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Step 1 notes > Step 1- systems-GI > Flashcards

Step 1- systems-GI Flashcards

1
Q

Define: foregut, midgut, hindgut developmental base for midgut pathology such as omphalocele, etc?

A

foregut - pharynx to duodenum midgut - duodenum to proximal 2/3 of transverse colon hindgut - distal 1/3 transverse colon to anal canal above the pectinate line Midgut (from duodenum to proximal 2/3 of transverse colon) development: 6th week: herniate through umbilical ring 10th week: return to abdominal cavity + rotates around SMA （肠系膜上动脉） 如果这个过程受损，出现malroration of midgut, omphalocele (脐突出， 这个病和gastroschisis腹裂的鉴别要点在于脐突出表面有腹膜，腹裂没有）， intestinal atresia or stenosis, volvulus 肠扭传

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2
Q

Dx: a newborn presents with drooling, choking and vomiting with the 1st feeding. CXR may/or may not show gas-filled stomach (这两种情况鉴别诊断）； 稍后可能出现cyanosis

A

TEF （tracheoesophageal fistula, 气管食管瘘） with gas: EA (esophageal atresia, 食管闭锁）+ TEF, MC type (85%) without gas: pure/isolated EA

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3
Q

Dx: 2-6 wk baby (mostly male), palpable “olive” mass in epigastric region, non bilious projectile vomiting

A

congenital pyloric stenosis 1/600 live births, more often in firstborn males

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4
Q

celiac trunk3大分支， 然后更小的分支供应胃？

A

celiac trunk (T12) 3大分支: 1） 向上left gastric A (分出食管支，然后沿胃小弯下行） 2） 向左splenic A (入脾之前先分出left gastroepiploic A 左胃网膜A沿胃大弯下行，再分出short gastric A向上供应胃底部；short gastric A have poor anastomoses if splenic artery is blocked) 3). 向右分出common hepatic A （肝总再分为往上的hepatic proper A - then right gastric A， 沿胃小弯走行和胃左A连接; 向下的gastroduodenal - then right gastroepiploic A，沿胃大弯走行和左胃网膜A连接 ) 左右胃网膜A有很多吻合支 左右胃A也有很多吻合支

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5
Q

Portal triad是哪3个结构，在什么韧带内？

A

portal vein, proper hepatic artery, common bile duct (within hepatoduodenal ligament) border the omental foramen, which connects the greater and lesser sacs; pringle maneuver止血

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6
Q

greater and lesser sacs在左右的分隔？

A

左：gastrosplenic ligament 右：gastrohepatic ligament (手术中可以切开进入小网膜

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7
Q

gut wall从内向外的分层？

A

MSMS Mucosa (epithelium - absorption; lamina propria - support; muscularis mucosa - motility Submucosa - include submucosal nerve plexus (Meissner) Muscularis externa - include Myenteric nerve plexus (Auerbach) Serosa (when intraperitoneal) / adventitia (when retroperitoneal)

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8
Q

celiac trunk3大分支， 然后更小的分支供应胃？

A

celiac trunk (T12) 3大分支: 1） 向上left gastric A (然后沿胃小弯下行） 2） 向左splenic A (入脾之前先分出left gastroepiploic A 左胃网膜A沿胃大弯下行，再分出short gastric A向上供应胃底部；short gastric A have poor anastomoses if splenic artery is blocked) 3). 向右分出common hepatic A （肝总再分为往上的hepatic proper A - then right gastric A; 向下的gastroduodenal - then right gastroepiploic A)

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9
Q

肝脏从外向内：hepatic triad结构？- zone I, II, III - central vein （回流到哪里）？各个zone在什么情况下受损？

A

hepatic triad： bile duct + branch hepatic A + branch of portal V blood 从外向内流，胆汁从内向外流 zone I (periportal zone): viral hepatitis, ingested toxins (cocaine, etc) zone II: intermediate zone zone III: pericentral zone ischemia, alcoholic hepatitis, metabolic toxins, contain P450 system central vein drains to hepatic V, - systemic circulation

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10
Q

GI 从十二指肠段到小肠产生哪些细胞regulatory substances?

