肝硬化与肝肿瘤课件

肝硬化與肝腫瘤 內科核心課程 內科專科考試重點提示 vComplication of liver cirrhosis Encephalopathy Hepatorenal syndrome Portal hypertension, collateral circulation Ascites, SBP Causes of Cirrhosis vAlcohol vViral hepatitis vBiliary obstruction vVeno-occlusive disease vHemochromatosis vWilsons disease vAutoimmune vDrugs and toxins vMetabolic diseases vIdiopathic 九十一年度內科專科醫師考試筆試題 下列各項敘述,何者錯誤? vA.年輕的肝硬化患者,應考慮是否為Wilsons disease vB.肝硬化患者,經過有經驗的眼科醫師以slit lamp檢查,未發現 Kayser-Fleischers ring,則可排除Wilsons disease vC.Wilsons disease患者之血清ceruloplasmin濃度通常會降 低 vD.Wilsons disease是以autosomal recessive方式遺傳 vE.Wilsons disease患者接受penicillamine治療時,應同時服 用pyridoxine 九十一年度內科專科醫師考試筆試題 有關原發性膽道性肝硬化 (primary biliary cirrhosis) 之敘述,何 者為誤? (1) 抗粒腺體抗體 (AMA) 通常陽性 (2) 好發於女性 (3) 常合併發生於慢性潰瘍性結腸炎患者 (4) 發炎性纖維化侵犯肝內及肝外膽管 (5) ERCP 或 MRCP 為確定診斷方式 vA.1 + 2 + 3 vB.2 + 3 + 4 vC.3 + 4 + 5 vD.3 + 4 vE.4 + 5 DIAGNOSIS vDefinitive diagnosis is by biopsy or gross inspection of liver vNoninvasive methods include US, CT scan, MRI vIndirect evidence - esophageal varices seen during endoscopy 93 內專考題 醫生發現病人胸前有spider angiomata,關於其後續安 排檢查之敘述,何者錯誤? vA.抽血檢驗GOT及GPT即可確立其診斷 vB.安排腹部超音波檢查 vC.若高度懷疑有肝硬化,應檢驗CBC及platelet count vD.若高度懷疑有肝硬化,應檢驗prothrombin time vE.若高度懷疑有肝硬化,胃鏡檢查有其必要 COMPENSATED CIRRHOSIS DECOMPENSATED CIRRHOSIS Development of cirrhosis Development of complications Orthotopic liver transplant (OLT) Death Median survival 10 years Median survival 2 years Gines et al. Hepatology 1987. PROBABILITY OF DEVELOPING DECOMPENSATED CIRRHOSIS 257 patients with compensated cirrhosis 1)Ascites* 2)Jaundice 3)Encephalopathy* 4)Variceal hemorrhage* *result from portal hypertension VARICES CIRRHOSIS Mesenteric vessels flow Splanchnic vasodilatation SINUSOID SPLENO- MEGALY SINUSOID resistance portal pressure Effect of Hepatitis B and C Virus Infections on the Natural History of Compensated Cirrhosis: A Cohort Study of 297 Patients Fattovich G et al, Am J Gastro 2002;97:2886 Median f/u: 6.5 years Risks of Complications of Cirrhosis Cirrhosis Variceal Bleeding HCC Ascites Encephalopathy adapted from Bennett WG et al, Ann Intern Med 1997;127:855 0.4 % 1.5% 2.5 % 1.1% percent per year Death Liver Transplant 11 % ?20+% ?30+% Ascites RoutineOptionalUnusual Total proteinGram stainCytology AlbuminAmylaseAcid-fast bacillus smear and culture Cell count with WBC differential Lactate dehydrogenase Triglycerides Culture in blood culture bottles Glucose Analysis of Ascetics Fluid 93 內專考題 下列有關自發性細菌腹膜炎(SBP)之敘述,何者有誤? vA.與肝衰竭(hepatic failure)程度有關。 vB.血清膽紅素高及腹水蛋白質濃度低者易得。 vC.有時無典型腹膜炎症狀及徵候。 vD.腹水培養有多種細菌生長。 vE.預防性抗生素之使用,可減少復發。 下列有關自發性細菌性腹膜炎之敘述,何者為誤 ? vA.常發生於肝硬化合併腹水患者 vB.腹水內蛋白低且血清膽紅素高者易得 vC.通常為厭氧菌感染(anaerobic microorganism infection) vD.