31: Liver Flashcards
1
Q
Which kind of liver abscess usually appears as a single abscess in the right lobe of the liver and is associated with elevated LFTs?
A
Amebic liver abscess
[UpToDate: Amebic liver abscesses are most commonly found in the right lobe; 70% to 80% are solitary subcapsular lesions, although multiple lesions can be present. Localization in the left lobe predisposes to extension into the pericardial sac.
On ultrasound, the abscess appears as a round, well-defined hypoechoic mass. On CT scan, it appears as a low-density mass with a peripheral enhancing rim. On MRI, the abscess appears as low-signal intensity on T1-weighted images and high-signal intensity on T2-weighted images. After healing, the periphery of the abscess may calcify as a thin, round ring.
On gallium citrate and technetium-labeled sulfur colloid radionuclide liver scans, amebic abscesses are “cold” (with a bright rim in some cases), whereas pyogenic abscesses are “hot.” Radiographic findings must be interpreted in the appropriate clinical context with consideration of the differential diagnoses, including pyogenic abscess and malignancy.
Serial imaging is generally not helpful since lesions may appear to increase in size or number on ultrasound following initiation of treatment, even with appropriate therapy and clinical improvement. Treated lesions may become anechoic, calcified, or may persist as cystic-appearing lesions. Complete radiologic resolution may take two years or more. Therefore, persistent abnormalities on ultrasound imaging should not prompt retreatment or additional testing in a patient who is clinically well.
A chest radiograph abnormality will be observed in approximately 50% of patients with an amebic liver abscess, most commonly elevation of the right hemidiaphragm. This finding does not necessarily signal pulmonary involvement in the infection.]
2
Q
What is the sequence of breakdown products of hemoglobin?
A
Hemoglobin -> Heme -> Biliverdin -> Bilirubin
3
Q
What is the treatment for focal nodular hyperplasia of the liver?
A
Conservative therapy (No resection)
[UpToDate: The natural history of FNH is one of stability and lack of complications. Lesions generally do not change over time, although they occasionally become smaller. However, as mentioned above, enlargement of FNH in the setting of OCPs and during pregnancy have been reported. There is no evidence for malignant transformation of FNH.
Patients who are suspected of having FNH based upon the evaluation described above should be managed conservatively. If a diagnosis remains unclear, a liver biopsy may be helpful, but may also be misleading since only resection will be definitive. Follow-up studies at three and six months will often be sufficient to confirm the stability of the lesion and its benign nature, after which no long-term follow-up is required routinely. Surgery should be reserved for the rare, very symptomatic FNH lesion, and the highly suspicious lesion, which has eluded diagnosis by all other modalities.
We generally do not insist that oral contraceptives and other estrogen containing preparations should be discontinued. However, it is reasonable to obtain a follow-up imaging study in 6 to 12 months in women who continue taking these drugs. Small FNH do not appear to pose a significant risk to a successful pregnancy, although close observation is strongly recommended and resection may be prudent for large (>8 cm) FNH.]
4
Q
Diagnose:
- HBsAg: -
- Anti-HBs: +
- HBeAg: -
- Anti-HBe: -
- Anti-HBc: -
A
Immunized against HBV
5
Q
According to Couinaud’s nomenclature, which numbered liver segment is the superior right posterolateral segment?
A
Segment VII
6
Q
What is the treatment for hepatorenal syndrome?
A
Stop diuretics and give volume
[No good therapy other than a liver transplant]
[UpToDate: The ideal therapy for hepatorenal syndrome is improvement of liver function from recovery of alcoholic hepatitis, treatment of decompensated hepatitis B with effective antiviral therapy, recovery from acute hepatic failure, or liver transplantation. The ability of liver function to improve with abstinence from alcohol and effective antiviral therapy of hepatitis B is remarkable.
However, when improvement of liver function is not possible in the short term, we recommend that medical therapy be instituted in an attempt to reverse the acute kidney injury associated with hepatorenal syndrome. Our suggestions regarding the choice of medical therapy depend upon several factors, including: whether the patient is admitted to the intensive care unit; the availability of certain drugs, for which there is national and regional variability; and whether the patient is a candidate for liver transplantation:
In patients with hepatorenal syndrome who are critically ill, we suggest initial treatment with norepinephrine in combination with albumin. Norepinephrine is given intravenously as a continuous infusion (0.5 to 3 mg/hr) with the goal of raising the mean arterial pressure by 10 mmHg, and albumin is given for at least two days as an intravenous bolus (1 g/kg per day [100 g maximum]). Intravenous vasopressin may also be effective, starting at 0.01 units/min and titrating upward as needed to raise the mean arterial pressure as noted below.
In patients with hepatorenal syndrome who are not critically ill, our suggestions depend upon the availability of certain drugs:
- Where terlipressin therapy is available, we suggest initial treatment with terlipressin in combination with albumin. Terlipressin is given as an intravenous bolus (1 to 2 mg every four to six hours), and albumin is given for two days as an intravenous bolus (1 g/kg per day [100 g maximum]), followed by 25 to 50 grams per day until terlipressin therapy is discontinued.
- Where terlipressin therapy is not available (principally the United States), we suggest initial treatment with a combination of midodrine, octreotide, and albumin. Midodrine is given orally (starting at 7.5 mg and increasing the dose at eight-hour intervals up to a maximum of 15 mg by mouth three times daily), octreotide is either given as a continuous intravenous infusion (50 mcg/hr) or subcutaneously (100 to 200 mcg three times daily), and albumin is given for two days as an intravenous bolus (1 g/kg per day [100 g maximum]), followed by 25 to 50 grams per day until midodrine and octreotide therapy is discontinued.
In highly selected patients who fail to respond to medical therapy with the above regimens and who are considered well enough to undergo the procedure, transjugular intrahepatic portosystemic shunt (TIPS) is sometimes successful. However, this procedure is associated with numerous complications and, because of the need for intravenous contrast, it may cause acute kidney injury. For this reason, some experts prefer dialysis as a first option (continuous renal replacement therapy) in most cases, particularly for patients whose serum creatinine remains above 1.5 mg/dL despite medical therapy.
In patients who fail to respond to the above therapies, develop severely impaired renal function, and either are candidates for liver transplantation or have a reversible form of liver injury and are expected to survive, we recommend dialysis as a bridge to liver transplantation or liver recovery.]
7
Q
What is the usual energy source for the liver?
A
Ketones
[α-Ketoacids derived from the degradation of amino acids are the liver’s own fuel. In fact, the main role of glycolysis in the liver is to form building blocks for biosyntheses. Furthermore, the liver cannot use acetoacetate as a fuel, because it has little of the transferase needed for acetoacetate’s activation to acetyl CoA. Thus, the liver eschews the fuels that it exports to muscle and the brain.]
8
Q
Cultures of an amebic liver abscess typically show what?
A
Nothing
[Cultures of abscess often sterile -> Protozoa (Entamoeba Histolytica) exist only in peripheral rim]
9
Q
Most primary and secondary liver tumors are supplied by which artery?
A
Hepatic artery
10
Q
Which Hepatitis Virus causes high mortality in pregnant women
A
HEV
11
Q
Diagnose:
- HBsAg: +
- Anti-HBs: -
- HBeAg: +
- Anti-HBe: -
- Anti-HBc: IgM
A
Acute HBV
12
Q
What are the 4 boundaries of the Foramen of Winslow?
A
- Anterior: Portal triad
- Posterior: IVC
- Inferior: Duodenum
- Superior: Liver
13
Q
The portal triad (common bile duct, portal vein, and proper hepatic artery) enters which numbered segment(s) of the liver?
A
Segments IV and V
14
Q
What is the # 1 cause of hepatocellular carcinoma worldwide?
A
Hepatitis B
15
Q
Jaundice occurs when the total bilirubin reaches what level?
A
Greater than 2.5
16
Q
What is the treatment for postpartum liver failure with ascites?
A
Heparin and antibiotics
17
Q
A liver abscess caused by an infection with which organism is associated with a maculopapular rash, increased eosinophils, and variceal bleeding?
A
Schistosomiasis
[UpToDate: Manifestations of chronic infection are generally observed among individuals with ongoing exposure in endemic regions. Disease is caused by the host immune response to migrating eggs. In the bowel, inflammation can result in ulceration, blood loss, and scarring. In the liver, periportal fibrosis (Symmers’ pipestem fibrosis) can lead to portal hypertension and subsequent esophageal varices. In the bladder, granulomatous inflammation can result in development of pseudopolyps and/or urinary tract obstruction leading to renal failure.]
