Clinical Summary B9402030 陳建佑 B9402032 陳冠豪 B9402039 郭震亞

Clinical Summary

B9402030 陳建佑 B9402032 陳冠豪B9402039 郭震亞

Pretransplantation history 1st 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

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

Pretransplantation history

各種檢驗 Endoscopic exam

Congestive gastropathy CT scan

Hepatosplenomegaly Cardiac echography

Mild MR 、 adequate LV performance EKG

normal

Pretransplantation history

Liver biopsy ~ 6/30/1995 Consistent with primary biliary cirrhosis Hemochromatosis had to be ruled out

Candidate of liver transplantation

Pretransplantation history

2nd 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

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(-)

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L t t R n r’s sout onac a e i ge i清洗肝臟Portal vein 相接NaHCO3 、 CaCl2 注射

Transplantation History

手術過程： 鬆開止血夾Sudden cardiac arrestSudden cardiac arrest

Two shuts of Bosmin

Heart rate resumed

PCWP > 60 mmHg, PCWP > 60 mmHg,

Systolic pressure ~ 200 mmHgSystolic pressure ~ 200 mmHg

Liver congestion, stony hard and dark red

Transplantation History

手術過程：Nipride infusion30 min 後血壓下降Liver softer and color became betterhepatic artery and BCD 相接

Total ischemic time ~ 11 hrs Total blood loss ~ 800 c.c Liver biopsy showed necrosis on both lobes

Treatment after transplantation

Date:10/15~11/1,1995 Immunosuppression regimen cyclosporine, steroid, imuran, FK506 with Okt3

Prostaglandin E1:for the preservation injury (Oct.15~Oct.20)

Liver started to make better bile from Oct.20

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

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)

但 Doppler echography 不支持診斷 ( 未發現Liver abscess)

Changes of conditions in patients

Seizure at 10:10AM, Oct 28. Blood sugar 80mg% 治療 : 給予 anticonvulsant 與 insulin Liver function went down progressive jaundice, elevated enzyme

CT scan was hold because of patient’s poor condition

Changes of conditions in patients

病情不見好轉 Strong antibiotics were given vancomycin, imiperem, metronidazole, amphotericin 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

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

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 Ca2+ 濃度 , 防止 hyperkalemia

Bosmin

成份Epinephrine

用途Cause bronchodilation and vasoconstriction

Nipride

成份Sodium nitroprusside<Na2[Fe(CN)5NO]·2H2O> 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

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…..

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

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

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

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

Anticonvulsant

Antiepileptic drugs 統稱 , 簡稱 AEDs( 抗癲癇藥 )

具多種機制 Voltage-gated sodium channelsComponents of the GABA system, including GABA receptors, the GAT-1 GABA transporter, GABA transaminaseVoltage-gated calcium channels

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 .

Metronidazole

Nitroimidazole anti-infective medication 治療 anaerobic bacteria, protozoa 感染

Amphotericin B

Antifungal antibiotic 治療 deep mycotic infections

及 cutaneous 和 mucocutaneous candidiasis

Gross findings

1.Cardiovascular system Mild atheromatous change

2.Respiratory system

Diffuse consolidation and edema

3.Pancreas

Profound fat necrosis and hemorrhage Hematoma in retroperitoneum with infection

3.Pancreas

Profound fat necrosis and hemorrhage Hematoma in retroperitoneum with infection

3.Pancreas

Profound fat necrosis and hemorrhage Hematoma in retroperitoneum with infection

3.Pancreas

Profound fat necrosis and hemorrhage Hematoma in retroperitoneum with infection

4.Liver

Patchy hemorrhage infarct

5.Spleen

Splenomegaly with marked congestion Several patches of red infarct

5.Spleen

Splenomegaly with marked congestion Several patches of red infarct

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

MICRO FINDING

B9402034 陳怡婷B9402043 張琬菁

Acute pancreatitis

Edema

Fat necrosis/Saponification

Hematoma

Cardiovascular system

Atherosclerosis with plauqe

Internal elastic layer

Fibrous cap

necrotic center

Liver

Severe cholestasis

Centrilobular necrosis

Other organ

Esophagus varice

Splenic Infart

Peripheral hemorrhage

Spleen Coagulation necrosis

Normal with Congestion

Infarct with Hemorrhage

Lungtype II pneumocyte hyperplasia

Lunghyaline membrane V.S. edema

Lungfungal hyphae with angioinvasion

Lung ： Aspiration Pneumonia with foreign bodies

Final Anatomical Diagnosis

B9402036 陳佑丞B9402031 陳奕廷

(1) Pulmonary edema

(2) Pleural effusion

(3) Aspiration pneumonia

(4) Focal angioinvasive aspergillosis

(5) Tracheal ulcer

(6) Epicardial hemorrhage

(7) Atherosclerosis

(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

(1) Pulmonary edema

History(1):

第一次看診時檢查就有 mild MR

Left heart failure

Pulmonary congestion

Pulmonary edema

(1) Pulmonary edema

History(2):

While unclamping, sudden cardiac arrest

給予 Bosmin

H.R. resumed, but PCWP↑

pulmonary edema

(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

(1) Pulmonary edema

History(4):

Acute pancreatitis

septicemia

pulmonary vascular permeability↑

pulmonary edema

(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

(2) Pleural effusion

History:

MR, PCWP↑, aspiration pneumonia,

septicemia

Pleural effusion

(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

(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

(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

(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

(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

(4)Focal angioinvasive aspergillosis

History(1):

術後使用免疫抑制劑 : cyclosporine,

steroid, imuran

immunity↓

aspergillosis with angioinvasion

(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 necorsisUlcer

(5) Tracheal ulcer

History:

1. 1st Admission 之前，已有 vomiting 嘔吐物刺激氣管 inflammationUlcer

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 infectioninflammationUlcer

(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 bosminPCWP up to greater than 60mmhg ； Systolic pressure>200mmhg

coronary a. Pressure pericardiacophrenic a. Pressure

(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 hemorrhage2. 病人已 AtherosclerosisCoronary a.粥狀硬化 MIepicardial hemorrhage

(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

(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

Final Anatomical Diagnosis(2)

B9402037 陳名揚B9402033 陳長聖B9402035 陳京瑜

(1)Liver hemorrhage infarct, centrilobular necrosis, and cholestasis InfarctCentrilobular necrosisCholestasis 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

(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。

(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

(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↑

(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

(2)Spleen, hemorrhagic infarct

History(1): Before transplantation, CT scan showed

hepatosplenomegaly. (Caused by primary biliary cirrhosis)

Portal tract lesion

Portal hypertension

Splenomagely

(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

(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

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

(4)Generalized cholestasis and bile casts

定義： Bile pigment accumulates within the hepatic parenchyma

原因： Hepatic dysfunction Biliary obstruction

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

History 2: Oct. 28 liver function deteriorating

→ cholestasis

(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

(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

(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

(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？

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.