可引發腎功能衰退及肝腦病變 vE.腹水內多形核白血球(PMN)高於250/cmm 92 內專考題 九十一年度內科專科醫師考試筆試題 關於spontaneous bacterial peritonitis (SBP)之敘述, 何者錯誤? vA.抗生素治療,以10-14天為原則 vB.1年內再復發之機率達70% vC.腹水中之蛋白質濃度 1.1g/dL (1)Liver cirrhosis (2)Nephortic syndrome (3)Congestive heart failure (4)Tuberculous peritonitis (5)Fulminant hepatic failure vA.1+2+3 vB.1+2+4 vC.1+3+5 vD.2+3+4 vE.3+4+5 九十年度內科專科醫師考試筆試題 一位病人因發燒及腹脹而住院。理學檢查發現有shifting dullness,且腹部有壓痛及反彈痛之現象。抽取腹水送驗,發現 其內之WBC數目為1000個/L,其中PMN佔80%。下列各項 敘述,何者錯誤? A.本病人之腹膜炎,最可能是Gram-negative細菌所引起的 B.首選抗生素為Cefotaxime加aminoglycoside C.治療期間一般以5天為準 D.腹水中蛋白質濃度小於1g/dL者,應注意腹膜炎之復發 E.食道靜脈曲張(esophageal varices)出血者,較易發生細菌性 腹膜炎 vSAAG = serum albumin ascites albumin 97% accuracy vSAAG 1.1g/dl; portal hypertension Liver cirrhosis, heart failure, mixed ascites, fulminant liver failure. vSAAG 2000 IU/L。 vCytology: 7% positive and only in peritoneal carcinomatosis. vSmear: 0%, culture 50% for TB vAscites culture: 80% in blood culture bottles 50%30%20% Mechanism of action of different therapies for varices TreatmentFlowResist ance Portal pressure Vasoconstrictors (e.g. -blockers) Venodilators (nitrates) Vasoconstrictors + venodilators Endoscopic therapy TIPS/Shunt surgery Treatment of varices/variceal hemorrhage Portal hypertension No varices Varices No hemorrhage Variceal hemorrhage Recurrent hemorrhage Pre-primary prophylaxis Primary prophylaxis Control of hemorrhage Secondary prophylaxis Can the development of varices be prevented? The risk of first bleeding is not reduced by adding ISMN to -blockers (BB) .121 8 0.55 (0.23,1.31) Merkel (1996) 21.9 0.62 (0.32,1.22) DAmico (2003) 35.8 0.99 (0.54,1.85) Garcia-Pagan (2003) 42.3 0.74 (0.49,1.11) Overall (95% CI) 0.72 (0.38,1.35) Large varices Better ISMN + BB Better BB alone DAmico G. EASL 2003. Management of compensated cirrhosis Small varices No hemorrhage Med/ Large varices No hemorrhage No varices Repeat endoscopy in 2-3 years Repeat endoscopy in 1-2 years Endoscopy at diagnosis of cirrhosis 1) Non-selective -blockers (propranolol, nadolol) - Dose adjusted to HR 55-60 bpm - Continued indefinitely 2) EVL in patients who cannot take -blockers Budd-Chiari Syndrome vCaused by hepatic venous obstruction vAt the level of the inferior vena cava, the hepatic veins, or the central veins within the liver itself vresult of congenital webs (in Africa and Asia), acute or chronic thrombosis (in the West), and malignancy Caput Medusae 九十三年度內科專科醫師考試筆試題 一位患者有間歇性兩側下肢水腫,身體檢查發 現腹部有側枝循環,位於肚臍兩側如圖53, 其血流由下腹部流向上腹部,敲診無腹水 。下列敘述何者正確? vA.MRA(magnetic resonance angiography)有助於確立診斷。 vB.腹部超音波檢查對本病人之診斷沒有幫 助。 vC.使用loop diuretics有助於消除本病人之 症狀。 vD.此種病人常會發生心臟衰竭。 vE.建議病人接受TIPS(transjugular intrahepatic portosystemic shunting)。 