18
Q
What conjugates bilirubin to glucuronic acid in the liver?
A
Glucuronyl transferase
[Improves water solubility]
19
Q
What is a direct contraindication to a splenorenal shunt in a cirrhotic patient?
A
Refractory ascites
[This procedure can worsen ascites]
20
Q
Which numbered segments of the liver are fed by the left portal vein?
A
Segments II, III, and IV
21
Q
Best marker for chronic HBV infection
A
HBsAg longer than 6 months but no anti-HBs antibodies
22
Q
How is Budd-Chiari syndrome diagnosed?
A
Angiogram with venous phase, CT angiogram
[Liver biopsy shows sinusoidal dilatation, congestion, and centrilobular congestion]
23
Q
Which vein(s) act as collaterals between the portal vein and the systemic venous system of the lower esophagus (azygous vein)
A
Coronary veins
24
Q
What are the King’s College criteria of poor prognostic indicators for non-acetaminopen-induced acute liver failure?
A
INR > 6.5
Or any 3 of the following:
- Age less than 10 or greater than 40
- Drug toxicity or undetermined etiology
- Jaundice greater than 7 days before encephalopathy
- INR greater than 3.5
- Bilirubin greater than 17 mg/dL
25
What is the treatment for symptomatic splenic vein thrombosis?
Splenectomy
26
What role does propranolol have in treating esophageal varices?
May help prevent re-bleeding
[No good role acutely]
[UpToDate: Nonselective beta blockers lower portal pressure and reduce the risk of first bleeding in patients with esophageal varices. For these reasons, they are the treatment of choice for primary prophylaxis in patients at high risk for variceal bleeding.
Multiple clinical trials have compared beta blockers with placebo for the prevention of recurrent variceal hemorrhage. Approximately 75% of the patients enrolled had Child-Pugh class A cirrhosis. Alcohol was the most common cause of liver disease and many patients were excluded from participation due to contraindications to use of beta blockers. The most important variation among studies was the timing of entry into the studies from the index bleed (one day to one month). The doses of the drug used were also variable (10 to 480 mg/day of propranolol); the dose was generally titrated to achieve a 25% decrease in resting heart rate.
With a few exceptions, an overall improvement in rebleeding rate was noted, but a survival advantage was only shown in one study. Meta-analysis of these data suggests that the risk of bleeding is decreased by approximately 40%, while the risk of death is decreased by 20%. Propranolol has also decreased recurrent bleeding (35% vs 62% at one year) from portal hypertensive gastropathy.]
27
What are the 2 primary bile acids (salts)?
Cholic and chenodeoxycholic
28
Which type of benign liver tumor has a central stellate scar that may look like cancer?
Focal nodular hyperplasia
29
What is the Pringle maneuver?
Clamping the porta hepatis
[Will not stop hepatic vein bleeding]
30
The entrance to the lesser sac of the abdomen is called what?
Foramen of Winslow
31
What is the only DNA hepatitis virus?
Hepatitis B
32
What is the treatment for a pyogenic liver abscess?
CT-guided drainage and antibiotics
[Surgical drainage for unstable condition and continued signs of sepsis]
33
According to Couinaud's nomenclature, which numbered liver segment is the inferior left lateral segment?
Segment III
34
Which syndrome is characterized by a mild defect in glucuronyl transferase, leading to abnormal conjugation of bilirubin?
Gilbert's disease
35
Conjugated bilirubin is broken down by bacteria in which portion of the digestive tract?
Terminal ileum
36
What are the 5 scoring criteria for calculating the Child-Pugh Score?
* Albumin: Greater than 3.5 (1 ), 3-3.5 (2), Less than 3 (3)
* Bilirubin: Less than 2.5 (1), 2.5-4 (2), Greater than 4 (3)
* Encephalopathy: None (1), Minimal (2), Severe (3)
* Ascites: None (1), Treatable with meds, (2), Refractory (3)
* INR: Less than 1.7 (1), 1.7-2.3 (2), Greater than 2.3 (3)
(# of points)
37
How is a pyogenic liver abscess diagnosed?
Aspiration
[UpToDate: The diagnosis of pyogenic liver abscess is made by history, clinical examination, and radiographic imaging followed by aspiration and culture of the abscess material.]
38
80% of all liver abscesses fall into which category of abscess?
Pyogenic abscess
[15% mortality with sepsis]
39
What percent of symptomatic HBV infected individuals develop acute vs chronic disease
* Acute: 90%
* Chronic: 10%
40
Schistosomiasis, congenital hepatic fibrosis, and portal vein thrombosis are all causes of portal hypertension from what kind of obstruction (pre-sinusoidal, sinusoidal, post-sinusoidal)?
Pre-sinusoidal obstruction
[50% of portal HTN in children]
41
What does elevated conjugated bilirubin signify?
Secretion defects into bile ducts or excretion defects into GI tract
[Stones, strictures, tumors]
42
What is the associated percent mortality following shunt placement in each of the 3 categories of cirrhotic patients according to the Child-Pugh criteria?
* Child's A: 2% mortality
* Child's B: 10% mortality
* Child's C: 50% mortality
43
What is hepatorenal syndrome?
Progressive renal failure with same lab findings as prerenal azotemia
[Usually a sign of end-stage renal disease]
[UpToDate: Arterial vasodilatation in the splanchnic circulation, which is triggered by portal hypertension, appears to play a central role in the hemodynamic changes and the decline in renal function in cirrhosis. The presumed mechanism is increased production or activity of vasodilators, mainly in the splanchnic circulation, with nitric oxide thought to be most important.
As the hepatic disease becomes more severe, there is a progressive rise in cardiac output and fall in systemic vascular resistance; the latter change occurs despite local increases in renal and femoral vascular resistance that result in part from hypotension-induced activation of the renin-angiotensin and sympathetic nervous systems. Thus, the reduction in total vascular resistance results from decreased vascular resistance in the splanchnic circulation, perhaps in part under the influence of nitric oxide derived from the endothelium. Bacterial translocation from the intestine into the mesenteric lymph nodes may play an important role in this process. A review of the hemodynamic changes seen with progressive cirrhosis can be found in a separate topic review.
The decline in renal perfusion in this setting is associated with reductions in glomerular filtration rate (GFR) and sodium excretion (often to less than 10 meq/day in advanced cirrhosis) and a fall in mean arterial pressure, despite the intense renal vasoconstriction. The importance of splanchnic vasodilatation in these changes can be indirectly illustrated by the response to ornipressin, an analog of antidiuretic hormone (arginine vasopressin) that is a preferential splanchnic vasoconstrictor.]
44
What are two rare complications of a liver hemangioma?
1. Consumptive coagulopathy (Kasabach-Merritt syndrome)
2. Congestive heart failure
[These complications are usually seen in children]
45
Which 3 types of hepatocellular carcinoma have the best prognosis?
1. Clear cell
2. Lymphocyte infiltrative
3. Fibrolamellar
[UpToDate: Well-differentiated clear cell and fibrolamellar tumors and the presence of tumor encapsulation have been associated with a better prognosis. Some suggest the utility of using tumor grade to select patients for treatment (eg, liver transplantation), although this has not yet been accepted into practice.]
46
Splenic vein thrombosis is most often caused by what?
Pancreatitis
47
In order to perform a splenorenal shunt, which 5 veins must be ligated?
1. Left adrenal vein
2. Left gonadal vein
3. Inferior mesenteric vein
4. Coronary vein
5. Pancreatic branches of splenic vein
48
What size margin is required in the resection of hepatocellular carcinoma?
1 cm margin
[UpToDate: The importance of wide resection margins is debated. In a study of 225 patients who underwent resection for HCC, three-year survival was significantly better when a \>1 cm tumor-free margin was achieved (77% vs 21%, respectively). However, larger series suggest that a negative margin of \<1 cm is acceptable.]
49
According to Couinaud's nomenclature, which numbered liver segment is the left medial segment (quadrate lobe)?
Segment IV
50
What are the 5 main components of bile?
1. Bile salts
2. Proteins
3. Phospholipids
4. Cholesterol
5. Bilirubin
[Phospholipids include lecithin.]
51
Best markers for past HBV infection
IgG anti-HBs & IgG anti-HBc In the absence of HBsAg
52
Which treatment is required for refractory variceal bleeding?
