Porphyrins and Hemoglobin Flashcards
The primary purpose of porphyrins in the human body is to
a. Chelate free hemoglobin
b. Contribute to the synthesis of heme
c. Transport ferrous iron
d. Transport oxygen to tissues
b. Contribute to the synthesis of heme
The chemical structure of porphyrin is described as a(n)
a. Cyclic tetrapyrrole
b. Heterocyclic pyrrole
c. Linear tetrapyrrole
d. Oxygen-binding prosthetic group
a. Cyclic tetrapyrrole
Which statement correctly describes a porphyrin property?
a. Porphyrins absorb light in the visible region of the spectrum and
fluoresce.
b. Porphyrins are not soluble in aqueous solution.
c. Porphyrins are oxidized to porphyrinogens when exposed to air.
d. Porphyrins produced spontaneously are functional compounds.
a. Porphyrins absorb light in the visible region of the spectrum and
fluoresce.
The porphyrias can be classified according to disease symptoms as
a. Congenital or acquired
b. Erythropoietic or hepatic
c. Hematologic or muscular
d. Neurologic or cutaneous
d. Neurologic or cutaneous
Elevated urinary PBG concentration with negative fecal porphyrin results
indicates
a. Acute intermittent porphyria (AIP)
b. Erythropoietic protoporphyria (EPP)
c. Hereditary coproporphyria (HCP)
d. Porphyria cutanea tarda (PCT)
a. Acute intermittent porphyria (AIP)
Porphyria cutanea tarda (PCT) is identified by
a. Chromatographic analysis of a 24-h urine collection
b. Scanning fluorescence of plasma
c. Screening for PBG in a random urine specimen
d. Separation of fecal porphyrins in a 24-h specimen
a. Chromatographic analysis of a 24-h urine collection
Suspected erythropoietic protoporphyria (EPP) is evaluated by
a. Assessing plasma or whole blood for protoporphyrin
b. Enzymatic measurement of hydroxymethylbilane synthase
c. HPLC analysis of a urine specimen
d. Molecular testing for ferrochelatase
a. Assessing plasma or whole blood for protoporphyrin
Porphobilinogen often is measured in the urine using
a. Chromatography
b. Electrophoresis
c. Spectrophotometry
d. The Watson-Schwartz method
d. The Watson-Schwartz method
In the clinical laboratory, testing to identify specific porphyrins is performed
using
a. Chemical derivatization and spectrophotometry
b. Fluorometric emission scanning
c. Liquid chromatography with fluorescent detection
d. Thin layer chromatography
a. Chemical derivatization and spectrophotometry
Specimens collected for evaluation of porphyria should be
a. Allowed to clot at room temperature for an hour
b. Diluted to less than 25 mg/dL creatinine
b. Exposed to light and air to oxidize the analytes
d. Protected from light and stored cold
d. Protected from light and stored cold
Inherited disorders in which a genetic defect causes abnormalities in rate and
quantity of synthesis of structurally normal polypeptide chains of the
hemoglobin molecule are called
a. Hemoglobinopathies
b. Molecular dyscrasias
c. Porphyrias
d. Thalassemias
d. Thalassemias
Molecular diagnostic techniques that can help diagnose hemoglobin
disorders such as hemoglobinopathies and thalassemias include
a. Biochemical separation, identification, and quantification of mixture
components
b. Separation of macromolecules and their fragments, based on their size
and charge
c. DNA amplification, hybridization, and nucleotide sequencing
d. The use of specific monoclonal antibodies
c. DNA amplification, hybridization, and nucleotide sequencing
Which of the following abnormal hemoglobins, found frequently in
individuals from Southeast Asia, migrates with hemoglobin A2 on
cellulose acetate electrophoresis?
a. Hemoglobin C
b. Hemoglobin D
c. Hemoglobin E
d. Hemoglobin Lepore
c. Hemoglobin E
Which type of alpha-thalassemia results from deletion of three genes and
produces a moderate hemolytic anemia?
a. Hemoglobin Bart’s
b. Hemoglobin H disease
c. Hydrops fetalis
d. Thalassemia trait
b. Hemoglobin H disease
The most effective way to quantitate hemoglobin A2
is by
a. Alkali denaturation test
b. Citrate agar electrophoresis
c. Column chromatography
d. Densitometry
c. Column chromatography