Table 1.

Included studies reporting on the outcome of acute pain episodes

StudyDesignPopulationKey findings and clinical descriptionsDescription of physical activity/triggerDiagnosis of SCTIs description of episode diagnostic of pain/vaso-occlusive episode (as typically observed in SCD)?Is the reported population representative of the whole experience of the authors (as in a consecutive or random sample)?
Biedrzycki et al20  Case report A case report of 51-y-old Afro-Caribbean man with SCT The patient had diabetes and presented acutely with hyperosmolar nonketotic acidosis. He rapidly deteriorated with multiorgan failure after hospital admission. Autopsy demonstrated sickled erythrocytes within the vasculature. The authors also report that the patient had “acute chest syndrome” without any clinical information to support this diagnosis. No prodrome of pain is presented. Acute illness History No No 
Taksande et al19  Case report A case report of a single 35-y-old woman with “hemoglobin AS pattern on electrophoresis” from India The patient reported low back pain for 6 mo while walking with relief in the supine position. Examination revealed pallor, hemoglobin of 6.5 g/dL and MCV of 77 fl. Peripheral smear was consistent with microcytic hypochromic anemia. Peripheral blood smear shows hypochromic microcytic anemia. MRI of the spine showed marrow necrosis in lumbar vertebrae, suggestive of cystic hemorrhagic necrosis of bone, which the authors attribute to sickle cell status. Not described Laboratory, electrophoresis No No 
Dora et al18  Prospective cohort 119 peripartem females with SCT in India at an academic hospital The authors report 5 individuals with pain and 1 with “acute chest syndrome” without detailed clinical description of either pain episodes or acute chest syndrome cases. Pregnancy Laboratory, HPLC No Yes 
Souza et al17  Case-control 
 
Cases: 31 individuals with SCT from 2 cities in Brazil
Controls: archived frozen samples without SCT 
The authors report that 22 of 31 individuals with SCT reported pain in clinical examination. The authors note that pain is in the upper and lower limbs and joints. Beyond this, no other clinical description is provided. Not described Laboratory, PCR No No 
StudyDesignPopulationKey findings and clinical descriptionsDescription of physical activity/triggerDiagnosis of SCTIs description of episode diagnostic of pain/vaso-occlusive episode (as typically observed in SCD)?Is the reported population representative of the whole experience of the authors (as in a consecutive or random sample)?
Biedrzycki et al20  Case report A case report of 51-y-old Afro-Caribbean man with SCT The patient had diabetes and presented acutely with hyperosmolar nonketotic acidosis. He rapidly deteriorated with multiorgan failure after hospital admission. Autopsy demonstrated sickled erythrocytes within the vasculature. The authors also report that the patient had “acute chest syndrome” without any clinical information to support this diagnosis. No prodrome of pain is presented. Acute illness History No No 
Taksande et al19  Case report A case report of a single 35-y-old woman with “hemoglobin AS pattern on electrophoresis” from India The patient reported low back pain for 6 mo while walking with relief in the supine position. Examination revealed pallor, hemoglobin of 6.5 g/dL and MCV of 77 fl. Peripheral smear was consistent with microcytic hypochromic anemia. Peripheral blood smear shows hypochromic microcytic anemia. MRI of the spine showed marrow necrosis in lumbar vertebrae, suggestive of cystic hemorrhagic necrosis of bone, which the authors attribute to sickle cell status. Not described Laboratory, electrophoresis No No 
Dora et al18  Prospective cohort 119 peripartem females with SCT in India at an academic hospital The authors report 5 individuals with pain and 1 with “acute chest syndrome” without detailed clinical description of either pain episodes or acute chest syndrome cases. Pregnancy Laboratory, HPLC No Yes 
Souza et al17  Case-control 
 
Cases: 31 individuals with SCT from 2 cities in Brazil
Controls: archived frozen samples without SCT 
The authors report that 22 of 31 individuals with SCT reported pain in clinical examination. The authors note that pain is in the upper and lower limbs and joints. Beyond this, no other clinical description is provided. Not described Laboratory, PCR No No 

HPLC, high-performance liquid chromatography; MCV, mean corpuscular volume; MRI, magnetic resonance imaging; PCR, polymerase chain reaction.

Pain evaluation not comparative.

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