REFERAT

CUSHING SYNDROME
Ben Intoisus, S.Ked dr. Bambang Sri Nugroho, Sp.PD

DEPARTMENT OF INTERNAL MEDICINE SOEDARSO GENERAL HOSPITAL FACUTLY OF MEDICINE TANJUNGPURA UNIVERSITY 2013

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Contents
1 DEFINITION

2
3

EPIDEMIOLOGY
LITERATURE REVIEW definition classification etiopathogenesis pathophysiology clinical manifestation and diagnosis management APPROACH TO THE PATIEN
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HISTORY
1932: Harvey Cushing described a series of seven pts with basophilic adenomas of the pituitary --- CD
Rare cases or rare found?

1912
Over 100 years

2014

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DEFINITION
Chronic glucocorticoid excess whatever its causes, is accompanied by a wide range of signs and symptoms known as

Cushings syndrome
Boscaro M, Arnaldi G. Approach to The Patient With Paossible Cushings Syndrome. Journal of Clinical Endocrinology and Company Logo Metabolism. 2009 ; 3121-3131

EPIDEMIOLOGY

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CLASSIFICATION
Cushings Syndrome

ACTH-dependent(80% casses)

ACTH-independent(20%cases)

Cushings disease ACTH pituitary adenoma Ectopik ACRH secretion

Adrenal adenoma Adrenal carcinoma Bilateral adrenal hyperplasia Iatrogenic Caushings Syndrome

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ADRENAL STEROIDOGENESIS

CHOLESTEROL

PREGNENOLONE

PROGESTERON

DEOXY CORTICOSTERONE

CORTICOSTERON

ALDOSTERON

HSD11B1
17-HYDROXIL PREGNENOLON 17-OHP 11DEOXYCORTISOL CORTISOL CORTISONE

HSD11B2

DHEA

ANDROSTENEDION

TESTOSTERON

DHEAS

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ETIOLOGY
ACTH-dependent
Pituitary (CD) (70%)
Microadenomas (95%) Macroadenomas (5%)

ACTH-independent (Factitious) Unilateral

Adrenal adenoma (10%) Adrenal carcinoma (5%)

Ectopic ACTH or CRH (10%)

Small cell lung ca Carcinoids: lung, pancreas, thymus

Bilateral
Macronodular Hyperplasia (AIMAH) (<2%) Primary pigmented Micronodular Adrenal disease (PPNAD) (<2%) McCune Albright Syndrome (<2%)

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PATHOPHYSIOLOGY

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CLINICAL MANIFESTATION

GLUCOCORTICO ID
GLUCONEOGENESI S LIPOLOSIS PROTEIN CATABOLISM INCREASING 11HSPL OBESITY DEPRESION

GONADOTROPIN
HIPOGONADISM AMENOREA DECRESING TESTOSTERON

NIBERALOCORTI COID
DIASTOLIC HT HIPOCALEMIA EDEMA

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SURGICAL TEATMENT FOR CAUSHINGS SYNDROME

Done by neurosurgeons Remission rate of 80-90%

Cure is confirmed by demonstrating profound hypoadrenalism post-op (am cortisol <50 nmol/L)

Transphenoidal adenomectomy

Morbidity extremely low

Period of adrenal insufficiency requiring GC for up to 2 yrs (6-8 mo)
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