1. Clinical Summary B9402030 陳建佑 B9402032 陳冠豪 B9402039 郭震亞

2. Pretransplantation history 1 st admission: 6/21~7/8, 1995 女性， 44 歲 主訴病情： 黃疸、茶色尿將近 1 年 病程： 從 1994 年 9 月開始，黃疸、噁心、嘔吐、食慾不振、色素 沉積漸漸明顯

3. Pretransplantation history 各種檢驗 Hemogram Hb 、 Ht 、 MCV 、 MCH 、 MCHC Biochemistry GOT/GPT 、 ALP 、 rGT 、 Bilirubin(D,T) 、 Albumin 、 Cholesterol 、 Triglyceride 、 Ferritin 、 Ceruloplasmin Bone marrow study Erythroid hyperplasia 、 absent iron store

4. Pretransplantation history 各種檢驗 Endoscopic exam Congestive gastropathy CT scan Hepatosplenomegaly Cardiac echography Mild MR 、 adequate LV performance EKG normal

5. Pretransplantation history Liver biopsy ~ 6/30/1995 Consistent with primary biliary cirrhosis Hemochromatosis had to be ruled out Candidate of liver transplantation

6. Pretransplantation history 2 nd admission: 7/19~7/24, 1995 (from ER) 原因： 7/15 conscious disturbance attack, after rice wine and ativan drug 7/16 consciousness resumed

7. Pretransplantation history Liver function worsened GOT/GPT : 139/64 ALP : 912 Bilirubin(D/T) : 26.8/56.3 rGT : 196 Prothrombin 14.3/11.5 排除病毒性肝炎 HBsAg (-) 、 AFP<3 、 anti-δ(-) 、 anti-HCV(-)

8. Transplantation History 手術時間： 10/13 AM 手術過程： 接受者肝臟摘除  肝臟 SVC 和 left, middle hepatic vein 相接  Lactate Ringer’s solution 清洗肝臟 Lactate Ringer’s solution  Portal vein 相接  NaHCO 3 、 CaCl 2 注射 NaHCO 3 、 CaCl 2

9. Transplantation History 手術過程：  鬆開止血夾  Sudden cardiac arrest  Two shuts of BosminBosmin  Heart rate resumed  PCWP > 60 mmHg, Systolic pressure ~ 200 mmHg Systolic pressure ~ 200 mmHg  Liver congestion, stony hard and dark red

10. Transplantation History 手術過程：  Nipride infusion Nipride  30 min 後血壓下降  Liver softer and color became better  hepatic artery and BCD 相接 Total ischemic time ~ 11 hrs Total blood loss ~ 800 c.c Liver biopsy showed necrosis on both lobes

11. Treatment after transplantation Date:10/15~11/1,1995 Immunosuppression regimen cyclosporine, steroid, imuran, FK506 with Okt3 cyclosporineimuranFK506Okt3 Prostaglandin E1:for the preservation injury (Oct.15~Oct.20) Liver started to make better bile from Oct.20

12. Changes of conditions in patients Clear consiousness and stable vital signs at immediate postop period Serum test: WBC increased, bilirubin 41.3mg% Kidney function went down Abdominal distention 檢驗 : Chest X-ray (on Oct.23) right pleural effusion, pneumonic patch on right lung 治療 : 給予 vancomycin

13. Changes of conditions in patients Tachycardia on Oct.26 Metabolic acidosis 診斷 : 懷疑是 sepsis 治療 : 1. Intubation 2. CVVHD (Oct 27)CVVHD 但 Doppler echography 不支持診斷 ( 未發現 Liver abscess)

14. Changes of conditions in patients Seizure at 10:10AM, Oct 28. Blood sugar 80mg% 治療 : 給予 anticonvulsant 與 insulin anticonvulsant Liver function went down progressive jaundice, elevated enzyme CT scan was hold because of patient’s poor condition

15. Changes of conditions in patients 病情不見好轉 Strong antibiotics were given vancomycin, imiperem, metronidazole, amphotericin Bimiperemmetronidazoleamphotericin B 但未找到感染原因 免疫抑制劑劑量下降至 2mg of FK 506 Blood pressure dropped Cardiac arrest on Nov.1 The patient passed away at 7:45 PM

16. Chilled Lactate Ringer’s solution 成份 NaCl (sodium chloride) NaC3H5O3 (sodium lactate) CaCl2 (calcium chloride) KCl (potassium chloride) 用途 在手術中作體液補充用, 加 lactate 是為了中和 acute fluid loss or renal failure 所發生的酸中 毒

17. Sodium Bicarbonate Calcium Chloride Given Before Unclamping Research shows there’s a rise in potassium level and a drop in pH at reperfusion. 用途 Sodium Bicarbonate  防止 acidosis Calcium chloride  維持正常 Ca 2+ 濃度, 防止 hyperkalemia

