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HYPERCORTISOLISM CUSHING SYNDROME Shan Zhongyan 单忠艳 The Endocrionology Department,

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Presentation on theme: "HYPERCORTISOLISM CUSHING SYNDROME Shan Zhongyan 单忠艳 The Endocrionology Department,"— Presentation transcript:

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

2 Contents 1. What is Cushing syndrome? 2. What is the causes? 3. How to diagnose?  Clinical Features  Hormone levels  Functional test  Localization 4. How to treatment?

3 Contents 1. What is Cushing syndrome? 2. What is the causes? 3. How to diagnose?  Clinical Features  Hormone levels  Functional test  Localization 4. 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 1. What is Cushing syndrome? 2. What is the causes? 3. How to diagnose?  Clinical Features  Hormone levels  Functional test  Localization 4. How to treatment?

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

7 Cortisol Secretion Regulation ACTH CRH Cortisol Hypothalamus Pituitary Adrenal

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

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 1. What is Cushing syndrome? 2. What is the causes? 3. How to diagnose?  Clinical Features  Hormone levels  Functional test  Localization 4. How to treatment?

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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 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 Function Clinical Features

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15 Hormone Level 1. Cortisol levels ① Plasma cortisol rhythm (8Am, 4Pm, midnight) ② 24h Urinary free cortisol, 17-OHCS, 17-KS ③ Plasma ACTH rhythm 2. Functional Tests ① Low dose dexamethasone suppression test ② High dose dexamethasone suppression test ③ 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 Diagnosis Schedules (1) Cushing Syndrome suspected Cortisol rhythm, UFC, 17-OHCS, 17-KS ElevatedNormal Obesity Low dose dex. test Yes No Cushing Syndrome

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

21 Contents 1. What is Cushing syndrome? 2. What is the causes? 3. How to diagnose?  Clinical Features  Hormone levels  Functional test  Localization 4. 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 Cause incidence Cor. ACTH Low-Dex. High-Dex. Treatment Cushing D 80 No Yes Transsphenoidal Ectopic ACTH 20 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

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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 X10 9 /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 8 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 Cortisol Synthesis Process Cholesterol Pregnenolone Desoxycortic osterone Corticosterone Aldosterone 17-hydroxy pregnenolone Desoxycortisol Cortisol Dehydroepi- androsterone Androstend ione Estrone Androsten diol 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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