A

细胞(从上到下）：DISK 对应产生： D cells (pancreatic islets, GI mucosa): somatostatin 生长抑素- 降胃酸 I cells (duodenum, jejunum): cholecystokinin [CCK] S cells (duodenum) - secretin, ↑ HCO3- secretion K cells: (duodenum, jejunum): glucose-dependent insulinotropic peptide: ↑ insulin release, ↓ gastric H+ secretion

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11
Q

CCK (cholecystokinin): 产生细胞及其肠段？ 作用？ 调控？特点：

A

CCK 胆囊收缩素 - I cells (duodenum + jejunum) 受↑ FA, aa 刺激，需要加强脂肪和蛋白质消化时分泌： 1) contract gallbladder + relax sphincter of Oddi 2) ↑ pancreatic secretion 3) ↓ gastric emptying 特点：act on neural muscarinic pathways to cause pancreatic secretion

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12
Q

Gastrin: 产生细胞及其肠段？ 作用？ 调控？特点：

A

G cells (antrum of stomach 胃窦）， 生成胃酸 - Zollinger-Ellison syn (胃泌素瘤） - Phenylalanine and trytophan are potent stimulators

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13
Q

Glucose-dependent insulinotrophic peptide: 又名？产生细胞及其肠段？ 作用？ 调控？特点：

A

= GIP (gastric inhibitory peptide) 由 duodenum + jejunum K cells 产生 （gluKose = K cell) 在 ORAL glucose， FA, aa ↑升高时分泌， ↑ insulin secretion ↓ H+ secretion 刺激GIP分泌用oral glucose load最为有效

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14
Q

Motilin： 产生细胞及其肠段？ 作用？ 调控？特点

A

由small intestine产生， ↑ in fasting state, to produce MMCs (migrating motor complexes)促进肠蠕动 note: EPO是motilin receptor agonist, 可以用来促进肠蠕动

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15
Q

secretin: 产生细胞及其肠段？ 作用？ 调控？特点

A

由duodenum S cells (S in the “DISK”)产生， 由 ↑ acids, FA in the duodenum 刺激， 作用： 1）↑ pancreatic HCO3- secretion中和十二指肠中的胃酸，以免产生溃疡 2）↓ gastric acid secretion 3) ↑ bile secretion

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16
Q

intrinsic factor: 产生细胞及其肠段？ 作用？特点

A

stomach parietal cells VB12-binding protein, 缺乏导致慢性胃炎和恶性贫血

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17
Q

gastric acid: 产生细胞及其肠段？ 正负调控的因子？gastrinoma的Cx? 胃酸产生的3相？

A

产生细胞及其肠段 - stomach parietal cells (还产生intrinsic factor) 正负调控的因子: ↑ by histamine, Phenylalanine, Tryptophan; Ach (副交感兴奋导致胃酸分泌）， gastrin, ↓ secretin, somatostatin, GIP, prostaglandin gastrinoma的Cx: refractory ulcers 胃酸产生的3相： cephalic (thoughts of food, see, smell, taste, 神经刺激胃酸分泌）， gastric (gastrin ↑ histamine, - increase HCL), intestinal （食物进入小肠后需要抑制胃酸分泌，回场和结肠分泌YY, - ECL - ↓histamine)

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18
Q

pepsin 胃蛋白酶： 产生细胞及其肠段？ 作用？被酶原被什么激活？

A

stomach chief cells; digest protein pepsinogen activated by H+

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19
Q

Brunner glands： 产生细胞及其肠段？ 作用？disease?

A

产生细胞及其肠段: duodenal submucosa 作用 - secrete alkaline mucus disease - hypertrophy in peptic ulcer diseases

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20
Q

糖的吸收： Glucose, galactose taken up by? fructose taken up by? transported to blood by？

A

Glucose, galactose taken up by： SGLT1 (sodium-glucose linked transporter, Na+ dependent) fructose taken up by: facilitated diffusion by GLUT5 all transported to blood by GLUT2

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21
Q

use which test to distinguish GI mucosal damage from other causes of malabsorption?

