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FF GI > Liver pathology > Flashcards

Liver pathology Flashcards

1
Q

The ____ surface of hepatocytes faces the canaliculi, whereas the ____ surface faces the sinusoids.


A

Apical; basolateral


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2
Q

In a patient with viral hepatitis, what zone of the liver is first affected?


A

Zone I, the periportal zone


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3
Q

A patient suffers septic shock and becomes hypotensive. Which zone of the liver is first to suffer necrosis?


A

Zone III, the pericentral vein zone, which is a watershed area


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4
Q

Drugs metabolized by the P-450 system are most likely to be found in which zone of the liver?


A

Zone III


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5
Q

List the three parts of the liver that make up the portal triad.


A

Bile ductule, portal vein, and hepatic artery


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6
Q

Lymph drains from the liver via which histologic structure?


A

The space of Disse


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7
Q

A teenaged cocaine addict presents with elevated liver enzymes. What zone of the liver is cocaine most likely affecting?


A

Zone I

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8
Q

During an autopsy, a 52-year-old man from the United States is found to have a shrunken, nodular liver. What is the most likely etiology?


A

Alcohol (responsible for 60–70% of cirrhosis in the United States)


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9
Q

Esophageal varices and caput medusae are caused by ____ (pathologic state) and are partially alleviated by ____ shunts.


A

Portal hypertension; portosystemic


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10
Q

A patient with musty-smelling breath presents to the ED with confusion and asterixis. What skin findings do you expect on exam?


A

Common skin findings in liver failure are jaundice and spider nevi (the musty breath is fetor hepaticus) & may see caput medusae, edema


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11
Q

A patient with melena is found to have esophageal varices. Where else in the GI tract are you concerned for bleeding?


A

The stomach, due to bleeding peptic ulcers (or may be caused by portal hypertension)


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12
Q

Name at least three direct effects of portal hypertension.


A

Splenomegaly, caput medusae, ascites, esophageal varices with hematemesis, peptic ulcers, anorectal varices, portal hypertensive gastropathy


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13
Q

An alcoholic has cirrhosis due to liver failure. What do you expect to find on eye exam?


A

Scleral icterus


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14
Q

While seeing a patient with known cirrhosis, you cannot help but notice his musty breath. What is this called?


A

Fetor hepaticus


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15
Q

Visible dilated capillary proliferation within the skin secondary to the effects of liver failure and cirrhosis is called what?


A

Spider nevi


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16
Q

A man with liver failure also complains of gynecomastia and testicular atrophy. How did this likely develop?


A

Due to an increase in estrogen


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17
Q

A patient with cirrhosis has a coarse, flapping tremor of the hands. What is this called?


A

Asterixis


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18
Q

A cirrhotic patient should be cautioned about what kind of hematologic abnormality(ies) due to her liver cell failure?


A

Bleeding tendency (decreased production of clotting factors, increased prothrombin time) and anemia


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19
Q

Why do patients with cirrhosis have an increased tendency to bleed?


A

Liver cell failure decreases production of prothrombin and clotting factors


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20
Q

Is the bleeding tendency in cirrhosis considered an effect of portal hypertension or an effect of liver cell failure?


A

Liver cell failure (it is due to the inability to synthesize clotting factors)


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21
Q

Is the ankle edema of cirrhosis considered an effect of portal hypertension or an effect of liver cell failure?


A

Liver cell failure (it is due to the inability to synthesize albumin resulting in lack of oncotic pressure)


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22
Q

What is cirrhosis?


A

Cirrhosis is defined as diffuse fibrotic and nodular regeneration that disrupts the normal architecture of the liver

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23
Q

What two gastrointestinal enzymes are markers of injury to hepatocytes?


A

The aminotransferases, which are alanine aminotransferase and aspartate aminotransferase


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24
Q

A patient has viral hepatitis. What do you expect the ratio of aspartate aminotransferase to alanine aminotransferase to be?


A

Low (in viral hepatitis, AST

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25
Q

A patient has alcoholic hepatitis. What do you expect the ratio of aspartate aminotransferase (AST) to alanine aminotransferase (ALT) to be?


A

High (in alcoholic hepatitis, AST > ALT)


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26
Q

A 40-year-old man is intoxicated and not making any sense. Elevated serum γ-glutamyl transpeptidase may indicate which diagnosis?


A

Chronic alcoholism, along with possible Wernicke encephalopathy


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27
Q

A patient has elevated alkaline phosphatase. To differentiate between bone disease and liver pathology, what marker do you use?


