Question

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.”

Past Medical History

The patient was diagnosed with homozygous HbS disease at age 11 months. He first pre- sented with a severe case of dactylitis at 101⁄2 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% HbA2.

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.

Laboratory Blood Test Results

See Patient Case Table 89.2

Patient Case Table 89.2 Laboratory Blood Test Results
Na 141 meq/L	Hct 28.1%	Bilirubin—total 1.4 mg/dL
K 3.6 meq/L	Plt 225 ? 103/mm3	Bilirubin—indirect 0.9 mg/dL
Cl 101 meq/L	WBC 7.0 ? 103/mm3	Alb 3.7 g/dL
HCO3 22 meq/L	MCV 82.1 fL	Protein—total 6.3 g/dL
BUN 13 mg/dL	Retic 6.4%	Ca 9.3 mg/dL
Cr 1.2 mg/dL	AST 25 IU/L	Mg 1.9 mg/dL
Glu, fasting 87 mg/dL	ALT 22 IU/L	PO4 4.0 mg/dL
Hb 10.9 g/dL	Alk phos 75 IU/L	Folic acid 515 ng/mL

Patient Case Question 7. Identify three clinical blood test results from Table 89.2 that were elevated and briefly explain why they are elevated.

Answer 1

In spite of the fact that cerebral localized necrosis is the most regular neurological intricacy, various other possibly destroying focal sensory system sequelae. These include: intra-cranial discharge, segregated neuropathies, transverse myelitis, sound-related and visual signs, and spinal rope contribution. In the spinal rope there has remained a portrayal of rope pressure by extramedullary hemopoietin tissue notwithstanding uncommon case accounts of backbone rope localized necrosis.

In the non-sickle cell infection populace it gives the idea that spinal infarct is significantly less continuous than cerebral dead tissue too, and represents just around 1.0 percent of all CNS infarcts. Of those with spinal dead tissue, most seem, by all accounts, to be from awful or careful etiologies than other natural causes. Aortic malady is an incessant guilty party with many case reports itemizing unfriendly sequelae following careful repair of aneurysms, yet in addition aortic thrombosis, and aortic analyzation. Other non-horrendous, non-careful etiologies of spinal rope infarct include: worldwide hypotension and additionally blood vessel inadequacy, frequently after heart failure; transient ischemic assaults; fibrocartilaginous emboli; blood vessel vascular mutations; syphilitic arteritis and neighboring spinal malady.

There is extensive proof that sickle cell malady speaks to a hypercoagulable state. It gives the idea that almost every part of hemostasis is modified to some degree in. There is great proof that there is externalization of phosphatidylserine in SCD, which is thought to assume a huge part in advancing macrophage acknowledgment in erythro-phagocytosis and therefore setting off a flag for the coagulation procedure. Expanded phosphatidylserine presentation is likewise thought to be related with expanded tissue factor articulation. Notwithstanding, it stays hazy how or to what degree those variations from the norm add to ailment intricacies, for example, cerebral and spinal infarcts.

In the scan for conceivable qualities of this subpopulation, some have started to investigate factors that incline the all-inclusive community to coagulation variations from the norm and thrombophilia. In any case, encourage approval is required before these can be utilized to tentatively guide proposals for atomic hereditary testing or treatment. There is no known identifiable thrombophilia variation from the norm that predicts cerebral dead tissue in sickle cell ailment.