A

D-xylose absorption test D-Xylose is a monosaccharide, or simple sugar, that does not require enzymes for digestion prior to absorption. Its absorption requires an intact mucosa only. In contrast, polysaccharides require enzymes, such as amylase, to break them down so that they can eventually be absorbed as monosaccharides. In normal individuals, a 25 g oral dose of D-xylose will be absorbed and excreted in the urine at approximately 4.5 g in 5 hours. A decreased urinary excretion of D-xylose is seen in conditions involving the GI mucosa, such as small intestinal bacterial overgrowth and Whipple’s disease. In cases of bacterial overgrowth, the values of D-Xylose absorption return to normal after treatment with antibiotics. In contrast, if the D-xylose urinary excretion is not normal after a course of antibiotics, then the problem must be due to a non-mucosal cause of malabsorption (i.e., celiac disease).

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22
Q

吸收部位？ Iron? Folate? VB12?

A

Iron First, Bro Iron: absorbed as Fe2+ in duodenum Folate: jejunum and ileum B12: in terminal ileum, along with bile acid, requires intrinsic factor

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23
Q

Peyer patches: Define? 出现在哪个肠段哪一层？功能？

A

unencapsulated lymphoid tissue; in lamina propria and submucosa of ileum M cells to present Ag to immune cells, and B cells stimulated in the germinal centers of Peyer patches differentiated into IgA-secreting plasma cells; secretory IgA is transported across the epithelium to the gut to fight intraluminal Ag

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24
Q

Bile: 生成的限速酶是？ 功能？

A

Bile: 生成的限速酶是 cholesterol 7a-hydroxylase 功能: 1. digest and absorb lipids and fat-soluble vitamins 2. excrete cholesterol (body’s ONLY way to eliminate cholesterol) 3. antimicrobial activity via membrane disruption

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25
Q

MC salivary gland tumor? Cx? Benign or malignant? 组织学组成？

A

pleomorphic adenoma 多形性腺瘤 Cx: painless, mobile mass benign mixed tumor composed of chondromyxoid (软骨粘液样) stroma and epithelium 如果切除不干净，或者手术过程中破裂，非常容易复发。

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26
Q

Warthin tumor: 发生在哪个器官？良性恶性？病理特点？

A

= papillary cystadenoma lymphomatosum 沃辛肿瘤, 乳头状淋巴瘤性囊腺瘤, benign cystic tumor, 病理上可见germinal centers

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27
Q

MC malignant tumor of salivary gland? 病理特征？Cx?

A

mucoepidermoid carcinoma 粘液表皮样癌: MC malignant tumor of salivary gland 病理特征： has both mutinous and squamous components Cx: painless, slow-growing mass

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28
Q

Achalasia: cause for 1° and 2º? Cx? Barium swallow? Associated with which disease?

A

cause for 1°: lose myenteric (Auerbach) plexus - LES fails to relax and 2º: 来自中美洲、南美洲病人，出现吞咽困难，要怀疑Chagas disease (南美布氏锥虫病； 由kissing bugs传染，血液Gimsa染色在显微镜下可见） Cx: progressive dysphagia to solids and liquids (鉴别诊断: obstruction - solids Associated with ↑ risk of esophageal squamous cell carcinoma

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29
Q

Boerhaave syn

A

透壁性食管破裂，外科急症，不治疗死亡率100% “波尔have的综合征” transmural, usually distal esophageal rupture due to violent retching (i.e. , endoscopy or paraesophageal surgery）. surgical emergency Cx: severe retching and vomiting followed by excruciating retrosternal chest and upper abdominal pain Dx: CT可见纵膈气体 related with Mallory–Weiss syndrome (mucosa tearing)