A

γ-glutamyl transpeptidase (like ALP, GGT is elevated in liver and biliary disease, but unlike ALP, it is not elevated in bone disease)


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28
Q

Alkaline phosphatase, in addition to being a marker of liver disease, is also a marker of ____ disease.


A

Bone


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29
Q

Which two gastrointestinal enzymes are used as markers for acute pancreatitis? Which one is more specific?


A

Amylase and lipase; lipase is more specific


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30
Q

A patient has epigastric abdominal pain radiating to the back, fever, and nausea. What two enzymes will likely be elevated on lab studies?


A

Amylase and lipase will likely be elevated (the patient has acute pancreatitis)


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31
Q

Which serum protein is decreased in Wilson disease?


A

Ceruloplasmin


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32
Q

A patient has bronze rings in the irises, liver cirrhosis, muscle rigidity, and dystonia. What serum protein is most useful to check?


A

Ceruloplasmin is decreased, because this patient likely has Wilson disease (the bronze rings are Kayser-Fleischer rings)


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33
Q

A boy who has never received any vaccines comes to the ED with an elevated amylase level (lipase is normal). Name a likely diagnosis.


A

Mumps

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34
Q

A 5-year-old boy with seasonal flu is given aspirin for his fever and develops altered mental status and elevated LFTs. Diagnosis?


A

Reye syndrome


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35
Q

What pathologic liver change is associated with Reye syndrome?


A

Microvesicular fatty changes and, later on, hepatomegaly


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36
Q

A 7-year-old girl is thought to have Reye syndrome. What metabolic disturbance do you check for (it shows up on a basic metabolic panel)?


A

Hypoglycemia


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37
Q

A mother brings in her son for lethargy and vomiting. A week ago he had a viral infection treated with a drug. What was he likely given?


A

Aspirin or salicylates (the boy likely developed Reye syndrome, which can occur in children with viral infections treated with aspirin)


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38
Q

List two viral infections that are strongly associated with Reye syndrome.


A

Varicella zoster virus, influenza B


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39
Q

A mother asks if she can give her 4-year-old son aspirin for his fever of 101°F. What drug would you recommend instead?


A

Acetaminophen or ibuprofen


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40
Q

How does aspirin cause Reye syndrome in young infants?


A

Aspirin metabolites reversibly inhibit mitochondrial enzymes and cause a decrease in β-oxidation


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41
Q

What potentially reversible liver pathology can be seen with moderate alcohol intake?


A

Macrovesicular fatty changes of the liver (hepatic steatosis)


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42
Q

A patient with chronic alcoholism develops hepatitis. What microscopic changes do you expect to see on liver biopsy?


A

Swollen and necrotic hepatocytes with neutrophilic infiltration and Mallory bodies (he likely has alcoholic hepatitis)


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43
Q

You test the AST and ALT levels of a long-term alcoholic with sustained alcohol consumption. What do you expect them to be?


A

AST > ALT (the ratio is usually >1.5) (make a toast with alcohol)


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44
Q

A 48-y/o woman with chronic alcoholism crashes her car and lacerates her liver. She goes to the OR. How do you expect her liver to appear?


A

She will likely have a micronodular, irregularly shrunken liver (also called hobnail appearance) (this is alcoholic cirrhosis)


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45
Q

An alcoholic patient has jaundice, a “hobnail” liver, and hypoalbuminemia. If the patient quits drinking, will these symptoms go away?


A

No, as this describes a micronodular, shrunken liver appearance indicating alcoholic cirrhosis, which is irreversible

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46
Q

What is the AST:ALT ratio in non-alcoholic fatty liver disease?


A

ALT > AST (ALT = Lipids)


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47
Q

A non-alcoholic, type II diabetic patient has cirrhosis and a high ALT:AST ratio. What pathologic changes are associated with this disease?


A

Insulin resistance = fatty infiltration of hepatocytes & cellular ballooning on pathology (as seen in non-alcoholic fatty liver disease)


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48
Q

What type of syndrome is associated with non-alcoholic fatty liver disease?


A

Metabolic syndrome (insulin resistance)


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49
Q

A non-alcoholic man with a high ALT:AST ratio and metabolic syndrome should be advised that he is at risk for what type(s) of liver disease?


A

Cirrhosis and HCC (he has non-alcoholic fatty liver disease)

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50
Q

What are some triggers of hepatic encephalopathy?