18. Bosmin 成份 Epinephrine 用途 Cause bronchodilation and vasoconstriction

19. Nipride 成份 Sodium nitroprusside serves as a source of nitric oxide 用途 作用在 arteriole 和 venule, 緊急降低血壓 副作用 CN + thiosulfate  thiocyanate 由腎臟排除 CN intoxication ( 短期 ) 昏迷, 癲癇 Thiocyanate intoxication ( 長期 ) 嗜睡, 意識混亂

20. Cyclosporine 成份 11 個氨基酸的 cyclo-polypeptide 用途 一種免疫抑制劑 抑制 Th, 以及 IL-2, IL-3, INF-γ 轉錄 它並不抑制造血, 而且對吞噬細胞的功能沒有影響 副作用 peptic ulcers, pancreatitis, fever, vomiting, diarrhea, confusion, breathing difficulties…..

21. Imuran 成份 Azathioprine 用途 A purine synthesis inhibitor, inhibiting the proliferation of cells, especially leukocytes. 副作用 Nausea, fatigue, hair loss, and rash

22. FK506 成份 Tacrolimus; a macrolide antibiotic 巨分子抗生素 用途 免疫抑制劑, inhibits both T-lymphocyte signal transduction and IL-2 transcription 副作用 liver and kidney problems, seizures, tremors, hypertension, hypomagnesemia, diabetes mellitus, hyperkalemia, hyperglycemia, potentially increasing the severity of existing fungal infectious conditions

23. Okt3 成份 A murinemonoclonal IgG2a antibody 用途 免疫抑制劑, antigen 會辨認 T cell 上負責傳遞 foreign antigen 訊息, proliferation, 攻擊 foreign Ag 的 CD-3 complex 結合, 使之失效 副作用 pyrexia, myalgia, nausea and diarrhea

24. CVVHD Continuous Venous-Venous HemoDialysis 連續性腎替代性治療 (CRRT) 的一種 常用在休克及多種器官衰竭合併急性腎衰竭的 病患以達到降低尿毒、移除水分及供給靜脈營 養注射的目的

25. Anticonvulsant Antiepileptic drugs 統稱, 簡稱 AEDs( 抗癲癇藥 ) 具多種機制  Voltage-gated sodium channels  Components of the GABA system, including GABA receptors, the GAT-1 GABA transporter, GABA transaminase  Voltage-gated calcium channels

26. Imipenem 廣效性抗生素 Aerobic and anaerobic Gram(+) as well as Gram(-) bacteria 抗 Pseudomonas aeruginosa 以及 Enterococcus species 著名.

27. Metronidazole Nitroimidazole anti-infective medication 治療 anaerobic bacteria, protozoa 感染

28. Amphotericin B Antifungal antibiotic 治療 deep mycotic infections 及 cutaneous 和 mucocutaneous candidiasis

29. Gross findings

30. 1.Cardiovascular system Mild atheromatous change

31. 2.Respiratory system Diffuse consolidation and edema

32. 3.Pancreas Profound fat necrosis and hemorrhage Hematoma in retroperitoneum with infection

33. 3.Pancreas Profound fat necrosis and hemorrhage Hematoma in retroperitoneum with infection

34. 3.Pancreas Profound fat necrosis and hemorrhage Hematoma in retroperitoneum with infection

35. 3.Pancreas Profound fat necrosis and hemorrhage Hematoma in retroperitoneum with infection

36. 4.Liver Patchy hemorrhage infarct

37. 5.Spleen Splenomegaly with marked congestion Several patches of red infarct

38. 5.Spleen Splenomegaly with marked congestion Several patches of red infarct

39. others Body cavity :ascites Heart :unremarkable GI tract :unremarkable Gallbladder and bile duct : abscent Urogenital system : unremarkable Endocrine system : unmarkable Central nervous system : not examined

40. MICRO FINDING B9402034 陳怡婷 B9402043 張琬菁

41. Acute pancreatitis

42. Edema

43. Fat necrosis/Saponification

44. Hematoma

45. Cardiovascular system

46. Atherosclerosis with plauqe Internal elastic layer Fibrous cap necrotic center

47. Liver

48. Severe cholestasis

49. Centrilobular necrosis

50. Other organ

51. Esophagus varice

52. Splenic Infart

53. Peripheral hemorrhage

54. Spleen Coagulation necrosis

55. Normal with Congestion

56. Infarct with Hemorrhage

57. Lung type II pneumocyte hyperplasia

58. Lung hyaline membrane V.S. edema

59. Lung fungal hyphae with angioinvasion

60. Lung ： Aspiration Pneumonia with foreign bodies

61. Final Anatomical Diagnosis B9402036 陳佑丞 B9402031 陳奕廷

62. (1) Pulmonary edema (2) Pleural effusion (3) Aspiration pneumonia (4) Focal angioinvasive aspergillosis (5) Tracheal ulcer (6) Epicardial hemorrhage (7) Atherosclerosis

