-Female pseudohermaphroditism
*Male pseudohermaphroditism
-Nuclear sex is XY,but sexual character
and sex organs are female.
-Cause-Androgen insensitivity syndrome.
-5 alpha reductase deficiency.
*gonads are testes.(no ovaries or uterus).
*previous name for AIS- testicular feminization syndrome
15. MLI OF SEX
*Marriage , divorce , nullity of marriage ,
inheritance , rape, paternity,maternity,
employment and other civil rights.
• In 2014, supreme court directed that transgenders must be
recognized as 3rd gender.
• all documents must contain that category.