A

Increased NH3 production (protein intake, GI bleeding, constipation, infection), decreased NH3 removal (renal failure, diuretics, TIPS)


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51
Q

A patient with hepatic encephalopathy is prescribed lactulose. He is also a medical student who wonders what lactulose does. You say?


A

Lactulose increases NH4+ generation, which decreases NH3 that can contribute to hepatic encephalopathy


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52
Q

What two viral diseases are associated with an increased incidence of hepatocellular carcinoma?


A

Hepatitis B and C


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53
Q

Excessive exposure to what two substances is associated with an increased incidence of hepatocellular carcinoma?


A

Alcohol (alcoholic cirrhosis) and carcinogens such as aflatoxin


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54
Q

A 47-y/o man’s friend is diagnosed with hepatocellular carcinoma. The man asks what factors might increase his risk for this disease.


A

Hepatitis B and C, Wilson disease, hemochromatosis, α1-antitrypsin deficiency, alcoholic cirrhosis, carcinogens (e.g., aflatoxins)


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55
Q

A 52-y/o man comes in with RUQ abdominal pain and is convinced that this could be liver cancer. What signs/symptoms could you check for?


A

Jaundice, tender hepatomegaly, ascites, polycythemia, anorexia


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56
Q

A doctor suspects hepatocellular carcinoma in a patient. She orders what lab, expecting it to be elevated?


A

α-fetoprotein, because it is elevated in hepatocellular carcinoma


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57
Q

How might a hepatocellular carcinoma predispose a patient to Budd-Chiari syndrome?


A

Via polycythemia (a hypercoagulable state leading to thrombosis in the hepatic vein) or by direct compression of the hepatic vein


58
Q

A woman with hemochromatosis develops jaundice and ascites and is found to have elevated serum α-fetoprotein levels. What is the diagnosis?


A

Hepatocellular carcinoma

59
Q

Under what circumstances would a liver biopsy for a patient with a liver tumor be contraindicated?


A

If the tumor is thought to be a cavernous hemangioma due to potential aspiration causing a life-threatening hemorrhage


60
Q

A liver mass is incidentally discovered in a 34-year-old man with no risk factors for carcinoma. Do go you straight to biopsy?


A

No, as this may be a cavernous hemangioma, commonly occurring in people 30–50 y/o for whom biopsy is dangerous due to bleeding risk


61
Q

A patient on oral contraceptives is discovered to have a benign liver tumor. What is she most likely to have?


A

Hepatic adenoma


62
Q

A patient often works with arsenic and polyvinyl chloride. He is found to have a malignant liver tumor. What liver tumor do you suspect?


A

Angiosarcoma


63
Q

A woman has a liver mass seen on ultrasound, and you are concerned that this could be a metastasis from a malignancy of which organs?


A

GI, lung, and breast tumors (metastases are the most common liver tumors)

64
Q

Blood backup to liver, commonly caused by right-sided heart failure and Budd-Chiari syndrome, leads to what liver finding?


A

Nutmeg liver (mottled liver appearance)


65
Q

The liver of your patient with hepatic vein thrombosis is mottled, similar to nutmeg. If this condition persists, what outcome might occur?


A

Centrilobular congestion and necrosis can be expected, possibly leading to cirrhosis if it persists (this is Budd-Chiari syndrome)


66
Q

In Budd-Chiari syndrome, there is occlusion of the ____ vena cava and/or the ____ veins.


A

Inferior; hepatic


67
Q

What liver region becomes congested and necrotic in Budd-Chiari syndrome?


A

The centrilobular region


68
Q

A patient with polycythemia vera develops new-onset ascites, abdominal pain, and elevation of liver enzymes. What do you suspect?


A

Congestive liver failure secondary to Budd-Chiari syndrome


69
Q

What medical conditions are often associated with Budd-Chiari syndrome?


A

Polycythemia vera, pregnancy (postpartum state), hypercoagulable states, hepatocellular carcinoma


70
Q

A patient with known hypercoagulability has developed ascites with visible abdominal and back veins. What exam finding can rule out HF?


A

Absence of JVD would support Budd-Chiari syndrome (the occlusion is distal) instead of HF

71
Q

In your patient with prolonged expiratory time, what type of emphysema is more consistent with α1-antitrypsin deficiency than with COPD?


A

Panacinar emphysema caused by breakdown of elastic fibers of the lungs (as opposed to centriacinar [centrilobular] emphysema in COPD)


72
Q

α1-antitrypsin deficiency can be diagnosed histologically by seeing what in liver biopsy samples?


