ENDOCRINE
ADRENAL CORTEX Glomerulosa (Salt), mineralocorticoids ALDOSTERONE Fasciculata (Sugar), glucocorticoids CORTISOL Reticularis (Sex), gonadocorticoids ANDROGENS, ESTROGENS The deeper you go, the sweeter it gets (Madonna)
HYPERADRENALISM HYPERALDOSTERONISM (g) CUSHING SYNDROME (CORTISOL) (f) (most common of the three) ADRENOGENITAL (VIRILIZING) SYNDROME (r)
CUSHING SYNDROME CENTRAL OBESITY MOON FACIES WEAKNESS HIRSUTISM HYPERTENSION DIABETES OSTEOPOROSIS STRIAE Hyper fasciculata hormones, i.e., cortisol
EXOGENOUS STEROIDS (90%) CUSHING SYNDROME PITUITARY ACTH INCREASE TUMOR ACTH INCREASE HYPERPLASIA OF CORTEX ADENOMA OF CORTEX CARCINOMA OF CORTEX EXOGENOUS STEROIDS (90%)
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
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)
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
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
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
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
PRIMARY HYPERALDOSTERONISM (Conn’s Syndrome) Na+ RETENTION K+ EXCRETION HYPERTENSION Hyper glomerulosa hormones.
PRIMARY HYPERALDOSTERONISM CORTICAL NEOPLASM CORTICAL HYPERPLASIA FAMILIAL (rare)
SECONDARY HYPERALDOSTERONISM DECREASED RENAL PERFUSION EDEMA (HEART, LIVER, KIDNEY) PREGNANCY Once again, the amazing reninangiotensinaldosterone axis
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
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.
ADRENAL INSUFFICIENCY PRIMARY ACUTE (ADRENAL CRISIS) PRIMARY CHRONIC (auto-immune ADDISON DISEASE) SECONDARY (PITUITARY)
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
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
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
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
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
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 3-甲氧去甲肾上腺素 (normetanephrine, NMN) noradrenaline 3、4-二羟苦杏仁酸 adrenaline 3-甲氧肾上腺素 (metanephrine, MN) 3-甲氧-4羟苦杏仁酸 (vanillyl mandelic acid, VMA）
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