Case study- Sickle cell disease-A 15-year-old African-American female presents to the emergency room…

A 15-year-old African-American female presents to the emergency room with complaints of bilateral thigh and hip pain. The pain has been present for 1 day and is steadily increasing in severity. Acetaminophen and ibuprofen have not relieved her symptoms. She denies any recent trauma or excessive exercise. She does report feeling fatigued and has been having burning with urination along with urinating frequently. She reports having similar pain episodes in the past, sometimes requiring hospitalization. On examination, she is afebrile (without fever) and in no acute distress. No one in her family has similar episodes. Her conjunctiva and mucosal membranes are slightly pale in coloration.She has nonspecific bilateral anterior thigh pain with no abnormalities appreciated. The remainder of her examination is completely normal. Her white blood cell count is elevated at 17,000/mm3, and her hemoglobin (Hb) level is decreased at 7.1 g/dL. The urinalysis demonstrates an abnormal number of numerous bacteria.The blood smear (as shown below) is typical of sickle cell disease.

Sickle cell anemia

The molecular event triggering this disease is which of the following?

A. A loss of quaternary structure of the hemoglobin molecule

B. An increase in oxygen binding to hemoglobin

C. A gain of ionic interactions, stabilizing the “T” form of hemoglobin

D. An increase in hydrophobic interactions between deoxyhemoglobin molecules

E. An alteration in hemoglobin secondary structure leading to loss of the “α” helix

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