A

PAS-positive globules


73
Q

A patient has panacinar emphysema and a liver tumor. He has a certain protein deficiency. What is the pattern of inheritance of this trait?


A

Codominant (this patient has α1-antitrypsin deficiency, which is a codominant trait)

74
Q

Is the hyperbilirubinemia of an intrahepatic process (such as hepatitis or cirrhosis) conjugated, unconjugated, or mixed?


A

This is mixed (direct and indirect) hyperbilirubinemia


75
Q

Lab results show high direct and indirect bilirubin levels. Is this process conjugated, unconjugated, or mixed?


A

This is mixed (direct and indirect) hyperbilirubinemia


76
Q

Is the hyperbilirubinemia of biliary tract obstruction conjugated, unconjugated, or mixed?


A

This is conjugated (direct) hyperbilirubinemia


77
Q

Labs show high direct bilirubin and normal indirect bilirubin levels. What types of biliary disease process might cause this?


A

Obstructive processes such as primary sclerosing cholangitis or biliary cirrhosis (this is a conjugated [direct] hyperbilirubinemia)


78
Q

A 5-day-old infant has persistent hyperbilirubinemia despite phototherapy. Is it likely conjugated, unconjugated, or mixed?


A

This is unconjugated (indirect) hyperbilirubinemia, likely due to hemolysis


79
Q

Labs show high indirect bilirubin levels. Does this represent a conjugated, unconjugated, or mixed hyperbilirubinemia?


A

Unconjugated (indirect) hyperbilirubinemia


80
Q

A patient with Dubin-Johnson syndrome comes in to your office. What type of hyperbilirubinemia does she have?


A

Conjugated (direct) (Dubin-Johnson is a defect of bile excretion)


81
Q

A patient presents with Crigler-Najjar syndrome. What sort of hyperbilirubinemia does she have?


A

Unconjugated (indirect) (Crigler-Najjar is a defect of conjugation)


82
Q

Name some causes of an unconjugated (indirect) hyperbilirubinemia.


A

Hemolysis, physiologic (newborns), Crigler-Najjar syndrome, Gilbert syndrome


83
Q

Name some causes of a conjugated (direct) hyperbilirubinemia that are due to biliary tract obstruction.


A

Gallstones, cholangiocarcinoma, pancreatic or liver cancer, liver flukes


84
Q

Name some causes of a conjugated (direct) hyperbilirubinemia that are due to biliary tract disease but are not obstructive.


A

Primary sclerosing cholangitis, primary biliary cirrhosis


85
Q

Name some causes of a conjugated (direct) hyperbilirubinemia that are due to bile excretion defects.


A

Dubin-Johnson syndrome, Rotor syndrome

86
Q

A newborn has a yellowish hue for 12 hours after birth. The doctor says it is a benign condition. What is its molecular basis?


A

Immature UDP-glucuronosyltransferase in the infant, leading to increased unconjugated bilirubin levels and jaundice


87
Q

A newborn has a yellow hue for 10 hours after birth. The doctor says it is due to immature UDP-glucuronosyltransferase. Treatment?


A

Phototherapy, which converts the unconjugated bilirubin into a water-soluble form that can be excreted in urine


88
Q

A newborn has severe physiologic neonatal jaundice. A doctor wants to start phototherapy to “protect the brain.” What does he mean by this?


A

Untreated unconjugated hyperbilirubinemia can cause kernicterus (bilirubin deposition in the brain, particularly in the basal ganglia)

89
Q

A college student with no prior medical history presents with scleral icterus during final exams. What is the likely diagnosis?


A

Gilbert syndrome, likely precipitated by fasting or stress secondary to studying


90
Q

The parents of a child with Gilbert syndrome want to know what complications to expect. What do you tell them?


A

Gilbert syndrome has no clinical consequences other than jaundice


91
Q

A woman occasionally has jaundice instigated by stress that goes away without treatment. What is the pathogenesis of this benign condition?


A

Slightly decreased UDP-glucuronosyltransferase activity or decreased bilirubin uptake by hepatocytes (this is Gilbert syndrome)


92
Q

Which levels are elevated in Gilbert syndrome: conjugated bilirubin, unconjugated bilirubin, or both?


A

Unconjugated bilirubin (since UDP-glucuronosyltransferase activity is decreased)


93
Q

A 1-y/o girl has a new unconjugated hyperbilirubinemia with no laboratory indications of hemolysis. What asymptomatic condition is likely?