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30
Q

Mallory-Weiss Syn

A

“gastro-esophageal laceration syndrome” mucosal tearing at the junction of the stomach and esophagus, usually caused by severe alcoholism 【mallory都和酒精有关】, bulimics (暴饮暴食），retching, coughing, or vomiting. Cx: periodic hematemesis 从症状和食管撕裂的深度（mucosa vs. transmural)和Boerhaave syn鉴别

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31
Q

eosinophilic esophagitis

A

infiltration of eosinophils in the esophagus. food allergens - dysphagia, heartburn, strictures unresponsive to GERD therapy

32
Q

esophagitis的病因？

A

几乎全在免疫缺陷（如AIDS)病人中出现，3大感染病原体，Cx无法区别，都表现为吞咽疼痛。鉴别在于内窥镜和显微镜： Candida: white pseudomembranes HSV-1: punched-out ulcers CMV: linear ulcers

33
Q

Plummer-Vinson Syn (PVS)

A

triad of dysphagia: Plummbers DIG 1) dysphagia (due to esophageal web) 2) iron-deficiency anemia 3) glossitis

34
Q

sclerodermal esophageal dysmortility, as part of CREST

A

CREST syndrome—Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia. Limited skin involvement, often confined to fingers and face. More benign clinical course. Associated with antiCentromere antibody (C for CREST). 食管病理：esophageal SMC atrophy - ↓ LES pressure and dysmortility - acid reflux and dysphagia - stricture

35
Q

Barrett esophagus: 病因？病理改变？↑ risk of which tumor?

A

病因：chronic acid reflux (GERD) 病理: 食管活检发现肠上皮的组织学特征： “glandular metaplasia” - replacement of stratified squamous epithelium with intestinal epithelium (non ciliated columnar with goblets cells) in the distal esophagus. ↑ risk of esophageal adenocarcinoma

36
Q

esophageal cancer: 两种瘤，是哪两种？ risk factors? 各自associated with which kind of tumor?

A

squamous cell carcinoma (upper 2/3), or adenocarcinoma (lower 1/3) risk factors: AABCD, EFFGH achalasis alcohol - squamous Barrett esophagus - adeno Cigarettes - both Diverticular (e.g., Zenker) - squamous esophageal web食管蹼 - squamous familial fat (obesity) - adeno GERD - adeno Hot liquids - squamous worldwide: squamous more common US: adeno more common

37
Q

chronic gastritis 分型？ 部位？

A

type A vs. type B A: autoimmune, auto-Ab against parietal cells, pernicious anemia, achlorhydria 胃酸缺乏 B: B = bacteria; MC type, caused by H. pylori infection ↑ risk of MALT lymphoma and gastric adenocarcinoma A comes before B: A: fundus/body B: autrum

38
Q

Ménétrier’s disease

A

hypoproteinemic hypertrophic gastropathy gastric hypertrophy + protein loss + parietal cell atrophy, ↑ mucous cells precancerous 病理特点：rugae of stomach are so hypertrophied that they look like brain gyri

39
Q

Dx: Virchow node?

A

stomach cancer淋巴结转移 to left supraclavicular node

40
Q

卵巢病理切片见signet ring cells：Dx?

A

Krukeberg tumor - stomach diffused adenocarcinoma, bilateral metastases to ovaries

41
Q

胃癌3大转移？

A

1。左锁骨上淋巴结 Virchow node 2. 双侧卵巢： Krukenberg tumor 3. Sister Mary Joseph nodule : subcutaneous periumbilical metastasis

42
Q

Celiac sprue: auto-Ab? associated with HLA哪个位点？ 病理特点？ Dx? associated with malignancy? Rx? 和tropical sprue鉴别？

A

auto-Ab: anti-tissue transglutaminase, anti-gliadin (wheat) Ab associated with HLA-DQ2, DQ8, northern European descent 病理特点: 远端十二指肠 and/or 近端空肠 blunting of villi，lymphocytes in the lamina propria, crypt hyperplasia Dx - anti-tissue transglutaminase ↑ associated with malignancy： ↑ risk for T-cell lymphoma Rx: gluten-free diet 和tropical sprue鉴别: 多发于热带居民或者去过热带的旅行者，病因不明，但是respond to antibiotics

43
Q

Abetalipoproteinemia: 病因？Cx? Patho?

