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ENDOCRINE
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ADRENAL CORTEX Glomerulosa (Salt), mineralocorticoids
ALDOSTERONE Fasciculata (Sugar), glucocorticoids CORTISOL Reticularis (Sex), gonadocorticoids ANDROGENS, ESTROGENS The deeper you go, the sweeter it gets (Madonna)
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HYPERADRENALISM HYPERALDOSTERONISM (g)
CUSHING SYNDROME (CORTISOL) (f) (most common of the three) ADRENOGENITAL (VIRILIZING) SYNDROME (r)
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CUSHING SYNDROME CENTRAL OBESITY MOON FACIES WEAKNESS HIRSUTISM
HYPERTENSION DIABETES OSTEOPOROSIS STRIAE Hyper fasciculata hormones, i.e., cortisol
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EXOGENOUS STEROIDS (90%)
CUSHING SYNDROME PITUITARY ACTH INCREASE TUMOR ACTH INCREASE HYPERPLASIA OF CORTEX ADENOMA OF CORTEX CARCINOMA OF CORTEX EXOGENOUS STEROIDS (90%)
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3) 糖代谢紊乱: 外周组织糖利用减少 肝糖输出增多 糖异生增加 糖耐量受损 继发性 (类固醇性) 糖尿病
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4) 电解质紊乱: 机制:过多Cortisol致潴钠排钾,高血压,低血钾(去氧皮质酮盐皮质样作用)、水肿及夜尿增加,低血钾性碱中毒(异位ACTH综合征和肾上腺皮质癌)
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5) 心血管病变 导致高血压的原因: Cortisol盐皮质样作用 容量扩张 血管活性物加压反应增强 血管舒张受抑制
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6)全身及神经系统 肌无力、不同程度的神经、情绪反应。可有类偏狂 7)对感染抵抗力下降 免疫功能抑制 抗体形成受阻 中性粒细胞吞噬减弱
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8) 血液改变 多血质:(RBC,WBC增多) 淋巴组织萎缩 淋巴细胞和白细胞百分比率减少
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9) 性腺功能障碍 机制:肾上腺雄激素产生过多及皮质醇抑制垂体促性腺激素。女性多囊卵巢综合征:月经紊乱或闭经、痤疮、多毛、男性化(生须、喉结增大、乳房萎缩、阴蒂肥大—肾上腺癌?) 男性性功能低下:阴茎缩小, 睾丸变软
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PRIMARY HYPERALDOSTERONISM (Conn’s Syndrome)
Na+ RETENTION K+ EXCRETION HYPERTENSION Hyper glomerulosa hormones.
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PRIMARY HYPERALDOSTERONISM
CORTICAL NEOPLASM CORTICAL HYPERPLASIA FAMILIAL (rare)
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SECONDARY HYPERALDOSTERONISM
DECREASED RENAL PERFUSION EDEMA (HEART, LIVER, KIDNEY) PREGNANCY Once again, the amazing reninangiotensinaldosterone axis
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临床表现 高血压:主要的表现,早期可出现。一般不呈恶性经过。 BP:170/100mmHg左右 早期:
高血压、醛固酮增多、肾素-血管紧张素被抑制 第二期: 高血压、轻度低钾 第三期: 高血压、严重低钾肌麻痹
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ADRENOGENITAL SYNDROME
VIRILIZATION/feminization CORTICAL NEOPLASM CORTICAL HYPERPLASIA 21-Hydroxylase Deficiency, with buildup of 17-hydroxy progesterone Congenital adrenal hyperplasia is generally synonymous with adrenogenital syndrome.
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ADRENAL INSUFFICIENCY
PRIMARY ACUTE (ADRENAL CRISIS) PRIMARY CHRONIC (auto-immune ADDISON DISEASE) SECONDARY (PITUITARY)
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PRIMARY ACUTE RAPID WITHDRAWAL OF STEROIDS
MASSIVE ADRENAL HEMORRHAGE (WATERHOUSE-FRIDERICHSEN, if it follows infection [meningo, staph, H. flu] and shock) Newborns with DIFFICULT DELIVERY ANTICOAGULANT RX POSTSURGICAL DIC PATIENTS
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PRIMARY CHRONIC Most of Addison disease is auto-immune adrenalitis [ACAs]) INFECTIONS (fungal diseases, histo-) METASTASES (adrenals are an amazingly preferred site for early lung carcinoma metastases) GENETIC DISORDERS
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ADRENAL MEDULLA PHEOCHROMOCYTOMAS, aka, primary tumors of the adrenal medulla 10% arise in an MEN setting 10% are EXTRA-adrenal 10% are bilateral 10% are malignant 10% are in childhood You can only call them malignant if they metastasize, but this is no bad thing, because they are all removed anyway
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嗜铬细胞瘤起源于肾上腺髓质、交感神经节和其他部位的嗜铬组织。分泌大量儿茶酚胺,作用于肾上腺素能受体,引起以高血压及代谢紊乱为主的综合征候群,严重时并发高血压危象、休克、颅内出血、心力衰竭、心室纤颤、心肌梗死等
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嗜铬细胞瘤分泌的其他多肽激素及其作用 舒血管肠肽、P物质 — 面部潮红 鸦片肽、生长抑素 — 便秘 舒血管肠肽、血清素、胃动素 — 腹泻
神经肽Y —血管收缩、面色苍白 舒血管肠肽、肾上腺髓质素 — 低血压、休克
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儿茶酚胺的代谢 Tyrosine 酪氨酸羧化酶(tyrosine carboxylase) dopa
多巴脱羧酶(dopa decarboxylase) dopamine β-羟化酶 甲氧去甲肾上腺素 (normetanephrine, NMN) noradrenaline 、4-二羟苦杏仁酸 adrenaline 甲氧肾上腺素 (metanephrine, MN) 3-甲氧-4羟苦杏仁酸 (vanillyl mandelic acid, VMA)
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肾上腺外嗜铬细胞瘤 腹主动脉旁(约10%~15%)、肾门、肾上极、肝门区、肝-下腔静脉之间、胰头部、髂窝或附近、血管旁(直肠后、卵巢、膀胱内) 胸腔后纵膈、左右腰椎旁间隙、腹腔神经丛 颈部、颅内
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