A

Gilbert syndrome, which can be triggered by fasting and stress in predisposed individuals


94
Q

A newborn has defective activity of UDP-glucuronosyltransferase, usually resulting in death within a few years of birth. Diagnosis?


A

Crigler-Najjar syndrome type I


95
Q

A 2-week-old boy is diagnosed with Crigler-Najjar syndrome type I. What do you tell the parents about the prognosis?


A

Death occurs in childhood


96
Q

A patient has jaundice, kernicterus, and high unconjugated bilirubin levels. He dies at age 3 years. What enzyme is missing in his disease?


A

UDP-glucuronosyltransferase (the patient has Crigler-Najjar syndrome type I)


97
Q

The parents of a jaundiced newborn are concerned that he has Crigler-Najjar syndrome. What type of bilirubinemia would rule this out?


A

Conjugated hyperbilirubinemia (as Crigler-Najjar causes impaired conjugation, leading to an unconjugated hyperbilirubinemia)


98
Q

What is kernicterus?


A

Bilirubin deposition in the brain


99
Q

What would a neonate with absent UDP-glucuronosyltransferase and cerebral palsy be treated with?


A

Plasmapheresis and phototherapy (this is Crigler-Najjar syndrome)


100
Q

A baby has hyperbilirubinemia from low UDP-glucuronosyltransferase. How can a drug-enhancing liver enzyme synthesis help you prognosticate?


A

Phenobarbital enhances enzyme synthesis in Crigler-Najjar syndrome type II (less severe/a better prognosis), but has no effect for type I


101
Q

A newborn has persistent hyperbilirubinemia despite phototherapy. What type of bilirubinemia supports a diagnosis of Dubin-Johnson syndrome?


A

Conjugated hyperbilirubinemia (Dubin-Johnson is a defect of hepatic excretion of bile)


102
Q

What physiologic process is defective in patients with Dubin-Johnson syndrome?


A

Excretion of bilirubin from hepatocytes


103
Q

A patient has defective excretion of direct bilirubin. Gross pathology shows a black liver. Diagnosis?


A

Dubin-Johnson syndrome, which has the characteristic black liver on gross pathology


104
Q

You diagnose Dubin-Johnson syndrome in a neonate with conjugated hyperbilirubinemia. How do you explain the prognosis to the parents?


A

There are no clinical consequence, as this condition is benign


105
Q

A patient has disease symptoms similar to Dubin-Johnson syndrome, but you do not find a grossly black liver. Does the prognosis change?


A

No (this is Rotor syndrome, which is a milder form of Dubin-Johnson syndrome)


106
Q

A 22-year-old new to your practice has a history of benign unconjugated hyperbilirubinemia. Which hepatic enzyme is likely affected?


A

Glucuronosyltransferase (specifically UDP-glucuronosyltransferase in the case of Gilbert syndrome)


107
Q

Unconjugated bilirubin is formed from ____.


A

Hemoglobin


108
Q

What cells within the liver are part of the mononuclear phagocyte system, aiding in the conversion of hemoglobin to unconjugated bilirubin?


A

Kupffer cells


109
Q

Describe the properties of circulating bilirubin (in normal healthy individuals). What allows it to be transported through the bloodstream?


A

Circulating bilirubin is unconjugated and water-insoluble; must be bound to albumin for transport


110
Q

To enter hepatocytes for processing, what two spaces must circulating bilirubin pass through?


A

Junctions between endothelial cells in the hepatic sinusoid, followed by the space of Disse


111
Q

A man has a rare mutation that slightly inhibits passage of bilirubin through the space of Disse. This presents similarly to what syndrome?


A

Gilbert syndrome (can consider this to be a form of impaired bilirubin uptake)


112
Q

Within hepatocytes, what enzyme is tasked with converting unconjugated bilirubin into conjugated bilirubin? What function does it perform?


A

UDP-glucuronosyltransferase, it conjugates the bilirubin


113
Q

After being acted on by UDP-glucuronosyltransferase, how does bilirubin differ from a previously circulating form?


A

It is now conjugated (bilirubin diglucuronide) and is more water soluble


114
Q

Conjugated bilirubin is secreted into what structure so that it may become a part of bile?


A

The lumen of the bile canaliculus


115
Q

A patient has jaundice and RUQ pain. A common bile duct gallstone is detected. This most directly impairs what step of bilirubin metabolism?


A

Secretion of conjugated bilirubin into the lumen of the bile canaliculus due to obstruction/stasis

116
Q

opper normally enters the blood circulation in what form?


