Pathophysiology Identify two subnormal clinical values from

Pathophysiology: Identify two subnormal clinical values from the laboratory blood test results in Table 89.2 and briefly explain why they are abnormal

CASE STUDY 89 SICKLE CELL ANEMIA For the Disease Summary for this case study see the CD-ROM PATIENT CASE s Chief Complaints My arms and legs hurt a lot more than usual today. The ibuprofen has stopped working and I need something stronger. I may have overdone it at the gym.\" History I.T. is an 18-year-old African American male with sickle cell anemia who presents with a one day history of increasing pain localized to his arms and legs. His pain has not been relieved with 400-mg ibuprofen every 6 hours for the past 24 hours. He rates his current pain inten- sity level as 8 out of 10. He reports that he always seems to have some pain in his arms, legs and back, but the pain intensified approximately 36 hours after a workout at the gym that was more vigorous than usual. \"I know that I shouldn\'t have done that,\" he confesses, \"but the guys needed a 6th player for a game of 3-on-3. And, on top of that, I forgot to take my water bottle to the gym The patient was diagnosed with homozygous Hbs disease at age 11 months. He first pre sented with a severe case of dactylitis at 10% months with significant swelling and pain in both hands and feet. Electrophoresis revealed a hemoglobin proportion of 86.5% Hbs, 11.3% HbF, and 2.2% HbA At age 16 months, the patient was hospitalized with acute sequestration syndrome and splenomegaly, treated, and released. At age 30 months and again at age 34 months, the patient was hospitalized for Streptococcus pneumoniae pneumonia, treated with IV antibi- otics, and released. He also experienced one episode of acute staphylococcal osteomyelitis of the left knee at age 13. Since the age of 3 years, the patient has averaged three painful crises per year but has had as many as 10 attacks in a year. He has experienced chronic low-grade pain in his arms, legs, and back since becoming a teenager but has been able to cope by tak ing ibuprofen when needed. Other medical conditions and surgeries of note include Born with hypospadias, which was surgically corrected at 8 months of age Tonsillectomy as a child

Solution

Sickle cell anemia:

Point mutation - result in sickle cell anemia (11th chromosomal co dominance effect)

It has observed that 147th position of beta chain of the hemoglobin- substitution of valine for glutamic acid. This is leading to polymerization of hemoglobin S in deoxygenated or hypoxic conditions to form gel like components in the erythrocytes finally abnormal hemoglobin in sickle cell disease may cause the erythrocyte to deform in hypoxic conditions result in damage to “microcirculation”

Two subnormal clinical values from the laboratory blood test results in Table 89.2 and briefly explain why they are abnormal

Hb content in the above given patient who has diagnostic condition sickle-cell anemia that induced low hemoglobin (10.9g/dL) content compared to the control or normal Hb (12 to 15 in women whereas in men 13 to 17) content in human population. The content of Hct normal is for men, 38.8 to 50% whereas for women, it will be 34.9 to 44.5% but in the above case, it has observed that Hct content will be 28.1% due to death of red blood cell finally causes low affinity for oxygen to carry & finally causes low blood pressure