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Cortex Adrenal

OH

O
Adrenal Glands
HH Acronym P Horm TGO

CRH ACTH Hydrocortison

TRH TSH T3 & T4


GnRH LH, FSH Androgen, estrogen
progestins
GHRH GH IGF-I, IGF-II
GHRIH/SRIH GH (TSH IGF-I, T3,T4
FSH,ACTH) others?
PRIH-PIH PRL Neurohormones
Pituitary Adrenal Axis
HORMONE TYPES
 Glucocorticoids
 Cortisol

 Androgens
 Dehydroepiandrosterone (DHEA)

 Minelaocorticoids
 Aldosteron

 11 deoxycortocosterone
The Adrenal Cortex Hormone
C/O:
1. Zona fasiculata and zona reticularis:
a. Glucocorticoid:
 Adaptation to severe stress
 Anti inflammatory agent
b. Androgen and precursor androgen
 Sex development
2. Zona glomerulosa:
Mineralocorticoid
 Balance of normal Na+ and K+
Glucocorticoid
(21 carbon steroid)

C/O:
1. Cortisol - predominant in
human
2. Corticosteron - less
abundant in human
Function:
Promote gluconeogenesis
Mineralocorticoid
(21 carbon steroid)
- Aldosterone

Prymary action
Promote Na retention and
K+ and H+ excretion,
particularly in kidney.
Androgen
(19 carbon steroid)

Androstenedion (weak) important precursor


of estrogen coverted by peripheral
aromatization in menopausal women
Precursor androgen:
Dehidroepiandrosterone (DHEA)
These steroid are converted in to more
potent androgen in extra adrenal tissue
Estrogen are not made in significant amount
in normal condition
Cholesterol from the plasma: is basic precursor
(much of cholesterol esterified and stored in
cytoplasmic lipid droplets.
a small portion synthesis in situ from acetyl CoA.
When ACTH (cAMP) stimulation to adrenal,
esterase is activated, and the free cholesterol formed
is transported to mitochondrion.
Cytochrome P450 side chain cleavage (P450scc)
convert cholesterol to pregnennolone.
Important
18 hydroxilase and 18 hydroxysteroid
dehydrogenase, which are required for
aldosterone synthesis are found only in
glomerulosa cells, so that biosynthesis of
this mineralocorticoid is certainly
confine on this region
LH Steroidogenesis, LH, & StAR
StAR
(Steroidogenic

Congenital adrenal hyperplasia


acute
regulatory
protein

(CAH)
Cyp21A2

Phimosis
hyposphadia
Scretion
Cortisol release with periodicity that regulated by
the diurnal rhythm of ACTH release
Consequently cortisol level highest in the morning
shortly after awakening and lowest in the late
afternoon and early evening

circulate in free and protein-bound


(transcortin/CBG)
most of cortisol bound to CBG tightly and a small
amount bound to albumin
half life: 1.5 - 2 hours
Mineralocorticoid

Does not have the specific


plasma transport protein, but
form very weak association
with albumin
Synthesis hormone regulated

Cortisol:
regulated by ACTH which in turn regulated by
CRH
these hormone linked by a classic negative
feedback loop
Mineralocorticoid
Renin angiotensin system and potasium
(primary)
Sodium, ACTH, and neural mechanism
Action of ACTH
Lipid Droplet

LDL
Cholesteryl esters
CEH
Cholesterol
A cAMP
C ATP
+ Pi
TH

R
AC

GS
PK

cortisol preg

11deoxy 17-OH
cortisol preg
Congenital Adrenal Hyperplasia
(CAH)

Virilization in a female child with


US images shows
congenital adrenalenlargement
hyperplasiaofsecondary
both the
lefttoand right adrenal deficiency
21-hydroxylase glands (arrows) with
presenting
Congenital Adrenal Hyperplasia “cerebriform”
with ambiguous appearance
genitaliaand
andmaintained
salt wasting
corticomedullary differentiation
. .
 Disorders of sex development (DSDs) can be
classified into four categories on the basis of
gonadal histologic features:
 Female pseudohermaphroditism (46,XX with two
ovaries);
 Male pseudohermaphroditism (46,XY with two testes);
 True hermaphroditism (ovotesticular DSD) (both
ovarian and testicular tissues);
 Gonadal dysgenesis, either mixed (a testis and a streak
gonad) or pure (bilateral streak gonads).
 The causes of female pseudohermaphroditism (46,XX
DSD) include CAH and transplacental androgen
exposure.
 CAH is the most common cause of ambiguous genitalia . It manifests as
various degrees of virilization in girls and as precocious puberty in boys.
 Most cases are secondary to21-hydroxylase deficiency.
 An elevated 17-hydroxy- progesterone level indicates the diagnosis of

CAH.
 Adrenal glands with a limb over 20 mm long and 4 mm wide and with

normal corticomedullary differentiation are suggestive of CAH and a


cerebriform appearance of the adrenal glands is reportedly specific for
CAH.
 A normal uterus and ovaries are seen at US in female

pseudohermaphroditism.
 Some boys with CAH may present with intratesticular nodularity

(hyperplasia of Leydig cells )


Symptoms of the severe form of CAH may
include:
 Dehydration
 Low blood pressure
 Low blood sugar level
 Trouble keeping enough salt in their bodies
 Altered development of the external genitalia in girls which is
noted at birth and may require surgery to correct
 Shorter height than their parents
 Early signs of puberty (in children)
 Irregular periods and possible trouble getting pregnant (in women)
 Excess facial hair (in women)
 Benign testicular tumors and infertility (in men)

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