A

↓ synthesis of apolipoprotein B - inability to generate chylomicrons - ↓ secretion of cholesterol, VLDL into blood - ↑ accumulation of fat in enterocytes 病理切片见clear or foamy cytoplasm Cx: early childhood failure to thrive, steatorrhea, acanthocutosis, ataxia, night blindness

44
Q

pancreatic insufficiency: 常见于哪些疾病？ 引起什么后果？ 用什么试验诊断？

A

常见于哪些疾病 - cystic fibrosis, chronic pancreatitis, obstructing cancer 引起什么后果 - malabsorption of fat, fat-soluble vitamins (DAKE) - VA缺乏常引起CF病人胰腺导管squamous keratinization 用什么试验诊断 - D-xylose absorption test: 在本病中正常！和intestinal mucosa defects, bacterial overgrowth鉴别

45
Q

鉴别Crohn’s disease vs. ulcerative colitis

A

Crohn’s disease : transmural (透壁，会引起fistula), creeping fat noncaseating granulomas and lymphoid aggregates (Th1) cobblestone skip lesions, rectal sparing “fat granny and an old crone skipping down a cobblestone road away from the wreck (rectal sparing) ulcerative colitis: mucosal and submucosal only no granulomas (Th2) pseudo polyps with intervening ulcerated, inflamed mucosa，病灶连续 (真息肉一般有正常mucosa间隔） always with rectal involvement “ulcerative colitis causes ULCCCERS” ulcers, large intestine, continous colorectal carcinoma, crypt abcesses, extended proximally, red diarrhea, sclerosing cholangitis (胆管炎（

46
Q

Zenker diverticulum: 发生部位？真憩室还是假憩室（两者区别）？Cx? CT所见？

A

发生部位 - pharyngoesophageal, 咽食管的 (和Meckel憩室区别：这是真憩室， persistence of the vitelline duct 卵黄管， GI最常见的先天性异常） 真憩室还是假憩室（两者区别）： false 【only mucosa and submucosa out pouch] vs. true: all the wall layers involved Cx - MC in elderly, high position dysphagia (food “at the throat”), cough, choke, obstruction, nasal regurgitation; food aspiration may cause pneumonia, foul breath from trapped food

47
Q

Meckel diverticulum: 发生部位？真憩室还是假憩室？Cx? The five 2’s

A

MC congenital GI anomaly true diverticulum: persistence of the vitelline duct (卵黄管） May contain ectopic acid-secreting gastric mucosa and/or pancreatic tissue may cause: Melena 黑粪症， RLQ pain, intussusception 肠套叠，volvulus 肠扭转 The five 2’s: 2 inches long 2 feet from the ileocecal valve 2% of the population present in first 2 years of life may have 2 types of epithelia (gastric/pancreatic)

48
Q

Hirschsprung disease: cause? Cx? associated with gene mutation? associated with which disease? Dx? Rx?

A

记忆法： a giant spring that has sprung in the colon - congenital megacolon Cause: neural crest cells fail to migrate - lack of ganglion cells/enteric nervous plexuses (Auerbach and Meissner plexus) in intestinal biopsy Cx: bilious emesis 胆汁性呕吐，abdominal distention, fail to pass meconium in the first 48 hrs, ultimately present as chronic constipation; involves rectum associated with gene mutation - RET associated with which disease -Downs Dx - rectal suction biopsy Rx: resection

49
Q

colonic polyps哪种病理分型是precursor to colorectal cancer?

