71 terms

Liver, Porphyrins, and Hemoglobin

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The "port wine" color of some urines can be attributed to the following?
porphyrins
p-dimethylaminobenzaldehyde is a constituent of which reagent?
Ehrlich
Both porphobilinogen and urobilinogen form a red-colored compound with Erlich's reagent. How can they be differentiated?
urobilinogen is soluble in chloroform
Prehepatic bilirubin has which of the following for a protein carrier?
albumin
Direct-reacting bilirubin is also known as what substance?
bilirubin diglucuronide
An increase in indirect-reacting bilirubin is suggestive of which condition?
hemoglobin breakdown
Bilirubin is converted in the intestine to which of the following substances?
urobilinogen
Which of the following will interfere with Evelyn Malloy method for bilirubin?
hemolysis
Which precaution should be followed in regard to specimen handling instructions for bilirubin determination?
protect from light
Which protein transports the majority of copper in the bloodstream?
ceruloplasmin
Increased serum copper is seen in which disease?
Wilson's disease
A break down product of hemoglobin is
bilirubin
Hemoglobin S can be separated by hemoglobin D by
electrophoresis on a different medium and acidic pH
On electrophoresis at alkaline pH, what is the slowest migrating hemoglobin
Hgb C
The hemoglobin that is resistant to alkali (KOH) denaturation is
hemoglobin F
Urobilinogen is formed in the
intestine
In bilirubin determination the purpose of adding a concentrated caffeine solution or methyl alcohol is to
allow indirect bilirubin to react with color reagent
If the total bilirubin is 3.1 mg/dL and the conjugated bilirubin is 2.0 mg/dL, the unconjugated bilirubin is
1.1 mg/dL
The principle of the tablet test for bilirubin in urine or feces is
chemical coupling of bilirubin with a diazonium salt to form a purple color
A serum sample was assayed for bilirubin at 10 am, and the result was 12 mg/dL, the same sample was retested at 3 pm, the result is now 8 mg/dL, the most likely explanation for this discrepency is
the sample was exposed to light
In the liver bilirubin is converted into
bilirubin diglucuronide
In which of these disease states is conjugated bilirubin a major serum component?
biliary obstruction
Kernicterus is an abnormal accumulation of bilirubin in
brain tissue
In which condition does decreased activity of glucoronyl transferase result in increased unconjugated bilirubin and kernicterus in neonates?
Crigler-Najjar syndrome
What substance gives feces it's normal color?
urobilin
A condition in which erythrocyte protoporphyrin is increased is
iron deficiency anemia
Which compounds originally condense to form aminolevulinic acid?
succinyl coenzyme A and glycine
What compound chelates iron and is the immediate precursor of heme formation?
protoporphyrin IX
Qualitative screening test for porphobilinogen that may be performed to aid in the diagnosis of the porphyrias?
Watson-Schwartz
What compound may be detected by observing it orange-red fluorescence in acid solution?
coproporhyrin
What is the immediate precursor of bilirubin formation?
biliverdin
To quantify serum bilirubin levels, it is necessary that bilirubin couples with diazotized sulfanic acid to form what complex?
azobilirubin
What enzyme catalyzes the conjugation of bilirubin?
uridine diphosphate glucuronyltransferase
What breakdown product of bilirubin metabolism is produced in the colon from the oxidation of urobilinogen by micro-organisms?
urobilin
Functions as transport protein for bilirubin in the blood
albumin
What term is used to describe the accumulation of bilirubin in the skin?
jaundice
In the condition kernicterus, the abnormal accumulation of bilirubin occurs in what tissue?
brain
As a reduction product of bilirubin catabolism, this compound is partially reabsorbed from the intestine through the portal circulation for reexcretion by the liver. What is this compound?
urobilinogen
Which bilirubin fraction is unconjugated and covalently bound to albumin?
delta
What reagent system contains the components sulfanilic acid, hydrochloric acid, and sodium nitrite?
diazo
Which of the following enzymes would best aid in identifying hepatobiliary disease?
alkaline phosphatase (ALP)
In what cells does the conjugation of bilirubin take place?
hepatocytes
Which of the following enzymes is responsible for the conjugation of bilirubin?
UDP-glucuronyl transferase
What fraction of bilirubin is water soluble and reacts with diazo reagent without the addition of an accelerator?
conjugated bilirubin
Which form of hepatitis is caused by a DNA virus?
hepatitis B
What enzyme is most useful in establishing the hepatic origin of an elevated serum alkaline phosphatase?
5'-nucleotidase
Hepatitis E is most likely to cause serious consequences in
pregnant women
Worldwide, most primary malignant tumors of liver are related to
alcoholism
The reagent p-dimethylaminobezaldehyde is used to measure
urobilinogen
What condition would result in elevations in primarily conjugated bilirubin
Dubin-Johnson syndrome
A urinalysis dipstick test indicated that urobilinogen was absent. What condition does that support
biliary obstruction
A patient presents with elevated levels of IgG anti-HAV while levels of IgM and anti-HAV are non detectable. This patient is likely to
have an immunity to HAV
What is the "rate limiting" step of heme synthesis?
aminolevulinicacid (ALA) synthase
Laboratory indicators of acute liver disease are serum bilirubin levels that are____, serum AST that is_____, and serum ALT that is____.
increased, increased, increased
______ is the current diagnostic marker for hepatitis B virus infection, with detection being less than _______ after infection
HBsAg, 4 weeks
For hepatitis delta virus to be present, what other hepatitis virus must the patient have
...
Characteristics of hepatitis C virus
-transmitted by blood, needle sharing, sexually
-primary cause of chronic liver disease, such as cirrhosis
-majority of HCV-infected pts are asymptomatic
What would be the laboratory interpretation of the following laboratory data for a hepatitis B serology profile?
HBsAg-negative
Anti-HBcT-negative
Anti-HBs positive
immune due to vaccination
version of the Western blot technique and is used to confirm the presence of antibodies to hepatitis C virus in a pt's serum
recombinant immunoblot assay (RIBA)
Common causes of acute hepatitis
hepatitis A,B,C
Laboratory findings in Wilson's disease include _____ceruloplasmin, ______ total plasma copper, ________free plasma copper, and _______urine copper
decreased, decreased, increased, increased
Which of the following syndromes has a mutation in the upstream promoter region of the gene for UDP-glucuronyl transferase 1 (UGT1) and results in reduced production of the enzyme
Gilbert's syndrome
Screening/ confirmatory test for Wilson's disease
screening test: ceruloplasmin, serum/ urine copper
confirmatory test: liver biopsy, genetic studies
Characteristics of icteric phase of an acute, symptomatic, viral hepatitis infection
-yellowing of the sclera of the eye
-decreased ability of the injured liver to clear conjugated bilirubin into the bile duct
-increasing serum levels of conjugated bilirubin
-dark yellow urine
The liver
-primary organ responsible for the metabolism of carbohydrates, proteins, lipids, pophyrins, bile acids
-responsible for synthesizing immunoglobulins
-principle storage site of lipids and vitamins
Theories of mechanism by which ethanol causes damage to the liver
-induction of the CyP450 generation of free radicals
-lesions known as Mallory bodies
-stimulation of an immune response
first pass
every substance that is absorbed in the gastrointestinal tract must first pass through the liver
Wilson's disease
defect in the ATP7B protein
Hereditary Hemochromatosis
C282Y mutation in the HFE protein
Gilbert's syndrome
mutation in the UGT1A1 gene
Mallory body
-inclusion found in the cytoplasm of liver cells
-most common in alcoholic hepatitis and alcoholic cirrhosis