病人因吐血被送至急診室,初步處置後接受內視鏡檢查,被發現 有食道靜脈曲張出血。你是這位病人的主治醫師,幫病人安排 腹部超音波檢查,請問你希望執行腹部超音波檢查之醫師幫你 証實下列各種可能的病況,除了: vA.肝硬化 vB.上腸繫膜靜脈(superior mesenteric vein)狹窄 vC.肝門靜脈栓塞(portal vein thrombosis) vD.肝動脈與肝門靜脈異常交流,導致肝門靜脈血流逆流 vE.肝靜脈(hepatic vein)及下腔靜脈(inferior vena cava)堵塞 或異常,即所謂Budd-Chiari syndrome 九十二年度內科專科醫師考試筆試題 Hepatic encephalopathy Pathogenesis Theories vEndogenous Neurotoxins Ammonia Mercaptans Phenols Short-medium fatty acids vIncreased Permeability of Blood-Brain Barrier vChange in Neurotransmitters and Receptors GABA Altered BCAA/AAA ratio vOther Zinc defficiency Manganese deposits Drugs Benzodiazepines Narcotics Alcohol Increased Ammonia Production, Absorption or Entry Into the Brain Excess Dietary Intake of Protein GI Bleeding Infection Electrolyte Disturbances (ie., hypokalemia) Constipation Metabolic alkalosis Dehydration Vomiting Diarrhea Hemorrhage Diuretics Large volume paracentesis Portosystemic Shunting Radiographic or surgically placed shunts Spontaneous shunts Vascular Occlusion Portal or Hepatic Vein Thrombosis Primary Hepatocellular Carcinoma Precipitants of Hepatic Encephalopathy Pathogenesis Theories: Change In Neurotransmitters and Receptors vGamma-Aminobutyric Acid (GABA) vBCAA-Ammonia Connection 93 內專考題 有關肝腦病變(Hepatoencephalopathy)之陳述,下列何 者有誤? vA.血氨濃度增加。 vB.中樞神經GABA濃度降低。 vC.使用Flumazenil (Anexate)可能有效。 vD.基底核(Basal ganglia)內,錳過度沈積,為致病因子 之一。 vE.電解質不平衡,尤其低鉀血症為一導致因素。 95 內專考題 肝硬化患者突然發生hepatic encephalopathy,可能是 因為下面各種狀況,除了 vA.肝炎急性發作(acute exacerbation) vB.過度使用loop diuretic vC.胃腸道出血 vD.腹瀉 (45次天) vE.攝食過多蛋白質 95 內專考題 一位病患因意識不清而住院,檢驗結果發現血中ammonia數值明顯升高 ,Alb=3.6g/dL(3.5), Bil(T)/(D)=1.1/0.4mg/dL,GOT=35(2.5mg/dl in 2 weeks): median survival 2 weeks vType II: slower rise, Cr 1.5mg/dl v Management: Ensure Diagnosis Liver Transplantation 肝硬化患者併發腹水,住院中被發現其尿量日漸減少,且血 清BUN及Cr慢慢升高,hepatorenal syndrome被高度懷 疑。下列各項檢驗結果,那一項不符合hepatorenal syndrome之診斷? vA.Urine sodium concentration 20 mmol/L vB.Urine / plasma creatinine ratio 40:1 vC.Urine osmolality 較 plasma osmolality高100 mOsm 以上 vD.FENa (fractional excretion of sodium) 20:1 九十一年度內科專科醫師考試筆試題 Differential Diagnosis Prerenal Azotemia HRSAcute Renal Failure Urine Na (mEq/L) 30 Urine/Plasma Creatinine ratio 30:1 plasma 100 mOsm plasma Equal to plasma Urine sedimentNormalUnremarkable Casts, Cellular debris Hgb: 9 Wbc: 3.0 Plt: 85 Bun: 10 Cr: 1.0 Lytes: nl AP: 150 Tbili: 1.7 Alb: 2.6 AST: 60 Dbili: 1.0 ALT: 50 Tprot: 5.5 A 63 y/o woman has a 3-month history of abdominal distention and 4.5 Kg weight gain despite maintaining her normal diet. She denies abdominal discomfort or a change in bowel habits. She has chronic hepatitis C contracted from a blood transfusion