A

adenomatous the more villous the polyp, the more likely it is to be malignant (villous = villainous) 其他的病理分型： - hyperplastic (MC non-neoplastic polyp), - juvenile (< 5 yrs): 单个息肉不会恶变，多个息肉 ↑ risk of adenocarcinoma - hamartomatous: Peutz-Jeghers Syn (AD, GI多个非恶性错构瘤 + hyperpigmented mouth, lips, hands, genitalia), ↑ risk of colorectal cancer and other visceral malignancies

50
Q

familial adenomatous polyposis (FAP) 家族性腺瘤性息肉病: 什么gene mutation? which chromosome? chance to progress into CRC (colorectal cancer)?

A

AD, mutation of APC gene on 5q 2-hit hypothesis: 100% progress into CRC unless resected thousands of polys arise at a young age, pancolonic, always involve rectum

51
Q

Gardner syn: Cx?

A

FAP + osseous and soft tissue tumors, congenital hypertrophy of retinal pigment epithelium

52
Q

Turcot syn

A

FAP + malignant CNS tumor (Turcot = Turban 穆斯林头巾）

53
Q

Whipple: 病原体特点？累及器官和部位？症状？

A

Tropheryma whipplei 惠普尔养障体， 最小的细菌，只能用电镜看到。gram +, PAS + foamy macrophage in intestinal lamina propria 多系统器官累及：CAN (cardiac symptoms, arthralgias, neurological symptoms), 多发于older men “Foamy Whipped cream in a CAN”

54
Q

HPNCC/Lynch syn: 隐性/显性遗传？什么gene mutation? chance to progress into CRC (colorectal cancer)? 累及部位？

A

AD mutation of DNA mismatch repair genes 80% progress to CRC proximal colon is always involved

55
Q

CRC： colorectal cancer 结肠3段发生几率？各自症状？ Dx? CEA marker的意义？

A

结肠3段发生几率：rectosigmoid > ascending> descending 各自症状: “right side bleeds, left side obstructs” - ascending: exophytic mass (向外生长），iron deficiency anemia, weight loss - descending: infiltrating (钡餐见典型“apple core” lesions, partial obstruction, colicky pain, hematochezia Dx: iron deficiency anemia in males (especially > 50) and postmenopausal females 要注意，这群人需要结肠镜检和粪便隐血试验 CEA marker的意义 - good for monitoring recurrence, not used for screening

56
Q

!!! Molecular pathogenesis of CRC: what are the 2 molecular pathways leading to CRC? what are their gene mutations? difference between colitis-associated CRC vs. sporadic CRC?

A

pathway 1: microsatellite instability pathway ( 15%): DNA mismatch repair gene mutations - cause both sporadic and HNPCC [mutations accumulate, but no defined morphologic correlates] pathway 2: APC/beta-catenin (chromosome instability) pathway (85%) - sporadic CRC order of gene events - AK53 difference between colitis-associated CRC vs. sporadic CRC: 1） in younger pts (with a IRB/CRC family history 2) develop cancer from non-polypoid dysplasia; multifocal in nature 3) gene events顺序反过来：early p53 mutation, late APC mutation 4) higher histological grade

57
Q

most specific diagnostic serum marker for acute pancreatic tis?

A

lipase 另外特异性稍差的marker还包括amylase

58
Q

Reye syn: cause unknown, but association? Cx? histological findings?

A

associated with aspirin (especially when treating VZV and influenza B viral infection), although cases also reported without aspirin - avoid aspirin in children, except for Kawasaki disease!! Cx: 肝、脑损伤 fatty liver (microvesicular fatty change), vomit, hypoglycemia, hepatomegaly, coma Mechanism: aspirin metabolites ↓ beta-oxidation by reversible inhibition of mitochondria enzyme

59
Q

alcoholic hepatitis: histological change? AST, ALT change?