Transjugular Intrahepatic Portosystemic Shunt (TIPS)
53
What causes hepatic encephalopathy?
Liver loses ability to metabolize waste, leading to a build up of ammonia, mercantanes, and false neurotransmitters
54
What is a normal portal vein pressure?
Less than 12mmHg
55
According to Couinaud's nomenclature, which numbered liver segment is the caudate lobe?
Segment I
56
Recurrent flapping tremor of the arms, like the action of a bird's wings, that occurs as a result of a brain condition associated with liver failure is called what?
Asterixis
[A sign that liver failure is progressing]
57
What is the treatment for an amebic liver abscess?
Flagyl
[Aspiration only if refractory and surgery only if free rupture]
[UpToDate: For circumstances in which amebic liver abscess is suspected based on epidemiology, clinical manifestations, and radiographic findings, it is reasonable to initiate empiric treatment pending further diagnostic evaluation (including confirmatory antigenic or serologic testing and evaluation for the parasite in stool and liver abscess pus).
In general, amebic liver is managed with a tissue agent and a luminal agent (to eliminate intraluminal cysts).
* Tissue agents — Patients with an amebic liver abscess should be treated with metronidazole (500 to 750 mg orally three times daily for 7 to 10 days) or tinidazole (2 g once daily for 5 days). The cure rate with this therapy is \>90%. Shorter duration of metronidazole is not generally recommended. Metronidazole is well absorbed from the gastrointestinal tract; intravenous therapy offers no significant advantage as long as the patient can take oral medications and has no major defect in small bowel absorption. Alternatives to metronidazole or tinidazole include ornidazole and nitazoxanide. Nitazoxanide (500 mg twice daily for 10 days) has been shown to be effective in a small case series. In the setting of slow response to metronidazole or relapse following therapy, therapeutic aspiration, percutaneous catheter drainage, and/or a prolonged course of metronidazole may be warranted.
* Luminal agents — Following therapy for invasive amebiasis, treatment with a luminal agent to eliminate intraluminal cysts is warranted, even if stool microscopy is negative. Intraluminal infection can be treated with one of the following regimens: paromomycin (25 to 30 mg/kg per day orally in three divided doses for 7 days), diiodohydroxyquin (650 mg orally three times daily for 20 days for adults and 30 to 40 mg/kg per day in three divided doses for 20 days for children), or diloxanide furoate (500 mg orally three times daily for 10 days for adult and 20 mg/kg per day in three divided doses for 10 days for children).
Uncomplicated amebic liver abscess has a mortality rate of \<1% if diagnosed and treated early. In one study of 135 patients with amebic liver abscess in India with overall mortality rate of 17%, independent risk factors for increased mortality included bilirubin level \>3.5 mg/dL, serum albumin \<2.0 g/dL, large volume of the abscess cavity, multiple abscesses, and encephalopathy.]
58
Which clotting factors are not made in the liver?
Von Willebrand factor and factor VIII
[Some factor VIII is produced in liver sinusoidal cells, but much of it is produced by endothelial cells outside of the liver throughout the body]
59
What runs within the falciform ligament?
Remnant of the umbilical vein
60
Diagnose:
* HBsAg: +
* Anti-HBs: -
* HBeAg: +
* Anti-HBe: -
* Anti-HBc: IgG
High infectivity Chronic HBV
61
What is the main biliary phospholipid?
Lecithin
62
Urine turns dark because of excess of what?
Urobilinogen
63
Which part of the liver receives separate right and left portal and arterial blood flow and drains directly into the IVC via separate hepatic veins?
Caudate lobe (Segment I)
64
What does the Child-Pugh score correlate with?
Mortality after open shunt placement
[These classes correlate with one- and two-year patient survival: class A: 100 and 85 percent; class B: 80 and 60 percent; and class C: 45 and 35 percent.]
[UpToDate: The Child-Pugh classification has been used to assess the risk of non-shunt operations in patients with cirrhosis. It is a modification of the Child-Turcotte classification, which incorporated five variables that were designed to stratify the risk of portacaval shunt surgery in patients with cirrhosis. The variables included the serum albumin and bilirubin, ascites, encephalopathy, and nutritional status. The Child-Pugh classification replaces nutritional status with prothrombin time. The score ranges from 5 to 15. Patients with a score of 5 or 6 have Child-Pugh class A cirrhosis (well-compensated cirrhosis), those with a score of 7 to 9 have Child-Pugh class B cirrhosis (significant functional compromise), and those with a score of 10 to 15 have Child-Pugh class C cirrhosis (decompensated cirrhosis).
In a review of 92 patients with cirrhosis who underwent abdominal surgery, the mortality rate was 10% for patients with Child-Pugh class A cirrhosis, 30% for patients with Child-Pugh class B cirrhosis, and 82% for patients with Child-Pugh class C cirrhosis. Other studies have validated the utility of the Child-Pugh classification for the assessment of surgical risk.
The Child-Pugh classification system also correlates with survival in patients not undergoing surgery; 1-year survival rates for patients with Child-Pugh class A, B, and C cirrhosis are approximately 100%, 80%, and 45%, respectively. Child-Pugh class is also associated with the likelihood of developing of complications of cirrhosis. As an example, patients with Child-Pugh class C cirrhosis are much more likely to develop variceal hemorrhage than those with Child-Pugh class A cirrhosis.]
65
What is the treatment for an asymptomatic hepatic adenoma?
Stop OCPs (or any causative agent), If no regression, patient needs resection of the tumor
[UpToDate: Hepatic adenomas are uncommon benign epithelial liver tumors that develop in an otherwise normal-appearing liver. They are seen predominantly in young women (20 to 44 years old), are frequently located in the right hepatic lobe, and are typically solitary (70% to 80%), although multiple adenomas have been described in patients with prolonged contraceptive use, glycogen storage diseases, and hepatic adenomatosis. Adenomas range in size from 1 to 30 cm. Symptoms such as abdominal pain are more likely with larger lesions.
We recommend resection of all symptomatic hepatic adenomas and adenomas \>5 cm (Grade 1C). Adenomas that do not resolve or enlarge after discontinuation of steroid medication should also be considered for surgical resection after discussion with the patient. In women taking oral contraceptives, we suggest repeating the imaging study six months after stopping oral contraceptives. If there is no change, or if the presumed adenoma has grown in size, we suggest surgery.]
66
Which two pharmaceutical agents can be used to temporize actively bleeding esophageal varices?
1. Vasopressin (Splanchnic artery contriction)
2. Octreotide (Decrease portal pressure by decreasing blood flow)
[UpToDate: The first step in stopping variceal bleeding is the initiation of pharmacologic therapy. Pharmacologic therapy should be started in all patients with upper gastrointestinal bleeding who have varices or who are at risk for having varices (eg, patients with advanced cirrhosis). Pharmacologic therapy should not be delayed pending confirmation that the source of bleeding is indeed from varices. In the United States, pharmacologic therapy typically consists of an octreotide bolus (50 mcg intravenous [IV]) followed by a continuous infusion (50 mcg IV per hour). Where available, terlipressin is often used. Terlipressin is administered at an initial dose of 2 mg IV every four hours and can be titrated down to 1 mg IV every four hours once hemorrhage is controlled. Pharmacologic therapy is typically continued for three to five days following cessation of bleeding.
For patients with esophageal varices, acute bleeding is typically managed with endoscopic variceal ligation, though occasionally endoscopic sclerotherapy is used. The goal should be to perform an upper endoscopy within 12 hours of presentation. If the bleeding cannot be controlled endoscopically, treatment options include transjugular intrahepatic portosystemic shunt (TIPS) placement or surgical shunting. For bleeding gastric varices, treatment is with cyanoacrylate injection where available. If cyanoacrylate injection is not an option, TIPS placement is typically used. Balloon tamponade is an option for temporarily stopping bleeding from esophageal or gastric varices while definitive treatment is being arranged, but it is associated with serious complications including esophageal rupture. Bleeding ectopic varices may be managed with TIPS placement or surgery.
Vasoactive medications decrease portal blood flow and are used for the treatment of acute variceal hemorrhage. They include vasopressin, somatostatin, and their analogs (terlipressin and octreotide, respectively). As a group, vasoactive medications have been shown to decrease mortality and improve hemostasis in patients with acute variceal bleeding. However, terlipressin is the only agent individually shown to reduce mortality. Pharmacologic therapy should be started at the time of presentation in a patient who has known varices or is at risk for varices. It should not be held pending confirmation of the diagnosis. Terlipressin is the preferred agent in many countries outside of the United States. It is initially given as 2 mg intravenous (IV) bolus every four hours. Octreotide is the agent available in the United States and is given as a 50 mcg IV bolus, followed by a continuous infusion at a rate of 50 mcg per hour. Pharmacologic therapy should be continued for three to five days.]
