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The Endocrionology Department,

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Presentation on theme: "The Endocrionology Department,"— Presentation transcript:

1 The Endocrionology Department,
HYPERCORTISOLISM CUSHING SYNDROME Shan Zhongyan 单忠艳 The Endocrionology Department,

2 Contents What is Cushing syndrome? What is the causes?
How to diagnose? Clinical Features Hormone levels Functional test Localization How to treatment?

3 Contents What is Cushing syndrome? What is the causes?
How to diagnose? Clinical Features Hormone levels Functional test Localization How to treatment?

4 Definition Cushing Syndrome
A diverse symptom complex resulting from excess steroid hormone (cortisol) production Cushing Disease A hypercortisolism resulting from pituitary ACTH hypersecretion

5 Contents What is Cushing syndrome? What is the causes?
How to diagnose? Clinical Features Hormone levels Functional test Localization How to treatment?

6 Adrenal Structure Capsula Glomerulosa Aldosterone Fasciculata Cortisol
Reticularis Androgen Medulla Catecholamine

7 Cortisol Secretion Regulation
ACTH CRH Cortisol Hypothalamus Pituitary Adrenal

8 Causes of Cushing Syndrome
Hypothalamus Cushing syn. CRH Ectopic CRH syndrome Hypothalamus Cushing disease (CD) ACTH Ectopic ACTH syndrome Pituitary Adrenal adenoma Adrenal carcinoma Micronodular adrenal disease Massive macronodular adrenal disease Cortisol Adrenal

9 Causes of Cushing syndrome
1. Endogenous Cushing syndrome (1) ACTH-dependent Cushing disease (CD) Ectopic ACTH syndrome Ectopic CRH syndrome (2) ACTH-independent Adrenal adenoma Adrenal carcinoma Micronodular adrenal disease Massive macronodular adrenal disease (3) Pseudocushing syndrome 2. Exogenous Cushing syndrome

10 Contents What is Cushing syndrome? What is the causes?
How to diagnose? Clinical Features Hormone levels Functional test Localization How to treatment?

11

12 Function Clinical Features
Functions Clinical Features Fat Central obesity, “moon face” “buffalo hump” Protein Thin extremities, muscle weakness, Purple striae, bruising, osteoporosis Glucose IGT, DM Electrolyte Hypertension, hypokalemia, alkalosis

13 Function Clinical Features
Functions Clinical Features Immunity Infection, impaired wound healing Sex gland Hirsutism, acne, menstrual dysfunction, Erectile dysfunction Bone marrow Increased RBC and WBC,plethoric Mental Increased lability Skin Hyperpigmentation

14

15 Hormone Level 1. Cortisol levels
Plasma cortisol rhythm (8Am, 4Pm, midnight) 24h Urinary free cortisol, 17-OHCS, 17-KS Plasma ACTH rhythm 2. Functional Tests Low dose dexamethasone suppression test High dose dexamethasone suppression test Metyrapone test

16 Localization Adrenal CT

17 Localization Pituitary MRI

18 Diagnosis 1. Confirmation of hypercortisolism (Y/N)
Plasma cortisol rhythm (8Am, 4Pm, midnight) 24h Urinary free cortisol, 17-OHCS, 17-KS Low dose dexamethasone suppression test 2. Differentiation of Cushing syndrome (What) Plasma ACTH level High dose dexamethasone suppression test Metyrapone test CRH stimulation test 3. Localization (Where) Adrenal CT, Pituitary MRI

19 Cortisol rhythm, UFC, 17-OHCS, 17-KS
Diagnosis Schedules (1) Cushing Syndrome suspected Cortisol rhythm, UFC, 17-OHCS, 17-KS Elevated Normal Low dose dex. test Obesity No Yes Cushing Syndrome

20 Diagnosis Schedules (2)
Cushing Syndrome ACTH rhythm Elevated suppressed ACTH-dependent ACTH-independent High dose dex. test Glucocorticoid taken No Yes Yes No Ectopic ACTH/CRH Cushing Disease Exogenous Cushing Adrenal Tumor/Nodule

21 Contents What is Cushing syndrome? What is the causes?
How to diagnose? Clinical Features Hormone levels Functional test Localization How to treatment?

22 Treatment Cause Treatment Cushing D Transsphenoidal
Ectopic ACTH Surgery+drug Ectopic CRH Surgery +drug Adrenal Aden Surgery Adrenal Carc Surgery +drug Micronodular Surgery +replace Macronodular Surgery +replace Nelson Syndrome

23 Summary Cause incidence Cor. ACTH Low-Dex. High-Dex. Treatment
Cushing D No Yes Transsphenoidal Ectopic ACTH No No Surgery+drug Ectopic CRH Rare No No Surgery +drug Adrenal Aden No No Surgery Adrenal Carc No No Surgery +drug Micronodular Less No No Surgery +replace Macronodular Rare No No Surgery +replace Exogenous More No No Stop taking Obesity Yes Yes Diet + exercise Summary

24

25 病例分析 张**,女,27岁 主述:体重增加2年 现病史:病人2年来体重逐渐增加,尤近半年明显,由60公斤增至76公斤。伴疲乏无力,头迷,近3个月未来月经,觉汗毛加重,长胡须,面部经常起痤疮。病来无明显多食, 活动量未减少,无多饮多尿,睡眠正常, 无视物障碍及缺损,无怕冷少汗,无软瘫。 体格检查:Bp 190/110mmHg , P 86次/分, T 36.8℃,H 1.58m,BW 76Kg。神志清晰,腹型肥胖。皮肤菲薄,满月脸,多血质面容,毛发浓密多油腻,发际下移,可见胡须,颜面可见痤疮。背部毛囊角化明显,下腹部及双下肢近端多条粉红色紫纹,呈梭型,四肢均可见片状瘀斑。甲状腺不大,心律规整,腹部未扪及包块。锁骨上、肾区均未闻及血管杂音。 问题:1、根据病人临床症状,你的初步诊断是什么? 2、如何检查明确诊断?

26 病例分析 检查结果:尿pH 6.0,尿蛋白阴性。血WBC 8.6 X109/L, Hb 12g/L。血pH 7.42,血钾3.32mmol/L,BUN、Cr正常。 血皮质醇 8 Am 2084 nmol/L,3pm 2162 nmol/L。 ACTH Am <10 pmol/L, 3pm < 10pmol/L。 OGTT BG 0’ 5.6 mmol/L,120’ 8.9 mmol/L。 小剂量地塞米松抑制试验:皮质醇 前2284 ,后 2146 nmol/L。 大剂量地塞米松抑制试验:皮质醇 前2146 ,后 1984 nmol/L。 肾上腺增强CT:左侧肾上腺占位病变。 问题:3、该病人完整的诊断包括什么?    4、如何选择治疗方案? 

27 Desoxycorticosterone 17-hydroxy pregnenolone Dehydroepi-androsterone
Cortisol Synthesis Process Cholesterol Pregnenolone Desoxycorticosterone Corticosterone Aldosterone 17-hydroxy pregnenolone Desoxycortisol Cortisol Dehydroepi-androsterone Androstendione Estrone Androstendiol Testosterone Estradiol

28 Cortisol Synthesis Process
胆固醇 孕烯醇酮 去氧皮质酮 皮质酮 醛固酮 17-羟孕烯醇酮 去氧皮质醇 皮质醇 去氢表雄酮 雄烯二酮 雌 酮 雄烯二醇 睾 酮 雌二醇 孕 酮 17-羟孕酮


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