A

1) swollen and necrotic hepatocytes 2) neutrophilic infiltration 3) mallory bodies (intracytoplasmic eosinophilic inclusion) AST > ALT (ratio > 1.5) [make a toAST to alcohol]

60
Q

hereditary hyperbilirubinemias: 4种类型： 名称，病因，表现？

A
  1. Gilbert syn: mild ↓ UDP-glucuronosyltransferase conjugation activity; very common, 无症状或轻微黄疸， no clinical consequences 2. Crigler-Najjar syn, type I: 比Gilbert严重，彻底没有UDP-glucuronosyltransferase， present early in life and die within a few years; type II: less severe than type I, respond to phenobarbital (which ↑ liver enzyme synthesis) 3. Dubin-Johnson syn: 结合胆红素排不出肝脏，在肝里积累（black liver), 良性 4. Rotor syn: similar to Dubin-Johnson but even milder, no black liver
61
Q

hepatic encephalopathy成因？Rx?

A

cirrhosis → portosystemic shunts → NH3 metabolism ↓ → neuropsychiatric dysfunction tiggers are with ↑ NH3 production or ↓ NH3 removal Rx: lactulose (乳果糖 ↑ NH4+ production) + low protein diet + rifaximin (利福昔明，抗生素，杀死肠道细菌）

62
Q

Budd-Chiari syn: 成因？表现？association?

A

occlusion of ICV or hepatic veins with centrilobular congestion and necrosis, → congestive liver disease Budda 大肚罗汉- 腹水 association: hyper coagulable states, polycythemia vera真性红细胞增多， pregnancy, HCC

63
Q

nutmeg liver 肉豆蔻肝

A

due to backup of blood into liver MCC: right-sided HF, Budd-Chiari syn can result in cardiac cirrhosis

64
Q

a1-antitrypsin deficiency: 在肝脏和肺中分别的表现？

A

肝脏： misfolded protein aggregates in hepatocellular ER → cirrhosis with PAS+ globules in liver (席夫氏碱染色阳性） codominant trait lung: uninhibited elastase in alveoli → panacinar emphysema

65
Q

Wilson disease - 联想House里那个误诊为精神分裂的病例 病因，表现, Rx?

A

肝脏无法排除coppor， 在肝、脑、肾脏和关节里沉积 Copper is BAD as Hell: C: ↓ ceruloplasmin, cirrhosis, corneal deposits (Kayser-Fleischer rings), Copper accumulation, Carcinoma B: basal ganglia degeneration (parkinsonian sym) A: asterixis D: dementia, dyskinesia, dysarthria H: hemolytic anemia Rx: Penicillamine 青霉胺， trientine 曲恩汀

66
Q

hemochromatosis： cause? gene mutation? association? Cx? Rx?

A

cause: iron (hemosiderin) deposit if 1°: AR, C282Y or H53D mutation of HFE gene if 2°: chronic transfusion therapy (beta-thalassemia) ↑ ferritin, iron, ↓ TIBC → ↑ transferrin saturation 【注意：iron loss thru menstruation slows down progression in women] association: HLA-A3 Cx - 三联症 (CDS: cirrhosis, diabetics, skin pigmentation [“bronze diabetes”- Gray’s anatomy里有这个病例】 Rx: repeated phlebotomy, deferoxamine 去铁胺， deferasirox

67
Q

acute pancreatitis: cause? Cx? Labs (which enzyme is more specific)? complication?

A

cause: GET SMASHED Gallstones, ethanol trauma steroids mumps autoimmune disease scorpion sting hypercalcemia (注意：高钙血症会引起急性胰腺炎，但是炎症发生之后因为Ca2+ collects in pancreatic calcium soap depositis, 反而会表现为低钙血症） hypertriglyceridemia (> 1000 mg/dL), ERCP (endoscopic retrograde cholangiopancreatogram, used to diagnose and treat conditions of the bile ducts and main pancreatic duct) drugs (e.g., sulfa drugs) Cx: epigastrci pain, radiating to back, anorexia, nausea Labs: ↑ amylase, lipase (more specific) complications: pseudocyst, DIC, hypocalcemia, ARDS (Acute respiratory distress syndrome ), diffuse fat necrosis, hemorrhage, infection, multiple organ failure