67
What percent of bile is composed of bile salts?
85%
68
Can a benign focal nodular hyperplasia become malignant?
No
[Also very unlikely to rupture]
[UpToDate: Focal nodular hyperplasia (FNH) is the most common non-malignant hepatic tumor that is not of vascular origin. It is now generally accepted to be a hyperplastic (regenerative) response to hyperperfusion by the characteristic anomalous arteries found in the center of these nodules.
FNH is most often solitary (80% to 95%) and usually less than 5 cm in diameter. Only 3% are larger than 10 cm.
The majority of reports have found that symptoms or signs directly attributable to FNH are infrequent.
The diagnosis of FNH is usually made by demonstrating its characteristic features on imaging tests and excluding other lesions. The latter can typically be accomplished by assessing the context in which FNH is detected and by obtaining specific radiologic and laboratory testing.
The natural history of FNH is one of stability and a lack of complications. Thus, we suggest that patients who are suspected of having FNH based upon the evaluation described above be managed conservatively (Grade 2B).
FNH may be responsive to exogenous estrogens. We generally do not insist that oral contraceptives and other estrogen-containing preparations should be discontinued. However, it is reasonable to obtain a follow-up imaging study in 6 to 12 months in women who continue taking these drugs.]
69
Which numbered segments of the liver are drained by the left hepatic vein?
Segment II, III, and superior portion of IV
70
How effective are banding and sclerotherapy at treating esophageal varices?
95% effective
71
What is the treatment for a liver abscess caused by Schistosomiasis?
Praziquantel
[Control any variceal bleeding]
[UpToDate: Treatment of schistosomiasis serves three purposes: reversing acute or early chronic disease, preventing complications associated with chronic infection, and preventing neuroschistosomiasis. The goal of treatment is reduction of egg production via reduction of worm load.
We recommend that patients with schistosomiasis be treated promptly with praziquantel, in the presence or absence of clinical manifestations (Grade 1B). A single dose of praziquantel reduces the parasite burden substantially though is not curative for moderate or severe infection.
Dosing of praziquantel for treatment of infection due to S. haematobium, S. mansoni, or S. intercalatum consists of 40 mg/kg (in one or two divided doses). Praziquantel dosing for treatment of infection due to S. japonicum or S. mekongi consists of 60 mg/kg (in two divided doses).
Follow-up after treatment includes monitoring of clinical manifestations, eosinophilia, and microscopy evaluation for eggs in stool or urine. In endemic areas, follow-up microscopy should be performed no sooner than six weeks following treatment. In nonendemic areas, follow-up microscopy may be performed three to six months after treatment. Persistence of viable eggs after treatment with praziquantel warrants repeat treatment.]
72
Which Hepatitis viruses carry a risk of Hepatocellular Carcinoma
HBV, HCV, & HDV
73
In which 4 circumstances is Transjugular Intrahepatic Portosystemic Shunt (TIPS) indicated?
1. Protracted bleeding
2. Progression of coagulopathy
3. Visceral hypoperfusion
4. Refractory ascites
74
When would you consider surgery to treat an amebic liver abscess?
Free rupture of the abscess
75
What correlates with tumor size in hepatocellular carcinoma?
AFP level
[UpToDate: A rise in serum AFP in a patient with cirrhosis or hepatitis B should raise concern that HCC has developed. It is generally accepted that serum levels greater than 500 mcg/L (normal in most laboratories is between 10 and 20 mcg/L) in a high-risk patient is diagnostic of HCC. However, HCC is often diagnosed at a lower AFP level in patients undergoing screening.]
76
What percent of the liver can be safely resected?
75%
77
What is the treatment for an asymptomatic liver hemangioma?
Conservative treatment
[UpToDate: Asymptomatic patients, particularly those with lesions \<1.5 cm, can be reassured and observed. As discussed above, long-term follow-up of hemangiomas in such patients has confirmed that most do not grow or develop complications. We do not recommend follow-up imaging in patients with hemangiomas ≤5 cm in size, provided there is certainty of the diagnosis based on radiologic and on clinical details. On the other hand, rapid growth of a hemangioma has been reported, justifying radiologic follow-up of patients with lesions \>5 cm, particularly those in a sub capsular location.]
78
What is the second most common hepatic artery variant?
Left hepatic artery off left gastric artery
[Found in gastrohepatic ligament medially]
79
What is the only water-soluble vitamin stored in the liver?
B12
80
Which numbered segments of the liver are fed by the right portal vein?
Segments V, VI, VII, and VIII
81
What is the cause of sinusoidal obstruction leading to portal hypertension?
Cirrhosis
82
Hepatic adenomas are more commonly found in which lobe?
Right lobe
[UpToDate: Seen predominantly in young women (20 to 44 years old), are frequently located in the right hepatic lobe, and are typically solitary (70% to 80%), although multiple adenomas have been described in patients with prolonged contraceptive use, glycogen storage diseases, and hepatic adenomatosis.]
83
What is the name of the peritoneum that covers the liver?
Glisson's capsule
84
Best markers for current acute HBV infection
IgM anti-HBc & HBsAg
85
Which test should be performed pre-operatively in a patient with an echinococcal liver abscess (hydatid cyst) who has jaundice, increased LFTs, or cholangitis?
ERCP to check for communication with biliary system
86
Which numbered segments of the liver are drained by the right hepatic vein?
Segment VI, VII, and VIII
87
What is the serious complication of Transjugular Intrahepatic Portosystemic Shunt (TIPS)?
Encephalopathy
[UpToDate: The primary complication due to shunting of blood from the portal to systemic circulation is the development of portosystemic encephalopathy (PSE). In addition, transjugular intrahepatic portosystemic shunt (TIPS) placement has also been associated with acute pulmonary edema and heart failure.
The incidence of PSE following TIPS is approximately 30% to 35% and is similar to that reported after surgical portal diversion. In one study, for example, 9 of 30 patients receiving TIPS had 24 episodes of PSE during follow-up; in comparison, only six episodes of PSE occurred in 25 concurrent controls treated only with sclerotherapy. Increasing age, advanced liver failure, the presence of minimal hepatic encephalopathy at the time of TIPS placement, and a history of overt hepatic encephalopathy prior to TIPS placement have all been associated with an increased risk of developing PSE after TIPS.
The diameter of the shunt may influence the development of PSE. The resistance to flow through the shunt decreases exponentially with increasing shunt diameter, resulting in increases in shunt flow and portosystemic shunting. The probability of PSE increases dramatically at shunt diameters above 10 mm. However, one randomized trial that assigned 45 patients to receive either 8 or 10 mm diameter stents failed to show an increased incidence of PSE in the patients with larger diameter shunts. At one year, the probability of being free of PSE was 43% in the 8 mm stent arm and 47% in the 10 mm stent arm. In addition, the trial was stopped prior to enrolling its goal of 114 patients because of a significantly higher portosystemic pressure gradient in patients who received the smaller shunts (8.9 vs 6.5 mmHg). This correlated with a decrease in the probability of remaining free from complications due to portal hypertension at one year in the 8 mm group compared with the 10 mm group (42% vs 83%).]
88
What is the mortality rate for acute liver failure (fulminant hepatic failure)?
80% mortality
[Outcome determined by the course of encephalopathy]
[UpToDate: Overall, survival rates in patients treated for acute liver failure are greater than 60%. Approximately 55% of patients will survive without needing a liver transplantation.
The prognosis of patients who are listed for transplantation is variable and cannot always be predicted accurately. An illustrative study from the United States included 308 patients with acute liver failure, of whom 67% survived. Of 135 patients who had been listed for transplantation, 66% received a transplantation, 22% died while awaiting transplantation, and 12% recovered without transplantation. In other series, death rates among patients listed for transplantation are as high as 40%.
Following liver transplantation, the one-year survival rate is approximately 80%. The majority of deaths among patients who undergo liver transplantation are due to neurologic complications or sepsis and occur within three months of the transplantation.]