63. (1) Pulmonary edema 定義 : 肺泡聚積組織液 原因 : 1.Hydrostatic pressure↑ -Mitral regurgitation (MR) -Left heart failure 2.Capillary permeability↑ -pneumonia -septicemia

64. (1) Pulmonary edema History(1): 第一次看診時檢查就有 mild MR  Left heart failure  Pulmonary congestion  Pulmonary edema

65. (1) Pulmonary edema History(2): While unclamping, sudden cardiac arrest  給予 Bosmin  H.R. resumed, but PCWP↑  pulmonary edema

66. (1) Pulmonary edema History(3): 病人主訴自從 83 年 9 月開始就有 nausea, vomiting 等症狀  aspiration pneumonia  pulmonary vascular permeability↑  pulmonary edema

67. (1) Pulmonary edema History(4): Acute pancreatitis  septicemia  pulmonary vascular permeability↑  pulmonary edema

68. (2) Pleural effusion 定義 : 組織液滲出至肋膜腔 原因 : 1.Hydrostatic pressure↑ -Mitral regurgitation (MR) -Left heart failure 2.Capillary permeability↑ -pneumonia -septicemia

69. (2) Pleural effusion History: MR, PCWP↑, aspiration pneumonia, septicemia  Pleural effusion

70. (3) Aspiration pneumonia 定義 : 因吸入嘔吐物 ( 含胃酸 ) 所造成的肺炎, 通常發生在意識不清者, 好犯右肺, 在 CXR 呈現 patch( 一片白 ) 成因 : 胃酸 + 混合性感染 (S. aureus + Gram(-) bacilli)  bronchial (lobular) pneumonia

71. (3) Aspiration pneumonia History(1): 病人主訴自從 83 年 9 月開始就有 nausea, vomiting 等症狀  aspiration pneumonia

72. (3) Aspiration pneumonia History(2): 手術過程中, 病患處於意識不清狀態, 容易吸入 自己嘔吐物  aspiration pneumonia

73. (3) Aspiration pneumonia History(3): 【 84.10.23 】術後胸部 X 光顯示 right pleural effusion with possible pneumonic patch on right lung field

74. (4)Focal angioinvasive aspergillosis 定義 : 由 Aspergillus 感染造成, 常發生在免疫 抑制的人, 會造成 angioinvasion Pathology: -Septated, branching fungal hyphae -Fungus ball

75. (4)Focal angioinvasive aspergillosis History(1): 術後使用免疫抑制劑 : cyclosporine, steroid, imuran  immunity↓  aspergillosis with angioinvasion

76. (5) Tracheal ulcer 定義 : 氣管內黏膜發生局部發炎性壞死 原因 : 手術過程因麻醉導致意識不清  吸入胃酸及消 化道混合物 ( 包含細菌 )  刺激氣管黏膜  inflammatory necorsis  Ulcer

77. (5) Tracheal ulcer History: 1. 1st Admission 之前，已有 vomiting  嘔吐物 刺激氣管  inflammation  Ulcer 2. 於肝臟移植手術中接受麻醉  失去 reflex ，無 法將流進氣管的消化道混合物咳出  infection  inflammation  Ulcer

78. (6)Epicardial hemorrhage 定義 : Epicadium (visceral layer of pericardium) bleeding. 原因 : 1. 病人於肝臟移植手術時，為了排除 cardiac arrest ，注射 two shots of bosmin  PCWP up to greater than 60mmhg ； Systolic pressure>200mmhg  coronary a. Pressure pericardiacophrenic a. Pressure

79. (6)Epicardial hemorrhage coronary a. Pressure pericardiacophrenic a. Pressure 高壓仍維持 30mins( 直到注射之 Nipride 發揮效 用 )  epicardial hemorrhage 2. 病人已 Atherosclerosis  Coronary a. 粥狀硬 化  MI  epicardial hemorrhage

80. (7) Atherosclerosis 定義 : 由於脂肪、血栓、結締組織和碳酸鈣在血管（主 要是動脈，但也包括靜脈）沉積所造成的一種對人體 有害的狀態 原因 : (a)LDL 攜帶之膽固醇堆積於內皮細胞  內皮細胞損傷  Macrophage 聚集，吞吃膽固醇，成為 foam cell ； 平滑肌增生 (b) 形成粥腫 (atheroma) ：由膽固醇、 foam cell 與 collagen 形成 (c) 形成血栓 (thrombosis) ：由血纖維蛋白 + 血球、血小 板

