Thursday 30 June 2011

Cushing Syndrome

Anatomy of ADRENAL GLAND
-          Cortex
Hormone
Layers
Functions
Glucocorticoid
 E.g. cortisol
zona glomerulosa
Affect carbohydrate, lipid and protein metabolism
Mineralocorticoid
zona fasciclata
Sodium & potassium balance
Androgen
Zona reticularis
Weak effect until peripheral conversion to testosterone & dihydrotestosterone

-          Medulla
o   Secretes adrenaline, noradrenaline, dopamine
o   Responds to symphatetisdc preganglionic neuron

CUSHING’SYNDROME
Def: glucocorticoid excess

Aetiology
*      Exogenous (most common)
o   Steroid treatment
*      Endogenous
o   ACTH dependant causes (↑ ACTH)
1.       Cushing’s disease          85%
Due to Pituitary microadenoma
à ↑ACTH à bilateral adrenal hyperplasia
·         F>M
·         30-50 yo
2.       Ectopic ACTH production
·         d/t        small cell lung ca
carcinoid syndrome
·         special features
                                                                                                               i.      weight loss
                                                                                                             ii.      pigmentation (d/t ↑ACTH)
                                                                                                            iii.      hyperglycaemia
                                                                                                           iv.      hypokalaemic metabolic alkalosis
(↑ cortisol à mineralocorticoid activity)
3.       Ectopic CRF production ( rare)
·         d/t        medullary thyroid ca
Prostate ca
4.       Iatrogenic : ACTH administration

o   ACTH independant causes ( ACTH d/t –ve FB)
1.       Iatrogenic
·         Steroid (common)
2.       Adrenal adenoma / carcinoma
·         a/w abdominal pain, virilisation
3.       Adrenal nodular hyperplasia
4.       Rare      Carney complex
Mc Cune Albright syndrome

Clinical features
C             central obesity, + proximal weakness
U            unusual bruising
S             striae
H             hypertension, hyperglycaemia, buffalo hump
I              impotence (M), irregular menses (F), infection
N
G            growth retardation
+            
2M         moon face
                Mood changes
                             i.      Depression
                           ii.      Lethargy
                          iii.      Irratibility

Investigations

Treatments
Depends on cause
1.       Iatrogenic
§  Stop medication
2.       Cushing’s disease
§  Selective removal of pituitary adenoma via transphenoidal approach
§  Bilateral adrenalectomy if
                                                               i.      Source cant be located
                                                             ii.      Recurrent
§  Radiotherapy of pituitary
3.       Adrenal adenoma/carcinoma
§  Adrenalectomy
§  If cancer             -radiotherapy
-adrenolytic drug e.g. mitotane
4.       Ectopic ACTH
§  Tumor resection

Prognosis
1.       Treated
§  Good prognosis
§  Resolution of physical and psychological feature
2.       Untreated
§  ↑ mortality rate
d/t cardiovascular complication

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