89
What percent of symptomatic HCV infected individuals develop acute vs chronic disease
* Acute: 20%
* Chronic: 80%
90
Which Hepatitis viruses are transmitted by fecal oral route
HAV & HEV
91
Diagnose:
* HBsAg: -
* Anti-HBs: -
* HBeAg: -
* Anti-HBe: +
* Anti-HBc: IgM
HBV Window Period
92
Which syndrome is characterized by a deficiency in secretion ability that leads to high conjugated bilirubin?
Dubin-Johnson syndrome
[UpToDate: Dubin-Johnson syndrome is characterized clinically by mild icterus. Otherwise, patients are asymptomatic, although mild constitutional complaints such as vague abdominal pains and weakness can occur. Icterus can be so mild as to be noted only during intercurrent illnesses, pregnancy, or consumption of oral contraceptives. Pruritus is not seen in this condition. Physical examination is usually normal except for the icterus, although hepatosplenomegaly is seen occasionally.
Routine clinical laboratory tests, including the complete blood count, serum albumin, cholesterol, alanine and aspartate aminotransferases, alkaline phosphatase, and prothrombin time, are normal. Fasting and postprandial bile acid levels, which are sensitive indices of liver disease, are normal, reflecting the fact that the genetic abnormality does not affect the transport of most bile acids. Serum bilirubin concentrations are usually between 2 and 5 mg/dL but may decline to normal levels or be as high as 20 to 25 mg/dL. Approximately 50% of the serum bilirubin is conjugated. Of the conjugated bilirubin, a major fraction is bilirubin diglucuronide. In addition, covalently albumin-bound fraction is found in plasma. Bilirubinuria is common.]
93
What are 6 strategies to treat ascites in the cirrhotic patient?
1. Water restriction (1-1.5L/d)
2. Decrease NaCl (1-2 g/d)
3. Diuretics (spironolactone counteracts hyperaldosteronism seen with liver failure)
4. Paracentesis
5. TIPS
6. Prophylactic antibiotics (norfloxacin) to prevent spontaneous bacterial peritonitis (used if previous SBP or current UGI bleed)
94
What can lead to isolated gastric varices without elevation of pressure in the rest of the portal system?
Splenic vein thrombosis
95
Which organism is most commonly responsible for pyogenic liver abscess?
E.Coli (Most pyogenic abscesses are from gram negative rods)
[UpToDate: Most pyogenic liver abscesses are polymicrobial; mixed enteric facultative and anaerobic species are the most common pathogens. Anaerobes are probably under-reported because they are difficult to culture and characterize in the laboratory.]
96
How is focal nodular hyperplasia of the liver diagnosed?
Abdominal CT (hypervascular tumor)
[Has kupffer cells, so will take up sulfur colloid on liver scan as opposed to a hepatic adenoma which does not take up sulfur colloid]
[UpToDate: U/S is able to identify the central scar in only 20 percent of cases. A properly timed dynamic, triphasic, helical CT scan performed without contrast, and with contrast during the hepatic arterial and portal venous phases, will often be highly suggestive of the diagnosis. There may be little to distinguish FNH from normal liver on standard MRI, since it is composed of the same elements as normal liver.]
97
Which Hepatitis virus requires infection with which other hepatitis virus
HDV requires HBV infection
98
Where is jaundice first evident?
Under the tongue
99
Which animals harbor echinococcus?
Sheep are carriers, Dogs are definitive hosts
100
What does elevated un-conjugated bilirubin signify?
Prehepatic cause such as hemolysis or hepatic deficiencies in uptake or conjugation of bilirubin
101
Can an isolated colon cancer metastasis to the liver be resected?
Yes, if enough liver will remain for the patient to survive
[35% 5-year survival after resection for cure]
[UpToDate: Resection offers the greatest likelihood of cure for patients with liver-isolated colorectal cancer (CRC). In surgical case series, 5-year survival rates after resection range from 24% to 58%, averaging 40%, and surgical mortality rates are generally \<5%. Subgroups with advanced age, comorbid disease, and synchronous hepatic and colon resection may have higher procedure-related mortality and worse long-term outcomes. As an example, 5-year survival rate was only 25% in a population-based retrospective report of 3957 US Medicare enrollees undergoing hepatic resection for CRC liver metastases. Even so, 5-year survival rates with the most active systemic chemotherapy regimens are only 10% to 11%, only about one-fifth of whom have a sustained disease remission.
Approximately one-third of 5-year survivors suffer a cancer-related death, while those who survive 10 years appear to be cured. In an analysis of 612 consecutive patients who underwent resection of CRC liver metastases and were followed for at least 10 years, there were 102 actual 10-year survivors (17%), and only one patient experienced a disease-specific death after 10 years of survival.
Because of its clear survival impact, surgical resection is the treatment of choice when feasible. The percentage of patients with isolated hepatic metastases who are amenable to potentially curative resection is a bit of a moving target since surgeons may have different interpretations of "resectable." It is safe to say that a majority of patients, with metastatic disease are not curative surgical candidates because of tumor size, location, multifocality, or inadequate hepatic reserve.]
102
What is the name of the ligament that carries the 3 structures of the portal triad?
Hepatoduodenal ligament (porta hepatis)
103
What is the most common type of benign liver tumor?
Hemangioma (Most are asymptomatic)
[UpToDate: Hepatic hemangiomas are the most common benign mesenchymal hepatic tumors. In one series 72 percent were hemangiomas.]
104
Where is the primary infection in a patient with an amebic liver abscess?
The colon (amebic colitis)
[Reaches liver via portal vein]
[UpToDate: Amebiasis is caused by the protozoan Entamoeba histolytica. Most infections are asymptomatic; clinical manifestations include amebic dysentery and extraintestinal disease. Extraintestinal manifestations include amebic liver abscess and other more rare manifestations such as pulmonary, cardiac, and brain involvement. Amebic liver abscess is the most common extraintestinal manifestation of amebiasis. Amebae establish hepatic infection by ascending the portal venous system.]
105
What does a positive Casoni skin test indicate?
Echinococcal liver abscess (hydatid cyst)
[Wikipedia: The Casoni test is an immediate hypersensitivity skin test used in the diagnosis of hydatid disease introduced by casoni. The test involves the intradermal injection of 0.25 ml of sterilised fluid from hydatid cysts/human cyst and sterilised by Seitz filtration on forearm and equal volume of saline injected on the other forearm. Observations made for next 30 mins and after 1 to 2 days. A wheal response occurring at the injection site within 20 minutes is considered positive. (Immediate hypersensitivity). Delayed hypersensitivity reaction usually read after 18-24 hours. The test is positive in about 90% of cases of hydatid disease affecting the liver, but positive in less than 50% of patients with hydatid disease elsewhere in the body; false positive results are also common. Being a type I hypersensitivity reaction, anaphylactic reaction tray must be kept ready before carrying out the test. Consequently, serological tests are now generally used.]
106
What is the treatment for a symptomatic hepatic adenoma?
Tumor resection for bleeding and malignancy risk (Embolization if multiple and unresectable)
[UpToDate: Surgical resection should be recommended for all patients with symptoms attributable to the adenoma and those with large lesions \>5 cm. Liver transplantation should be reserved for patients in whom surgical resection is not possible due to tumor size/location and those with adenomatosis. Mortality from elective surgical resection is \<1 percent, and resection of some peripherally located adenomas can even be accomplished laparoscopically.]
107
What are the 3 risk factors for spontaneous bacterial peritonitis?
1. Prior SBP
2. UGI bleed (variceal hemorrhage)
3. Low-protein ascites
108
What is the treatment for spontaneous bacterial peritonitis?
3rd-generation cephalosporins
[patients usually respond within 48 hours]
[UpToDate: In patients with suspected spontaneous bacterial peritonitis (SBP), empiric therapy should be initiated as soon as possible to maximize the patient's chance of survival. However, antibiotics should not be given until ascitic fluid has been obtained for culture. Most cases of SBP are due to gut bacteria such as Escherichia coli and Klebsiella, though streptococcal and staphylococcal infections can also occur. As a result, broad-spectrum therapy is warranted until the results of susceptibility testing are available. We prefer cefotaxime 2 g intravenously every eight hours because it has been shown to produce excellent ascitic fluid levels. In addition to antibiotic therapy, patients with SBP who are taking a nonselective beta blocker should have the medication discontinued.]
109
What extends from the falciform ligament and carries the obliterated umbilical vein to the undersurface of the liver?
Ligamentum Teres
110
Can a benign hepatic adenoma become malignant?
