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Sickle-cell disease: Quiz


Question 1: This crisis is triggered by ________, which directly affects erythropoiesis (production of red blood cells) by invading the red cell precursors and multiplying in them and destroying them.
AdenoviridaeRotavirusParvovirus B19Virus disease

Question 2: This is particularly common in patients with co-existent ________.
Haemophilia BSideroblastic anemiaGlucose-6-phosphate dehydrogenase deficiencyHaemophilia A

Question 3: The gene defect is a known mutation of a single nucleotide (see single-nucleotide polymorphism - SNP) (T to A) of the β-globin gene, which results in ________ being substituted by valine at position 6.
Nitrous oxideGlutamic acidDizocilpineGlutamine

Question 4: ________ (bacterial bone infection), Salmonella is the second most common organism behind Staphylococcus aureus which remains first in both the general population and those with sickle cell.
Osteochondritis dissecansOsteoporosisOsteomyelitisAvascular necrosis

Question 5: Overwhelming post-(auto)splenectomy infection (OPSI), which is due to functional asplenia, caused by encapsulated organisms such as Streptococcus pneumoniae and ________.
Haemophilus influenzaePathogenic bacteriaTyphoid feverCholera

Question 6: Therefore, a urinalysis to detect an ________ urinary tract infection, and chest X-ray to look for occult pneumonia should be routinely performed.
ReligionMagic (paranormal)MysticismOccult

Question 7: Abnormal haemoglobin forms can be detected on haemoglobin electrophoresis, a form of ________ on which the various types of haemoglobin move at varying speeds.
ProteinWestern blotSDS-PAGEGel electrophoresis

Question 8: The origin of the mutation that led to the sickle-cell gene was initially thought to be in the ________, spreading to Asia and Africa.
Middle EastEuropeArabian PeninsulaWestern Asia

Question 9: Sickle-cell disease, usually presenting in childhood, occurs more commonly in people (or their descendants) from parts of tropical and sub-tropical regions where ________ is or was common.
AIDSBabesiosisPlasmodium falciparumMalaria

Question 10: Micro-: ________ (Plummer-Vinson syndrome)
Sickle-cell diseaseIron deficiency anemiaHenoch–Schönlein purpuraSerum iron

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