Diseases of suprarenal gland

Fedor Šimko

Institute of Pathophysiology LFUK

Disclimer slide –prednáška je určená len na

výučbové potreby medikov LFUK v Bratislave

Fedor Šimko

Hormones of suprarenal gland

Glucocorticoids – fasciculata layer

Mineralocorticoid – glomelurose layer

Androgenes (estrogenes)– reticular layer

Catecholamines- suprarenal medulla

(chromafine cells)

Syntheis of Glk and ALD –in the suprarenal cortex

cholesterol progesteronepregnenolone

11-deoxycorticosterone

Corticosterone

ALDOSTERONE

17-hydroxypregnenolone

11-deoxycortizol

Cortizol

Min

era

loco

rtic

oid

eff

ects

Androgenes synthesis

cholesterol progesteronepregnenolone

androstendione

TESTOSTERONE

11-hydroxy-

androstendion

Dehydroepi-

androsterone

Regulation of corticoid secretion

(ACTH-RH –ACTH-- Gluk – neg. feedback)

Regulation of aldosterone secretion renin – Ang II- AT1R- aldosterone

Androgens regulation

ACTH –trophic effect on suparenal gland

glucocorticoids

ACTH (TSH,STH) ACTH (TSH,STH)

Hypothalamo-adenohypophyseal system

Releasing factors

RF

Trophic hormones-TH

adenohypophysis adenohypophysis

inhibiting factors

IF

Aldosterone-

stimulation

Angiotensin II

HyperkalemiaAdrenocorticothrophic

hormone

Glucocorticoid functions

- gluconeogenesis ( from amino acids)

- Reduction of glycolysis (hexokinase

inhibition)

- Glycogenolysis activation

- inhibition of insuline binding to receptors

- Reduction of glucose reabsrobtion in

distal tubes

•Proteosynthesis / proteolysis

•Lipolysis/lipogenesis- fat redistribution

Hyperglykémia - glykosúria

Glucocorticoid functions

-Atiinflammatory and antialergic effects:

-Proteocatabolism in lymfatic tissue

-Stabilisation of lysosomes

-Reduced capillary permeability

-Reduced bradykine production (by inhibition of

kalikrein in lysosomes)

-Stimulation of hematopoesis

Hyperglykémia - glykosúria

Aldosterone functions

1. Reabsorbtion of Na+ and H2O in

dist. tubuli of kidnies (also in salivary or

swet gland)

2. Na+/K+ exchange – hypokalemia, alkalosis

3. Hypertrophy of vascular musculature and fibrotic proliferation

Androgen functions

-Proteosynthesis

-Musculature growth

-Bone growth – enlargement of bone

longevity – (before closure of growth gaps)

Sex organs growth and maturation

Secondary sex signs

Libido development

Cushing´s sy. a Cushing´s disease

Def: overproduction of corticoids (cortisol)

Etiology and classification:

-Primary (Cush.sy.)- adenoma /

adenocarcinoma of suprarenal cortex – ACTH

reduced

-Secondary (Cushing´s disease)– excessive

production of CRH/ACTH – TU of hypophysis /

hypothalamus / or ectopic production – ACTH

increased

Cushing´s sy. a Cushing´s disease

Clinical manifestations:

Hyperglycemia /steroid diabetes mell.

Hypertension (volume enlargement)

Hypernatremia/ hypokalemia + alkalosis

Strie rubrae – weekness of subcutaneous fibrotic tissue- hemorrhagy

Fat tissue redistribution – buffle type obesity / moon face

Cushing´s sy. / Cushing´s disease

Clinical manifestation

- Osteoporosis (glukoneogenesis from bone amino-acids) + reduced

calcium resorbtion

- Atrophy of musculature and of lymfatic tissue

- Peptic ulcer – (increased HCl + reduced mucin production)

- Polyglobulia + eozinopenia

- Face pletora- (polyglobulia + arteriolar dilatation)