Yes
[UpToDate: The risk of malignant transformation to hepatocellular carcinoma is difficult to ascertain from the literature. Two small studies reported the development of malignant transformation in 8% to 13% of patients, suggesting that it is not a rare occurrence. An increase in size on sequential imaging studies or a rise in serum AFP levels should raise concern that malignant transformation has developed.]
111
What is the most common cause of liver failure?
Cirrhosis
[Palpable liver, jaundice, and ascites]
112
Which hepatocytes are most sensitive to ischemia?
Central lobular (Acinar zone III)
113
Pyogenic liver abscess is most commonly secondary to what?
Contiguous infection from biliary tract
[UpToDate: A considerable proportion of pyogenic liver abscesses follow one or more episodes of portal vein pyemia, usually related to bowel leakage and peritonitis. Another important route is direct spread from biliary infection. Underlying biliary tract disease such as gallstones or malignant obstruction is present in 40% to 60% of cases.]
114
Budd-Chiari syndrome, constrictive pericarditis, and CHF are all causes of portal hypertension from what?
Post-sinusoidal obstruction
115
Should an echinococcal cyst be aspirated?
No
[Can cause leakage and anaphylactic shock]
116
Which life-threatening syndrome is characterized as a severe deficiency in glucuronyl transferase (inability to conjugate bilirubin), leading to high unconjugated bilirubin?
Crigler-Najjar disease
117
What is the treatment for an echinococcal liver abscess (hydatid cyst)?
Pre-op albendazole for 2 weeks followed by surgical removal
[Intra-op can inject cyst with alcohol to kill organisms, then aspirate out. Must get entire cyst wall]
[UpToDate: Management options for cystic echinococcosis (CE) include surgery, percutaneous management, drug therapy, and observation. Surgery has been the traditional approach for treatment of CE; subsequently, alternative approaches have been introduced and have replaced surgery as the treatment of choice in some cases.
In general, clinical approach depends on the World Health Organization (WHO) diagnostic classification. Stage CE1 and CE3a cysts have a single compartment; such cysts that are \<5 cm may be treated with albendazole alone. In settings where albendazole treatment with follow-up monitoring is not feasible, definitive management with percutaneous treatment via puncture, aspiration, injection, and reaspiration (PAIR) is an acceptable alternative approach. Stage CE1 and CE3a cysts that are \>5 cm may be treated with albendazole in combination with PAIR. In situations where albendazole treatment is not feasible, percutaneous treatment with PAIR (in the absence of adjunctive drug therapy) is an acceptable alternative approach. Issues related to drug therapy are discussed below.
Stage CE2 and CE3b cysts have many compartments that require individual puncture; patients with such cysts commonly relapse after PAIR. Therefore, management of these cysts requires either modified catheterization technique (eg, non-PAIR percutaneous therapy) or surgery (with adjunctive drug therapy). The optimal choice between these approaches is uncertain and further study is needed.
Stages CE4 and CE5 are inactive cysts that may be managed with observation.
There are few randomized trials comparing different treatments, and the level of evidence supporting the use of one therapeutic modality over another is low. In one study of 32 patients with hepatic cysts comparing percutaneous drainage alone, albendazole alone, and percutaneous drainage in combination with albendazole, those who received combination therapy had the greatest size reduction observed ultrasonographically. Subsequently, a randomized trial performed among 50 patients demonstrated that outcomes among those who underwent percutaneous drainage (in combination with albendazole) were comparable to outcomes among patients who underwent surgery (with no albendazole therapy).]
118
Portal hypertension in children is usually caused by what?
Extra-hepatic portal vein thrombosis
119
What is the mechanism of ascites in cirrhosis?
Hepatocyte destruction --\> Fibrosis and scarring of liver --\> Increased hepatic pressure --\> Portal venous congestion --\> Lymphatic overload --\> Leakage of splanchnic and hepatic lymph into peritoneum --\> Ascites
120
Cirrhosis is a cause of what kind of portal hypertension (pre-sinusoidal, sinusoidal, post-sinusoidal)?
Sinusoidal obstruction
121
What are the 2 secondary bile acids (salts)?
Deoxycholic and lithocholic
[Dehydroxylated primary bile acids by bacteria in gut]
122
What is the treatment for Budd-Chiari syndrome?
Porta-caval shunt
[Needs to connect to the IVC above the obstruction]
[UpToDate:
Initial treatment includes:
* Correcting underlying disorders that predisposed to the development of Budd-Chiari syndrome (when possible)
* Initiating anticoagulation unless there are contraindications
* Treating complications of portal hypertension (if present)
Additional treatments that may be appropriate for some patients include:
* Thrombolysis for patients with acute Budd-Chiari syndrome if a well-defined clot is present
* Angioplasty/stenting for symptomatic patients with venous obstructions that are amenable to angiographic treatment
* Liver transplantation for patients with acute liver failure; patients with acute liver failure should be referred to a liver transplantation center if possible for evaluation and management
If initial treatments fail, options include:
* TIPS placement or surgical shunting to decompress the liver
* Liver transplantation]
123
Which category of cirrhotic patient according to the Child-Pugh criteria may be considered for splenorenal shunt?
Child's A cirrhotics who present just with bleeding
[Rarely used anymore]
124
What causes physiologic jaundice of the newborn?
Immature glucuronyl transferase resulting in high unconjugated bilirubin
125
What percent of hepatic adenomas are symptomatic?
80%
[20% risk of significant bleeding (rupture)]
[UpToDate: Hepatic adenomas are uncommon benign epithelial liver tumors that develop in an otherwise normal-appearing liver. They are seen predominantly in young women (20 to 44 years old), are frequently located in the right hepatic lobe, and are typically solitary (70% to 80%), although multiple adenomas have been described in patients with prolonged contraceptive use, glycogen storage diseases, and hepatic adenomatosis. Adenomas range in size from 1 to 30 cm. Symptoms such as abdominal pain are more likely with larger lesions. Adenomas are typically diagnosed because of abdominal pain localized to the epigastrium or right upper quadrant, during an imaging study done for unrelated reasons, and (less commonly) because of detection of a mass on clinical examination or as a sudden, life-threatening hemodynamic collapse associated with rupture and intraabdominal bleeding.]
126
According to Couinaud's nomenclature, which numbered liver segment is the inferior right posterolateral segment?
Segment VI
127
What is the 5-year survival rate with resection of hepatocellular carcinoma?
30%
[UpToDate: There is a wide variability in outcomes after resection for HCC, attributable to both tumor-related factors and the underlying liver disease.
Long-term overall survival rates of ≥40% can be achieved with limited hepatic resections for small tumors (\<5 cm) in patients with Child-Pugh class A cirrhosis. The best outcomes are reported in carefully selected patients who have solitary lesions without intrahepatic metastasis or vascular invasion (gross or microscopic invasion of branches of the portal or hepatic veins), tumor diameter ≤5 cm, and a negative surgical margin of \>1 cm; 5-year survival rates are as high as 78%. Recent series demonstrate prolonged survival for more advanced HCC with proper patient selection despite major hepatectomy.
In contrast, studies with lower long-term survival rates usually include populations of patients with severe cirrhosis (Child-Pugh class B or C) or tumors greater than 8 cm in diameter, which require resection of more hepatic parenchyma. These patients have higher operative morbidity and mortality, and their intrinsic liver disease is a major contributor to reduced long-term survival.
Several staging systems have been developed to prognostically stratify outcomes from treatment of HCC. Some, like the tumor node metastasis (TNM) staging system of the American Joint Committee on Cancer (AJCC), are based entirely upon pathologic disease extent, while others, such as that of the Cancer of the Liver Italian Program (CLIP), take underlying liver function into account. There is ongoing debate as to which staging system is optimal for use in patients who do not undergo hepatic resection. Among hepatologists, the Barcelona Clinic Liver Cancer (BCLC) staging classification, is the most widely used staging system other than the AJCC staging classification, but many groups challenge the validity of the system because of good results in selected higher stage BCLC patients treated with surgery (where BCLC advises nonsurgical therapy).]
128
What is the treatment for hepatic encephalopathy?
1. Lactulose titrated to 2-3 stools daily (it is a cathartic that gets rid of bacteria in the gut and acidifies the colon to prevent NH3 uptake by converting it to ammonium)
2. Limit protein intake (less than 70 g/day)
3. Branched-chain amino acids (metabolized by skeletal muscle, may be of some value)
4. Neomycin (gets rid of ammonia-producing bacteria from the gut)
129
The liver stores large amounts of water-soluble or fat-soluble vitamins?