A

As ceruloplasmin


117
Q

In a patient with Wilson disease, which metal tends to accumulate in the liver, brain, cornea, kidneys, and joints?


A

Copper


118
Q

Why would a patient with Wilson disease have asterixis?


A

Wilson patients eventually develop liver failure, which can present with asterixis because the liver cannot metabolize ammonia


119
Q

A man with a defective ATP7B gene is seen by a neurologist for parkinsonian symptoms. What portion of the brain is most likely degenerated?


A

The basal ganglia (due to copper deposition from Wilson disease)


120
Q

You suspect Wilson disease in a patient referred to your neurology practice. What substance is classically decreased in lab studies?


A

Ceruloplasmin


121
Q

A man has Wilson disease. His physician informs him that which hepatic changes will likely occur?


A

Liver failure, cirrhosis, hepatocellular carcinoma


122
Q

What cancer is associated with Wilson disease?


A

Hepatocellular carcinoma


123
Q

A patient with Wilson disease presents with choreiform movements. This is due to copper deposition in which area of the brain?


A

The basal ganglia


124
Q

The parents of a child with Wilson disease ask about the chances that their future children also having the disease. How do you respond?


A

There is a 25% chance, as Wilson disease has autosomal recessive inheritance


125
Q

What type of anemia is associated with Wilson disease?


A

Hemolytic anemia


126
Q

A 30-year-old patient has new-onset dementia, elevated liver function tests, and discolored rings around his irises. Diagnosis?


A

Wilson disease


127
Q

A patient has new dementia, high liver function tests, and discolored rings around his irises. What other lab result confirms the diagnosis?


A

Low serum ceruloplasmin levels (the patient has Wilson disease)


128
Q

Copper is normally excreted into bile by a hepatocyte copper transporting ____, which is coded by the ____ gene on chromosome ____.


A

ATPase, ATP7B, 13


129
Q

A patient with Wilson disease that affects the kidneys will present with what syndrome?


A

Fanconi syndrome


130
Q

How does the mnemonic “Copper is Hella BAD” help you remember the classic features of Wilson disease?


A

Ceruloplasmin, Cirrhosis, Corneas, Copper Carcinoma (HCC), Hemolysis, Basal ganglia, Asterixis, Dementia Dyskinesia, Dysarthria

131
Q

Hemochromatosis classically affects which three organs, causing which three classic symptoms?


A

Liver (symptom of micronodular cirrhosis), skin (pigmentation), and pancreas (bronze diabetes)


132
Q

A man recently diagnosed with insulin-dependent diabetes mellitus has pigmented skin and elevated LFTs. What does liver biopsy show?


A

Liver biopsy will diagnose it with a positive Prussian blue stain (this is hemochromatosis)


133
Q

A 42-year-old woman with HLA-A3–related iron disease could be at risk of what cardiac complication? How?


A

Congestive heart failure; secondary to cardiomyopathy (caused by iron deposition in the heart, from hemochromatosis)


134
Q

A patient new to your practice has known hemochromatosis. For which cancer should you be vigilant because of this?


A

Hepatocellular carcinoma


135
Q

What is the pattern of inheritance of primary hemochromatosis?


A

Autosomal recessive


136
Q

Primary hemochromatosis is due to ____ or ____ mutation on the HFE gene.


A

C282Y, H63D


137
Q

Primary hemochromatosis is associated with this type of human leukocyte antigen (HLA).


A

HLA-A3


138
Q

Secondary hemochromatosis is mainly caused by what?


A

Chronic transfusion therapy, which can occur in the setting of disorders such as β-thalassemia major


139
Q

Describe ferritin levels, iron levels, total iron-binding capacity, and transferrin saturation in patients with hemochromatosis.


A

High, high, low, high


140
Q

A patient has a C282Y mutation on the HFE gene that causes symptoms. What are some of his treatment options?


A

Repeated phlebotomy, chelation with deferasirox, deferoxamine, deferiprone (oral) (this is hereditary hemochromatosis)


141
Q

A woman undergoing menstruation notices that her “bronze” diabetes is not as severe. Skin pigmentation is also decreased. Why?


A

Iron is lost through menstruation, slowing the progression of hemochromatosis in women


142
Q

A man with liver disease triggers a metal detector while passing through airport security, but no metal is found on him. Explain.


A

He likely has hemochromatosis with a total body iron level >50 g, which can set off metal detectors at airports

FF GI (10 decks)

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