82. (7) Atherosclerosis 危險因子 (risk factors): (1) 高血脂 (2) 高血壓 (3) 糖尿病 (4) 抽菸 (5) 男性 (6) 家族史 (7) 年齡、肥胖、少運動 病人因 PBC 導致肝功能不良  長時間肝功能不良 ；肝臟代謝膽固醇功能失調  膽固醇累積於週邊血管  atherosclerosis History 第一次住院時， Cholesterol 317 Triglyceride 302

83. Final Anatomical Diagnosis(2) B9402037 陳名揚 B9402033 陳長聖 B9402035 陳京瑜

84. (1)Liver hemorrhage infarct, centrilobular necrosis, and cholestasis Infarct  Centrilobular necrosis  Cholestasis Infarction – 動脈或靜脈阻塞所造成的缺血性壞死 Hemorrhage infarct Tissues with dual circulations Tissues previously congested because of sluggish venous outflow Centrilobular necrosis – 灌流不足導致肝末端靜脈區域的壞死 Caused by hypoperfusion. With superimposed passive congestion(right side heart failure), with hemorrhage. Cholestasis – 膽汁鬱積包含 bilirubin, bile salt, cholesterol 的累 積 Hepatocellular dystunction

85. (1)Liver hemorrhage infarct, centrilobular necrosis, and cholestasis History(1): 手術中血壓攀升， liver congestion  centrilobular necrosis 縫合前的 biopsy 顯示 liver 有 necrosis 。

86. (1)Liver hemorrhage infarct, centrilobular necrosis, and cholestasis History(2): Oct. 26 產生敗血 1. peritoneum sepsis 會造成 portal vein thrombosis 2. Liver 灌流不足  centrilobular necrosis

87. (1)Liver hemorrhage infarct, centrilobular necrosis, and cholestasis History(3) Oct. 28 liver function deteriorated 持續的壞死造成 progressive jaundice & enzyme↑

88. (2)Spleen, hemorrhagic infarct Splenic infarcts – In splenomegaly Blood supply can’t keep up with the increased demands of spleen. Congestive states related to portal hypertesion

89. (2)Spleen, hemorrhagic infarct History(1): Before transplantation, CT scan showed hepatosplenomegaly. (Caused by primary biliary cirrhosis) Portal tract lesion  Portal hypertension  Splenomagely

90. (2)Spleen, hemorrhagic infarct History(2) Oct. 26 產生敗血  peritoneum sepsis 會造成 portal vein thrombosis  Portal hypertension  Splenomagely  hemorrhagic infarct

91. (3)Ascites, turbid, 2000c.c. 定義： The collection of excess serous fluid in the peritoneal cavity. 原因： Hepatic sinusoidal hypertension Percolation of hepatic lymph into the peritoneal cavity

92. History 手術時打兩針 Bosmin ，造成 PCWP 過高 → 右心壓力高 → 肝臟 congestion 、壓力高 → 腹水

93. (4)Generalized cholestasis and bile casts 定義： Bile pigment accumulates within the hepatic parenchyma 原因： Hepatic dysfunction Biliary obstruction

94. History 1: → 手術時肝臟高壓超過 30 分鐘 → Hepatic necrosis, worse on left side → cholestasis

95. History 2: Oct. 28 liver function deteriorating → cholestasis

96. (5)Esophageal varices, mild 定義： extremely dilated sub-mucosal veins in the esophagus. 原因： Liver cirrhosis  long term hypertension of portal vein  側枝循環的形成 血流：胃的 coronary vein  食道的 sub-mucosal veins  azygos veins

98. (5)Esophageal varices, mild History: June 30, 1995. Liver biopsy 顯示出病人有 primary biliary cirrhosis 會造成 liver cirrhosis ，故有 esophageal varices

99. (6)Acute pancreatitis, severe, (with huge retroperitoneal hematoma) 定義： a sudden inflammation of the pancreas. It can have severe complications and high mortality despite treatment. 原因： 胰蛋白酶 (trypsin) 在胰臟中被活化  tissue proteolysis, lipolysis, hemorrhage(  hematoma) 膽結石塞到 Ampulla of Vater 是常見的原因 Acinar cell 被破壞是可能的原因，缺血和外傷都會 破壞 acinar cells

100. (6)Acute pancreatitis, severe, (with huge retroperitoneal hematoma) History: During the surgery, total ischemic time was about 11 hours 醫原性傷害？開刀過程中的傷害？

101. Final Comments The cause of death is due to acute pancreatitis. Huge retroperitoneal hematoma with infection caused mutiple organ failure Acute pancreatitis may be related to the major operation (cardiac arrest) The anastomosis sites of liver transplantation were good, and there was no obvious rejection.