Fat-soluble vitamins
130
What are the 3 risk factors for hepatic sarcoma?
1. Polyvinyl chloride (PVC)
2. Thorotrast (radiocontrast agent in medical radiography in the 1930s and 1940s)
3. Arsenic (rapidly fatal)
[UpToDate: Angiosarcoma is a high-grade malignant vascular neoplasm, and it is the most common sarcoma arising in the liver. It occurs in older patients (\>60 years old) and is more common in men. It can be caused by exposure to vinyl chloride, arsenic, anabolic steroids, radiation, and thorium dioxide. Patients typically present with abdominal pain, fatigue, jaundice, ascites, and weight loss. Rarely, patients present with Budd-Chiari syndrome. Hepatomegaly with or without splenomegaly and thrombocytopenia are common findings. Metastases occur frequently and rapidly to the spleen, lymph nodes, lung, bone, and adrenals. Liver failure and intraabdominal bleeding due to liver rupture are common causes of death.
Angiosarcoma forms numerous poorly defined variably sized nodules that are soft, spongy, hemorrhagic, and necrotic. The entire liver is frequently involved. The tumor is composed of pleomorphic spindle or epithelioid cells, often with bizarre or multinucleated forms; mitoses are evident. Better-differentiated areas may show large cavernous spaces that are lined by atypical endothelial cells. The tumor cells grow along and into sinusoids, peliotic areas, and branches of portal and hepatic veins. Immunostaining shows positivity for vascular markers, such as the ERG transcription factor, CD31, CD34, and Factor VIII antigen.]
131
What is Budd-Chiari syndrome?
Occlusion of hepatic veins or IVC
[This leads to RUQ pain, hepatosplenomegaly, ascites, fulminant hepatic failure, muscle wasting, and variceal bleeding]
132
What portion of hepatic blood flow is supplied by the portal vein?
2/3
133
Diagnose:
* HBsAg: +
* Anti-HBs: -
* HBeAg: -
* Anti-HBe: +
* Anti-HBc: IgG
Low infectivity Chronic HBV
134
What are the two most common problems with hepatic resection?
1. Bleeding
2. Bile leak
135
Where in the hepatocytes of the liver is alkaline phosphatase normally located?
In the canalicular membrane
[The highly specialized canalicular (or apical) membrane of the hepatocyte is rich in microvilli and comprises 10-15% of the membrane surface area of the hepatocyte]
[UpToDate: Increased serum alkaline phosphatase is derived from tissues whose metabolism is either functionally disturbed (the obstructed liver) or greatly stimulated (placenta in the third trimester of pregnancy and bone in growing children). The cause in hepatobiliary disease has been debated. Two theories were proposed in the past: the damaged liver regurgitates hepatic alkaline phosphatase back into serum; and the damaged liver, particularly if due to obstructive jaundice, fails to excrete the alkaline phosphatase made in bone, the intestines, and the liver. This long-standing debate was resolved in favor of the regurgitation of liver alkaline phosphatase into serum. The data supporting this theory include the following:
* Only hepatic alkaline phosphatase is found in the serum of patients with liver disease, particularly cholestasis.
* The clearance rates of infused placental alkaline phosphatase are the same in patients with bile duct obstruction and healthy controls.
* In experimental bile duct obstruction in rats, the entire increase in serum alkaline phosphatase activity is due to the leakage of hepatic alkaline phosphatase into serum. The increased serum activity is paralleled by a striking elevation in hepatic alkaline phosphatase activity and is not accounted for by biliary retention of alkaline phosphatase.
Thus, the elevation in serum alkaline phosphatase in hepatobiliary disease results from increased de novo synthesis in the liver followed by release into the circulation. Retained bile acids appear to play a central role in this process; they induce the synthesis of the enzyme and may cause it to leak into the circulation, perhaps by disruption of hepatic organelles and solubilization of phosphatase bound to such membranes.
The precise manner in which the alkaline phosphatase reaches the circulation is unclear. In some patients with cholestasis, small vesicles that contain many basolateral (sinusoidal) membrane enzymes still bound to these membranes, including alkaline phosphatase, have been found in serum.]
136
What would an abdominal CT of a patient with an echinococcal liver abscess (hydatid cyst) reveal?
Ectocyst (calcified) and endocyst (double-walled cyst)
137
Which Hepatitis viruses are transmitted by blood or sexual contact
HBV, HCV, & HDV
138
Where in the hepatocytes of the liver does nutrient uptake occur?
Sinusoidal membrane
139
Conjugated bilirubin is broken down by bacteria in the terminal ileum and free bilirubin is reabsorbed and converted to what?
Urobilinogen
[Urobilinogen is eventually released in the urine as urobilin]
140
What is the difference in vascularity of primary liver tumors and metastatic liver tumors?
* Primary liver tumors are hypervascular
* Metastatic liver tumors are hypovascular
141
The middle hepatic artery is most commonly a branch of which artery?
Left hepatic artery
142
Fulminant hepatitis (acute liver failure) can occur with which types viral hepatitis?
B, D, and E
[Very rare with hepatitis A and C]
143
Which types of viral hepatitis can cause chronic hepatitis and hepatoma (hepatocellular carcinoma)?
B, C, and D
144
Diagnose:
* HBsAg: -
* Anti-HBs: +
* HBeAg: -
* Anti-HBe: +
* Anti-HBc: IgG
Recovery from HBV infection
145
What are some (10) risk factors for hepatocellular carcinoma?
1. Hepatitis B (#1 cause worldwide)
2. Hepatitis C
3. Ethanol
4. Hemochromatosis
5. Alpha-1-antitrypsin deficiency
6. Primary sclerosing cholangitis
7. Alfatoxins
8. Hepatic adenoma
9. Steroids
10. Pesticides
[Primary biliary cirrhosis and Wilson's disease are not risk factors]
146
How are hepatic adenomas diagnosed?
Sulfur colloid scan (no Kupffer cells in adenomas, thus no uptake (cold) on sulfur colloid scan)
[U/S characteristics are often non-specific and may mimic other benign or malignant liver lesions. CT imaging often reveals areas of hemorrhage, necrosis, or fibrosis, giving adenomas a heterogeneous appearance. MRI demonstrates a hypervascular tumor]
[UpToDate: The diagnosis should be made based upon the clinical setting, a combination of imaging studies, and/or surgical resection. Percutaneous liver biopsy or fine-needle aspiration are usually not indicated because adenomas have a tendency to bleed following biopsy and because the amount of tissue obtained using these methods is frequently insufficient for establishing a diagnosis.]
147
How do you diagnose a liver hemangioma?
MRI and CT scan show peripheral to central enhancement (Appears as a hypervascular lesion)
[UpToDate: The lesions classically opacify on CT after a delay of three or more minutes and remain isodense or hyperdense on delayed scans. MRI has emerged as a highly accurate, noninvasive technique for diagnosing hemangiomas with a sensitivity of approximately 90 percent and a specificity of 91 to 99 percent. The typical MRI appearance is a smooth, well-demarcated, homogeneous mass that has low signal intensity on T1-weighted images and is hyperintense on T2-weighted images. The presence of intratumoral fibrosis results in areas of low intensity on T2-weighted images.]
148
The gallbladder lies under which numbered segment(s) of the liver?
Segments IV and V
149
What are the King's College criteria of poor prognostic indicators for acetaminopen-induced acute liver failure?
Arterial pH less than 7.3
Or all of the following:
* INR greater than 6.5
* Creatinine greater than 3.4mg/dL
* Grade III/IV encephalopathy
150
What is a Sengstaken-Blakemore esophageal tube used for?
It has a balloon used to control variceal bleeding
[Risk of rupture of the esophagus so hardly used anymore]
151
According to Couinaud's nomenclature, which numbered liver segment is the superior left lateral segment?
Segment II
152
What is the best indicator of synthetic function of the liver in a cirrhotic patient?
Prothrombin time (PT)
153
Which two vessels join to form the portal vein?
1. Superior mesenteric vein
2. Splenic vein
154
What are the 3 most common bacteria causing spontaneous bacterial peritonitis?
1. E. Coli
2. Pneumococci
3. Streptococci
[Most commonly mono-organism infection; if not, need to worry about bowel perforation]
155
Into which vessel does the inferior mesenteric vein empty?
Splenic vein
156
Which three structures make up the portal triad?