- Oligomenorrhea + loss of libido

- Endocrinne psychosyndrome

Addisson´s syndrome - hypocorticism

Def: insufficiency of suprarenal cortex (defficiency

of Glk, Ald, Andr)

Etiology and classification:

-TBC, hemorrhage, autoimune damage,

meningococcus sepsis

-Sudden withdrawal of corticoid therapy

-pathogenesis: signs of deficit of Glk, Ald, Andr

+ insufficient response to stressors

Addisson´s disease

Classification

- Primary – destruction of suprarenal cortex

ACTH is increased – hyperpigmentations (MSH)

-Secondary – destruction of hypothalamus / hypophysis

-ACTH is decreased, reduced propiomelanocortin – pallor

-Chronic – Addison´s disease

-Acute – Waterhaus-Friderichsen sy. – hemorrhagic

destruction of suprarenal gland / sudden interruption of

hormone - replacement therapy

Addison´s disease – peripheral,

central

Manifestations:

-Weekness, fatigue, loss of weight

-Hypotension/hypovolemia, hypoglycemia

-Anemia / leukopenia /eozinophilia, muscular a-

trophy

-Abdominal pain, vomiting (by reduced NaCl)

-Low Na+, high K+, acidosis

-hyperpigmentations –peripheral / pallor –central

-Bradypsychism, anxiety, end. psychosyndrome

Addison´s crisis

Acute destruction of suprarenal cortex

Etiology: infections-sepsis + hemorrhagy,adrenalectomy/sudden interruption of corticoid therapy

Manifestations:

Depletion of NaCl, hyperkalemia, acidosis hypovolemia

Abdominal pain, weekness

Polyuria, vomitting, diarrhea,

hypotension, shock

Somnolency, sopor

Primary hyperaldosteronism –

Cohn´s sy.

Deffinition: Autonomic increase of

aldosterone production

Etiology: adenoma/adenocarcinoma of

suprarenal cortex

Primary hyperladosteronism –

Cohn´s sy.

Clinical manifestations:

Hypokalemia - muscular weekness,

obstipation, dysrrhytmias

Hypokalemic nephropathy: polyuria,

proteinuria, nykturia, polydipsia, hypo / izostenuria,

alkalosis

Hypernatremia, hypertension (volume), hypertensive heart

Secondary hyperladosteronism

Def: compensatory mechanism in states

with hypovolemia and hypotension

Etiology: heart failure, nephrotic syndrome, liver

cirrhosis

Manifestations:

-hypotension

-hypokalemia

-Increased both - renin and aldosteron

Cause of Ald increase in

HF / liver cirrhosis / nephrotic sy.

Kidney

hypoperfusion-

renin release

decreased

aldosteron degradation

In damaged liver

Secondary hyperaldosteronismheart failure, nephrotic syndr, liver cirrhosis

extravasal loss of fluid –

hypovolemia + hypotension

Renin – angiotensin II – aldosterone

vasoconstriction + fluid retention

increased BP and volume

- hemodynamic improvement fibrotic

remodelling

Feochromocytoma

Def: excessive production of catecholamines (adr, NA)

Etiology: adenoma of suprarenal medulla /adenoma

of sympathetic ganglions

Classification:

paroxysmal (Adr) / continual form (NA)

adenoma / adenocarcinoma(10%)

in suprarenal medulla / in ganglions (10%)

Feochromocytoma

Manifestation:

Paroxysmal hypertension, hypertension crisis

(Adr)

Continual hypertension (NA)

Hypotension, paroxysmal hypotension – Adr

Vasodilatation via β2 receptors

hypovolemia – via postcapillary vasoconstriction followed

by intestitial edema (reduced volume / BP, swetting,

vomitus)

Feochromocytoma

Clinical manifestations:

Hypertension / hypertensive crisis:

- cefalea

- stenocardia, palpitations, tachycardia, dyspnea

- nausea / vomitting / abdominal pain

- pallor / cold swetting

- nervousness, anxiety, anger

- Increased catecholamines in blood and urine

Adr- paroxysmal, NA- continuous