68
Q

chronic pancreatitis: cause? Cx? Labs (和急性胰腺炎鉴别)? CT所见？

A

cause - alcohol abuse, idiopathic Cx - pancreatic insufficiency (steatorrhea, Vit DAKE deficiency (fat-soluble), ↑ pancreatic adenocarcinoma Labs (和急性胰腺炎鉴别): amylase and lipase may or may not ↑ (acute: always ↑) CT所见: atrophy and calcification of the pancreas

69
Q

Misoprostol的机制？用于哪些疾病？

A

synthetic PGE1 analog, 1. prevent NSAID-induced peptide ulcers: 作用于parietal cells - GPCR - inhibit Adenyl cyclase - ↓ cAMP - inhibit gastric acid synthesis 2. maintain PDA 3. induce labor (所以在有可能怀孕的妇女中不能用）

70
Q

H2 blockers代表药物？治疗胃溃疡的机制？毒副作用？

A

H2 blockers代表药物： “Take H2 blockers before you DINE. Table for 2” cimetidine, ranitidine, famotidine, nizatidine 治疗胃溃疡的机制: reversibly inhibit histamine H2 receptors → ↓H+ secretion by parietal cells 毒副作用(主要是cimentidine, ranitidine)： 1） cimentidine: P450 potent inhibitor 2） cimentidine: anti-androgen (prolactin release, gynecomastia, impotence, ↓ libido in male) 3) can cause BBB (confusion, dizziness, headaches) and placate 4) ↓ renal excretion of creatinine

71
Q

prazole是什么类型药物？Mechanism? Cx? toxicity?

A

proton pump inhibitors (pr = Proton) Omeprazole, lansoprazole, esomeprazole, pantoprazole, dexlansoprazole, Mechanism: inreversibly inhibit H+/K+ ATPase in stomach parietal cells Cx: peptic ulcer, gastritis, esophageal reflux, Zollinger-Ellison syn toxicity: ↑ risk of C. difficile infection, pneumonia, hip fracture, ↓ serum Mg2+ with long term use

72
Q

antiacid代表药物？各自的副作用？

A

所有的抗酸剂都可引起hypokalemia 1. aluminum hydroxide 氢氧化铝: constipation (minimum of feces), hypophosphatemia; proximal muscle weakness osteodystrophy, seizures 2. calcium carbonate: hypercalcemia, rebound acid ↑ can chelate and ↓ other drugs’ effectiveness (eg. tetracycline) 3. Magnesium hydroxide: diarrhea (Mg = must go to the bathroom) hyporeflexia, hypotension, cardiac arrest

73
Q

sulfasalazine: 机制？治疗什么疾病？

A

水杨酸偶氮磺胺吡啶 a combination of sulfapyridine (antibacterial) and 5-aminosalicylic acid (anti-inflammatory) activated by colonic bacteria 用于：Crohn disease, and 溃疡性结肠炎

74
Q

ondansetron: 机制？治疗什么疾病? toxicity?

A

奥坦西隆,枢复宁 5-HT3受体拮抗药, ↓ vagal stimulation, 强效中枢性镇吐药 用于控制手术后或者化疗病人呕吐 “at a party but feeling queasy? Keep on dancing with ondansetron!” toxicity: headache, constipation

75
Q

Metoclopramide： 机制？治疗什么疾病? toxicity?

A

甲氧氯普胺,灭吐灵 D2 receptor antagnoist: ↑ resting tone, contractility, LES tone, motility (所以可以用于治疗食管、胃动力不足等） 治疗什么疾病： 1）diabetic and post-surgery gastroparesis 2) antiemetic 止吐药 toxicity： 1) ↑ parkinsonian effects (contradicted in pts with small bowel obstruction or PD); 2) restlessness, drowsiness, fatigue, depression, nausea, diarrhrea, 3) drug interaction with digoxin and diabetic agents

76
Q

O157:H7 Shiga-like toxin-producing E.coli strains和别的E. Coli有2大区别：

A

1）do NOT ferment orbital -containing MacConkey agar, (different from other other ~ 80% E. coli strains) 2） do not produce glucuronidase

Step 1 notes (22 decks)

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