1. Common bile duct
2. Portal vein
3. Proper hepatic artery
157
What markers will be present in an HBV vaccinated individual
IgG anti-HBs but no anti-HBc
158
What are 3 causes of pre-sinusoidal obstruction leading to portal hypertension?
1. Schistosomiasis
2. Congenital hepatic fibrosis
3. Portal vein thrombosis
[UpToDate: Schistosomiasis is one of the most common causes of noncirrhotic portal hypertension worldwide. Of the three main Schistosoma species, S. japonicum and S. mansoni are known to cause liver disease. S. hematobium affects mainly the urinary tract, although at advanced stages the liver can develop portal fibrosis. The acute stage of schistosomiasis mimics acute bacterial infection and is accompanied by marked eosinophilia. Chronic hepatic schistosomiasis is characterized by features of portal hypertension: esophageal varices, hepatomegaly, and splenomegaly with hypersplenism. The diagnosis of schistosomiasis can be made by the detection of schistosomal ova in the stool. Management includes treating underlying parasitic infection and preventing or treating the consequences of portal hypertension.
S. japonicum is distributed widely throughout the world, predominantly in Asia. S. mansoni is endemic in Egypt, Africa, the Middle East, and South America. Two other species (S. mekongi and S. intercalatum) also cause hepatic infections in endemic areas in Southeast Asia and Western Africa, respectively.
Pathophysiology — The infection occurs when the schistosomal cercariae enter the body through the skin. Adult worms eventually find their way to inhabit tributaries of the inferior (S. mansoni) or superior (S. japonicum) mesenteric veins, where they produce hundreds to thousands eggs per day for several years before the end of their lifespan. Some of these eggs pass through the intestinal mucosa and are excreted in the urine or feces to continue their life cycle. Others flow into the portal vein tributaries and become trapped in the terminal portal venules, where they induce chronic inflammation that is subsequently followed by marked fibrosis. S. japonicum is capable of producing far more eggs than S. mansoni and thus causes more severe liver disease.
In the early stages of infection, the portal resistance is mainly presinusoidal. However, as the fibrotic changes in the portal tracts progress, lobular distortion at the sinusoidal level occurs. This results in an increase in resistance to portal venous flow, as evidenced by an increased wedged hepatic venous pressure in advanced cases. Hemodynamic studies in patients with hepatic schistosomiasis have demonstrated a hyperdynamic systemic and splanchnic circulation with normal hepatic venous pressure gradient and total hepatic blood flow.
Praziquantel and oxamniquine are active against all forms of human schistosomiasis and are effective in eradicating the worms in the acute stage of the disease. Cure rates vary from 60% to 90% for S. mansoni and 60% to 80% for S. japonicum. Even if treatment does not kill all the worms, it will result in a sharp reduction in egg laying. In patients with chronic schistosomiasis, the worms no longer lay eggs and the patient may not require any specific antihelminthic treatment.]
159
Fever, abdominal pain, ascites with PMNs \> 250, positive culture of ascites fluid are all indicative of what?
Spontaneous bacterial peritonitis
160
Where is urea synthesized?
In the liver
161
How much albumin should be replaced following paracentesis for ascites?
1 gram of albumin for every 100cc removed
162
Which hormone is elevated in liver failure patients?
Aldosterone
[Secondary to impaired hepatic metabolism and impaired GFR]
163
Which benign liver tumor has walls with a characteristic blue hue?
Solitary cyst
[Congenital, seen in women, right lobe, most can be left alone because rarely cause complications]
164
Which numbered segments of the liver are drained by the middle hepatic vein?
Segment V and inferior portion of IV
165
What are the 3 categories of cirrhosis patients according to the Child-Pugh Score?
* Child's A: 5-6 points
* Child's B: 7-9 points
* Child's C: 10+ points
166
What is the treatment for a symptomatic liver hemangioma?
Surgery +/- pre-op embolization (Steroids, possible XRT for unresectable disease)
[UpToDate: Asymptomatic patients, particularly those with lesions \<1.5 cm, can be reassured and observed. Long-term follow-up of hemangiomas in such patients has confirmed that most do not grow or develop complications. We do not recommend follow-up imaging in patients with hemangiomas ≤5 cm in size, provided there is certainty of the diagnosis based on radiologic and on clinical details. On the other hand, rapid growth of a hemangioma has been reported, justifying radiologic follow-up of patients with lesions \>5 cm, particularly those in a sub capsular location. Our approach is to repeat imaging in 6 to 12 months for such lesions, using the imaging modality that best showed the hemangioma previously (eg, computed tomography scan or magnetic resonance imaging). If there is no change in the size of the lesion, we do not perform additional imaging. In the absence of symptoms, the risk of bleeding is too low to justify prophylactic resection.
Patients who have pain or symptoms suggestive of extrinsic compression of adjacent structures should be considered for surgical resection. However, it is important that all other causes of pain have been evaluated and excluded prior to surgery. This approach is supported in one report in which 25% of patients had persistence of symptoms after surgical resection. Overall, indications for surgical intervention have decreased to approximately 2% and include the development of complications such as rupture and intraperitoneal bleeding, which has a 20% mortality rate, incapacitating symptoms due to large hemangiomas, or failure to exclude a malignancy by radiologic studies. Giant hemangiomas only rarely turn out to be malignant vascular tumors when resected.]
167
According to Couinaud's nomenclature, which numbered liver segment is the inferior right anteromedial segment?
Segment V
168
What are 3 causes of post-sinusoidal obstruction leading to portal hypertension?
1. Budd-Chiari syndrome (hepatic vein occlusive disease)
2. Constrictive pericarditis
3. CHF
169
What should immediately be considered if a patient meets King's College criteria for liver failure?
Urgent liver transplant listing
170
What is the most common cancer worldwide?
Hepatocellular carcinoma
171
Which syndrome is characterized by a deficiency in storage ability that leads to high conjugated bilirubin?
Rotor's syndrome
[UpToDate: The syndrome is characterized by chronic conjugated and unconjugated hyperbilirubinemia without evidence of hemolysis. Although Rotor syndrome had been thought previously to be a variant of Dubin-Johnson syndrome, the two conditions are now understood to be different entities. In Rotor syndrome, there is a defect in hepatic storage of conjugated bilirubin, which leaks into the plasma, resulting in hyperbilirubinemia. Rotor syndrome is also a benign condition.
As in Dubin-Johnson syndrome, Rotor syndrome does not require treatment. However, the diagnosis is important to avoid confusion with other hepatobiliary diseases.]
172
What is the most common cause of massive hematemesis in children?
Portal hypertension
[Usually due to extra-hepatic portal vein thrombosis]
173
What does Cantlie's line separate?
The right and left lobes of the liver
174
What is the most common hepatic artery variant?
Right hepatic artery off superior mesenteric artery
[Courses behind pancreas, posterolateral to the common bile duct]
175
According to Couinaud's nomenclature, which numbered liver segment is the superior right anteromedial segment?
Segment VIII
176
What causes postpartum liver failure with ascites?
Hepatic vein thrombosis
[Has an infectious component]
177
Which type of benign liver tumor is common in women (especially those taking OCPs) and in individuals with a history of steroid use?
Hepatic adenoma
[UpToDate: Hepatic adenoma is a benign epithelial liver tumor that usually occurs in a noncirrhotic liver. It is most commonly seen in premenopausal women older than 30 years of age. The majority of patients have used oral contraceptives for more than two years prior to diagnosis. Hepatic adenoma has also been noted in patients with type 1 glycogen storage diseases. Hepatic adenoma has a small risk of neoplastic transformation.]
178
What are bile acids conjugated to in the production of bile salts?
Taurine and Glycine
[Improves water solubility]
[Wikipedia: Prior to secreting any of the bile acids (primary or secondary, see below), liver cells conjugate them with one of two amino acids, glycine or taurine, to form a total of 8 possible conjugated bile acids. These conjugated bile acids are often referred to as bile salts because of their physiologically-important acid-base properties. The pKa of the unconjugated bile acids are between 5 and 6.5, and the pH of the duodenum ranges between 3 and 5, so when unconjugated bile acids are in the duodenum, they are almost always protonated (HA form), which makes them relatively insoluble in water. Conjugating bile acids with amino acids lowers the pKa of the bile-acid/amino-acid conjugate to between 1 and 4. Thus conjugated bile acids are almost always in their deprotonated (A-) form in the duodenum, which makes them much more water-soluble and much more able to fulfil their physiologic function of emulsifying fats.]
